HEME quizlet #1 - 5/3/20

Darwin Charles, a 50-year-old male, presents to your clinic today with a complaint of fatigue for the past 4 weeks. He also states he has had a cough for around the same amount of time. In Mr. Charles chart, you notice is a smoker of 30 years. Mr. Charles denies fever, night sweats or unexplained weight loss. On physical exam, you note an enlarged, non-tender, fixed cervical lymph node. Mr. Charles denies every noticing it was there. You decide to do an incisional biopsy on the lymph node and subsequently you diagnose Darwin Charles with Non-Hodgkin's Lymphoma. How are you going to treat Mr. Charles? (A) Careful observation (no treatment) (B) Radiation therapy (C) R-CHOP chemotherapy (D) stem cell bone marrow transplant (E) All of the above are treatment options depending on the type and subtype of Mr. Charles' NHL

A10. E. NHL is treated differently depending on what type and subtype of disease you are diagnosed with. A through D are all options in how to treat NHL.

"Q10. You are seeing a 36 y/o male on your ICU rounds who was admitted yesterday with septic shock. He has since developed a lower GI bleed that has not stopped and multiple purpura on his chest and upper extremities. You order a STAT CBC, INR and PTT. The CBC reveals a platelet count of 15k/uL (normal 150-450 k/uL) INR and PTT are both elevated. You immediately administer norepinephrine and start a 2 L bolus of NS. What is the next most appropriate step in treating this patient? (A) Give platelets (B) Give FFP (C) Give heparin (D) a and b (E) all of the above

A10. The answer is d. a and b. This patient is presenting with DIC secondary to a septic shock. Because he is currently bleeding (GI) and has a critically low plt count, he should receive both FFP and platelets. Anticoagulants such as heparin would not be beneficial as this patient is having bleeding symptoms.

John is a 62 year old male, following up with you in the GI clinic for his cirrhosis, who complains today of increased dyspnea on exertion, weakness, and fatigue over the past 3 months since his last visit with you. Remainder of your ROS is negative. Physical exam reveals mild hepatomegaly and shifting dullness, unchanged from his last examination. You decide to order a reticulocyte count and a CBC, which reveals a mild microcytic anemia, new when compared to CBC obtained at his last visit. Iron studies are negative for iron deficiency anemia. Peripheral blood smear reveals a mild microcytic anemia. Which of the following would NOT be helpful in treatment of John's microcytic anemia? A.Iron replacement. B.Treatment of John's Cirrhosis C. Referral to Hematologist for long term management D. All of the above would be helpful for John.

A10: Answer is a. This is supposed to represent a patient with anemia of chronic disease. Can rule out iron deficiency anemia, thalassemia and genetic sideroblastic anemia from his normal hemoglobin at his last visit, and non-diagnostic mild microcytic anemia. He also has cirrhosis, which alongside renal disease is the most common chronic etiology. Iron replacement has generally NOT been helpful in treatment, but referral to hematologist, and identification and tx of underlying disease are appropriate.

Opal, a 28-year-old female, is bringing in her daughter, Turquoise, for her 12-month check-up. Opal tells you she is concerned about her daughter's growth. She has been noticing her daughter's appetite has decreased and she is unable to gain weight. Upon exam, you find that Turquoise is below the 20th quartile of growth for her age and notice marked conjunctival pallor. On palpation you are able to feel her liver and spleen and growing more concerned, you decide to order some labs. They come back as follows: Reticulocyte count - low MCV - microcytic Iron studies - WNL PBS - microcytosis, hypochromia, nucleated RBC's Erythrocyte porphyrin test - WNL Hemoglobin electrophoresis - primarily increased hemoglobin F When reading the labs, you confirm that Turquoise has the diagnosis you suspected. You explain this to her mother Opal and begin explaining the treatment for this disease entails lifelong blood transfusions with chelation therapy, bone marrow transplants and possibly a splenectomy due to her enlarged spleen. What is Turquoise's most likely diagnosis? (A) beta-thalassemia minor (B) alpha thalassemia mutation 4 out of 4 (C) beta thalassemia major (D) alpha thalassemia 1 out of 4

A10: C. Immediately we can rule out alpha thalassemia 4 out of 4 mutations because we know this is incompatible with life. We also know that 1 of 4 mutations is considered a silent carrier, and thus Opal would not present with such severe symptoms. When deciding between beta-thalassemia major or minor the biggest giveaway lies in the hemoglobin electrophoresis and treatments given. In major, the hemoglobin electrophoresis shows increased hemoglobin F, in minor its increased hemoglobin A2. The treatment for major includes the treatments listed above, while in minor usually requires no treatment at all. Thus, B-thalassemia major is the correct diagnosis. Note specifically to B-thalassemia major, it commonly appears within the first 2 years of life, occurring with hepatosplenomegaly, poor growth, and skeletal abnormalities.

Which of the following regarding erythropoiesis is concerning for pathology? A.22 year old female, with erythropoiesis occurring in the vertebrae. B.4 month old male, with erythropoiesis occurring in the liver. C. 3 year old female, with erythropoiesis occurring in the spleen. D. 56 year old male, with erythropoiesis occurring in the ribs. E. None of the above are concerning for pathology, all represent the normal physiologic process of erythropoiesis.

A1: Answer is c. This question is regarding the normal physiologic location of erythropoiesis. Prior to bone marrow, hematopoiesis occurs in the fetal yolk sac, followed by the liver and spleen. By 7 months of age, it should be occurring in the bone marrow. In adults, this is typically the axial skeleton. After infancy --> Extramedullary hematopoiesis is pathologic. Therefore, 3yo female, should not be having erythropoiesis occurring in the spleen, and would be concerning for pathology.

Which of the following is false in regard to Hairy Cell Leukemia? (A) monocytopenia is commonly seen. (B) Chemotherapy leads to remission in 2/3 of patients. (C) It affects the B-cells. (D) lymphadenopathy is commonly seen.

A1: D. Hairy cell leukemia is an uncommon B-cell leukemia. Pancytopenia is a common manifestation - especially monocytopenia. Chemotherapy shows long term remission in 2/3's of patients. BUT lymphadenopathy is a very UNcommon finding in this cancer!

Thalassemia disruptes which of the follow component(s)? A. alpha globin chains B. beta globin chains C. protophorphyrin D. A and B E. all of the above

A1: D. Thalassemia is defined as decreased production or structural abnormality of either of the two normal globin chains (alpha or beta type). Sideroblastic anemia is define as microcytic anemia due to failure to synthesize the porphyrin ring - thus answer C.

Harry, a 50-year-old male, presents to your office today for his annual wellness check-up. Brad has no concerns for his health and his PMH is unremarkable. Upon physical exam you become concerned due to the presence of splenomegaly and conjunctival pallor. You decide to run some labs and they come back as follows: CBC: monocytopenia, normocytic anemia PBS: enlarged lymphocytes with villous cytoplasmic projections Bone marrow biopsy: mild fibrosis and diffuse cellular infiltrate of B-cells w/ villous cytoplasmic projections Which of the following cancers does Brad most likely have? (A) Hairy cell leukemia (B) acute myeloid leukemia (C) chornic myeloid leukemia (D) chronic lymphocytic leukemia

A2. A. Brad is presenting with hairy cell leukemia. This cancer is very uncommon, but if seen, is most common in men ages 40 to 60. It's common for these patients to present with splenomegaly, an infection or anemia. Monocytopenia is especially common in these patients. The PBS is the biggest giveaway. It is showing "hairy" B-cells, as does the bone marrow biopsy.

Abnormal formation of an erythrocyte can occur from deficiency in all of the following, except? A.Folic acid B.Iodine C.Iron D.Vitamin B12

A2: Answer is b. This question is asking about the building blocks for an erythrocyte. The building blocks include iron (needed to make hgb), vitamin B12 (needed for DNA stimulated synthesis of RBC/maturation and development of precursor cells), and folic acid (needed for DNA stimulated synthesis of RBC/maturation and development of precursor cells). Iodine deficiency would make you think thyroid problems.

Which of the following is NOT true of alpha - thalassemia with mutations in 3 of 4 genes? (A) termed Hemoglobin H Disease (B) results in mild microcytic anemia (C) it allows excess beta chains to form tetramers (D) Specially stained PBS allow this to be seen on the inside of RBC's (E) all of the above are true

A2: B. Alpha thalassemia that results due to 3 of 4 gene mutations results in a moderate to severe microcytic anemia. Alpha thalassemia that has 2 of 4 gene mutations results in mild microcytic anemia. If you recall, 1 of 4 gene mutations is a silent carrier, thus you have no symptoms and 4 of 4 gene mutations is incompatible with life. The remaining options are all true of alpha-thalassemia with mutations in 3 of 4 genes.

Q31: Which of the following is NOT a myeloproliferative disorder? A. Essential Thrombocytosis B.Myelofibrosis C.Waldenstrom's Macroglobulinemia D.Polycythemia Vera

A31: The answer is C. The myeloproliferative disorders include polycythemia vera, essential thrombocytosis, and myelofibrosis. Waldenstrom's Macroglobulinemia is a rare plasma cell malignancy, characterized by the presence of IgM monoclonal macroglobulin.

Q32: Which of the following statements about myeloproliferative disorders is FALSE? A.Myeloproliferative disorders usually have an indolent course and are chronic in nature. B. Myeloproliferative disorders cause proliferation of the myeloid cell lineage, including RBC's, platelets, monocytes, and leukocytes. C.Myeloproliferative disorders have a risk of transformation into acute myeloid leukemia. D. Myeloproliferative disorders can present with elevation of >1 myeloid cell line and some degree of bone marrow fibrosis.

A32: The answer is b. B is false because although myeloproliferative disorders are a proliferation of the myeloid cell lineage, they do not include proliferation of leukocytes. That is leukemia! The remainder are true of myeloproliferative disorders.

Q33: Which of the following is would be considered an appropriate treatment option for someone with essential thrombocytosis?A. Anagrelide in a 70yo female, with a history of CAD. B. Epoetin and Darbepoetin in a 50yo male. C.Aspirin and Hydroxyurea in a 70 yo female, with risk factors for thrombosis. D.Phlebotomy in a 60yo male, with no risk factors for thrombosis.

A33: The answer is c. This question is asking about appropriate treatment options for the myeloproliferative disorder, essential thrombocytosis. Remember treatment is controversial and only certain populations qualify for certain treatments. The correct answer is c, because the combination of aspirin and hydroxyurea can be used to prevent a thrombotic event, in a patient over 65 with a previous event or risk factors for thrombosis. Anagrelide can only be used in non-elderly without heart disease, making A wrong. Epoetin and Darbepoetin are treatment for myelodysplastic syndromes, to try and stimulate erythrocyte production, making B wrong. Phlebotomy is used for an excess of RBC, not excess of platelets, making D wrong.

Q34: Which of the following is FALSE about primary myelofibrosis? (A) Primary myelofibrosis is characterized by myeloid proliferation, and excessive fibrosis leading to bone marrow failure. (B) JAK mutation may be positive. (C) The major clinical manifestation is prominent hepatosplenomegaly, with subsequent abdominal pain and early satiety. (D) The prognosis is good, with a 95% 5 year survival rate.

A34: The answer is d. Primary myelofibrosis has a poor prognosis, with the average survival rate being 2-5 years. Also 8-10% of primary myelofibrosis patients also transform into AML which is rapidly fatal.

Q35: Which of the following is TRUE about the JAK2 mutation? A.If the JAK2 mutation is positive, you can make the diagnosis of Polycythemia Vera. B. The JAK2 mutation is positive in 95% of patients with essential thrombocytosis. C.The JAK2 mutation leads to a decrease in RBC production. D. The JAK2 mutation is not all that helpful in making the diagnosis of primary myelofibrosis.

A35: The answer is d. The only true statement is that the JAK2 mutation is not helpful in making the diagnosis of primary myelofibrosis, and that is because the JAK2 mutation is only positive in 50% of myelofibrosis patients and also can be positive in patients with Polycythemia Vera and essential thrombocytosis. A is false because you need 2 major criteria and 1 minor criteria or 2 minor and 1 major criteria to make the diagnosis of PV, not just a positive JAK2 mutation. B is wrong because the JAK2 mutation is only positive in 50% of patients with ET. C is wrong because JAK2 mutation leads to an increase in RBC production.

Q36: PBS reveals nucleated teardrop RBC's. Which of the following disorders is high on your differential diagnosis? A.Polycythemia Vera B.Iron deficiency anemia C.Primary myelofibrosis D.Multiple myeloma E.Chronic myeloid leukemia

A36: The answer is c. PBS with nucleated teardrop RBC should make you think primary myelofibrosis.

Q37: Which of the following is NOT an explanation for the prominent hepatosplenomegaly in primary myelofibrosis? A. Megakaryocytes proliferation and production of fibroblastic growth factors. B.Excess collagen production causing bone marrow fibrosis. C.Increased cell turnover leads to elevated uric acid levels. D.Impaired bone marrow function causes hematopoiesis to take place extramedullary.

A37: The answer is c. Primary myelofibrosis is a result of dysplastic megakaryocytes producing fibroblast growth factors that stimulate collagen production, leading to bone marrow fibrosis and impaired bone marrow function. This causes hematopoiesis to take place extramedullary and leads to hepatosplenomegaly. Although there is elevated uric acid levels from increased cell turnover, this will lead to things like Gout, not hepatosplenomegaly.

Q38: Ashley is a 70 year old female, with past medical history significant for HTN, CAD, Type 2 Diabetes, and hx DVT presenting to the clinic today for her annual physical. She has no complaints today. Her physical exam is unremarkable. You obtain routine labs for maintenance of her Type 2 Diabetes. CBC presents with the following results: RBC: 4.0 million cells/mcL (normal 3.9-5.03 million), Hemoglobin: 14grams/dL (normal 11.5 - 15grams/dL), Hematocrit: 39% (normal 35.5 - 44.9%), White blood cell count: 8,000 cells/mcL (normal 3,400-9,600cells/mcL), Platelet Count: 1,000,000/mcL (normal 160,000 - 380,000/mcL). You see the following results. Which of the following displays the most appropriate counseling to provide the patient at this time? A. Your platelet count is significantly elevated today, based off these findings, I am diagnosing you with essential thrombocytosis. We will now discuss some treatment options. B.Your platelet count is significantly elevated today, making me concerned for a myeloproliferative disorder called essential thrombocytosis. I would like to order one more test, looking for a gene mutation called JAK2, and if this returns positive, it will confirm my suspicion. C.Your platelet count returned quite elevated today. This could mean one of many things and I would like to re check this in 1 month. If it is still elevated, we will order more tests to rule out any other possible etiologies, such as iron deficiency anemia, infections, or any other concerning findings. D. Your platelet count is just a bit elevated today, I do not think we need to do any further testing today, but I would like to see you back in 6 months, and have this reevaluated.

A38: The correct answer is C. Yes, you should be concerned for essential thrombocytosis, however you must rule out other causes such as (IDA, inflammation, infection, and cancer) prior to making the diagnosis and beginning treatment. Absence of the Philadelphia chromosome would help us rule out CML. Remember, with an elevated platelet level, we should re check in 1 month to make sure the findings are not spurious.

Q39: In the vignette above, you decide to follow through and order a full evaluation for Ashley. There is no evidence of Iron deficiency anemia, inflammation, infection, or cancer, and no Philadelphia chromosome. You do get genetic testing and see the JAK2 mutation. Given this information, you diagnose Ashley with Essential Thrombocytosis. Which of the following is NOT the most appropriate treatment option for her? Pick the best answer. A. Anagrelide B. Monitoring for signs and symptoms of microvascular thrombi or erythromelalgia C. Combination of Aspirin and Hydroxyurea D. Monitor for progression into acute myeloid leukemia.

A39: The answer is a. Anagrelide is a treatment option for essential thrombocytosis, but can only be used in non-elderly patients without heart disease making this the wrong choice. D should also make you think, because although progression into AML is rare in ET, there is still a very small but real risk associated given it is a myeloproliferative disorder, so you do need to monitor for this.

Beta - thalassemia trait/minor has an increased level of _____________ by up to 3-6%. (A) Hemoglobin A2 (B) Hemoglobin F (C) Hemoglobin H (D) None of the above

A3: A. Remember that beta thalassemia trait/minor is a gene mutation in 1 or 2 genes and primarily increases levels of hemoglobin A2 by 3-6% (normal is 2%). It also causes a mild microcytic anemia and in 20% of patient's splenomegaly. Beta-thalassemia major is associated with hemoglobin F. Alpha-thalassemia with mutations in 3 of 4 genes is called hemoglobin H disease.

Patient presents complaining of an increase in fatigue, weakness, and shortness of breath, that is exacerbated by walking a flight of stairs. On examination, you note conjunctival pallor as well as pallor to the nailbeds. Assuming hematopoiesis is not impaired, you would you expect this patient's reticulocyte count to? A.Elevated B.Normal C.Decreased D.I would not order a reticulocyte count.

A3: Answer is a. This is a patient presenting with the signs and symptoms common to all anemias. Assuming hematopoiesis is not impaired (normal bone marrow, normal kidneys/EPO, normal precursors), the normal physiologic response to anemia is for the reticulocyte count to increase.

Which of the following is seen on PBS of hairy cell leukemia? (A) enlarged lymphocytes (B) monocytopenia (C) lymphocytes w/ villous cytoplasmic projections (D) B and C (E) All of the above

A3: E. In hairy cell leukemia, PBS shows large lymphocytes and villous cytoplasmic projections or "hairs." Hairy cell leukemia commonly is shown to have pancytopenia - but especially monocytopenia.

Q40: Suzy is a 60 year female who presents to your clinic today because she has "not been feeling herself" lately. When asked what she means, she explains that for the past 6-9 months she has been feeling overall fatigued, with an occasional headache and what seems to be an increased work of breathing at times. She additionally states "I feel full after I eat a small amount of food" and "I think I am losing weight because of it". You do a full physical exam: General: Appears fatigued but otherwise is in NAD. Alert and oriented x3. Skin: Color pink. Nails without clubbing or cyanosis. HEENT: Head is normocephalic atraumatic, Visual fields full by confrontation. Conjunctival pallor. Sclera white. Corneas clear. PERRLA. EOMs intact without nystagmus. Neck: Neck supple, full ROM. No palpable adenopathy. Trachea midline. Thyroid gland not enlarged, no palpable masses or nodules. Pulmonary: No signs of respiratory distress. Chest wall expansion and diaphragmatic excursion symmetric. Lung sounds are clear to auscultation in all lobes bilaterally without crackles, rhonchi, or wheezes. Cardiovascular: Heart is regular rate and rhythm. Normal S1 and S2. No murmurs or extra heart sounds. Abdominal: Abdomen is soft. Tenderness over the LUQ, without rebound or guarding. Prominent hepatosplenomegaly. Umbilicus is midline without herniation. No dullness to percussion. Positive bowel sounds in all four quadrants. Extremities: Extremities are warm without edema. Calves are supple and nontender. Neurologic: Patient is alert, well groomed and cheerful. Speech is fluent and words are clear. Thought processes are coherent and insight is good. Oriented to person, place and time. Based on her physical exam findings, you order a CBC which reveals a leukoerythroblastic anemia and band neutrophils and a PBS which returns with nucleated teardrop RBC's. What is the most likely diagnosis? A. Chronic myeloid leukemia B.Acute myeloid leukemia C. Polycythemia vera D.Primary myelofibrosis E. Essential thrombocytosis

A40: The answer is D. The patient's presentation with fatigue/SOB/headaches, as well as early satiety and weight loss are signs and symptoms of an anemia and some sort of cancer. The prominent hepatosplenomegaly on exam leads you to believe primary myelofibrosis confirmed by the nucleated teardrop RBC's on PBS.

Which of the following is correct in regards to normal hemoglobin? A.Hemoglobin A2 is the major contributor to adult hemoglobin, and is a tetramer of two alpha and two delta chains. B.Hemoglobin A is a minor contributor of normal hemoglobin in adults, and is a tetramer with two alpha chains and two gamma chains. C.Hemoglobin F is a tetramer made up of two alpha chains, and two gamma chains. D.A and B are correct. E. All of the above are correct.

A4: Answer is c. A is wrong (Hemoglobin A2 is a MINOR <3% contributor to hemoglobin amounts, and made of two alpha and two delta chains). B is wrong (Hemoglobin A is the MAJOR contributor of normal hemoglobin in adults, and is a tetramer of two alpha and two BETA chains). C is correct.

Which of the following is NOT a cause of sideroblastic anemia? (A) lead (B) occult bleeding (C) genetics (D) alcohol (E) all of the above are causes

A4: B. Remember that sideroblastic anemia can be either congenital or acquired. When we say something is congenital, we refer to genetics (answer C). The acquired causes of sideroblastic anemia were primarily alcohol and lead (answer A and D). Thus, answer B - occult bleeding is not a cause. This is commonly a primary cause of Iron deficiency anemia.

Which of the following is true regarding lymphoma? (A) Non-Hodgkin's lymphoma (NHL) is less common than Hodgkin's lymphoma (HL). (B) B symptoms are more commonly seen in NHL (C) NHL is one of the most curable forms of cancer. (D) 85% of NHL are T-cel type, 15% are B-cell type (E) None of the above

A4: E. NHL is more common than HL. B symptoms are more predominately seen in HL, although NHL can exhibit these too. HL is one of the most curable forms of cancer. 85% of NHL are B-cell type, while 15% are T-cell type. Thus, answer E, none of the above is correct.

Which of the following is the most common hematologic malignancy in the world? (A) NHL (B) CLL (C) AML (D) ALL

A5: A. NHL is the most common hematologic malignancy in the world. CLL is the most common type of leukemia in the US. AML is the most common acute leukemia in adults. ALL is the most common malignancy of childhood.

You are concerned your patient has iron deficiency anemia. You order iron studies including a ferritin, total iron, total iron binding capacity, and a % saturation. What would you expect the results to be? A.High ferritin, high total iron, high % saturation, low total iron binding capacity. B.High ferritin, low total iron, high % saturation, high total iron binding capacity. C.Low ferritin, low total iron, low % saturation, low total iron binding capacity. D.Low ferritin, low total iron, low % saturation, high total iron binding capacity.

A5: Answer is d. In iron deficiency anemia, iron indices reveal. 1. low ferritin (wouldn't need to go on to obtain other iron studies, this is diagnostic for IDA). 2. low total iron. 3. low % saturation. 4. High total iron binding capacity.

Which of the following are you likely to find upon diagnosing Sideroblastic Anemia? (A) elevated bilirubin (B) microcytic anemia (C) bone marrow ring sideroblasts on PBS (D)microcytosis (E) All of the above

A5: E. All of the above are true of sideroblastic anemia. Elevated bilirubin may or may not be seen due to recycling/destruction of faulty RBC's. Both microcytic anemia and microcytosis are traits of sideroblastic anemia. Bone marrow ring sideroblasts on PBS is a buzz word for sideroblastic anemia!

Patient presents with a microcytic anemia and bone marrow ring sideroblasts on peripheral blood smear. You should elicit more history regarding? A.Family history of sideroblastic anemia B.Alcohol use C.Exposure to lead E.Drug use F.All of the above

A6: Answer is e. Sideroblastic Anemia is microcytic anemia due to the failure to synthesize the porphyrin ring. It can be congenital (genetic), or acquired from drugs/toxins including alcohol and lead.

Miska is a 21-year-old female, who presents to your clinic today with a concern that she has a lump growing in her neck. Before you saw Miska today, you noticed her PMH was positive for a history of IBD, for which she currently takes an immunomodulator (azathioprine) for and all else is unremarkable. Miska complains she has been waking up at night with night sweats and a fever and tells you she is constantly fatigue. On physical exam, you note she has an enlarged, fixed, non-tender, cervical lymph node on the right side. You order some labs and they come back as follows: CBC: shows anemia PBS: Reed Sternberg cells ESR: elevatedAlbumin: decreasedLDH: elevatedUric acid: elevated Alk Phos: elevated What disease does Miska have? (A) NHL (B) HL (C) ALL (D) CLL

A6: B. The most common presenting symptom of HL is painless lymphadenopathy in the cervical region. Other common symptoms are night sweats, unexplained fever, unexplained weight loss, persistent fatigue, etc. Miska also had a hx of IBD for which she took an immunomodulator for - if you remember, these cause immunosuppression. Immunosuppression is a risk factor for HL - as is, genetic predisposition, HIV and EBV. All of the labs listed are consistent with HL - most notably, notice Reed Sternberg cells!

Anemia of Chronic Disease's underlying etiology is caused from inflammation that decreases which of the following? (A) EPO production (B) Hepcidin production (C) Bone marrow responsiveness to EPO (D) A and C (E) all of the above

A6: D. Hepcidin is a positive acute phase reactant and thus increases due to inflammation. Although cytokines are not listed, they too increase in the presence of inflammation, thus inhibiting RBC production. Both EPO production and bone marrow responsiveness to EPO decrease in the presence of inflammation. Remember, the OVERALL effect is decreased RBC production and survival in ACD!

Erythrocytes are synthesized in the ___ and recycled in the ___? A.Bone marrow; spleen B.Spleen; bone marrow C.Blood; bone marrow D.Bone marrow; liver

A7: Answer is a. Erythrocytes are synthesize in the bone marrow (after 7mo of age), and recycled in the spleen.

Which of the following is a B symptom of Hodgkin's Lymphoma? (A) persistent fatigue (B) unexplained fever (C) persistent cough (D) lymphadenopathy (E) Anorexia

A7: B. Remember the B symptoms of HL are drenching night sweats, unexplained weight loss, and unexplained fever - these symptoms are used to help stage the disease and determine prognosis. All other symptoms listed, are symptoms that may be seen in HL.

Which of the following is NOT true of anemia of chronic disease? (A) Diagnosis includes the presence of a chronic disease (B) MCV commonly shows a normocytic anemia (C) All causes of microcytic anemia must be excluded upon work-up (D) Iron replacement is generally helpful in treatment (E) All of the above are true

A7: D. We know that ACD has to include the presence of a chronic disease - it says it in its name! ACD is tricky in that normally the MCV shows a normocytic anemia, but in some cases can be a microcytic anemia! When we diagnose ACD, it's almost like a diagnosis of exclusion. We need to rule out Fe deficiency and other causes such as thalassemia or sideroblastic anemia. Lastly, when treating ACD, we treat the underlying disease and refer to a hematologist - Fe replacement is generally NOT helpful in treating this.

Donald, a 72-year-old male, is complaining of generalized fatigue, headaches and shortness of breath. On file you notice he has a history of HTN, DM and RA. Family history is negative for hemoglobin abnormalities and social history is negative for alcoholism or lead exposure. Upon examination, you notice conjunctival pallor. Vitals were normal, besides a rather rapid heart rate of 110. You decide to run some lab work and it comes back as follows: Reticulocyte count - low MCV - microcytic Iron studies - WNL PBS - non-diagnostic except for microcytic anemia What is Donald's most likely diagnosis? (A) Anemia of chronic disease (B) sideroblastic anemia (C) thalassemia (D) Iron deficiency anemia

A8: A. Due to presentation, we should be concerned about some type of anemia. It's important to note here about his PMH. He has a few chronic diseases, so we cannot rule out ACD and his FH is negative for hemoglobin abnormalities, enabling us to cross sideroblastic anemia and thalassemia off our list. When we look at the labs, notice that Fe studies come back normal, excluding Iron deficiency anemia as our cause. That leaves us with ACD. ACD is typically a diagnosis of exclusion plus the presence of a chronic disease.

Which of the following is the most common type of indolent Non-Hodgkin's Lymphoma? (A) Follicular lyphoma (B) Marginal zone B-cell lymphoma (C) Cutaneous T-cell lymphoma (D) Waldenstrom macroglobulinemia (E) Gastric mucosa-associated lymphoid tissue (MALT) lymphoma

A8: A. Follicular lymphoma is the most common type of indolent NHL. All other forms listed are other types of indolent lymphoma that Professor Baumgartner asked us to know!

You are seeing a 30 year old female with a chief complaint of fatigue. She describes it as "feeling run down and weak". When asked about her activity level she tells you she has become mildly SOB with activity. She denies any significant headache or urinary complaints. She does endorse a longstanding history of diarrhea as well as abdominal bloating. She denies blood in the stool. Physical Examination: Vitals BP 122/78, RR 14, Pulse 110bpm, T 37.0 C. General: Alert. NAD. Appears mildly restless. Skin: Nailbed pallor. HEENT: Mild conjunctival pallor. EOMI, PERRL. Cardiovascular: Mildly tachycardic at 110bpm. Normal S1 and S2 without murmur. Pulmonary: Clear to auscultation bilaterally. Abdominal: Abdomen without masses. Abdomen is mildly distended with active bowel sounds. Nontender to palpation. Remainder of examination is normal. CBC is ordered and reveals a hemoglobin of 10.3 (Normal 12-16). MCV is 75 (normal 90-100). Ferritin is obtained and returns at 9 ng/mL (normal 12-150ng/mL). What is the next best step in management of this patient? A.Order peripheral blood smear. B.Order iron studies including total iron, total iron binding capacity, % saturation. C. Order EGD and a Colonoscopy. D.Order a stool hemoccult, tTG, and UA. E. Prescribe ropinirole to treat patient's restless leg syndrome.

A8: Answer is d. This is supposed to be a patient presenting with iron deficiency anemia general signs/symptoms of anemia. Labs reveal microcytic anemia, with low ferritin revealing Iron Deficiency Anemia. Next best step would be to search for the cause --> order stool hemoccult, tTG, and UA. Should be thinking celiac disease given underlying diarrhea and abdominal bloating.

Which of the following is false in regard to Non-Hodgkin's Lymphoma? (A) The presence of B symptoms does not have prognostic signifiance (B) Fine needle aspiration is used to diagnose NHL (C) high grade, aggressive NHL is more likely to be cured compared to low grade, indolent NHL (D) CHOP or R-CHOP is the mainstay of treatment (E) All of the above are true.

A9. B. A, C & D are all true of NHL. Answer B is false because FNA does not supply enough tissue to be biopsied. Rather we need to use an incisional or excisional biopsy of the lymph node to diagnose NHL.

Peter is a 4yo male, presents to the clinic with his mother, who is concerned he has been more fatigued than she would expect for his age. Mother tells you she feels he has not transitioned out of napping, like her nephew who is of similar age. Mother states the patient continues to nap twice per day. She has no other complaints regarding Peter. She wants to know if this is normal. Physical examination is unremarkable. You decide to order a reticulocyte count and a CBC. Reticulocyte count WNL. CBC reveals a microcytic anemia, not requiring transfusion at this time. You go on to order iron studies which reveal a normal ferritin, an elevated TIBC, a normal total iron, and a normal % saturation. Peripheral blood smear is obtained and reveals a microcytic anemia with poikilocytosis. What is the next best step in the evaluation and management of Peter at this time? A. Reassure mother, no further management required. B.Obtain an erythrocyte porphyrin test and hemoglobin electrophoresis. C. Order a bilirubin level. D.Treat patient's iron deficiency anemia with 150-200mg of elemental iron.

A9: Answer is b. This patient has microcytic anemia due to one of the four causes. We can rule out iron deficiency anemia based on his iron studies, and anemia of chronic disease based on his age. Peripheral blood smear is not helpful and just tells us information we already know (he has a microcytic anemia). We need a test that will help us differentiate between Thalassemia and Sideroblastic anemia, making the correct choice b. An erythrocyte porphyrin test is normal in thalassemia, but may be elevated in sideroblastic anemia due to lead poisoning. Hemoglobin electrophoresis will tell us how much of each type of hemoglobin present, revealing thalassemia. A bilirubin is would not be helpful in distinguishing, even though it can sometimes be elevated in sideroblastic anemia due to recycling/destruction of faulty RBC.

Jovahn, a 45-year-old male, presents to you complaining of difficulty breathing while normal daily activities and a generalized fatigue and weakness he's noticed over the last few months. On file you notice he has a history of GERD and longstanding alcohol abuse. On examination, he appears fatigued with conjunctival and nailbed pallor. Vital signs are normal, so you decide to run some lab work and it comes back as follows: Reticulocyte count - low MCV - microcytic Iron studies - WNL PBS - bone marrow ring sideroblasts What is the best way to treat Jovahn's diagnosis? (A) usually no treatment is necessary (B) bone marrow transplant (C) 200mg/day elemental Fe (D) stop drinking and prescribe pyridoxine (Vit. B6)

A9: D. Due to Jovahn's presentation, we should be concerned about some type of anemia. When looking at his labs, the big giveaway is that he has bone marrow ring sideroblasts on PBS - indicating sideroblastic anemia. If you remember, sideroblastic anemia is either genetic or due to an underlying drug or toxin - namely alcohol or lead. If we look in his PMH, it says he is an alcoholic. Thus, the best way to combat his anemia would be to stop drinking alcohol and prescribe Vit B6 (as some patients respond to this).

In a 55 yo female patient, you incidentally find significantly elevated ALT/ AST levels during a workup for a different problem. After a thorough workup, you find a ferritin level of 200 ng/mL, a transferrin saturation of 48%, and identify a homozygous HFE genotype in this patient. You make the diagnosis of hereditary hemochromatosis. What is the most appropriate treatment for this patient? (A) phlebotomy (B) plasmapheresis (C) prednisone (D) azathioprine (E) copper chelating agents

Answer A. Standard treatment for hereditary hemochromatosis is phlebotomy, with a goal ferritin level between 50-100 ng/mL and a goal transferrin saturation <30%. Additionally, patients should not take iron or vitamin C supplements and should avoid utilization of cast-iron cookware.

Female patient, age 25, presents with a complaint of painful vesicles and bullae on each of her hands, which presented after spending time at the beach last week. At first, she thought it was a severe form of sunburn. You run some tests and find elevated urine and plasma porphyrins and a normal ALT/ AST. What is the best treatment option for this patient's condition? (A) plasmapheresis (B) avoidance of triggering events (C) iron supplementation (D) low dose corticosteroids (E) more than 1 answer is correct

Answer B. This patient presents with porphyria; most likely, porphyria cutanea tarda (PCT), due to her skin manifestations and their emergence with sun exposure. The patient's elevated urine and plasma porphyrin levels further support this diagnosis. Standard treatment for porphyrias is avoidance of triggering events, including sunlight, drugs (alcohol, OCP's, etc.), infection, fasting, and surgery.

Male patient, age 42 yo, presents to your primary care clinic for a routine Type 2 DM check-up. The majority of his exam is normal, aside from some vision blurriness after long work days staring at the computer, which you attribute to his poor sugar control, and skin hyperpigmentation or a "bronze" appearance to the skin. After inquiring about some other symptoms, the patient said he has been having erectile dysfunction recently, which is a new problem for him. Which laboratory value would be MOST helpful in making the diagnosis? (A) CBC (B) ALT/ AST (C) transferrin saturation (D) peripheral blood smear (E) skin biopsy

Answer C. This patient most likely is presenting with hereditary hemochromatosis, as he fits the most common patient presentation characteristics (male, 40s) and has three common symptoms of the condition: DM, skin hyperpigmentation ("bronze" appearance), and impotence. 3 measures used to make the diagnosis of hereditary hemochromatosis include transferrin saturation (> 45), ferritin (>200 ng/mL in men and >150 ng/mL in women), and HFE genotype homozygous or compound heterozygous.

Hereditary hemochromatosis involves __________ in the body, potentially leading to organ dysfunction. (A) iron excess (B) iron deficiency (C) copper excess (D) copper deficiency (E) vitamin C deficiency

Answer: A. Hereditary hemochromatosis is a condition of iron excess, caused by abnormalities in the HFE gene (leading to abnormalities in the HFE protein, which is responsible for regulating iron absorption). A means of remembering this is the gene name itself: HFE stands for High Fe, or high iron. Of note, option C (copper excess), is the pathology seen in Wilson's disease.

Which is NOT part of the classic pentad of thrombotic thrombocytopenic purpura (TTP)? (A) thrombocytosis (B) neurologic changes (C) AKI (D) microangiopathic hemolytic anemia (E) fever

Answer: A. TTP involves non-immunologic platelet destruction, triggered by an event or substance. The classic pentad of TTP is thrombocytopenia, microangiopathic hemolytic anemia, neurologic changes (including headache, confusion, and focal changes), acute kidney injury, and fever.

Which of the following is NOT an early finding of hereditary hemochromatosis? (A) impotence (B) hyperpigmentation or "bronze skin" (C) fatigue (D) arthralgias (E) A&B are both findings of hereditary hemochromatosis

Answer: B. Impotence, fatigue, and arthralgias are all early findings of hereditary hemochromatosis. Because iron abnormalities occur before symptoms of organ abnormalities, late findings of the disease include hyperpigmentation or "bronze" skin (the correct answer), liver failure, elevated liver enzymes, DM, restrictive cardiomyopathy, and arrhythmias.

The 4 T's score is commonly used to determine the clinical likelihood of HIT Type 2. Which of the following would NOT increase the likelihood of a patient having HIT type 2? (A) Timing- onset 5-10 days after exposure or <1 day if prior heparin exposure in the last 30 days (B) Temperature - T>38.0C (C) Thrombosis (D) No other potential causes (E) Thrombocytopenia

Answer: B. The 4 T's are thrombocytopenia (a clinical feature of HIT type 2), timing (5-10 days after exposure or a prior exposure increase the likelihood), thrombosis (feature of HIT type 2), and other causes (no other potential causes increases the likelihood of HIT type 2). An increased temperature is not part of the 4 Ts score and does not increase the likelihood of having HIT type 2.

What is the standard treatment for TTP/ HUS in adults? (A) platelet transfusions (B) plasmapheresis (C) iron supplementation (D) supportive cares (monitoring, IVF if needed) (E) heparin

Answer: B. Treatment for TTP/ HUS in adults involves daily plasmapheresis and the possible addition of corticosteroids, ASA, or rituximab if needed. In children, treatment is supportive cares without plasmapheresis. Of note, transfusions are contraindicated for any individual with TTP/ HUS.

Which of the following is TRUE: (A) tx of hereditary hemochromatosis includes iron supplementation (B) African Americans are more likely to present with hereditary hemochromatosis (C) early diagnosis of hereditary hemochromatosis is important in order to prevent organ damage (D) in symptomatic patients w/o treatment, the 5-year survival rate of hereditary hemochromatosis is near 60% (E) hereditary hemochromatosis has an autosomal dominance inheritance pattern

Answer: C. By the time most patients with hereditary hemochromatosis present, they already have evidence of organ damage (most commonly, liver damage). Early diagnosis can help prevent progression to organ damage. Answer A is incorrect, as phlebotomy is the primary treatment of hereditary hemochromatosis; iron supplementation, Vitamin C supplementation, and cast-iron cookware should be avoided in these patients. Answer B is incorrect, as white Americans are most likely to develop hereditary hemochromatosis. Answer D is incorrect, as the 5-year survival rate in symptomatic patients without treatment is actually 10%. Answer E is incorrect, as the disease has an autosomal recessive inheritance pattern.

Which of the following lab findings would you NOT expect to see in TTP/ HUS? (A) PBS- schistocytes (B) CBC- hemolytic anemia (C) INR/ PT- elevated (D) UA- proteinuria (E) LDH- increased

Answer: C. Coagulation studies, such as INR/ PT, are usually normal in patients with TTP/ HUS. Microangiopathic hemolytic anemia, a characteristic of TTP, can present as hemolytic anemia on a CBC and increased LDH. Proteinuria and hematuria may be seen, as they are signs of renal failure (a characteristic of HUS). Finally, a peripheral blood smear commonly shows schistocytes, or small RBC fragments, a result of hemolytic anemia.

A 58 year old patient presents with diffuse petechiae, significant bruising, fatigue, and new onset of headaches. You order some labs and find thrombocytopenia and hemolytic anemia on CBC, elevated LDH, and low haptoglobin. You strongly suspect this patient has thrombotic thrombocytopenic purpura (TTP). Which of the following would confirm your diagnosis? (A) JAK2 mutation (B) increased ADAMTS13 (C) decreased ADAMTS13 (D) presence of Philadelphia chromosome (E) presence of the Pittsburgh chromosome

Answer: C. One cause of TTP is decreased activity of the ADAMTS13 enzyme, which normally degrades von Willebrand factor. A JAK2 mutation is commonly seen in patients with essential thrombocythemia, and the Philadelphia chromosome is a hallmark of CML (chronic myeloid leukemia).

3 yo female patient presents with extreme fatigue and decreased urination, notably following a 4 day course of bloody diarrhea and abdominal pain. On exam, you note some non-blanchable purpura. You order labs and see hemolytic anemia & significant thrombocytopenia on CBC, elevated creatinine, and normal PT/ PTT. What is the best treatment option for this patient? (A) antibiotics (B) daily plasmapheresis (C) observation (D) refer to hematology (E) more than one of the above is correct

Answer: C. This patient's most likely diagnosis is HUS. HUS is more common in kids from 6 mo. to 4 years old and is most commonly caused by a prodromal EHEC infection (bloody diarrhea and abdominal pain). This patient's symptoms of fatigue, decreased urination, and non-blanchable purpura correspond to the presentation of HUS. Coupled with labs showing hemolytic anemia, a low platelet count, and elevated creatinine (signs of renal failure, which is characteristic of HUS), the case for HUS is strengthened. The presence of normal coags (PT/ PTT) helps to rule out DIC. HUS should be considered in any child presenting with renal failure following a course of diarrhea. HUS in kids is often self-limited and should be treated with observation and IV fluids (if necessary) to maintain renal perfusion.

Most cases of isolated hemolytic uremic syndrome (HUS) in children are caused by: (A) autoimmune disease (B) viral infection (C) S. pneumo (D) E. coli serotype O157:H7 (E) idiopathic

Answer: D. E. coli serotype O157:H7, otherwise known as enterohemorrhagic E. coli (EHEC) causes most cases of isolated HUS in kids. Reversely, about 2-7% of EHEC infections lead to HUS.

Which of the following is a characteristic of heparin induced thrombocytopenia (HIT) Type 2? (A) direct interaction between heparin and platelets, leading to platelet aggregation (B) occurs <2 days following heparin exposure (C) occurs in 25% of people exposed to heparin (D) can lead to thrombotic complications and death (E) is typically short-lived and reversible

Answer: D. HIT Type 2 is a more severe form (compared to HIT Type 1), characterized by immunoglobulin-mediated platelet activation and typically occurs 5-10 days after exposure to heparin, or with prior exposure. HIT Type 2 has a high risk of thrombotic complications and mortality. The other answer options are all characteristics of HIT Type 1.

"When are symptoms of organ dysfunction, due to hereditary hemochromatosis, most likely to present? (A) infancy (B) adolescence (C) young adulthood, ~age 21 (D) middle-age (E) older adulthood

Answer: D. Iron abnormalities occur before organ abnormalities present in patients with hereditary hemochromatosis. In most cases, organ abnormalities present during middle-aged individuals, around age 40 in men and shortly after menopause in females.

Why is dark, reddish urine sometimes seen in patients with porphyrias? (A) a lack of heme in the urine (B) build up of heme in the urine (C) poor kidney function (D) build-up of intermediates in the heme pathway (E) it is not commonly seen and is cause for additional investigation

Answer: D. Porphyrias are a group of genetic or acquired diseases characterized by the deficient synthesis of heme. When heme cannot be formed effectively, intermediates in the heme synthesis pathway can be toxic and present as a dark, reddish urine sometimes seen in patients with porphyrias.

Which of the following is NOT a triggering event of porphyrias? (A) infection (B) surgery (C) fasting (D) cold weather (E) drugs

Answer: D. Porphyrias can be triggered by drugs (including alcohol and OCPs), infections, fasting, and surgery. In some porphyrias, sun exposure can also lead to a rash, vesicles, or bullae. Cold weather is not a known triggering event of porphyrias.

What is the treatment of HIT Type 2? (A) stop heparin usage (B) blood transfusion (C) start alternative non-heparin anticoagulant (i.e. argatroban, fondaparinux, DOAC) (D) options A&C (E) all of the above

Answer: D. Treatment for HIT Type 2 is to stop heparin usage and never restart it, along with initiation of a non-heparin anticoagulant, such as argatroban, fondaparinux, or (sometimes) a DOAC. A blood transfusion is not part of standard treatment.

Male patient, 45, was started on heparin one week ago. Today, his labs show a significant drop in platelet levels. You find his "4 T's" score to be 6, which warrants additional investigative testing for suspected HIT. What is the next best test to order? (A) creatinine (B) SRA- serotonin release assay (C) HIPA- heparin-induced platelet aggregation (D) PT/ INR (E) ELISA immunoassay

Answer: E. After conducting a "4 T's" score, if the possibility of HIT is not ruled out, an ELISA immunoassay (antibody detection) is the next test conducted. The test is highly sensitive, but not very specific. Therefore, a functional assay test (such as SRA or HIPA) may be used if confirmatory testing is needed, due to their high specificity. Between these two functional assay tests, SRA is often preferred.

Which of the following is NOT a general symptom of porphyrias? (A) rash (B) neurovisceral pain (C) anxiety (D) seizures (E) all of the above are symptoms of porphyrias

Answer: E. General symptoms of porphyrias include acute attacks of neurovisceral pain (especially in the abdomen), neuropathies, CNS symptoms (including anxiety, depression, and seizures), and skin manifestations (rash, vesicles, and/ or bulla), especially following sun exposure.

"Q2. 20-30% of patients with MDS will eventually develop which cancer? (A) AML (B) CML (C) Non-Hodgkin's Lymphoma (D) ALL

The answer is a. AML. Non-Hodgkin's lymphoma and ALL are both cancers of the lymphoid cell line, and therefore would not be affected by MDS. Chronic Myeloid Leukemia (CML) is not associated with MDS.

All of the following tests would be useful in diagnosing hereditary spherocytosis except: a. Fluorescence in situ hybridization (FISH) b. EMA binding test c. Osmotic fragility test d. Acidified glycerol lysisest (AGLT)

The answer is a. Fluorescence in situ hybridization (FISH). FISH is a test that maps the genetic material of an individual's cells and is not used in the diagnosis of hereditary spherocytosis. All of the other tests listed can be used in the diagnosis of this disorder.

Which of the following statements regarding DIC is true? a. In acute DIC, bleeding is a more common finding than microthrombosis b. In chronic DIC, bleeding is a more common finding than thrombosis c. Heparin is usually given to patients with DIC d. DIC is a genetic condition

The answer is a. In acute DIC bleeding is a more common finding than microthrombosis. (b.) is false, as thrombosis is more common than bleeding in chronic DIC. (c.) is false, as anticoagulants are usually avoided in patients with DIC. (d.) is false, as DIC is an acquired thromboembolic disorder.

"Q5. Which of the following treatments is potentially curative of MDS? (A) Allogenic hematopoietic stem cell transplant (HSCT) (B) Autologous stem cell transplant (auto-SCT) (C) Chemotherapy (D) Radiation therapy

The answer is a. allogenic HSCT. While chemotherapy may be useful to slow the progression of MDS, it is not curative. Auto-SCT and radiation therapy are not routinely used to treat MDS. It is worth noting that allogenic HSCT is reserved for younger patients high-risk disease as it has a high mortality related to transplant.

"Q4. Which of the following patients with MDS is a good candidate for Allogenic hematopoietic stem cell transplantation (HSCT)? (A) 30 y/o female with moderate-risk disease (B) 37 y/o male with high-risk disease (C) 67 y/o female with high-risk disease (D) 70 y/o male with low-risk disease

The answer is b. 37 y/o male with high-risk disease. Due to the high rate of transplant-related mortality, HSCT is reserved for MDS patients who are younger, and have high-risk disease. Patient a. is younger, but not high risk. Patients c. and d. are older and would most likely have more harm than benefit from HSCT.

Most cases of HUS in children are caused by: a. Hemorrhagic colitis b. E. coli O157:H7 c. Penicillin d. Idiopathic e. Congenital enzyme deficiency

The answer is b. E. coli O157:H7. All listed options are potential causes of HUS in children, but the most common cause is E. coli. The most common presenting symptom in these children is diarrhea (90%).

A 67 y/o male is brought into your emergency department by his spouse. She states that he has been acting confused all morning. She also states that he has been more sleepy than usual, as he couldn't stay awake to watch the Vikings game at noon yesterday, which he would never usually miss. You obtain his vital signs and notice that has a temperature of 39﮿C. All other vitals are WNL. On physical exam you find multiple non-blanchable purple spots ranging from 5 to 10 mm. You obtain a head CT followed by an LP, both of which come back normal. You then obtain a CBC and BUN/Cr. The CBC shows a hemolytic anemia and thrombocytopenia. Creatinine levels are decreased indicating decreased renal function. Which of the following treatments would be contraindicated in this patient? a. Plasma exchange b. PRBC transfusion c. Corticosteroids d. Rituximab e. Aspirin

The answer is b. PRBC transfusion. This patient is presenting with typical signs and symptoms of TTP/HUS: confused, fatigued, fever, purpura, thrombocytopenia, hemolytic anemia and decreased renal function. Transfusions are contraindicated in patients with TTP/HUS. Daily plasma exchange is the preferred treatment with corticosteroids, aspirin, and rituximab all being potential add-ons.

"Q1. In a patient with Myelodysplastic syndrome (MDS) you could see disordered hematopoiesis in all the following cell lines except: (A) erythrocytes (B) Neutrophils (C) Lymphocytes (D) Megakaryocytes

The answer is c. Lymphocytes. Lymphocytes arise from the lymphoid cell line, while all the other options arise from myeloid cell lines. MDS is classified as disordered hematopoiesis in one or more of the myeloid cell lines.

Which of the following is not part of the "classic pentad" associated with TTP? a. Thrombocytopenia b. Neurologic changes c. Macrocytic anemia d. Microangiopathic hemolytic anemia e. Acute kidney injury

The answer is c. Macrocytic anemia. The classic pentad of TTP is: Thrombocytopenia, Microangiopathic hemolytic anemia, neurologic changes, AKI, and fever. The first three clinical manifestations listed in this pentad are common, while AKI and fever happen in roughly 50% of people. Therefore, seeing the full pentad is uncommon.

"Q9. You are following-up with a 48 y/o male patient in your GI clinic who is in remission 2 years post being diagnosed with stage II rectal adenocarinoma. He was underwent surgery and a 6 month regimine of chemotherapy. Recently, he has been feeling fatigued, dyspneic, and has reported that he has been bruising more easily than normal. On his abnominal exam you note hepatosplenomegaly, but the rest of his exam is normal. You obtain a CBC and find that he has a macrocytic anemia, throbocytopenia, and neutropenia. PBS shows severely dysplastic megakaryocytes. You are most concerned that he has which of the following? (A) Sideroblastic anemia (B) Primary MDS (C) Seconday MDS (D) Sickle cell anemia

The answer is c. Secondary MDS. Cytopenias and normocytic or macrocytic anemias are common findings in MDS. A PBS showing dysplastic megakaryocytes further increases our concern that this patient has MDS. This MDS would be classified as secondary and not primary due to the history of chemotherapy and the patient being of a younger age. Primary MDS is most often seen in the mid to late 60s and is not assoicated with any inciting events. Sideroblastic anemia would show sideroblasts on PBS, so this can be ruled out. Sickle cell anemia would show sickle cells on PBS, so this can also be ruled out.

Which of the following is not a common cause DIC? a. Sepsis b. Pancreatic cancer c. Amniotic fluid embolism d. HIV e. Trauma

The answer is d. HIV. The five common causes of DIC are Sepsis/infections, malignancy, obstetric complications, trauma, and massive hemolysis. Autoimmune disorders such as HIV are uncommon causes of DIC.

"Q6. Which of the following statements about patients with MDS is false? (A) Most cases of MDS are primary in origin (B) Median age of onset is mid to late 60s (C) MDS before age 50 may be due to a genetic defect (D) Life-threatening bleeding from thrombocytopenia is a common complication (E) Neutropenic infection is the leading cause of death

The answer is d. Life-threatening bleeding from thrombocytopenia is a common complication. This statement is false, as life-threatening bleeding from thrombocytopenia is less common in patients with MDS than other causes of mortality. All of the other statements are true regarding patients with MDS.

What is only definitive treatment for hereditary spherocytosis? a. Daily folic acid supplementation b. Weekly EPO injections c. PRBC transfusions d. Splenectomy e. Stem cell transplant

The answer is d. Splenectomy. Splenectomy is the only definitive treatment for hereditary spherocytosis. Answers (a), (b), and (c) are all potential treatments for hereditary spherocytosis but will not cure it. Stem cell transplant is the definitive treatment for PNH.

"Q3. The International prognostic scoring system (IPSS) for MDS is based on all the following factors that affect prognosis except: (A) Cytogenetics of bone marrow cells (B) Percentage of bone marrow blasts (C) Number of cytopenias (RBC, WBC, Platelets) (D) The presence of Auer Rods on PBS

The answer is d. the presence of Auer Rods on PBS. While Auer rods are typically seen in AML, are not included the IPSS for MDS. The presence of Auer Rods in an individual with MDS would indicate a worse prognostic factor, as the patient most likely has developed AML, but the IPSS does not account for this in their scoring system. All the other options listed (a, b, c) are included within the scoring system.

Which of the following is not an expected symptom in a patient with TTP/HUS? a. Fever b. Confusion c. Purpura d. Jaundice e. Petechiae

The answer is e. Petechiae. TTP/HUS are associated with all of the other symptoms listed, along with renal failure, which is the main feature of HUS. Both purpura and petechiae are non-blanching purple spots on the skin, but petechiae are much smaller and not associated with TTP/HUS. As the name implies, Thrombotic Thrombocytopenic Purpura (TTP) is associated with purpura.

"Q7. Which of the following patients is at risk for developing secondary MDS? (A) a 70 y/o male with 40 pack-year smoking history (B) a 65 y/o female with a history of breast cancer treated with chemo and radiation therapy (C) a 40 y/o male with a history of prostate cancer treated with radiation therapy (D) a 45 y/o female with a history of alcoholism (E) b and c

The answer is e. b and c. Secondary MDS is also called "therapy related MDS" and is induced by exposure to radiation therapy or to chemotherapy with alkylating agents. Smoking and alcohol use are not known to be associated with MDS.

Which of the following is TRUE regarding EDTA? (a) EDTA is an anticoagulant used in CBC tubes to preserve blood cells (b) EDTA should be used instead of sodium citrate when there has been a spurious reading of thrombocytopenia (c) Checking a peripheral blood smear after a spurious result on CBC is not useful (d) EDTA is a type of stain used when assessing for thrombocytopenia on a PBS

The correct answer is a. EDTA is an anticoagulant used in CBC tubes to preserve blood cells. EDTA generally results in more accurate readings, but with some patients' blood, it may actually cause platelet clumping. If this leads to a spurious result, you can either recheck the CBC using sodium citrate instead of EDTA, or you can check a peripheral blood smear.

You are concerned that a thrombocytopenia reported out on one of your patient's CBC results is spurious. Which finding on a peripheral blood smear would support your suspicion? (a) Platelet clumping (b) Very few platelets/HPF (high-powered field) (c) Schistocytes (d) None of the above. You need to repeat the CBC to confirm your suspicion.

The correct answer is a. platelet clumping. A spurious reading of thrombocytopenia may be due to EDTA clumping the platelets together. This large platelet clump is then read as only a single platelet by the machine. This platelet clump can be seen on PBS. Answer b. would support the thrombocytopenia reading. Schistocytes suggest hemolysis. Answer d. is incorrect as well because both a PBS and repeating the CBC are options. However, repeating the CBC with sodium citrate would be relatively quicker than performing a PBS.

You are seeing a 38-year old female patient for new-onset fatigue. As part of your work-up, you get a CBC to assess for anemia. When you get the results back, you note that the platelet count is 42 K/uL (normal: 150-450 k/uL). On your physical exam, you had not noted any petechiae, bleeding from mucous membranes, or ecchymoses. The spleen was not palpable. There is no family history of platelet disorders. What is the best next step? (a) Urgent platelet transfusion (b) Recheck the CBC, but ask the lab to use sodium citrate this time (c) Check PT, INR, and a D-dimer (d) Recheck the CBC, but ask the lab to use EDTA this time

The correct answer is b. Recheck the CBC, but ask the lab to use sodium citrate this time. Because this patient does not show any signs or symptoms or thrombocytopenia, this reading may have been spurious due to platelet clumping due to EDTA use. We would need to confirm that this is a true thrombocytopenia before treating this patient. Checking a PT, INR, and D-dimer would give us information about the coagulation cascade rather than platelet function.

Which of the following is FALSE regarding hypersplenism? (a) Diseases such as myelofibrosis and thalassemias can cause hypersplenism. (b) Splenectomy is usually the only effective treatment in most cases of hypersplenism. (c) Patients s/p splenectomy should receive empiric antibiotics if they develop a fever. (d) Hypersplenism can lead to thrombocytopenia.

The correct answer is b. Splenectomy is usually the only effective treatment in most cases of hypersplenism. The first-line treatment is always to treat the underlying disease that is causing hypersplenism. A splenectomy is only indicated in certain severe cases of hypersplenism, such as if the degree of platelet sequestration is causing significant bleeding problems. Splenectomy should be avoided whenever possible due to the serious risk of infection. All other answer options are true.

Approximately what percentage of platelets are sequestered in a normal-sized spleen of a healthy person? (a) 5% (b) 15%) (c) 30% (d) 40% (e) 45-55%

The correct answer is c. 30%. About 1/3 of platelets are sequestered in the spleen and exchanged for damaged platelets or released as needed. The spleen may sequester more platelets if it is enlarged.

Which of the following is NOT a cause of hypersplenism? (a) Lymphomas (b) CHF (c) Cat scratch disease (d) Lyme disease (e) a and c

The correct answer is c. Cat scratch disease. Cat scratch disease is caused by Bartonella henselae and leads to a tender, swollen lymph node near the site of the cat scratch. All other answer options are causes of hypersplenism. Other causes of hypersplenism include cirrhosis, thrombosis of splenic veins, mononucleosis, hepatitis, endocarditis, TB, SLE, and myelofibrosis.

You are seeing a 29-year old pregnant G1P0 female for routine prenatal care at 32 weeks gestation. Her vitals are as follows: T: 98.8F BP: 128/81 P: 82 RR: 14 As part of your routine evaluation, you got a CBC today. You note that her platelets are at 143. Looking back on CBCs previously obtained on this patient, you note that her platelets tend to run in the 160s. What is the most likely cause for her low platelets? (a) Preeclampsia (b) HELLP syndrome (c) Gestational thrombocytopenia (d) DIC

The correct answer is c. Gestational thrombocytopenia. During pregnancy, the number of platelets tends to decrease slightly, and there is also hemodilution of the platelets as plasma volume increases. As a side note, even though platelets decrease, they have increased function during pregnancy (throwback to OB). This patient is not showing signs of preeclampsia, HELLP syndrome, or DIC. HELLP syndrome and DIC also generally tend to occur later in pregnancy or in the postpartum period.

Which of the following organisms is NOT routinely vaccinated for in patients s/p splenectomy? (a) Streptococcus pneumoniae (b) Neisseria meningitidis (c) Haemophilus influenzae (d) Bordetella pertussis

The correct answer is d. Bordetella pertussis. B. pertussis is part of the Tdap and DTaP vaccines that are routinely administered in childhood and adolescence. It is not a routine vaccination for an individual s/p splenectomy. The other answer options are all encapsulated organisms. The spleen plays a significant role in immunity against encapsulated organisms, so individuals s/p splenectomy are at a significantly increased risk of severe infection with these organisms.

You are meeting with a 54-year old male s/p splenectomy. He had surgery last week and is recovering well. His spleen was removed because it was markedly enlarged from chronic cirrhosis. Which of the following is FALSE regarding this patient? (a) If he develops a fever, he should receive empiric antibiotics. (b) He needs to be vaccinated against encapsulated organisms, including N. meningitidis (c) The splenomegaly must have been severe, as splenectomy is a last-resort treatment (d) This patient will likely have a transient thrombocytopenia following surgery

The correct answer is d. This patient will likely have a transient thrombocytopenia following surgery. After a splenectomy, there is usually a transient thrombocytosis as some of the platelets that were stored in the spleen are released. Answer a. is correct because this patient is at high risk for a serious infection, so fevers are treated empirically. The other answer options are correct as well.

Which is the following is NOT an obstetric cause of thrombocytopenia? (a) Preeclampsia (b) Dilution of platelets (c) HELLP syndrome (d) DIC (e)None of the above

The correct answer is e. None of the above. All answer options are potential causes of thrombocytopenia during pregnancy or in the postpartum period. Dilution of platelets due to increased plasma volume is not a true thrombocytopenia, but CBC results will show decreased platelets because the concentration has decreased. HELLP stands for Hemolysis, Elevated Liver enzymes, and Low Platelets. It is a complication of eclampsia. DIC can occur due to fetal death, an amniotic fluid embolism, preeclampsia/eclampsia, post-partum hemorrhage, or placental abruption.

Ferritin is a ________ acute phase reactant (a) Positive (b) Negative

a. Positive

What is another term for schistocyte? (a) Pencil cell (b) Fragment (c) Ecchinocyte (d) Sickle cell (e) Elliptocyte

b. Fragment

Pagophagia (eating ice) is associated with which of the following types of anemia? (a) Thalassemia (b) Iron deficiency (c) Sideroblastic (d) Anemia of chronic disease (e) All of the above

b. Iron deficiency

A 54-year-old male presents for a follow-up appointment for his iron deficiency anemia. He has been treated with oral replacement therapy for the past week. Initial reticulocyte count was 1.2% What would you hope his reticulocyte count to be today? (a) 1.0% (b) 1.2% (c) 2.2% (d) 4.2%

c. 2.2% (it should increase by about 1% each week with treatment)

Which of the following is NOT seen on the CBC in iron deficiency anemia? (a) Hypochromic RBCs (b) Reactive thrombocytosis (c) Decreased RDW (d) Microcytosis (e) All of the above are seen on the CBC

c. Decreased RDW (there will be an INcreased RDW)

Which of the following would be expected as a normal physiologic response to anemia? (a) Decreased reticulocytes (b) Increased hemoglobin (c) Increased reticulocytes (d) Increased blood volume

c. Increased reticulocytes

Which of the following is the stimulus for erythropoietin? (a) Low tissue CO2 (b) High plasma CO2 (c) Low tissue O2 (d) High plasma O2

c. Low tissue O2 (tissue hypoxia sensed in kidneys)

Which of the following is NOT associated with MICROcytic anemia? (a) Iron deficiency (b) Thalassemia (c) Sideroblastic anemia (d) B12 deficiency (e) Anemia of chronic disease

d (this causes MACROcytic anemia)

Which of the following is NOT a part of the myeloid cell line? (a) Thrombocyte (b) Basophil (c) Macrophage (d) Plasma cell (e) All of the above a part of the myeloid cell line

d. Plasma cell (this is lymphoid cell line)

Which of the following steps of RBC production is erythropoietin involved in? (a) Proerythroblast --> erythroblast (b) Erythroblast --> reticulocyte (c) Reticulocyte --> Erythrocyte (d) Both a & b (e) All of the above

e (EPO does it all)

Which of the following cell types can be seen on the PBS in iron deficiency anemia? (a) Microcytes (b) Pencil cells (c) Target cells (d) Both a & b (e) All of the above

e. All of the above

Which of the following is a cause of iron deficiency anemia? (a) Bleeding (b) Celiac disease (c) Short bowel syndrome (d) Pregnancy (e) All of the above

e. All of the above

Which of the following symptoms are common to all anemias? (a) Fatigue (b) Pallor (c) Palpitations (d) Headache (e) All of the above

e. All of the above