IM - HEME

Gallstones - Pt most likely presents w/ Hereditary spherocytosis -A) Etiology ----1) Usually presents in younger pts (<30) ----2) Usually asymptomatic -A) PBS findings ---1) Spherocytes ---2) Basophils ------a) The large cell is a NORMAL BASOPHIL ---3) Target cells ------a) Expected on any pt w/ anemia (normal response) -B) Signs/Symptoms ---1) Splenomegaly ---2) Hemolytic anemia ---3) ± jaundice ------a) During stressful situations/conditions -C) Complications ----1) Pigmented gallstones -D) MGMT ----a) Dx tests -------1) PBS ----b) TMT -------1) Supportive -----------a) Folic acid -----------b) Blood transfusions Acute leukemia - Also presents in younger pts - However, pt has no signs/symptoms of leukemia (i.e., non-tender lymphadenopathy)

(UWS2E1) A 24F, presents for a routine check-up. She has no sig PMHx. BP is 120/74 & pulse is 76/min. BMI is 32. The lungs are clear to auscultation. A normal S1 & S2 are heard on cardiac exam. The abd is soft & non-tender. The spleen tip is palpated in the left upper abd quadrant. There is no peripheral edema. A PBS is attached. The pt is at greatest risk for which of the following conditions? (Acute leukemia VS Gallstones)

Renal tubular damage - Pt most likely presents w/ Multiple myeloma -A) Signs/Symptom → (focus on CONSTELLATION OF SYMPTOMS) -----1) Constitutional symptoms -----2) Hypercalcemia -----3) Normocytic anemia ---------(MCV is in normal range 80-100) ----4) Renal insufficiency ---------a) Common in pts w/ MM → d/t multiple reasons: ------------1) Monoclonal light chain deposits that clog tubules in kidney (most common reason) ----------------a) presents as myeloma cast nephropathy) ----------------b) little-to-no protein, No hematuria, NO associated renal tubular damage casts ------------2) Amyloidosis ----------------(presents as nephrotic syndrome) ------------3) Monoclonal immunoglobulin deposits ----------------(presents as nephrotic syndrome) Renal artery stenosis - Usually presents in pts w/ HTN - However, key differences include: ----1) Abd exam would reveal a abd bruit ----2) Pt would have a Hx of HTN despite TMT ----3) Pt would have Hx of atherosclerotic disease ----4) Usually presents w/ -------a) RECURRENT flash pulmonary edema -------b) Often associated w/ SEVERE HTN → (≥180/120) -----------[Pts BP is 146/92 & he DOES NOT have pulm edema] ----5) RAS DOES NOT present w/: ---------Wt loss ---------Hypercalcemia

(UWS2E1) A 63M, presents d/t progressive fatigue over the last 6 months. The pt feels "exhausted" by the end of the day & has lost 10-lbs. He was hospitalized w/ pneumonia 7 months ago & notes that "everything deteriorated" thereafter. He has no fevers, chills, abd pain, or urinary symptoms. PMHx is sig for HTN treated w/ lisinopril. He is a lifetime non-smoker but drinks socially. BP is 146/92 & pulse is 80/min. The pt is obese. Mucous membranes are pale. Abd exam is normal, but rectal exam shows a mildly enlarged, non-tender prostate. Labs show: - Hb--------------------------------------9.2 - MCV------------------------------------86 - PLTS------------------------------------150K - Leukocyte Ct---------------------------9,2K - Na+--------------------------------------137 - K+----------------------------------------3.9 - Cl----------------------------------------102 - Ca+--------------------------------------11.8 - BUN-------------------------------------30 - Cr----------------------------------------2.2 Urinalysis shows no hematuria or proteinuria. Which of the following is the most likely cause of this pts kidney injury? (Renal artery stenosis VS Renal tubular damage)

Prior surgery - Pt most likely presents w/ REACTIVE (SECONDARY) thrombocytosis -A) RF ----1) Prior BAT --------a) Probable splenectomy -B) Pathophysio ----1) Pts usually have dramatic thrombocytosis following splenectomy → However, this resolves w/in weeks/months ----2) However, INFECTIONS can cause reactive thrombocytosis -------(pt had recent pneumonia infection) Essential thrombocytosis - Would also cause thrombocytosis - However, Key differences include: ---1) PT would have PERSISTENT thrombocytosis ------a) Pt would have had a Hx of it ------b) Usually > 600K ---2) Other symptoms of essential thrombocytosis include: ------a) Hx of Thrombosis ------b) Hx of Hemorrhage ----------[pt has none of these symptoms]

(UWS2E1) A 65M, presents for a routine follow-up 3 months after a short hospitalization for pneumonia. He feels well & has no acute problems. The pt has a PMHx sig for HTN, hyperlipidemia, & knee OA. He underwent an emergency laparotomy 5 years ago for BAT from a MVC. He has been on lisinopril & amlodipine for HTN since his recent hospitalization. The pt smokes a pack of ciggs daily & drinks alcohol on weekends. BP is 160/90, & pulse is 90/min. Cardiopulmonary auscultation reveals clear lung fields & normal first & second heart sounds. The abd is soft, w/out organomegaly. A well-healed, midline abd surgical scar is present. Labs show: --------------NOW------------3 months ago------- - Hb----------14.4----------------14.1 - PLTS--------500K--------------490K - Leukos-----7K------------------14,2K Follow-up CXR today shows complete resolution of the left lower lobe consolidation seen 3 months ago. Which of the following is the most likely cause of this pts thrombocytosis? (Essential thrombocythemia VS Prior surgery)

CD20 cell surface antigen - Pt presents w/ CLL -A) Etiology -----1) Most common leukemia in Western world -----2) Usually presents in males ~70 years old -A) Signs/Symptoms -----1) Frequently asymptomatic -----2) Lymphadenopathy → --------(MOST COMMON PRESENTING SYMPTOM) ---------(cervical, supraclavicular, axillary) -----3) Hepatosplenomegaly -----4) Mild thrombocytopenia & anemia -----5) Purpura -C) Associated -------1) Warm autoimmune hemolytic anemia -C) MGMT -----a) Dx tests ---------1) Immunohistochemical staining & PBS -------------a) Smudge cells -------------b) Prominent lymphocytosis w/ mild pancytopenia -------------------bb) >30% lymphocytes in BM biopsy --------------c) B-cells (CD19, CD20, CD23) & CD5+ -----b) TMT ---------1) Radiation --------------Localized disease --------------Staging based on RAI & Binet systems ---------2) Chemotherapy ------------a) For advanced, symptomatic CLL ------------b) Fludarabine, cyclophosphamide and rituximab BCR-ABL tyrosine kinase - Associated w/ CML (Chronic myelogenous leukemia) - Also presents w/ hepatosplenomegaly & Leukemia in older Males - However, key differences include --A) Presents w/ KEY ADDITIONAL SYMPTOMS including: -----1) Early satiety → (enlarged spleen pushes on stomach) -----2) Constitutional symptoms → (fever, wt loss, malaise) -----3) LUQ pain → (d/t splenic infarction/splenomegaly) ---------[pt has none of these symptoms] --B) Abnormal labs include: -----1) Basophilia -----2) Negative LAP stain (decreased LAP score) -----3) 9:22 chromosome ---------[NONE appear on pts labs] --C) MGMT ------a) Dx tests ----------1) Karyotyping ----------2) FISH ----------3) RT-PCR ------b) TMT ----------1) BCR-ABL TK inhibitors --------------a) (Imatinib, dasatinib, Nilotinib, Bosutinib)

(UWS2E1) A 67M, presents to the office concerned about several swollen LN in his axilla. The LN are non-painful but have been present >3 weeks. He has had no recent fevers, chills, or wt loss. The pt is an investment banker & is careful w/ his health, noting that he exercise daily & eats organic food when possible. He does not smoke, drink, or use drugs. He has been married to his wife for 45 years. Temp is 98.2F, BP is 156/92, & pulse is 68/min. He is fit & well nourished. There are several enlarged, non-tender, mobile LN's in his bilateral cervical & axially chains. Chest exam is normal. With deep exhalation, a spleen tip is palpable under the left lower ribs. Labs show: - Hb--------------------------------------8.5 - MCV------------------------------------89 - PLTS------------------------------------90K - Leukocyte Ct---------------------------56,8K ---- Neutrophils--------------------------8% ---- Lymphocytes------------------------88% ---- Monocytes--------------------------4% - BUN-------------------------------------12 - Cr----------------------------------------1.1 This pt would benefit most from therapy directed against which of the following? (BCR-ABL tyrosine kinase VS CD20 cell surface antigen)

Smudge cells - Pt Most likely presents w/ CLL -A) Etiology -----1) Most common leukemia in Western world -----2) Usually presents in males ~70 years old -A) Signs/Symptoms -----1) Frequently asymptomatic -----2) Lymphadenopathy → --------(MOST COMMON PRESENTING SYMPTOM) ---------(cervical, supraclavicular, axillary) -----3) Hepatosplenomegaly ----------(pt has splenomegaly) -----4) Mild thrombocytopenia & anemia -----5) Purpura -C) Associated -------1) Warm autoimmune hemolytic anemia -C) MGMT -----a) Dx tests ---------1) Immunohistochemical staining & PBS -------------a) Smudge cells -------------b) Prominent lymphocytosis w/ mild pancytopenia ------------------->30% lymphocytes in BM biopsy --------------c) B-cells (CD19, CD20, CD23) & CD5+ -----b) TMT ---------1) Radiation --------------Localized disease --------------Staging based on RAI & Binet systems ---------2) Chemotherapy -------------For advanced, symptomatic CLL --------------(fludarabine, cyclophosphamide and rituximab) Auer rods - Seen on PBS of CML - CML Also, presents w/ leukemia in older pts - However, key differences include: --A) Presents w/ KEY ADDITIONAL SYMPTOMS including: -----1) Early satiety → (enlarged spleen pushes on stomach) -----2) Constitutional symptoms → (fever, wt loss, malaise) -----3) LUQ pain → (d/t splenic infarction/splenomegaly) ---------[pt has none of these symptoms] --B) Abnormal labs include: -----1) Basophilia -----2) Negative LAP stain (decreased LAP score) -----3) 9:22 chromosome ---------[NONE appear on pts labs]

(UWS2E1) A 68M, presents w/ cough productive of green sputum for the last week. The pt has had no fevers or chills. He was treated for a viral URI at an urgent care 2-weeks ago, & was prescribed a cough suppressant that does not seem to be working. He has had no SOB or dyspnea on exertion. The pt does not use tobacco, drink, or use drugs. Temp is 98.7F, BP is 148/92, & pulse is 92/min. Pulse Ox is 97% on room air. PE shows mild mucosal pallor. There are several palpable, non-tender LNs in the cervical chain & axilla. His chest & abd exams are normal other that slightly prominent spleen tip w/ deep exhalation. Labs show: - Hb--------------------------------------9.5 - MCV------------------------------------87 - PLTS------------------------------------127K - Leukocyte Ct---------------------------38,8K --- Neutros-------------------------------7% --- Lymphos------------------------------92% --- Monos---------------------------------1% Which if the following is most likely to be seen on PBS? (Auer rods VS Smudge cells)

Increased inflammatory cytokines - Pt most likely has Anemia of chronic disease (ACD) -A) Etiology includes: ----1) ACD is associated w/ any CHRONIC condition, including: --------a) Hodgkins -B) Pathophysio ----1) ACD develops d/t long-term elevations in serum inflammatory cytokines -C) Signs/Symptoms of Hodgkins ----1) Biomodal distribution (<25 & >55) ----2) Painless mediastinal mass ----3) Fever, night sweats ----4) Wt loss ----5) Can be asymptomatic -D) Labs ----a) Slight microcytic anemia ----b) Low reticulocyte response → (should be elevated if a pt has anemia) -E) MGMT ----a) Dx tests ------1) LN Biopsy ??? ----b) TMT → mnemonic "ABVD" -------A → Adriamycin (doxorubicin) -------B → Bleomycin -------V → Vinblastine -------D → Dacarbazine Increased iron recycling by M∅ - ACD is marked by REDUCED iron recycling - INCREASED iron recycling may be seen in Hemochromatosis

A 24F, presents d/t a month of progressive exertional dyspnea. The pt does not have chest pain or palpitations. She has no CMCs & takes no meds. Temp is 98.6F, BP is 114/68, pulse 90/min, RR is 12/min. BMI is 23. Exam shows mucosal pallor & cervical lymphadenopathy. Cardiopulmonary auscultation reveals clear lung fields & no murmurs. Labs show: - Hb---------------------------------9 - MCV-------------------------------76 - Reticulocytes---------------------0.5% - Plts--------------------------------240K - Leukos----------------------------7,5K - Creatinine-------------------------0.8 CXR shows a mediastinal mass. What is the most likely cause of this pts anemia? (Increased inflammatory cytokines OR Increased iron recycling by M∅'s)

Imatinib - Pt presents w/ signs concerning for ALL -A) Associated Translocations ----1) 9:22 (BCR-ABL) → most common in adults ----2) 12:21 (BRC-ABL) → most common in children ----3) Cells -------a) BALL → CD10, CD19, CD20 -------b) TALL → CD3, CD7 -C) Associated labs ----1) +TdT ----2) +PAS staining -D) MGMT ----a) Dx tests -------1) Molecular & cryogenic markers → to determine prognosis ----b) TMT -------1) Combo Chemo -------2) Prophylactic intrathecal chemo + methotrexate -----------a) For prophylaxis against METs -------3) Tyrosine kinase inhibitor (imatinib, desatinib) All-trans retinoic acid - This is the TMT for acute promyelocytic leukemia (a subset of AML) - However, key differences includes: ---1) ETIOLOGY ------a) Usually presents in males >65 ---------[pt is 5 years old] ---2) TRANSLOCATION ------a) associated w/ 15:17 translocation ---3) PBS -------a) Auer rods ---4) MGMT -------a) Dx tests ----------1) BM biopsy -------b) TMT ----------1) All-trans retinoic acid ----------2) ± Chemo ----------3) Supportive --------------a) PLTS & RBC transfusions --------------b) Antibiotics --------------c) Uric acid lowering agents

A 5-year-old African-American boy is brought to the physician because of fatigue and night sweats for the past month. During this time, he has also lost 3 kg (6.6 lbs). Before the onset of symptoms, he had been healthy except for a febrile seizure as an infant. His brother had chickenpox 2 months ago. He is at the 75th percentile for height and 50th percentile for weight. He appears markedly fatigued. His temperature is 38°C (100.4°F), pulse is 95/min, respirations are 19/min, and blood pressure is 100/60 mm Hg. Lung and cardiac examination is normal. There are enlarged, nontender lymph nodes bilaterally in the neck. The abdomen is soft and nontender. A complete blood count shows: - Hb---------------------------------9.1 - Leukos-----------------------------8K - HCT--------------------------------26.9 - PLTS-------------------------------34K - Na+--------------------------------135 - K+----------------------------------4.5 - Cl----------------------------------101 - HCO3------------------------------27 - BUN--------------------------------9 - Cr-----------------------------------0.7 - Ca-----------------------------------8.8 PCR testing demonstrates a 9:22 chromosomal translocation. Which of the following is the most appropriate pharmacotherapy? (All-trans retinoic acid VS Imatinib)

Over proliferation of plasma cells - Pt most likely has hypercalcemia d/t underlying Multiple myeloma -A) Etiology of hypercalcemia >14 ----a) Only occurs w/ MALIGNANCY -------1) Multiple myeloma -------2) Squamous cell carcinoma of lung -A) Signs/Symptoms → (of MM) ----a) MOST COMMON presenting signs are: --------1) Anemia -----------a) fatigue & lethargy --------2) Bone pain -----------a) hip pain --------3) Elevated Cr --------4) Hypercalcemia ------------a) Confusion ------------b) GI upset (constipation, nausea) Decreased renal excretion of Ca+ - Decreased renal excretion of Ca+ can be caused by multiple conditions - Most often it is seen w/ hydrochlorothiazide use ------[Hypercalcemia d/t Hydrochlorothiazide would only be around Serum Ca+ 11, Pt has a Serum Ca+ of 14.7] ****SIDE NOTE → DECREASED renal excretion DOES NOT describe CKD - In CKD we would see an INCREASED renal excretion of Ca+ → (b/c NORMALLY the kidney RE-ABSORBS Ca+)

A 70-year-old man is brought to the emergency department by his wife because of lethargy, confusion, and nausea for the past 2 days. He has previously been healthy and has no past medical history. His only medications are a daily multivitamin and acetaminophen, which he takes daily for hip pain. Vital signs are within normal limits. He is disoriented to place and time but recognizes his wife. The remainder of his physical examination shows no abnormalities. Laboratory studies show a hemoglobin concentration of 9.1 g/dL, a serum calcium concentration of 14.7 mg/dL, and a serum creatinine of 2.2 mg/dL (previously 0.9 mg/dL). Which of the following is the most likely underlying mechanism of this patient's condition? (Decreased renal excretion of Ca+ VS Over-Proliferation of plasma cells)

vWF - Pt presents w/ MORE signs supporting a Dx of vWF (Vs hemophilia -A) Coagulation times -----1) ↑ Bleeding time ---------a) MOST SPECIFIC for vWF -----2) INCREASED/normal aPTT -----3) Normal PT -B) Signs/Symptoms ----1) Mucocutaneous bleeding → MOST COMMON PRESENTING symptom Hemophilia A -Coagulation times ------1) NORMAL Bleeding time ----------a) b/c PLTS are NOT affected ------2) INCREASED aPTT ----------a) MOST SPECIFIC FOR HEMOPHILIA ------3) Normal PT - Most commonly presents w/ hemarthrosis as FIRST SYMPTOM

AMBOSS A 25-year-old man comes to the physician for the evaluation of recurrent episodes of nosebleeds over the past 6 months. The nosebleeds occur spontaneously and stop after 10 minutes after pinching the nose at the nostrils. He has no history of serious illness except for prolonged bleeding following wisdom teeth extraction 2 years ago. He does not smoke or drink alcohol. He takes no medications. Vital signs are within normal limits. Examination of the nose shows no abnormalities. There are several bruises on the lower extremities. The remainder of the examination shows no abnormalities. Laboratory studies show: - Hb---------------------------------------15 - Leukocytes------------------------------6K - Bleeding time---------------------------9 min (N 2-7 mins) - PT time----------------------------------13 sec (N 11-13) - aPTT-------------------------------------55 (N 35-45) Which of the following is the most likely Dx? (vWF VS Hemophilia A)

Oral prednisone - Pt presents w/ signs concerning for Warm autoimmune hemolytic anemia (W-AHA) -A) RFs ----1) Symptoms began 3 days after taking antibiotics → (cephalexin is a known trigger for WAHA) -B) Associated labs ----1) POSITIVE for Direct Coombs test -C) MGMT ----a) Dx tests -------1) CBC -------2) PBS -------3) LFTs ----b) TMT -------1) Glucocorticoids (PREFERRED INITIAL TMT) -------2) Splenectomy ----------a) Reserved for pts REFRACTORY to glucocorticoids or severe disease -------3) Cytotoxic agents ----------a) For pts who refuse/have CI to splenectomy Plasmapheresis - TMT for COLD autoimmune hemolytic anemia - Also presents w/ POSITIVE DIRECT Coombs test - However, key differences include: ---1) CHARACTERISTIC SYMPTOMS OF C-AHA include: -------a) COLD trigger -----------[Pt does not describe cold as a trigger for symptoms] ---2) MGMT → TMT -------1) Cold avoidance -------2) Cytotoxic therapy, plasmapheresis ------------[splenectomy & steroids are NOT EFFECTIVE for C-AHA]

AMBOSS A 27-year-old man comes to the physician because of severe fatigue that started 1 week ago. Ten days ago, he finished a course of oral cephalexin for cellulitis. He does not take any medications. He appears tired. His temperature is 37.5°C (99.5°F), pulse is 95/min, and blood pressure is 120/75 mm Hg. Examination shows scleral icterus and pallor of the skin and oral mucosa. The spleen tip is palpated 1 cm below the left costal margin. The remainder of the examination shows no abnormalities. Laboratory studies show: - Hb---------------------------------------10.5 - HCT--------------------------------------32% - Reticulocyte Ct--------------------------5% - LDH--------------------------------------750 - Na+---------------------------------------140 - Haptoglobin-----------------Undetectable - Direct Anti-globulin test ----------------pos A peripheral blood smear shows spherocytes. Which of the following is the most appropriate next step in treatment? (Oral prednisone VS Plasmapheresis)

Primidone - Pt has signs concerning for Acute intermittent porphyria -A) Signs/Symptoms → 6 P's of AIP: ----1P → Painful abdomen ----------a) ± decreased bowel sounds ----------b) Diffuse and constant pain ----2P → Polyneuropathy, ----3P → Psychologic disturbances ----------a) Especially depression ----------b) Migraines ----4P → Purple-red pee, ----5P → Precipitated by triggers ----------a) Meds --------------aa) Primidone (for Essentail Tremor) --------------bb) Cytochrome p450 INDUCERS ----------b) Alcohol ----------c) Starvation ----6P → Palpitations -B) Pathophys -----1) Autosomal dominant disorder characterized by deficiency of porphobilinogen deaminase ---------a) AKA hydroxymethylbilane synthase or uroporphyrinogen I synthase -C) MGMT ----a) Dx tests -------1) Urinary tests ----------a) ↑ Porphobilinogen ----------b) ↑ ALA (aminolevulinic acid) ----------c) ↑ Porphyrins (e.g. uroporphyrin, coproporphyrin) -----b) TMT --------1) Glucose loading + IV Hemin Amitriptyline -Not a known trigger for AIP

AMBOSS A 28-year-old woman comes to the emergency department because of increasing abdominal pain for 2 days. The pain is diffuse and constant, and she describes it as 7 out of 10 in intensity. She has also had numbness in her lower extremities for 12 hours. She has type 1 diabetes mellitus, migraine with aura, and essential tremor. She appears uncomfortable. She is oriented to place and person only. Her temperature is 37°C (98.6°F), pulse is 123/min, and blood pressure is 140/70 mm Hg. Examination shows a distended abdomen with no tenderness to palpation. Bowel sounds are decreased. Muscle strength and sensation is decreased in the lower extremities. There is a tremor of the right upper extremity. Urinalysis shows elevated levels of aminolevulinic acid and porphobilinogen. Which of the following is the most likely cause of this patient's symptoms? (Primidone VS Amitriptyline)

Excisional biopsy - Pt presents w/ lymphoma -A) MGMT ----a) Dx tests -------1) Excisional biopsy (DIAGNOSTIC) -----------a) Must differentiate between Hodgkins & non-Hodgkins lymphoma ------2) BM aspiration -----------a) Used as second test to STAGE lymphoma

AMBOSS A 60-year-old man comes to the physician because of a 2-month history of chest pain, dry cough, and shortness of breath. He describes two painless masses in his neck, which he says appeared 4 months ago and are progressively increasing in size. During this time, he has had week-long episodes of fever interspersed with 10-day periods of being afebrile. He reports that his clothes have become looser over the past few months. He drinks alcohol occasionally. His temperature is 38°C (100.4°F), pulse is 90/min, and blood pressure is 105/60 mm Hg. Physical examination shows two nontender, fixed cervical lymph nodes on either side of the neck, which are approximately 2.2 cm and 4.5 cm in size. The tip of the spleen is palpated 3 cm below the left costal margin. An x-ray of the chest shows discrete widening of the superior mediastinum. Which of the following is most appropriate to confirm the diagnosis? (BM aspiration VS Excisional biopsy)

Desmopressin - Pt presents w/ a coagulopathy dysfunction - The question specifically asks how to treat the pts INCREASED BLEEDING -A) Pathophysio ----1) Increased bleeding in CKD is d/t Uremia-induced coagulative PLTS dysfunction -A) MGMT → of Uremia can be split into "SHORT term VS LONG term TMTS ---1) SHORT TERM TMTs -------a) Desmopressin (PREFERRED INITAL TMT) ---------aa) Can help to correct some of the PLTS dysfunction ---2) LONG TERM TMTs -------a) Hemodialysis -------b) Cryoprecipitate -------c) Conjugated estrogens Plasmapheresis - NOT a TMT for bleeding d/t uremic coagulopathy

AMBOSS A 63-year-old Hispanic man presents for elective laparoscopic cholecystectomy. He was hospitalized three weeks ago for abdominal pain, found to have multiple small gallstones, and has now decided to undergo definitive treatment. His past medical history includes hypertension, type 2 diabetes mellitus, and gastroesophageal reflux disease. His medications include aspirin, lisinopril, hydrochlorothiazide, glargine, metformin, and omeprazole. The surgery is completed without complications; however, on post-operative day one, he has oozing from his wound and IV sites. His temperature is 37.0ºC (98.6ºF), pulse is 74/minute, respiratory rate is 17/minute, and blood pressure is 146/102 mmHg. He denies any pain but does complain of nausea. Serum laboratory results are shown below. - Na+-------------------------------------142 - K+---------------------------------------4.1 - Cl---------------------------------------104 - HCO3-----------------------------------25 - BUN-------------------------------------38 - Cr---------------------------------------2.5 - Glucose--------------------------------138 - Leukocytes-----------------------------8,9K - Hb---------------------------------------12.6 - HCT-------------------------------------37% - PLTS-------------------------------------203K - MCV------------------------------------93 - PT time----------------------------------12 sec (N 11-13) - aPTT-------------------------------------24 (N 35-45) Which of the following should be included to treat his increased bleeding? (Desmopressin VS Plasmapheresis)

AV malformation in the colonic wall - Pt has signs & RFs concerning for angiodysplasia -A) RFs ----1) Age (>60) ----2) ESRD B) Signs/Symptoms ----1) Most common cause of hematochezia in older patients (>60) Malignant transformation - Describes colon cancer - Also presents w/ hematochezia & fatigue - However, key differences include: ---1) MISSING OTHER CHARACTERISTIC SYMPTOMS OF COLON CANCER, including: ------a) Fever, night sweats ------b) Wt loss ------c) Change in bowel habits

AMBOSS A 65-year-old man comes to the physician because of progressively worsening fatigue for 6 months. During this time, he has also had shortness of breath and palpitations on exertion. He has noticed blood in his stools on three separate occasions in the past 4 months. He has type 2 diabetes mellitus and end-stage renal disease. He drinks two to four beers daily. He does not smoke. His pulse is 95/min and blood pressure is 120/70 mm Hg. Examination shows pale conjunctivae. The abdomen is soft with no organomegaly. Rectal examination is unremarkable. His hemoglobin concentration is 7.2 g/dL, hematocrit is 32%, and mean corpuscular volume is 68 μm3. Which of the following is the most likely underlying cause of this patient's bleeding? (AV malformation in the colonic wall VS Malignant transformation in the colonic mucosal walls)

Whole Body CT - Pt has signs concerning for Multiple myeloma/MGUS -A) Serum protein electrophoresis findings → (KEY TO Dx) ----1) Reveals an M-spike -B) Signs/Symptoms ----1) Lower Back pain ----2) Fatigue -C) Associated labs ----1) ↑ Serum protein --------a) Pt has a 10 (N 6-7.8) -D) MGMT ----a) Dx tests -------1) BM biopsy → (DIAGNOSTIC TEST) ----------a) Clonal Plasma cells ≥10% -------2) PBS → GOOD INITIAL TEST -----------a) However, cannot distinguish between MM & MGUS -------2) Whole-body CT ----------a) Can help to distinguish between MGUS & MM ----b) TMT -------1) Stem cell transplant -------2) Chemotherapy -------3) Activity → should be encouraged to increase bone density -------4) Vaccines -----------a) S.pneumo, annual flu and HiB are encouraged, given their immunosuppression ------5) Radiation therapy ----------a) For Plasmacytomas → (Bony lesions that cause pain or compress the spinal cord) ------6) Proteasomal inhibitors ----------a) Bortezomib, carfilzomib, and ixazomib

AMBOSS A 71-year-old man comes to the physician for a health maintenance examination. Aside from occasional lower back pain in the last couple of years, he feels well. He had a right-sided myocardial infarction 4 years ago. Currently, he has no shortness of breath and has no anginal symptoms. He has a 30 pack-year history of smoking but stopped smoking 10 years ago and does not drink alcohol. His pulse is 59/min and blood pressure is 135/75 mm Hg. Physical examination reveals 1+ lower extremity edema. Cardiac and pulmonary auscultation show no abnormalities. There is no lymphadenopathy. His laboratory studies show a hemoglobin of 13.2 g/dL and serum protein of 10.1 g/dL. ECG shows known Q wave abnormalities unchanged since the last ECG one year ago. A serum protein electrophoresis with immunofixation is shown. Which of the following is the most appropriate next step to establish the diagnosis? (PBS VS Whole Body CT)

Multiple myeloma - Pt presents w/ signs concerning for Amyloidosis - Multiple myeloma is a common cause of Light-chain amyloidosis -A) Signs/Symptoms → (of Amyloidosis) ----1) Restrictive cardiomyopathy --------a) Heart failure ------------aa) dyspnea, orthopnea, pulmonary edema --------b) Preserved ejection fraction, --------c) Low-voltage on ECG, --------d) Symmetrical wall thickening with reduced diastolic filling ----2) Macroglossia --------a) W/ scalloping of the lateral tongue from contact with teeth -B) Associated Labs → (associated w/ Multiple myeloma) ----1) ↑ ESR ----2) Anemia Systemic sclerosis - Also can cause restrictive cardiomyopathy - However, key differences include: ---1) MISSING KEY SYMPTOMS of systemic sclerosis (CREST) ------a) Esophageal dysmotility ------b) Calcification ------c) Raynauds ------d) Sclerodactyly (Skin tightening) ------e) Telangiectasias ---2) AGE ------a) Usually occurs in females between ages 20-50

AMBOSS A 71-year-old woman comes to the physician because of progressive shortness of breath and swollen legs for 4 weeks. She has tried sleeping in a raised position using 2 pillows but still wakes up occasionally from a choking sensation. She returned from a safari tour in Tanzania 3 months ago. She has type 2 diabetes mellitus, arterial hypertension, and gastroesophageal reflux disease. Her sister has polymyalgia rheumatica. Her current medications include insulin, enalapril, and omeprazole. She has smoked one half-pack of cigarettes daily for 45 years. Her temperature is 37°C (98.6°F), pulse is 112/min, respirations are 22/min, and blood pressure is 119/76 mm Hg. Pulse oximetry on room air shows an oxygen saturation of 90%. Examination shows pitting edema below the knees and jugular venous distention. Crackles are heard at both lung bases. A photograph of her tongue is shown. Her hemoglobin concentration is 10.0 g/dL, leukocyte count is 6,100/mm3, and erythrocyte sedimentation rate is 62 mm/h. ECG shows sinus rhythm and low-voltage QRS complexes. Echocardiography shows symmetrical left ventricular hypertrophy, reduced diastolic filling, and an ejection fraction of 55%. Which of the following is the most likely cause of this patient's symptoms? (Systemic sclerosis VS Multiple myeloma)

Ruxolitinib - Pt presents w/ signs & labs concerning for primary myelofibrosis -A) Signs/Symptoms ----1) Extensive splenomegaly (KEY CLINICAL SIGN) --------a) ± LUQ pain --------b) ± Shoulder pain ----2) Palpable hepatomegaly --------a) Portal HTN (Ascites, esophageal varices, caput medusae) ----3) Pleura/pericardial effusions ----2) Constitutional symptoms --------a) (fatigue, weight loss, night sweats), ----2) Anemia, ----3) Thrombocytopenia, -B) MGMT -----a) Dx tests --------1) Mutation assay ------------a) Positive for JAK 2 mutation --------2) BM aspiration ------------a) Fibrosis/Dry tap (punctio sicca') --------3) PBS Findings ------------a) Dacrocytes (tear drop cells) on blood smear -----b) TMT --------1) Ruxolitinib ------------a) JAK 2 inhibitor --------2) Stem cell transplant ------------a) CI in this pt b/c of age & co-morbidities

AMBOSS A 72-year-old man comes to the physician because of a 2-month history of fatigue and worsening abdominal pain. During this period, he also has excessive night sweats and shortness of breath on exertion. Over the past 3 months, he has had a 5.6-kg (12-lb) weight loss. He had a myocardial infarction 3 years ago. He has hypertension, diabetes mellitus, and chronic bronchitis. His medications include insulin, aspirin, lisinopril, and an albuterol inhaler. He has smoked half a pack of cigarettes for the past 45 years. Vital signs are within normal limits. The spleen is palpated 6 cm below the left costal margin. Laboratory studies show: - Hb-------------------------------------6.4 - Leukocytes----------------------------5,2K - MCV-----------------------------------85 - PLTS-----------------------------------96K A blood smear is shown. Bone marrow aspiration shows extensive fibrosis and a few scattered plasma cells. A JAK 2 assay is positive. Which of the following is the most appropriate next step in management? (Stem cell transplant VS Ruxolitinib)

Gilberts syndrome - Pt Presents w/ signs concerning for Gilberts syndrome -A) Etiology -----1) Commonly occurs after a period of fasting/stress ---------a) Pt recently traveled & had a period of not eating ----2) Recent Antibiotic use -B) Signs/Symptoms ----1) Mild Jaundice -------a) W/ Mild excess of indirect hyperbilirubin (< 3mg) ----2) Scleral icterus ----3) Fatigue -C) Associated Labs ----1) NORMAL AST/ALT ----2) NORMAL GGT G6PD deficiency - Also presents Generalized fatigue, nausea, diminished appetite, mild jaundice & Presents in pts w/ recent Hx of antibiotic use - However, Key difference is ---1) PBS FINDINGS -------a) G6PD deficiency presents w/ HEINZ BODIES on PBS ------------[pt has NORMAL PBS]

TRUE LEARN A 16-year-old girl is brought to the physician because of yellowish discoloration of her eyes and generalized fatigue since she returned from a 2-week class trip to Guatemala 2 days ago. During her time there, she had watery diarrhea, nausea, and lack of appetite for 3 days that resolved without treatment. She also took primaquine for malaria prophylaxis. Three weeks ago, she had a urinary tract infection that was treated with nitrofurantoin. Her immunizations are up-to-date. Her temperature is 37.1°C (98.8°F), pulse is 82/min and blood pressure is 110/74 mm Hg. Examination shows scleral icterus. There is no lymphadenopathy. The remainder of the examination shows no abnormalities. Laboratory studies show: - Leukocytes------------------------------6,4K - Hb---------------------------------------12.1 - PLTS-------------------------------------234K - RC count--------------------------------1.1% - PT time----------------------------------12 sec (N 11-13) - INR---------------------------------------1 - Fibrin split products---------------------neg - Urea--------------------------------------20 - Cr-----------------------------------------1.1 - Total bili----------------------------------2.8 - Direct Bili---------------------------------0.2 - ALP---------------------------------------43 - ALT---------------------------------------17 - AST---------------------------------------16 - γ-GGT------------------------------------38 (N 5-50) - Anti-HAV IgG----------------------------pos - Anti-HBs---------------------------------pos A PBS shows no abnormalities. Which is the most likely Dx? (Gilberts syndrome VS G6PD deficiency)

All-trans retinoic acid -A) MGMT of AML (M3) includes: ----1) Chemotherapy ----2) All-trans retinoic acid -------a) Specific TMT for acute promyelocytic leukemia ----3) PLTS transfusions -------a) To prevent DIC in AML (M3) -----4) Red blood cell transfusions -----5) Antibiotics ---------a) for febrile patients -----6) Uric acid lowering agents Amoxicillin - Pt presents w/ pneumonia - However, Pt should be hospitalized based on her presentation, including (Acute presentation, higher fever, RR of 30/min, & underlying condition - AML) - Even if pt was treated as an outpatient for pneumonia, first line TMT of pneumonia is AZ/Doxy -----[NOT Amoxicillin]

TRUE LEARN A 22-year-old woman presents to the Emergency Department with dyspnea and fever for the past 24 hours. The symptoms developed fairly suddenly with fevers, chills and rigors in addition to productive cough. She also complains of frequent, intermittent nose bleeds and easy bruising for the past month. Her temperature is 39.3°C (102.7°F), pulse is 108/minute and regular, respirations are 30/minute, and blood pressure is 113/74 mmHg. Oxygen saturation is 92% on room air. There are rales over the right mid lung field. A chest x-ray shows a right middle lobe pneumonia with possible necrotizing changes. Laboratory results are below: - Leukocytes------------------------------1,1K - Hb---------------------------------------8.1 - PLTS-------------------------------------45K - PT time----------------------------------20 sec - aPTT-------------------------------------54 sec - INR---------------------------------------3.4 Fibrin split products Elevated A peripheral smear is highly suggestive of acute promyelocytic leukemia; cytogenetic studies are pending. Which of the following is the most appropriate initial treatment? All-trans retinoic acid VS Amoxicillin)

Bite cells - Pr presents w/ signs concerning for G6PD deficiency -A) RF ----1) MEDS "mnemonic - Hemolysis IS D PAIN" --------I → Isoniazid (INH) --------S → Sulfa meds --------D → Dapsone ------------a) Used to treat cellulitis --------P → Primaquine --------A → Aspirin --------I → Ibuprofen --------N → Nitrofurantoin -B) Signs/Symptoms ----1) Jaundice ----2) Splenomegaly ----3) Dark urine ----4) Sudden anemia -C) MGMT ----a) Dx tests -------1) Quantification of G6PD w/ enzyme assay -----------a) ONLY AFTER resolution of current hemolytic episode ----b) TMT -------1) Supportive Schistocytes - Usually seen in PBS of MAHA or aortic stenosis/valve replacement

TRUE LEARN A 24-year-old man presents to the Emergency Department with yellow skin and eyes for the past day. He had been feeling well and was in his usual state of health just prior to this sudden change in skin color, aside from starting treatment with an antibiotic for cellulitis. He denies any recent travel. He works as a patient transporter in a hospital. He denies any recent blood borne exposures. On physical examination, he is afebrile, his sclera are icteric and his skin is jaundiced. His abdomen is soft and nontender. There is no hepatomegaly. The spleen tip is palpable 4 cm below the left costal margin. The results of initial laboratory tests are below. - Hb-------------------------------7.9 - MCV-----------------------------83 - LDH------------------------------105 - Haptoglobin---------------------29 (N 41 - 165) - Total Bili-------------------------7.3 - Direct Bili------------------------6.9 Which of the following is most likely found on a peripheral blood smear? (Schistocytes VS Bite cells)

vWF deficiency - Pt presents w/ signs concerning for vWF deficiency -A) Signs/Symptoms ----1) Mucosal bleeding (MORE COMMON IN vWF) -B) Associated labs ----1) INCREASED Bleeding time ---------(b/c decrease in vWF factor inhibits the PLTS to work correctly) ----2) NORMAL/ELEVATED aPTT ---------a) b/c clotting factors are unaffected ----3) NORMAL PT ---------a) b/c clotting factors are unaffected Hemophilia A - Also presents w/ abnormal bleeding in young pts - Key differences include: ---1) LABS → Hemophilia presents w/: ------a) NORMAL Bleeding time ----------[Pt has INCREASED Bleeding time] ------b) NORMAL PT ------c) ↑ aPTT ---------aa) Intrinsic pathway is affected --------------[Pt has NORMAL aPTT] ---2) FIRST SYMPTOM ------a) Usually Hemarthrosis ---------[vWF presents w/ MUCOSAL bleeding]

TRUE LEARN A 25-year-old man comes to the physician for the evaluation of recurrent episodes of nosebleeds over the past 6 months. The nosebleeds occur spontaneously and stop after 10 minutes after pinching the nose at the nostrils. He has no history of serious illness except for prolonged bleeding following wisdom teeth extraction 2 years ago. He does not smoke or drink alcohol. He takes no medications. Vital signs are within normal limits. Examination of the nose shows no abnormalities. There are several bruises on the lower extremities. The remainder of the examination shows no abnormalities. Laboratory studies show: - Leukocytes------------------------------6K - Hb---------------------------------------15 - PLTS-------------------------------------220K - Bleeding time---------------------------9 min - PT time----------------------------------13 sec (N 11-13) - aPTT-------------------------------------55 (N 35-45) What is the most likely Dx? (Hemophilia A VS vWF deficiency)

Intravascular hemolysis - Pt most likely presents w/ Exertional hemoglobinuria → which is a type of INTRAVASCULAR hemolysis -A) Characteristics SPECIFIC to intravascular hemolysis include: -----1) ↑ LDH w/ ↓ haptoglobin -----2) Hemoglobinuria Extravascular hemolysis - Usually seen in the setting of Hereditary spherocytosis & other hemoglobinopathies - Key Differences include: ---1) NOT ASSOCIATED w/ hemoglobinuria

TRUE LEARN A 25-year-old woman comes to the physician for a routine examination. She is a recreational runner and for the past 3 months she has been training for a marathon. Yesterday she ran 17 km (10.5 mile). Menses occur at regular 28-day intervals and last 6 days. She drinks three to five beers when going out with friends. She follows a vegan diet. She appears well. Her temperature is 36.6°C (98.0°F), blood pressure is 110/72 mm Hg, pulse is 70/min, and respirations are 14/min. Physical examination shows no abnormalities. Laboratory studies show: - Hb---------------------------------------9.8 - MCV-------------------------------------91 - LDH-------------------------------------400 - PLTS-------------------------------------250K - RC count--------------------------------3% - Haptoglobin-----------------------------23 (N 41-165) - Iron---------------------------------------90 - Ferritin-----------------------------------170 - TIBC--------------------------------------220 (N 251-406) Urinalysis shows trace blood but no RBC's. Which of the following is the most likely cause of this pts anemia? (Extravascular hemolysis VS Intravascular hemolysis)

Increased Hemoglobin Barts Concentration - Newborn most likely presents w/ α-Thalassemia -A) Signs/Symptoms ----1) Microcytic anemia ----2) Jaundice ----3) Hepatosplenomegaly -B) Associated labs ----1) Microcytic anemia ----2) Hb DNA test -------a) 3 missing alleles is indicative of Barts hemoglobin -C) MGMT ----a) TMT -------1) Mainly supportive as newborn will most likely die Decreased Reticulocyte Ct - Either alpha-thalassemia OR Beta-Thalassemia would have an INCREASED reticulocyte CT → (d/t hemolytic anemia b/c this is the natural response) - A DECREASED reticulocyte count is associated w/ congenital infections w/ bone marrow suppression (parvo B-19, Rubella)

TRUE LEARN A 3080-g (6-lb 13-oz) male newborn is delivered at term to a 27-year-old woman, gravida 2, para 1. Pregnancy was uncomplicated. He appears pale. His temperature is 36.8°C (98.2°F), pulse is 167/min, and respirations are 56/min. Examination shows jaundice of the skin and conjunctivae. The liver is palpated 2-3 cm below the right costal margin, and the spleen is palpated 1-2 cm below the left costal margin. The lungs are clear to auscultation. No murmurs are heard. His hemoglobin concentration is 10.6 g/dL and mean corpuscular volume is 73 μm3. Hemoglobin DNA testing shows 3 missing alleles. Which of the following laboratory findings is most likely present in this patient? (Decreased reticulocyte Ct VS Increased hemoglobin Barts concentration)

Hep C antibody testing - Pt presents w/ signs concerning for ITP -A) Etiology -----a) ITP can have 3 major etiologies --------1) Primary -----------a) Anti-PLTS Antibody against GIIb/IIIa -----------b) Idiopathic --------2) Secondary -----------a) Associated w/ underlying medical condition --------3) Drug-induced/drug-dependent antibodies -----------a) Heparin-induced (HIT) -B) Secondary ITP Associated medical conditions: -----1) Hep C -----2) Autoimmune disease -----3) Lymphoproliferative disorders -----4) HIV -C) MGMT ----a) Dx Tests -------1) Testing focus should be on RULING OUT these diseases Anti-PLTS antibody - Would be associated w/ primary (idiopathic) ITP - However, you must FIRST RULE OUT other disease before Dx ITP as primary (you must ASSUME ITS SECONDARY)

TRUE LEARN A 31-year-old woman comes to the doctor because of episodic nosebleeds and gingival bleeding for the past 2 weeks. She has no history of serious illness and takes no medications. She is sexually active with two male partners and uses condoms inconsistently. Vital signs are within normal limits. Examination shows punctate, non-blanching macules on the chest. The remainder of the examination shows no abnormalities. The hemoglobin concentration is 13.1 g/dL, leukocyte count is 6600/mm3, and platelet count is 28,000/mm3. A peripheral blood smear shows reduced platelets with normal morphology. HIV test is negative. Which of the following is the most appropriate next step in management? (Anti-plts antibody testing VS Hep C antibody testing)

Splenectomy - Pt has ITP that is REFRACTORY to first-line TMTs -A) Signs/Symptoms → REFRACTORY ITP: ----1) Persistent & severe thrombocytopenia ----2) Recent Hx of multiple episodes of uncontrolled bleeding -B) MGMT ----a) Dx Tests -------1) HIV-1/2 immunoassay -------2) HCV antibody -------3) PBS review -----------a) ↓ PLTS (<30K) -------4) BM aspiration -----------a) NORMAL/INCREASED Megakaryocytes ----b) TMT - BASED ON PRESENTATION ------1) Mainstay of TMT ----------a) IV Corticosteroids (PREFERRED)/IVIG ----------b) Splenectomy (elective) --------------aa) PLTS ct must be >50K --------------bb) IVIG administered a week prior to splenectomy ------2) Pts refractory to first-line TMT who are actively bleeding intracerebral hemorrhage + PLTS <20K ------------a) Splenectomy (PREFERRED) ------------b) Rituximab/TPO agonist (Romiplostim, eltrombopag, avatrombopag) ------3) PTs w/ severe bleeding (intracerebral hemorrhage) & PLTS <30K ----------a) PLTS transfusion ----------b) IVIG ----------c) Glucocorticoids ----------d) Romiplostim (TPO analog) Observation & follow-up - Mainstay TMT of TTP

TRUE LEARN A 38-year-old woman comes to the physician for a follow-up examination. She was diagnosed with immune thrombocytopenic purpura at the age of 37 years and has been treated with glucocorticoids and intravenous immune globulin. She has visited the emergency department 3 times in the past 4 months for nose bleeds, which required cauterization. Her platelet counts on her previous visits were 18,320/mm3, 17,500/mm3, and 19,100/mm3. Current medications include dexamethasone and a multivitamin. She has no children. Her immunizations are up-to-date. Vital signs are within normal limits. Examination shows petechiae on the bilateral lower extremities. Cardiopulmonary examination shows no abnormalities. The abdomen is soft and nontender; there is no organomegaly. Her hemoglobin concentration is 13.3 g/dL, leukocyte count is 8,100/mm3, and platelet count is 13,000/mm3. Her blood type is A negative. Serology for hepatitis C and HIV is negative. Which of the following is the most appropriate next step in management? (Splenectomy VS Observation & follow-up)

Reassurance - Pt presents w/signs concerning for beta-thalassemia minor -A) Associated Labs ----1) Decreased MCV (KEY LAB FINDING) ----2) Normal RDW (KEY LAB FINDING) ----3) Mildly decreased Hb ----4) Target cells (on PBS) ----5) MILDLY ELEVATED HbA2 levels -B) Signs/Symptoms ----1) NO SYMPTOMS except asymptomatic anemia -C) MGMT ----a) Dx tests -------1) PBS -------2) Electrophoresis -----------a) ↑ HbA2 is CONFIRMATORY ----b TMT -------1) Supportive/Observation (Reassurance) Folic Acid - Would be used in a TMT for MACRO-cytic anemia and/or β-thalassemia MAJOR

TRUE LEARN A 4-year-old boy is brought to the physician for the evaluation of fatigue since he returned from visiting family in South Africa one week ago. The day after he returned, he had fever, chills, and diffuse joint pain for 3 days. His symptoms improved with acetaminophen. He was born at term and has been healthy. His immunizations are up-to-date. His temperature is 37.6°C (99.68°F), pulse is 100/min, and blood pressure is 100/60 mm Hg. Examination shows conjunctival pallor. The remainder of the examination shows no abnormalities. Laboratory studies show: - Hb---------------------------------------10.8 - MCV-------------------------------------68 - RCDW------------------------------------14% (N 13-15%) - Hb A2------------------------------------6% (N <3.5%) A PBS shows microcytic, hypochromic erythrocytes, some of which have a darkly stained center & a peripheral rim, separated by a pale ring. Which of the following is the most appropriate next step in MGMT? (Folic acid therapy VS Reassurance)

Folate deficiency - Both Folate & Vit B1 deficiency can present w/ confusion, horizontal nystagmus, & ataxia - However, only folate deficiency would present w/: ---1) Macrocytic anemia (hyper-segmented neutrophils) ---2) INCREASED homocysteine levels - RF associated w/ folate deficiency: ----1) Chronic alcohol use/abuse Vit B1 deficiency - Can present w/ confusion, horizontal nystagmus, & ataxia - However, would NOT BE A CAUSE OF MACROCYTIC ANEMIA (or any anemia)

TRUE LEARN A 44-year-old woman is brought to the emergency department because of confusion and agitation. She was brought by police after she was found walking along a highway. The patient's brother comes to join her soon after her arrival. He says she has peptic ulcer disease and hypertension. He thinks she drinks around half a bottle of vodka daily. Her current medications include omeprazole and hydrochlorothiazide, although the brother is unsure if she takes them regularly. Her temperature is 37.1°C (98.7°F), pulse is 90/min, respirations are 16/min, and blood pressure is 135/90 mm Hg. On mental status examination, she is confused and not oriented to person, place, or time. Neurologic examination shows horizontal nystagmus. Her gait is wide-based with small steps. Her hemoglobin concentration is 9 g/dL. Her serum homocysteine concentration is elevated and her methylmalonic acid concentration is within the reference range. A peripheral blood smear shows hypersegmented neutrophils. Which of the following is the most likely cause of this patient's anemia? (Folate deficiency Vs Vit B1 deficiency)

Hemodialysis - Pt presents w/signs concerning for HUS -A) Signs/Symptoms → (PENTAD presentation) ----1) GI symptoms -------a) MORE COMMON IN HUS (Vs TTP) ----2) Acute renal failure/injury (PREDOMINANT FEATURE) ----3) Microangiopathic hemolytic anemia (MAHA), --------a) HALLMARK of the disease ----4) Thrombocytopenia --------a) Petechiae ----5) Neurologic abnormalities (altered mental status) -------a) RARE in HUS → more COMMON in TTP ----6) Fever --------a) Typically low/may even be absent -B) Associated labs ----1) Schistocytes --------a) d/t non-immune hemolysis of erythrocytes ----2) Normochromic & normocytic RBCs ----3) ADAMTS13 activity level <10% -C) MGMT ----a) TMT --------1) Plasma exchange ------------a) Until PLTS Ct & LDH normalize --------2) Hemodialysis ------------a) In pediatric pts →indications for hemodialysis include: ------------------1) Oliguria/Anuria ------------------2) Azotemia ------------------3) Hyperkalemia ------------------4) Acidosis ----------------------(pt is in Acidosis)

TRUE LEARN A 5-year-old girl is brought to the emergency department because of abdominal pain, vomiting, and diarrhea for 6 days. Her mother says that over the last 24 hours she has developed a rash and has been urinating less frequently than usual. One month ago, she had a 3-day episode of high fever and sore throat that subsided without medical treatment. She appears weak. Her temperature is 37.7°C (99.8°F), pulse is 120/min, respirations are 28/min, and blood pressure is 114/72 mm Hg. Examination shows petechiae on the trunk and jaundice of the skin. The abdomen is diffusely tender with no peritoneal signs. Neurological examination shows no abnormalities. Laboratory studies show: - Leukocytes------------------------------16,2K - Hb---------------------------------------8 - MCV-------------------------------------85 - PLTS-------------------------------------38K - BUN-------------------------------------43 - Cr-----------------------------------------2.9 - pH----------------------------------------7.0 Urine dipstick is positive for blood and protein. A blood smear shows schistocytes and normochromic, normocytic cells. In addition to supportive treatment, which of the following is the most appropriate next step in management of this patient? (Hemodialysis VS Plasma exchange therapy)

Mechanical destruction - Pt presents w/ signs concerning for mechanical destruction of RBCs -A) RF ----1) Aortic stenosis --------a) Late crescendo-decrescendo systolic murmur --------b) Bother that has a bicuspid aortic valve -B) PBS findings ----1) Schistocytes -------a) Indicate an extravascular hemolysis → AS causes a SHEARING of RBCs last which leads to schistocytes Erythrocyte enzyme defect - Describes G6PD - Associated w/ recent antibiotic use - BOTH G6PD & Mechanical destruction of RBC's would have TARGET CELLS (this indicates that INCREASED RBC synthesis is occurring) - Key to making correct Dx ---1) PBS FINDINGS → G6PD presents w/ ------a) Heinz bodies & bite cells ---------[NOT on this pts PBS] ------b) NOT ASSOCIATED w/schistocytes ---------[Pt DOES HAVE schistocytes on PBS]

TRUE LEARN A 52-year-old man comes to to the emergency department with fatigue and shortness of breath that has become progressively worse over the past week. He had an upper respiratory tract infection 2 weeks ago, for which he was given an antibiotic. He has hypertension, type 2 diabetes mellitus, and colonic polyps diagnosed on screening colonoscopy 2 years ago. His mother has systemic lupus erythematosus and his brother has a bicuspid aortic valve. He does not smoke cigarettes or drink alcohol. Current medications include lisinopril and metformin. His temperature is 37.3°C (99.1°F), pulse is 91/min, respirations are 18/min, and blood pressure is 145/84 mm Hg. His conjunctivae are pale. Cardiac examination shows a late systolic crescendo-decrescendo murmur at the right upper sternal border. Laboratory studies show: - Leukocytes------------------------------9,5K - HCT--------------------------------------24% - PLTS-------------------------------------178K - LDH--------------------------------------215 - Na+---------------------------------------140 - K+-----------------------------------------4.6 - Cl-----------------------------------------100 - HCO3------------------------------------25 - BUN--------------------------------------21 - Cr------i----------------------------------1.2 - Total bili----------------------------------1.9 - Haptoglobin-----------------------------22 (N 41-165) - LDH---------------------------------------215 A PBS is shown. Which of the following is the most likely cause of this pts anemia? (Mechanical destruction of RBC's VS Erythrocyte enzyme defect)

Imatinib - Pt has signs concerning for Lymphoma - Translocation confirms the Dx of CML -A) MGMT ----a) Dx test --------1) FISH --------2) Karyotyping --------3) RT-PCR ----b) TMT --------1) Imatinib (PREFERRED INITAL) ------------a) However can also use other BCR-ABL TK inhibitors including Dasatinib, Nilotinib, Bosutinib Infliximab -Monoclonal Ab against TNF-α - Used mainly in Inflammatory conditions (RA, Crohns)

TRUE LEARN A 60-year-old man presents to the physician's office for a routine physical examination. He has no specific complaints today and has been generally well-feeling aside from some fatigue which he attributes to "old age." On examination, he is afebrile and his blood pressure is 138/88 mm Hg. Examination is significant for generalized pallor and a spleen tip palpable 4 cm below the left costal margin. A complete blood count is below: - Leukocytes-----------------------------65K - Neutros---------------------------------70% - Lymphos--------------------------------13% - Monocytes------------------------------3% - Eosinophils------------------------------4% - Basophils--------------------------------8% - Hb---------------------------------------8.9 A peripheral smear is abnormal and reflective of the subsequent bone marrow biopsy, which reveals granulocytic infiltration with a predominance of neutrophils as well as eosinophilia, basophilia, and 2% blasts. The granulocytes are large and have a high nuclear: cytoplasmic ratio. Cytogenetic studies demonstrate a reciprocal translocation between chromosomes 9 and 22. Which of the following is the most appropriate therapy for this patient's disease? (Imatinib VS Infliximab)

Polycythemia - Pt presents signs & RF associated w/ polycythemia -A) RF -----1) Adults males, typically ~65 -----2) -B) Signs/Symptoms -----1) Aquagenic pruritus ---------a) Itching following exposure to warm water ---------b) ± Linear excoriations ----2) Erythromelalgia ---------a) Episodic blockage of blood vessels followed by burning pain in the extremities with erythema, cyanosis, pallor + INTACT distal pulses ---------b) ± Palpable cord in calf (DVT) ---------c) Responsive to aspirin -----3) Symptomatic gout ---------a) (5-10% of cases) -----4) Splenomegaly -----5) Blurry vision/headache -----6) Facial plethora --------a) Facial flushing, ruddy appearance -C) Complications -----1) Budd Chiari -D) Associated labs -----1) Thrombocytosis -----2) ↓ EPO levels Essential thrombocytosis - Also presents w/ thrombocytosis, splenomegaly, erythromelalagia - However, key differences include: ---1) THROMBOCYTOSIS is usually ≥ 600K --------[pts PLTS ct is 400K]

TRUE LEARN A 65-year-old man presents to the Emergency Department with leg pain that woke him from sleep this morning. It is severe and burning in quality, limited to his left calf. He also complains of headache, dizziness and malaise for the past three weeks. His only medical history is mild hypertension and benign prostatic hypertrophy for which he takes hydrochlorothiazide and tamsulosin. He drinks red wine occasionally and quit smoking 20 years ago after 30 years. On review of systems, he has several other complaints, including diffuse skin itching following hot showers for the past two months, and intermittent, sporadic burning pain and redness in his hands and feet that improves with aspirin. His temperature is 38.0ºC (100.4ºF), pulse is 85/minute, respirations are 14/minute, and blood pressure is 140/85 mmHg. His face is flushed and ruddy-appearing. On abdominal examination, his spleen tip is palpable 4 cm below the left costal margin. There are linear excoriations over his arms, legs and trunk. A palpable cord is appreciated in the left calf, which is exquisitely painful to touch and associated with overlying erythema and warmth. Doppler ultrasound of the left lower extremity confirms a deep vein thrombosis in a deep left calf vein extending to the popliteal vein. A complete blood count is shown below: - Leukocytes------------------------------11,000K - Hb----------------------------------------17.8 - PLTS--------------------------------------400K Which of the following is the most likely diagnosis in this patient? (Polycythemia VS Essential thrombocytosis)

Leukocytoclastic vasculitis - This is an "umbrella term" for vasculitides, including HSP -A) Signs/Symptoms → (CLASSIC HSP presentation) ----1) Preceding URI infection --------a) Pharyngitis 2 weeks ago ----2) Palpable purpura --------b) Non-blanching/non-pruritic skin lesions ----3) Abd pain & arthritis ----4) Occult bleeding/bloody stools -B) Associated labs ----1) RAISED PLTS Ct ----5) Nephritic syndrome --------a) Hematuria, proteinuria Juvenile idiopathic arthritis - Also presents w/ rash & joint pain in an adolescent patient - However, key differences include: ---1) JIA HAS SPECIFIC CRITERIA, including: -------a) Joint inflammation that lasts > 6 WEEKS ----------[Pt has had joint pain for 6 DAYS] ---2) NOT TYPICALLY SSOCIATED W/ JIA include: -------a) Abd pain -------b) Recent URI (pharyngitis) Acute RF - Acute RF also presents 2-4 weeks after a URI & presents w/ Joint pain & a rash , - However, key differences include: ---1) MISSING KEY SYMPTOMS OF ACUTE RF, including ------a) Chorea ------b) Sub-Q nodules ------c) Carditis (abnormal ECG/new murmur) ------d) Neuro involvement ---2) PT HISTORY ------a) The pt was treated w/ amoxicillin (2 weeks previously → amoxicillin is used to treat Acute RF so the pt should have had RESOLUTION OF SYMPTOMS

TRUE LEARN A 7-year-old girl is brought to the emergency department because she has had abdominal pain and vomiting for the past day. The pain is intermittent, diffuse in nature, and worse after meals. She does not have loose or bloody stools. She has had a nonpruritic rash on her legs and swelling of her ankles for 6 days. She was treated with oral amoxicillin for a sore throat 2 weeks ago. Her immunizations are up-to-date. Vital signs are within normal limits. Examination of the lower extremities shows non-blanching, raised erythematous papules. The left ankle joint is swollen and warm, and its range of motion is limited by pain. Abdominal examination shows tenderness to palpation in the left lower quadrant without guarding or rebound. Bowel sounds are normal. Test of the stool for occult blood is positive. Laboratory studies show: - Leukocytes------------------------------11,8K - Hb---------------------------------------10.1 - PLTS-------------------------------------431K - ESR--------------------------------------45 - Glucose----------------------------------72 - Cr-----------------------------------------0.9 Which of the following is the most likely Dx? (Juvenile idiopathic arthritis VS Leukocytoclastic vasculitis VS Acute RF)

Cryoprecipitate -A) MGMT for hemophilia includes: -----1) Cryoprecipitate/Factor VIII --------a) PREFERRED in BLEEDING pts -----2) Emicizumab​ -----3) Desmopressin acetate --------a) Mild disease only

TRUE LEARN A 9-year-old boy presents to the Emergency Department with dark colored urine. A few hours ago, he was playing with friends in the neighborhood when another boy accidentally tripped him and fell on his back. His mother states that he bruises easily and has a history of bleeding into his knee joint after a minor fall. His uncle (her brother) has a "blood disorder" and, like her son, bruises easily; her brother has a history of major bleeding during surgery. On examination, he is in no apparent distress but is complaining of back pain. His vitals are within normal limits. His lungs are clear, and heart has a normal S1 and S2 with regular rate and rhythm. His abdomen is soft and non-tender, there is no suprapubic tenderness. There is a large 6cm x 4cm ecchymosis on his left flank. There is gross blood evident in the urine specimen with no protein, glucose, or white cells. Which of the following is the next appropriate step in the management of this patient? (Cryoprecipitate VS Desmopressin)

TTP ***SIDE NOTE Fever, headache, nausea, abd discomfort, microangiopathic anemia, thrombocytopenia, & acute glomerular injury are symptoms of BOTH HUS & TTP*** -A) Features Unique to TTP are: -----1) RFs -------a) Pregnancy is a RF for TTP ------------aa) NOT A RF for HUS -----2) TTP usually presents w/ mucocutaneous bleeding as first symptom -----3) HUS is usually seen in children → w/ preceding bloody diarrhea

TRUE LEARN A previously healthy 35-year-old primigravid woman at 12 weeks' gestation comes to the physician because of a fever, persistent headache, nausea, and abdominal discomfort for 1 week. During this time, she has also noticed that her gums bleed while brushing her teeth. A month ago, she returned from a camping trip to Sri Lanka. Her temperature is 39.3°C (102.8°F), pulse is 104/min, respirations are 24/min, and blood pressure is 135/88 mm Hg. Examination shows pallor and mild scleral icterus. There are a few scattered petechiae over the trunk and back. There is no lymphadenopathy. Physical and neurologic examinations show no other abnormalities. Test of the stool for occult blood is positive. Laboratory studies show: - Leukocytes------------------------------10K - Hb---------------------------------------8.2 - PLTS-------------------------------------18K - INR---------------------------------------1.0 - Coomb's---------------------------------neg - Fibrin split products---------------------neg - Urea--------------------------------------20 - Cr-----------------------------------------1.1 - Total bili----------------------------------3.0 - Direct Bili---------------------------------0.8 - ALT---------------------------------------20 - AST----------------------------------------16 - LDH---------------------------------------900 -----URINE----- - Protein------------------------------------1+ - WBCs--------------------------------Occasional - RBCs------------------------------------50-60 - Bacteria----------------------------------n/a Blood & urine cultures are negative. What is the most likely Dx? (HUS VS TTP)

F VIII - Desmopressin promotes the release of vWF from endothelial cell storage sites - In doing so, the increased vWF binds available factor VIII → preventing its degradation → thus effectively INCREASING F VIII concentrations

TRUE LEARN An 11-year-old female is brought to the physician by her mother for excessive menstrual bleeding. She began her menstrual period three months ago. Her cycles are regular and occur every 24 days; however, each menstrual period lasts 7-8 days, and she needs to change sanitary pads 7-8 times a day and 2-3 times each night. She also complains that she cannot keep up with her classmates during gym class due to being easily fatigued. She has a history of multiple nosebleeds, and easy bruising, which "runs in the family." Her physical examination is unremarkable. Ristocetin cofactor activity reveals decreased von Willebrand Factor activity. She is prescribed desmopressin prior to menstruation to decrease bleeding. Which of the following factors increase following desmopressin administration? (F VIII VS F X)

TTP - Pt has signs concerning for TTP -1) RF ----1) Surgery -B) Signs/Symptoms → PENTAD presentation ----1) Microangiopathic Hemolytic anemia ----2) Neurological -------a) Decreased mental status ----3) Thrombocytopenia ----4) High fever ----5) Acute renal failure ----6) Purpura (Rash) DIC - Also presents w/ decreased mental status, petechiae, thrombocytopenia & fever - However, key differences include: ---1) MISSING KEY DIC SYMPTOMS, including: ------a) Bleeding from multiple sites ------b) Hemodynamically UN-stable ----------[pt is hemodynamically stable & does not report bleeding]

TRUE LEARN (Q1/2) A 38-year-old man is brought to the Emergency Department by his wife with fever and a several hour history of confusion. The patient's wife states that when he woke up this morning, he was very irritable and complaining of feeling hot. She also noticed a new rash on his legs and brought him to the hospital. His temperature is 39.6ºC (103.2ºF), pulse is 90/minute, respirations are 14/minute, and blood pressure is 110/68 mmHg. He is sleepy and irritable when aroused. Initial labs are below. - Hb---------------------------------8.3 - PLTS-------------------------------24K - MCV-------------------------------89 - Leukocytes------------------------8K Which of the following is the most likely diagnosis? (TTP VS DIC)

Plasma exchange -A) MGMT for TTP ----a) TMT -------1) Plasma exchange ----------a) Should be continued until PLTS ct & LDH normalize -------2) Plasma infusion ----------a) If plasma exchange is not available Fresh frozen plasma & whole blood transfusion - Used as alternative to plasma exchange when it is not available - Plasma exchange has been shown to be more efficacious then FFP or whole blood

TRUE LEARN (Q2/2) A 38-year-old man is brought to the Emergency Department by his wife with fever and a several hour history of confusion. The patient's wife states that when he woke up this morning, he was very irritable and complaining of feeling hot. She also noticed a new rash on his legs and brought him to the hospital. His temperature is 39.6ºC (103.2ºF), pulse is 90/minute, respirations are 14/minute, and blood pressure is 110/68 mmHg. He is sleepy and irritable when aroused. Initial labs are below. - Hb---------------------------------8.3 - PLTS-------------------------------24K - MCV-------------------------------89 - Leukocytes------------------------8K The pt is Dx'd w/ TTP. Intravenous access is obtained and the patient is started on fluids and empiric antibiotics for possible sepsis. Which of the following is the next appropriate step in the management of this patient? (Plasma exchange VS Fresh frozen plasma VS Whole blood transfusion)

Immune mediated hemolysis - Pt presents w/ anemia in the setting of CLL -A) Pathophysiology ----1) Most cases of anemia + HIGH reticulocyte Ct are associated w/: -------a) Acute bleeding -------b) Hemolysis ----2) Pts w/ CLL often have significant IMMUNE dysregulation, which triggers formation of IgG antibodies against the RBC membrane (warm agglutinins) → leading to Warm AUTOIMMUNE hemolytic anemia Leukemic BM infiltration - A high RC count suggests that: there are adequate levels of iron, folate, & Vit B12 - It also suggests that the BM is responding appropriately to the anemia by increasing the production of RBCs

UWORLD A 68M, presents d/t 2 weeks of worsening fatigue & exertional dyspnea. The pt was Dx'd w/ early-stage leukemia a year ago but has not required TMT b/c he has not had sig symptoms. Vitals are normal. PE shows generalized lymphadenopathy & hepatosplenomegaly. Labs results show: - Hb---------------------------------7.1 - Reticulocytes---------------------6% - PLTS-------------------------------210K - Leukocytes------------------------44,8K - Lymphocytes----------------------80% Which of the following is the most likely underlying cause of these pts anemia? (Leukemic BM infiltration VS Immune mediated hemolysis)