Hematology pt 2
A 45-year old man of 70kg is treated for hypertension with propranolol. The drug has F=0.25, Cl=50 L/h/70kg, Vd=270L/70kg, t1/2=4h. What dose of propranolol should be given every 12 hours to achieve steady-state plasma concentration of 20ug/L in this patient? a. 10 mg b. 20 mg c. 50 mg d. 80 mg e. 100 mg
20*270= 540ug
A 60-year old female presents with 6-months fatigue. CBC: Hb 8 g/dL, WBC 9K/uL, Plt 680 K/uL, Reticulocyte count 1%, MCV 68fL, RDW 22. On examination, she has koilonychia. She is at risk for the following: a. Plummer-Vinson syndrome b. Cold exposure triggering autoimmune hemolysis c. Neural deficits d. Hepatic metastasis e. GI complications including gastritis
A+E Always find cause of Fe-def in elderly
A 38-year old female with 1 month of fatigue has the following CBC: Hb 8 g/dL, WBC 9 k/uL, Plt 250 k/uL, Reticulocyte count 8%, MCV 98fl. The patient tests positive in direct Coombs test for IgG binding to red blood cells. She also has positive C3 Coombs test. The following is a treatment option for this patient: a. Oral prednisone b. Rituximab c. Avoid cold exposure d. Splenectomy e. Anti-complement therapy
A+D Warm antibody: Spherocytes on smear
Plummer-Vinson syndrome includes: a. Pica b. Iron deficiency anemia c. Hypomania d. Hypercoagulability e. Esophageal webs
B+E +glossitis
Drug idiosyncrasies are not immunologically mediated, but rather caused by genetic abnormalities in metabolic processing of a drug. The following is an example of drug idiosyncratic reaction: a. Hemolytic anemia after alpha-methyldopa b. Isoniazid-induced peripheral neuropathy c. Porphyrinuria after barbiturates d. Prolonged apnea after succinylcholine e. Red blood cell destruction after primaquine
B, C, D, E Succinylcholine + abnorm AChE -> Apnea Isoniazid + slow NAT -> Periph neuroPx Primaquine +G6PDH def ->Hemolytic ane Barbiturate + mut heme biosynth -> Porph
The figure depicts a chemical molecule with 4 labeled sites. Which can be reduced by reductases produced by gastrointestinal bacteria? A B C D
D - Nitro reduction
On laboratory analysis, HbA2 is found to comprise 5% of a patient's hemoglobin. The HbF is around 3%. This patient most likely has: a. Heterozygous Beta thalassemia b. Homozygous alpha thalassemia c. Aplastic anemia d. Pure red blood cell aplasia e. Iron deficiency anemia
a. Heterozygous Beta thalassemia Homozyg B-thal: >30% HbF
Spastic paraparesis, sensory ataxia, and paresthesia can result from following cause of anemia: a. Iron deficiency b. Beta thalassemia c. Alpha thalassemia d. Hemochromatosis e. Vitamin B12 deficiency
e. Vitamin B12 deficiency
A 4-year old boy presents with fatigue, facial and cranial bone enlargement and splenomegaly. On examination, his labs indicate anemia and hemochromatosis. He experienced several severe episodes of anemia that were treated successfully with transfussions. The following is the most likely diagnosis: a. Homozygous Beta thalassemia b. Heterozygous alpha thalassemia c. Aplastic anemia d. Anemia of chronic disease e. Folate deficiency f. Iron deficiency anemia
a. Homozygous Beta thalassemia bone/ face enlargement: 'crew cut' sign Low hepcidin -> 2o hemochromatosis + transfussions (Fe overload)
A 45-year old man of 70kg is treated for hypertension with propranolol. The drug has F=0.25, Cl=50 L/h/70kg, Vd=270L/70kg, t1/2=4h. How much propranolol is in the body 12 hours after CSS is reached if no further drug is given? a. 0.7 mg b. 1.35 mg c. 2.7 mg d. 5.4 mg e. 11 mg
a. 0.7 mg 12h = 3 t1/2, so 540->270->135->68
A 45-year old man of 70kg is treated for hypertension with propranolol. The drug has F=0.25, Cl=50 L/h/70kg, Vd=270L/70kg, t1/2=4h. If the concentration at steady-state is 20 ug/L, how much drug will be in the body at that time? a. 2.7 mg b. 5.4 mg c. 20 mg d. 50 mg e. 100 mg
a. 5.4 mg 20ug/L * 270L= 540ug
A 93-year old man with a history of medical problems presents with increasing irritability and restlessness. He has some muscle weakness, Hb of 9g/dL, HCT of 29%, Reticulocyte count 0.4%, MCV 110ug/dL, serum iron 40 ug/dL, serum ferritin 150 ng/mL, RBC folate 100 ng/dL, and serum vitamin B12 at 300 pg/mL. The other primary form of megaloblastic anemia would impair: a. DNA, lipid, and amino acid synthesis b. Lipid synthesis only c. Amino acid synthesis only d. Cytochrome oxidase function e. Myelin degradation
a. DNA, lipid, and amino acid synthesis Vit B12
A 45-year old woman with a history of heavy menses and extensive Ibuprofen use to control osteoarthritis pain presents with abdominal pain and fatigue. She is found to have extensive gastritis, hemoglobin of 8 g/dL, HCT of 27%, reticulocyte count of 2% and MCV of 75 ug/dL. Her serum iron is low at 40 ug/dL, serum ferritin is 9 ng/mL and total iron binding capacity is high at 450 ug/dL. She is given the appropriate treatment, but experiences acute poisoning due to overdose. The following is the 1st-line approach: a. Deferoxamine b. Whole bowel irrigation c. Oral ferrous sulfate d. Supportive care while the patient recovers e. Phlebotomy
a. Deferoxamine
A 38-year old male goes ice fishing and returns with cyanotic fingertips. His Hb is 8 g/dL, WBC 9K/uL, Plt 250K/uL, MCV 98 fL; U/A: Blood +; RBC- Neg. Reticulocyte count is 12%. The following is useful in confirming the suspected diagnosis: a. Direct Coombs test b. Blood smear c. Donath-Landsteiner test d. Hemoglobin electrophoresis e. Complement mutation genetics
a. Direct Coombs test DC: CD+, IgM+, IgG- Cold hemolytic dx: RBC agglutination
The following is an example of pharmacokinetic interaction affecting drug distribution: a. Dixogin displacing quinidine in tissues b. Cyclophosphamide-induced pancytopenia c. Aspirin-induced asthma d. Cholestyramine binding tolbutamide e. Succinylcholine-induced apnea
a. Dixogin displacing quinidine in tissues
A 66-year old woman is being treated with fludarabine and cyclophosphamide for CLL. She develops severe agranulocytosis due to the cyclophosphamide. Which of the following is responsible for her reaction? a. Drug cytotoxicity b. Drug hypersensitivity c. Drug idiosyncrasy d. Drug supersensitivity e. Drug tolerance
a. Drug cytotoxicity
A 32-year old African American male is receiving Dapsone for PCP prophylaxis. Shortly after starting, he experiences occasional bloody urine. On examination, his U/A is blood+, RBC- neg; Hb 9 g/dL, WBC 9K/uL, Plt 250K/uL, MCV 80 fl. What is the diagnosis? a. G6P deficiency b. Hereditary spherocytosis c. Paroxysmal nocturnal hemoglobinuria d. Paroxysmal cold hemoglobinuria e. Warm autoimmune hemolytic anemia
a. G6P deficiency Smear: Bite or blister cell
The following indicates Alpha-thalassemia in a symptomatic patient: a. HbA 98%, HbF 1%, HbC/A2 1% b. HbA 92%, HbF 3.5%, HbC/A2 4.5% c. HbS 100% d. HbA 60%, HbS 40% e. HbA 50%, HbF 50%
a. HbA 98%, HbF 1%, HbA2 1% Same as wt (normal)
Which of the following abnormal responses is due to drug hypersensitivity reaction? a. Hemolytic anemia after alpha-methyldopa b. Isoniazid-induced peripheral neuropathy c. Porphyrinuria after barbiturates d. Prolonged apnea after succinylcholine e. Red blood cell destruction after primaquine
a. Hemolytic anemia after alpha-methyldopa
The following distinguishes cold autoimmune hemolytic anemia from warm autoimmune hemolytic anemia: a. IgM antibodies binding RBC antigens b. IgG antibodies binding RBC antigens c. C3 coating RBCs d. Extravascular hemolysis e. Increased risk if taking Dapsone or alpha-methyldopa f. Optimal binding at 25C
a. IgM antibodies binding RBC antigens C3b-RBC -> Spleen MF -> extravasc hem C9-MAC attack -> intravasc hemolysis Optimal binding @0-4C
A 93-year old man with a history of medical problems presents with increasing irritability and restlessness. He has some muscle weakness, Hb of 9g/dL, HCT of 29%, Reticulocyte count 0.4%, MCV 110ug/dL, serum iron 40 ug/dL, serum ferritin 150 ng/mL, RBC folate 150 ng/dL, and serum vitamin B12 at 150 pg/mL. The following is true of this patient's anemia: a. Intrinsic factor can be used to treat pernicious anemia or the parietal cells that produce it b. Deficiency develops over 3-6 years c. Excretion occurs primarily in the urine d. The deficient compound is endogenously synthesized by humans e. Tapeworms are a common cause of vitamin B6 deficiency in the developing world
a. Intrinsic factor can be used to treat deficiency of IF or the parietal cells that produce it
Basophilic stippling of red blood cells on blood smear indicate: a. Lead poisoning b. Waldenstrom macroglobulinemia c. Cold autoimmune hemolytic anemia d. Paroxysmal nocturnal hemoglobinuria e. Iron deficiency anemia
a. Lead poisoning Can also indicate severe hemolysis
A 45-year old woman with a history of heavy menses and extensive Ibuprofen use to control osteoarthritis pain presents with abdominal pain and fatigue. She is found to have extensive gastritis, hemoglobin of 8 g/dL, HCT of 27%, reticulocyte count of 2% and MCV of 75 ug/dL. Her serum iron is low at 40 ug/dL, serum ferritin is 9 ng/mL and total iron binding capacity is high at 450 ug/dL. The following is a likely cause of her pathology: a. Low absorption of iron by the duodenum and proximal jejunum b. High levels of ferritin c. Ulcerative colitis affecting her colon d. Hyperchloridia and large amounts of ascorbic acid in her diet e. Low levels of transferrin
a. Low absorption of iron by the duodenum and proximal jejunum
Hereditary spherocytosis can be distinguished from paroxysmal nocturnal hemoglobinuria by the following: a. Mutation of ankyrin, spectrin, of band-3 b. Preserved biconcave structure unless hemoglobin is deoxygenated c. Cholelithiasis and increased osmotic fragility of RBCs to low ionic strength is rare d. Acquired hemolytic anemia e. Deficiency in glycosyl-phosphatidylinositol-linked glycoproteins f. Deficiency of CD55 (DAF) and CD59 (C3 convertase inhibitor) g. Mutation of the phosphotidylinositol glycan A gene h. Both extravascular and intravascular hemolysis
a. Mutation of ankyrin, spectrin, of band-3
Relative polycythemia (erythrocytosis) can be distinguished from absolute by the following: a. Normal red cell mass b. Increased red cell mass c. Normal plasma volume d. Increased plasma volume e. Hematocrit level
a. Normal red cell mass
A 58-year old man presents with several months of fatigue and progressive weakness. Labs reveal anemia with mild cytopenia, low ferritin, and high soluble ferritin receptor. He occasionally notices red urine. He is negative for occult blood in the stool. The following is the most likely cause of his symptoms: a. Paroxysmal nocturnal hemoglobinuria b. Gastritis c. Ulcerative colitis d. Colon cancer e. Pernicious anemia
a. Paroxysmal nocturnal hemoglobinuria
Low erythropoietin levels cause the following: a. Primary erythrocytosis b. Polycythemia vera c. Secondary erythrocytosis d. High-affinity hemoglobinopathy e. Autoimmune-mediated hemolysis
a. Primary erythrocytosis
A 30-year old man presents with finger and toe cyanosis, fatigue, and weakness. He is found to have anemia and tested for possible causes, which is determined to be autoimmune hemolysis. The following would be an appropriate treatment if a direct Coombs test is positive for C3b-bound red blood cells, but not if it is positive for IgG-bound red blood cells. a. Rituximab b. Oral prednisone c. Splenectomy d. Cold exposure e. Erythropoietin
a. Rituximab
Iprogressive fatigue and exercise intolerance. Labs return the following finding: Microcytic hypochromic anemia. The following would suggest anemia due to chronic disease: a. Upregulated hepcidin activity b. High soluble transferring receptor levels c. Excessive release of iron d. Downregulated transferrin e. Low serum iron f. Anisocytosis g. Low iron storage
a. Upregulated hepcidin activity
A 82-year old female with 6 months of some weakness and fatigue. On CBC: Hb 8 g/dL, WBC 3200/uL, Plt 116k/uL, Reticulocyte count 1%, MCV 112 fl. The top cause of this form of anemia is: a. Vitamin B12 or vitamin B6 deficiency b. Methotrexate c. Anti-retroviral drugs d. Hypothyroidism e. Myelodysplastic syndrome
a. Vitamin B12 or vitamin B6 deficiency Macrocytosis (not megaloblastic) anemia #1 cause alcoholism
Cytotoxic autoimmune response involving IgM and IgG targets the following organs with the following clinical manifestations: a. GI tract, skin, and lung: GI allergy, urticaria, and anaphylactic shock b. Circulating blood cells: Leukopenia, cytopenia, hemolytic anemia c. Blood vessels, joints, and kidneys: Nephritis, vasculitis, and arthritis d. Skin, lungs, CNS: Contract nephritis and dermatitis
b. Circulating blood cells: Leukopenia, cytopenia, hemolytic anemia Type 2
A positive direct coombs test can be used to diagnose the following: a. Paroxysmal nocturnal hemoglobinuria - lack of CD55 and CD59 receptors b. Positive Direct Coombs for C3b-bound RBCs in cold autoimmune hemolytic anemia c. Positive Direct Coombs for IgG-bound RBCs in cold autoimmune hemolytic anemia d. Negative Direct Coombs for IgG-bound RBCs in warm autoimmune hemolytic anemia
b. Positive Direct Coombs for C3b-bound RBCs in cold autoimmune hemolytic anemia
A 56-year old male with a prosthetic heart valve presents with fatigue. His CBC: Hb 8 g/dL, WBC 9K/uL, Plt 250K/uL, MCV 84 fl. Reticulocyte count is 8%. The following would be expected to confirm the suspected pathology: a. Spherocytes on blood smear b. Schistocytes on blood smear c. Deficiency of CD55 and CD59 on flow cytometry d. Positive Donath-Landsteiner test e. Positive Coombs test for IgG-RBC
b. Schistocytes on blood smear
A 30-year old man presents with fatigue and weakness following cold exposure after skiing for an hour. He is found to have anemia and tested for possible causes, which is determined to be autoimmune hemolysis. Identifying a Donath-Landsteiner antibody against a P blood group antigen indicates that this patient may also be at risk for: a. Warm hemolytic anemia b. Complement-mediated intravascular hemolysis c. IgG antibodies binding P antigen at 38C d. Complement being fixed to red blood cells at 0-4C e. Paroxysmal nocturnal hematuria
b. Complement-mediated intravascular hemolysis This is paroxysmal cold hemoglobinuria
The following predisposes a patient to folic acid deficiency: a. Vitamin B12 deficiency b. Phenytoin medication c. Smoking d. Diabetes e. Gastritis
b. Phenytoin medication
A 93-year old man with a history of medical problems presents with increasing irritability and restlessness. He has some muscle weakness, Hb of 9g/dL, HCT of 29%, Reticulocyte count 0.4%, MCV 110ug/dL, serum iron 40 ug/dL, serum ferritin 150 ng/mL, RBC folate 100 ng/dL, and serum vitamin B12 at 300 pg/mL. The following could be a cause of this patient's anemia: a. Smoking b. Alcoholism c. Pregnancy d. Diabetes e. Aplastic anemia
b. Alcoholism
A 30-year old man has sudden onset of fatigue, weakness, and pallor. He is brought to the emergency room and lab tests reveal IgG autoantibodies against red cell membrane antigens. The following predisposes this patient to this pathology: a. Dapsone medication or Fava bean consumption b. Alpha-methyldopa medication c. Deficiency of CD55 and CD59 d. Iron deficiency anemia e. Mutation in ankyrin, spectrin, or band-3 cytoskeletal proteins
b. Alpha-methyldopa medication warm autoi2 hemolytic anemia: -Hematopoietic neoplasia, autoi2 dx, a-methyldopa
A 65-year old male with rheumatoid arthritis presents with some weakness and fatigue. On CBC: Hb 9 g/dL, WBC 9k/uL, Plt 500k/uL, Reticulocyte count 1%, MCV 78 fl, RDW 20. Serum iron and TIBC are low, but ferritin is high. What is the diagnosis? a. Iron deficiency disease b. Anemia of chronic disease c. Thalassemia d. Paroxysmal nocturnal hemoglobinuria e. Spherocytosis
b. Anemia of chronic disease
The following is an example of overextension of pharmacological response: a. Dapsone-induced G6P deficiency anemia b. Atropine-induced dry mouth c. Aspirin-induced gastrointestinal toxicity d. Chloramphenicol-induced anemia e. Aminoglycoside-induced renal toxicity
b. Atropine-induced dry mouth
A 45-year old woman with a history of heavy menses and extensive Ibuprofen use to control osteoarthritis pain presents with abdominal pain and fatigue. She is found to have extensive gastritis, hemoglobin of 8 g/dL, HCT of 27%, reticulocyte count of 2% and MCV of 75 ug/dL. Her serum iron is low at 40 ug/dL, serum ferritin is 9 ng/mL and total iron binding capacity is high at 450 ug/dL. This patient would have problems with the following: a. Myoglobin phagolysis b. Cytochrome, catalase, and peroxidase function c. Glycolysis impairment d. Poikilocyte prevention e. Coagulation
b. Cytochrome, catalase, and peroxidase function
A 22-year old woman experiences severe asthma after taking 2 aspirin for a severe headache. Which of the following describes the adverse effect of aspirin in this patient? a. Drug allergy b. Drug idiosyncracy c. Drug toxicity d. Placebo response e. Tachyphylactic response
b. Drug idiosyncrasy NSAID --| COX: In this pt -> Shunt arachidonic acid to lipooxygenase pathway
The following is true of anemia treatment: a. Folic acid or leucovorin can be used to treat folic acid and vitamin B12 deficiencies b. Erythropoietic is an appropriate treatment for normocytic anemia c. Ferrous sulfate is preferred for microcytic anemia if iron storage levels are normal or high d. Macrocytic anemia can be treated with hematopoietic growth factors
b. Erythropoietic is an appropriate treatment for normocytic anemia GFs: Epo, GM-CSF (Sargramostim), Filgrastim(G-CSF), Oprelvekin (IL-11)
The following is true of anemia pathology: a. Factors that impair hemoglobin synthesis cause macrocytic anemia b. Factors that impair red blood cell maturation cause macrocytic anemia c. Factors that impair hematopoiesis or growth factors cause microcytic anemia d. Factors that impair red blood cell maturation cause normocytic anemia
b. Factors that impair red blood cell maturation cause macrocytic anemia
The following is true of hematopoietic growth factors used to treat normocytic anemia or before blood stem cell transplantation. a. Epo is specifically used to stimulate megakaryopoiesis b. Filgrastim is used similarly to Sargramostim to stimulate peripheral blood stem cells for transplantation c. Oprelvekin is used to treat anemia in patients with chronic renal failure d. Epo is used to treat congenital neutropenia and other inherited myeloid disorders
b. Filgrastim is used similarly to Sargramostim to stimulate peripheral blood stem cells for transplantation Epo GM-CSF: Sargramostim G-CSF: Filgrastim IL-11: Oprelvekin
Headache, blurred vision, or stupor become evident at what state of polycythemia or Waldenstrom macroglobulinemia? a. Hct >40% b. Hct >60% c. Hb >14 g/dL d. Hb >20 g/dL e. MHCV >45%
b. Hct >60% Hyperviscosity syndrome
A 39-year old man presents with severe anemia that is determined to be hemolytic on testing. The patient's blood smear indicates bite cells with a Wright stain and Heinz bodies with a cresyl blue stain. The following is true of this patient's pathology: a. A mutation in phosphotidylinositol glycan A predisposes him to oxidative stress b. Insufficiency of NADPH in red blood cells leaves GSH unreduced and incapable of metabolizing H2O2 c. Hemolysis is precipitated by low haptoglobin serum levels d. The patient's cytoskeletal proteins are mutated, causing the abnormal red blood cell morphologies seen on the smear e. Precipitated hemoglobin is causing red blood cells to appear as bite cells
b. Insufficiency of NADPH in red blood cells leaves GSH unreduced and incapable of metabolizing H2O2
Human teratogens involve the following: a. Acetaminophen, phenytoin, Warfarin b. Isotretinoin, phenytoin, valproic acid c. Aspirin, diazepam, atropine d. Dapsone, thalidomide, chloramphenicol e. Sulfonamide, tetracycline, atropine
b. Isotretinoin, phenytoin, valproic acid Sulfonamide: Kernicterus Chloramphenicol: Gray baby syndrome Tetracycline: Bone/tooth dx Other: Thalidomide, antifolates (MTX), phenytoin, warfarin, isotretinoin, Li, valproic acid, EtOH
A 25-year old male presents with lower extremity weakness and anemia. His MCV is 115 fL. What red blood cell abnormality is most likely on the blood smear? a. Increased central pallor b. Macroovalocytes c. Basophilic stippling d. Sickle-like appearance e. Target-like cells
b. Macroovalocytes
The figure depicts the concentration of drug X in venous blood of an experimental subject after rapid bolus IV injection of 10 mg/kg of drug X. Based on the graph, which of the following best describes the distribution of drug X in the subject? a. Mainly in extracellular fluid b. Mainly in total body water c. Highly concentrated in tissues d. Penetrates cellular membranes slowly e. Predominantly bound to plasma protein
b. Mainly in total body water Vd=Amt/[C] = 10/9 = 0.9L/kg = Tissues >0.9 L/kg: Tissues 0.6 L/kg: TBW 0.4 L/kg: plasma 0.2 L/kg: ECF <0.15: Plasma prot
The following pathological signs are associated with megaloblastic anemia: a. Low hemoglobin levels b. Marco-ovalocytes c. Hyposegmented neutrophils d. Low methylmalonic acid and homocysteine e. Low levels of GM-CSF, G-CSF, and IL-11
b. Marco-ovalocytes
The first sign of blood loss anemia is: a. Hemoglobin level b. Mean corpuscular volume c. Fluid shift from interstitial space to intravascular space d. Red blood cell mass
b. Mean corpuscular volume No immediate change Hb - 24-48h Hypovolemic shock Slow fluid shift: Interstit -> Intravasc space Slow RBC mass recovery
An anemic patient's blood smear shows microcytic anemia with target cells and basophilic stippling. The most common cause of this histopathology is: a. Autoimmune hemolytic anemia b. Mutated alpha hemoglobin genes c. Beta-thalassemia d. Pure red blood cell aplasia e. Iron deficiency anemia
b. Mutated alpha hemoglobin genes alpha-thalassemia
A 82-year old female with 6 months of some weakness and fatigue. On CBC: Hb 8 g/dL, WBC 3200/uL, Plt 116k/uL, Reticulocyte count 1%, MCV 112 fl. Her folatic acid and methylmalonic acid levels are normal. On blood smear, she has ringed sideroblasts. This likely indicates the following pathology: a. Anemia of chronic disease b. Myelodysplastic syndrome c. Aplastic anemia d. Thalassemia e. Methotrexate medication f. Chloramphenicol overdose
b. Myelodysplastic syndrome
A 45-year old woman with a history of heavy menses and extensive Ibuprofen use to control osteoarthritis pain presents with abdominal pain and fatigue. She is found to have extensive gastritis, hemoglobin of 8 g/dL, HCT of 27%, reticulocyte count of 2% and MCV of 75 ug/dL. Her serum iron is low at 40 ug/dL, serum ferritin is 9 ng/mL and total iron binding capacity is high at 450 ug/dL. The following is the preferred treatment: a. IV Ferrous gluconate b. Oral ferrous sulfate c. Parenteral iron dextran d. Oral cobalamin (Vitamin B12) e. Oral folate (Vitamin B6)
b. Oral ferrous sulfate
A 45-year old woman with a history of heavy menses and extensive Ibuprofen use to control osteoarthritis pain presents with abdominal pain and fatigue. She is found to have extensive gastritis, hemoglobin of 8 g/dL, HCT of 27%, reticulocyte count of 2% and MCV of 75 ug/dL. Her serum iron is low at 40 ug/dL, serum ferritin is 9 ng/mL and total iron binding capacity is high at 450 ug/dL. The following is the best indicator of her iron status: a. Hemosiderin granules in bone marrow b. Serum ferritin is 9 ng/mL c. Serum iron is 40 ug/dL d. Total iron binding capacity is 450 ug/ dL e. Transferrin saturation
b. Serum ferritin is 9 ng/mL
A 60-year old female presents with 6-months fatigue. CBC: Hb 8 g/dL, WBC 9K/uL, Plt 680 K/uL, Reticulocyte count 1%, MCV 68fL, RDW 22. Which of the following is the most helpful test to differentiate her diagnosis? a. Serum ferritin b. Total iron binding capacity c. Methylmalonic acid d. Direct Coombs test e. Hemoglobin electrophoresis
b. Soluble transferrin receptor Microcytic anemia Sol t-rec: Diff Fe def vs CDA vs thalassemia vs sideroblastic (congen or MDS) vs Pb toxicity
A 93-year old man with a history of medical problems presents with increasing irritability and restlessness. He has some muscle weakness, Hb of 9g/dL, HCT of 29%, Reticulocyte count 0.4%, MCV 110ug/dL, serum iron 40 ug/dL, serum ferritin 150 ng/mL, RBC folate 100 ng/dL, and serum vitamin B12 at 300 pg/mL. Which processes are impaired in this patient? a. Cytochrome oxidase function b. Synthesis of amino acids, purines, and pyrimidines c. Cell apoptosis d. MAP/ Erk/ Mek signaling e. GSH reduction
b. Synthesis of amino acids, purines, and pyrimidines
Penicillin-induced hemolytic anemia is the following type of hypersensitivity reaction: a. Type 1: IgE-mediated b. Type 2: IgG and IgM-mediated c. Type 3: Antigen-antibody complex mediated d. Type 4: Cytotoxic T-cell mediated
b. Type 2: IgG and IgM-mediated Any hemolytic anemia or pancytopenia is typically type 2 & med by IgG, IgM
A patient presents with fatigue and pallor. He has intermittent episodes of hematuria. Upon further testing, he is found to have a mutation of the phosphotidylinositol glycan A gene. This patient is predisposed to the following complications: a. Spherocytosis b. Venous thrombosis c. Microvascular veno-occlusive crisis d. Microcytic anemia e. Transformation to acute lymphoid leukemia
b. Venous thrombosis PNH (paroxysmal nocturnal hemoglobinuria) -> Anemia, hematuria, VTE, tx -> AML
The incidence of placebo response in clinical trials is: a. 0-10% b. 10-20% c. 20-40% c. 40-60% d. 60-75%
c. 20-40%
A 24-year old woman is being treated for cystitis with oral ampicillin, 250 mg every 6 hours. The volume of distribution is 20L, oral bioavailability is 0.7, and half-life is 1.3 hours. Which of the following best represents total body clearance of ampicillin in this patient? a. 50 mL/min b. 100 mL/min c. 200 mL/min d. 400 mL/min e. 600 mL/min
c. 200 mL/min C= (20)*(0.5) = 10L /60 = .16 mL/min ~200 mL/min C=Kel*Vd Kel = .693/1.3= 0.5
Drug Y has the following pharmacokinetic parameters: Urine concentration 15mg/mL, urine flow 2mL/min, total plasma concentration 1mg/mL, plasma protein binding 50%, volume of distribution 50L, bioavailability 0.5. What is the renal clearance of unbound drug Y? a. 15 mL/min b. 30 mL/min c. 60 mL/min d. 120 mL/min e. 600 mL/min
c. 60 mL/min 15*2 = 30mg/min removed *urine plasma conc = 1mg/mL -> Need to clear 30 mL of plasma/min -> Since 50% unbound, 60mL needed
A 75-year old woman weighing 40kg has a ventricular arrythmia and is treated with IV procainamide with a plasma half-life of 4 hours and volume of distribution of 2 L/kg. The effective therapeutic plasma level for cardiac dysrhythmia is 5 mg/L and the minimal toxic plasma concentration is 10 mg/L. What is the constant infusion rate needed to give a steady-state level of 5 mg/L in this patient? a. Less than 1 mg/hour b. About 7 mg/hour c. About 70 mg/hour d. About 0.7 g/hour e. More than 1 g/hour
c. About 70 mg/hour Calc clearance rate Cl=Vd*Kel = .35L/kg/h * =1.75*40=70mg/h 0.7/4h= 0.15? 5mg/L *40kg *2L/kg = 400mg = Load dose t1/2=4h -> Takes 4-5 t1/2 to reach Css
Point mutations in 1 or 2 beta genes that encode chains of hemoglobin cause the following: a. Extramedullary hematopoiesis b. Aggregation of unpaired Beta chains c. Aggregation of unpaired alpha chains d. Increased Beta chain production e. Increased alpha chain production
c. Aggregation of unpaired alpha chains Inadequate B chain production -> alpha chains aggregate
Combined toxicity is noted when the following drugs are co-administered: a. Ethanol and propranolol b. Naloxone and morphine c. Aminoglycoside and vancomycin d. Ethanol and vancomycin e. Ibuprofen and aspirin
c. Aminoglycoside and vancomycin
Severe anemia due to y4 hemoglobin that is usually fatal due to hydrops fetalis is characterized as: a. Hemoglobin H disease b. Heterozygous alpha thalassemia c. Hemoglobin Barts disease d. Heterozygous beta thalassemia c. Homozygous beta thalassemia
c. Hemoglobin Barts disease This is a homozyg alpha-thalassemia
A 93-year old man with a history of medical problems presents with increasing irritability and restlessness. He has some muscle weakness, Hb of 9g/dL, HCT of 29%, Reticulocyte count 0.4%, MCV 110ug/dL, serum iron 40 ug/dL, serum ferritin 150 ng/mL, RBC folate 150 ng/dL, and serum vitamin B12 at 150 pg/mL. The following treatment is appropriate for this patient: a. Oral ferrous sulfate b. IV iron dextran c. Intramuscular hydroxocobalamin d. Oral folate e. Intramuscular cyanocobalamin
c. Intramuscular hydroxocobalamin
The following is an example of pharmacokinetic interaction affecting drug absorption: a. Isoniazid-induced peripheral neuropathy b. Cyclophosphamide-induced pancytopenia c. Aspirin-induced asthma d. Cholestyramine-induced drug binding e. Succinylcholine-induced apnea
d. Cholestyramine-induced drug binding Atropine slows GI motility Antacids: Metals chelate many Rx
The following is true of vitamin B6 and B12 metabolism: a. Administration of folic acid can overcome vitamin B12 deficiency b. Administration of vitamin B12 can overcome folic acid deficiency c. Neurological abnormalities caused by vitamin B12 deficiency cannot be overcome by administration of folic acid d. Neurological abnormalities caused by vitamin B6 deficiency can be overcome by administration of vitamin B12
c. Neurological abnormalities caused by vitamin B12 deficiency cannot be overcome by administration of folic acid Pernicious anemia (vB12) can be treated w/ B6, but neuro Px persists
Subacute toxicity is distinguished from acute toxicity by the FDA as: a. Lasting more than 6 months b. Onset after a single dose c. Onset after 2-4 weeks d. Reproductive toxicity e. Carcinogenesis f. Pharmacological profile
c. Onset after 2-4 weeks
A 30-year old man has sudden onset of fatigue, weakness, and pallor. He is brought to the emergency room and lab tests reveal spherocytes on blood smear. He has systemic lupus erythematosus. The following diagnostic test would confirm the suspected diagnosis: a. Flow cytometry detecting a deficit in CD55 and CD59 cells b. Genetic mutation in hemoglobin beta chain c. Positive direct Coombs test for IgG bound to red blood cells d. Bite or helmet cells on blood smear e. Positive indirect Coombs test for IgM bound to red blood cells
c. Positive direct Coombs test for IgG bound to red blood cells warm autoi2 hemolytic anemia: + direct Coombs for IgG-RBC, C3-RBC 70% Tx: Oral prednisone, splenectomy
A 30-year old man has sudden onset of fatigue, weakness, and pallor. He is brought to the emergency room and lab tests reveal IgG autoantibodies against red cell membrane antigens. The following is expected in the pathology of this disease: a. Increased sensitivity of red blood cells to complement-mediated lysis b. X-linked recessive pattern of inheritance c. Spherocytes on blood smear d. Optimal binding at 25C e. Increased hemoglobin sensitivity to oxidative stress
c. Spherocytes on blood smear Autoi2 hemolytic anemia -Warm -Cold -Paroxysmal cold hemoglobinuria
Sulfonamides can trigger a severe form of immune vasculitis. This is what type of hypersensitivity reaction? a. Type 2: IgG and IgM-mediated b. Type 1: IgE-mediated c. Type 3: Antigen-antibody complex mediated d. Type 4: Cytotoxic T-cell mediated
c. Type 3: Antigen-antibody complex mediated
A 93-year old man with a history of medical problems presents with increasing irritability and restlessness. He has some muscle weakness, Hb of 9g/dL, HCT of 29%, Reticulocyte count 0.4%, MCV 110ug/dL, serum iron 40 ug/dL, serum ferritin 150 ng/mL, RBC folate 100 ng/dL, and serum vitamin B12 at 300 pg/mL. His anemia has likely developed over the past: a. 3-4 days b. Week c. 1-2 months d. 1-6 months e. 12 months
d. 1-6 months B6 anemia -> Megaloblastic anemia
An antibiotic is given by IV push at 100mg/d. Half-life is 36 hours with volume of distribution 10 liters. How long will it take before average plasma concentration is greater than 97% of the average steady-state concentration? a. 2 days b. 4 days c. 5 days d. 8 days e. 12 days
d. 8 days 50->75->87.5->94->97% 36->72->108->144->180 Takes 4-5 half lives: 36*5=180/24 = 7.5d
Regular hemoglobin synthesis can be characterized by the following: a. 95% HbA2 containing 2 alpha and 2 delta chains b. 95% HbF containing 2 alpha and 2 gamma chains c. 50% HbA containing 2 alpha and 2 Beta chains d. All isomers contain 2 alpha chains e. Alpha subunits are on chromosome 11; B and gamma chains are on chromosome 16
d. All isomers contain 2 alpha chains HbA a2B2 97% HbA2 a2d2 2% HbF a2y2 1%
A 39-year old man presents with severe anemia that is determined to be hemolytic on testing. He recently ate a restaurant and recalls eating a bean salad just prior to becoming severely tired and ill. The following is true of the suspected pathology: a. A deficiency of CD55 and CD59 predispose this patient to complement-mediated hemolysis b. A mutation in ankyrin or spectrin predispose this patient to spherocytosis c. This man is having a sickle cell microvascular occlusive attack d. An X-linked recessive deficiency in an erythrocyte enzyme is responsible e. These symptoms can be precipitated by Isoniazid or Phenytoin
d. An X-linked recessive deficiency in an erythrocyte enzyme is responsible GDPD deficiency; X-linked recessive; ->lack of NADPH -> Lack of GSH -> Accumulation of H2O2 -> Oxidative stress - Precip by Dapsone, fava beans
A dose of 80mg of drug X is administered to a 50kg woman. Before appreciable metabolism or excretion, the plasma concentration is found to be 8 mg/L. Which of the following best describes the distribution of drug X in this patient? a. Concentrated in tissues b. Confined to plasma c. Distributed in total body water d. Distributed into extracellular fluid e. Highly plasma protein-bound
d. Distributed into extracellular fluid 80mg -> 8mg/L: In 10L Relative to 70kg person, 10L is about 17L, which would be ECF
Cardiac toxicity is associated with the following: a. Long-term aspirin use b. Diazepam c. Atropine d. Doxorubicin e. Acetaminophen f. Aminoglycoside g. Dapsone
d. Doxorubicin
A 45-year old patient presents with fatigue and pallor. He has intermittent episodes of hematuria. What testing is appropriate to definitively diagnose this patient with the suspected pathology? a. Blood smear b. Iron level testing c. Homocysteine serum level d. Flow cytometry e. Staining with new methylene blue on bone marrow biopsy
d. Flow cytometry Detect deficiency of CD55 and CD59: Paroxysmal nocturnal hemoglobinuria
A 93-year old man with a history of medical problems presents with increasing irritability and restlessness. He has some muscle weakness, Hb of 9g/dL, HCT of 29%, Reticulocyte count 0.4%, MCV 110ug/dL, serum iron 40 ug/dL, serum ferritin 150 ng/mL, RBC folate 100 ng/dL, and serum vitamin B12 at 300 pg/mL. Which type of anemia does the patient have? a. Iron deficiency b. Anemia of chronic disease c. Pure red cell aplasia d. Folate (B6) deficiency e. Cobalamin (B12) deficiency
d. Folate (B6) deficiency
The Donath-Landsteiner test is correctly described as: a. Hemolysis occurs at 37C for 60 min, then no hemolysis once the temperature is lowered to 4C b. No hemolysis occurs at 37C for 60 min, then hemolsys once the temperature is lowered to 4C c. Hemolysis at both 37C and 4C d. Hemolysis only after the temperature has been increased back to 37C from 4C e. Hemolysis only after the exposure to 4C exceeds 60 minutes
d. Hemolysis only after the temperature has been increased back to 37C from 4C
The following is correct regarding the algorithm to diagnose absolute erythrocytosis: a. Normal red cell mass and low Epo indicate polycythemia vera b. Low red cell mass and increased pO2 indicate kidney cyst, hydroneprosis, or Epo-secreting tumor c. High red cell mass, high Epo, and high pO2 indicate high altitude, lung disease, heart disease, or high-affinity hemoglobin d. High red cell mass and low Epo indicate polycythemia vera
d. High red cell mass and low Epo indicate polycythemia vera ^HCT, RBC mass -> Epo: low -> PV -> Epo: high -> pO2 ok -> Renal/ Epo canc -> Epo: high -> pP2 low -> Lung/ heart/ altitude/ high-aff Hb
A 23-year old female presents with chronic fatigue and occasional red urine.Labs reveal that she has microcytic anemia with decreased CD55 and CD59 expression. What is the pathophysiological mechanism of this anemia? a. Increased sensitivity of red blood cells to oxidative stress b. Impaired DNA synthesis within erythroid progenitor cells c. Splenic trapping of red cells with polymerized hemoglobin d. Increased sensitivity of red cells to complement-mediated lysis e. Mutated cytoskeletal proteins causing red blood cell aggregation
d. Increased sensitivity of red cells to complement-mediated lysis
A 45-year old woman with a history of heavy menses and extensive Ibuprofen use to control osteoarthritis pain presents with abdominal pain and fatigue. She is found to have extensive gastritis, hemoglobin of 8 g/dL, HCT of 27%, reticulocyte count of 2% and MCV of 75 ug/dL. Her serum iron is low at 40 ug/dL, serum ferritin is 9 ng/mL and total iron binding capacity is high at 450 ug/dL. What type of anemia does the patient have? a. Beta thalassemia b. Folate, B6 deficiency c. Cobalamin, B12 deficiency d. Iron deficiency e. Anemia of chronic disease
d. Iron deficiency
A 32-year old female presents with progressive fatigue and exercise intolerance. Labs return the following findings: Microcytic hypochromic anemia with low ferritin and high transferrin and soluble transferrin receptor levels. The most likely diagnosis is: a. Hemochromatosis b. Anemia due to chronic disease c. Aplastic anemia d. Iron deficiency anemia e. Folate deficiency anemia f. Koilonychia
d. Iron deficiency anemia High transferrin + sol transferrin receptor
Sickle cell disease is characterized by the following: a. Intravascular hemolysis b. Hemoglobinopathy causing microcytic anemia c. G6V mutation in the Beta chain of hemoglobin predispose it to degradation d. Microvascular occlusion is due to increased HbS precipitation under deoxygenated state e. Elevated HbA2 on electrophoresis f. Treatment by transfusion or transplantation precedes hydroxyurea treatment
d. Microvascular occlusion is due to increased HbS precipitation under deoxygenated state
Extravascular hemolysis can be distringuished from intravascular hemolysis by: a. Increased reticulocyte count b. Increased LDH1, LDH2 c. Increased unconjugated bilirubin d. Normal serum haptoglobin e. Increased serum and urine hemoglobin
d. Normal serum haptoglobin
The following is correct regarding human clinical trials: a. Phase 1 tests clinical effectiveness in hundreds of patients b. Phase 2 tests efficacy and toxicity c. Phase 3 finds the maximum tolerated dose d. Phase 4 enabled detection of aplastic anemia in chloramphenicol therapy in tens of thousands of subjects e. An NDA is filed with the FDA during phase 1 f. Informed consent is required in writing for phase 1, 2, and 3
d. Phase 4 enabled detection of aplastic anemia in chloramphenicol therapy in tens of thousands of subjects
Which of the following statements is true regarding concentration of drug at steady-state Css? a. Fluctuations decrease with longer dose intervals b. Plateau concentration is directly related to Dl c. Plateau concentration is directly related to Kel d. Plateau concentration is inversely related to Vd e. Time to reach the plateau state is directly proportional to the administered dose
d. Plateau concentration is inversely related to Vd
Pharmacokinetic excretion is correctly described to be affected in the following ways: a. Alkalinization of urine by ammonium chloride enhances excretion of weak organic acids b. Alkalinization of urine by sodium bicarbonate decreases excretion of weak organic acids c. Acidification of urine by ammonium chloride decreases excretion of weak organic bases d. Acidification of urine by sodium bicarbonate increases excretion of weak organic bases d. Probenicid inhibits tubular secretion of penicillin
d. Probenicid inhibits tubular secretion of penicillin
Drug hypersensitivity is characterized by the following: a. Dose-response reaction b. Occurs in a majority of the population c. Manifestations are extensions of usual pharmacological or toxicological effects of the drug d. Require primary sensitization prior to immunological response e. Most drug are immunogenic as small molecules on their own
d. Require primary sensitization prior to immunological response
Synergistic effects are observed the following are coadministered: a. Ethanol and probenecid b. Isoniazid and chloramphenicol c. Ibuprofen and aspirin d. Valiuim and ethanol e. Naloxone and isoniazid
d. Valiuim and ethanol
The figure depicts the effects of urine pH on renal clearance of drug X in an experimental subject. Based on the results, the drug is most likely to have which structure? a. Non-ionizable compound b. Strong organic acid c. Strong organic base d. Weak organic acid e. Weak organic base
d. Weak organic acid Excretion is from filtration (vs secretion) -because it changes w/ pH
Paroxysmal nocturnal hematuria can be distinguished from paroxysmal cold hemoglobinuria by the following: a. Complement-mediated hemolysis b. IgG binds red blood cells at 0-4C c. Anemia and hemoglobinuria episodes following cold exposure d. Predisposition due to measles, mumps, or varicella viruses e. Deficiency of CD55 and CD59
e. Deficiency of CD55 and CD59
A 68-year old man with non-Hodgkin lymphoma is anemic and on blood smear, found to have red blood cell agglutination. What lab test is most useful in diagnosing the cause of his anemia? a. Hemoglobin electrophoresis b. Serum ferritin c. Serum methylmalonic acid d. Donath-Landsteiner test e. Direct Coombs test
e. Direct Coombs test Hb SPEP - Thalassemia, SCD Ser Fer - Fe def Ser methylmalonic acid - Vit B12 def D-L test: Paroxysmal cold hemoglobinuria Dir Coombs: Warm, cold autoi2 hemolytic anemia
The following predispose a patient to IgM antibodies binding RBC antigens precipitating autoimmune hemolytic anemia: a. Dapsone medication b. alpha-methyldopa medication c. Liver disorder d. Mutation of C3 complement e. EBV - Little i antigen f. Systemic lupus erythematosus g. Mycoplasma pneumoniae - Little i antigen
e. EBV little i antigen
The following distinguishes folic acid deficiency from vitamin B12 deficiency: a. Macroovalocytes b. Microcytic anemia c. Megaloblastic hematopoietic precursors d. Neurological deficits e. High homocysteine serum levels
e. High homocysteine serum levels
A 82-year old female with 6 months of some weakness and fatigue. On CBC: Hb 8 g/dL, WBC 3200/uL, Plt 116k/uL, Reticulocyte count 1%, MCV 112 fl. The following would be indicative of vitamin B12 deficiency versus folate deficiency: a. High LDH b. Low B12 level c. Low B6 level d. High homocysteine e. High methylmalonic acid
e. High methylmalonic acid
The following is an inappropriate cause of absolute erythrocytosis: a. Polycythemia vera b. High altitude c. Low erythropoietin levels d. High affinity hemoglobinopathy e. Hydronephrosis f. COPD
e. Hydronephrosis 1 Local kidney hypoxia: Cyst, hydronephrosis 2 Epo-secreting tumors
A patient with inherited hemochromatosis is experiencing liver and cardiac symptoms indicating uncontrolled disease. He is not anemic. The following approach is the most appropriate treatment: a. Deferoxamine b. Whole bowel irrigation c. Oral ferrous sulfate d. Supportive care while the patient recovers e. Phlebotomy
e. Phlebotomy
The following is a late sign of hemolytic anemia, typically occuring 1 week after onset: a. Lactate dehydrogenase, especially LDH1 and LDH2 b. Urine and free hemoglobin c. Decreased levels of haptoglobin since it binds free hemoglobin to form a hemoglobin-haptoglobin complex d. Unconjugated bilirubin e. Reticulocytosis
e. Reticulocytosis (vRBC mass -> ^Epo) Cardiac, kidney also have LDH Muscle, liver have LDH4, LDH5
A 60-year old female is asymptomatic, but CBC reveals Hb 10.3 g/dL, WBC 9k/uL, Plt 300K/uL, Reticulocyte count 1%, MCV 68fl, RDW 15. The serum iron is 160 (slightly low), TIBC is 300 and ferritin 150 (both normal). What is the diagnosis? a. Sideroblastic anemia due to myelodysplastic syndrome b. Iron deficiency anemia c. Anemia of chronic disease d. Paroxysmal nocturnal hemoglobinuria e. Thalassemia
e. Thalassemia
Schistiocytes on blood smear, DIC, or thrombocytic thrombocytopenic purpura indicate the following cause of anemia: a. Chronic disease b. Paroxysmal nocturnal hemoglobinuria c. Paroxysmal cold hemoglobinuria d. Warm autoimmune hemolytic anemia e. Trauma-related hemolytic anemia
e. Trauma-related hemolytic anemia
Helmet cells seen on blood smear resulting from microangiopathic hemolysis and trauma are similar to bite cells of what pathology? a. Paroxysmal nocturnal hemoglobinuria b. Warm autoimmune hemolytic anemia c. Spherocytosis d. Cold autoimmune hemolytic anemia e. Paroxysmal cold hemoglobinuria f. G6P deficiency
f. G6P deficiency
