Cytopenias
3) intercurrent viral infection
A 6 y/o boy with severe hemophilia B comes to the ER after falling two stories from an open window. He is unconscious and has an obvious hematoma on the back of his head. While the nurse is arranging a stat CT, you are calculating his dose of rFIX. He weighs 20 Kg. Given that rFIX dosing is higher than pd-FIX, the best approximate dose would be? 1) 2500 units; 2) 5000 units; 3) 1250 units; 4) 500 units
1) 2500 units
Inherited resistance to activated protein C can be diagnosed by requesting genetic testing of the gene for which coagulation protein? 1) factor V; 2) factor II; 3) factor VIII; 4) protein S; 5) protein C
1) factor V
10 wk old AAM is referred for abnormal newborn screen (FS). Family testing reveals that father has Hgb AS while mother has only Hgb A. You order Hgb electrophoresis on the infant which reveals FSA pattern. The most likely diagnosis for this infant that explains these labs is: 1) non-paternity; 2) sickle beta+ thal; 3) sickle beta zero thal; 4) sickle cell trait; 5) sickle cell anemia
2) sickle beta + thal
Platelet units can be derived from whole blood donations (RDPs) or by apheresis techniques (SDPs). Single donor platelet units: 1) are best delivered using a bedside leukoreduction filter; 2) require storage at 2-6 degrees Celsius; 3) contain the # of platelets equivalent to 6-8 units of RDPs; 4) can be stored up to 10 days; 5) are more likely than RDP to be contaminated with bacteria
3) contain the # of platelets equivalent to 6-8 units of RDPs
The major source of calories for an 18 month old girl is unpasteurized goat's milk from the family farm. Hgb is 7.5 g/dL, MCV 80, retic 2.2%. Which nutritional deficiency is most likely the cause of this child's anemia? 1) folate; 2) iron; 3) folate and iron; 4) folate, iron, and B12; 5) B12 and pyridoxine
3) folate and iron
Joint bleeding is a hallmark of the hemophilias (Factor VIII and IX deficiency). Which one of these less common factor deficiencies is also known to present with joint bleeding? 1) factor II; 2) factor V; 3) factor XI; 4) factor XII; 5) factor X
5) factor X
A 2 y/o ingests a warfarin-like rat poison and develops dark red-black necrotic skin lesions on his thighs and abdomen. Which of the following is the most likely explanation? 1) congenital protein C deficiency; 2) antithrombin III deficiency; 3) factor V Leiden heterozygous state; 4) lupus anticoagulant; 5) prothrombin gene mutation
1) congenital protein C deficiency
9 y/o Greek male with beta thal major receives q 3 wk pRBC transfusion. Mid-way through he develops fever, chills, hypotension, gross hematuria and mouth bleeding. Labs showed: WBC 21 (60%N, 15%B, 29%L), Hgb 4.5 g/dL, plat 75K, PT 30, PTT 55, fib 70, D-dimer >20. You presume a hemolytic transfusion reaction and 2ry DIC. Which is the most appropriate initial therapy? 1) NS, Abx, pRBC, FFP; 2) NS, ATIII, pRBC, FFP; 3) Aminocaproic acid, pRBC, platelets; 4) UFH, NS, pRBC, Cryo
1) NS, Abx, pRBC, FFP
Prophylactic platelet transfusions are reserved for patients who are profoundly thrombocytopenic (<10K). Which of the following patients with a platelet count <10K should be transfused? 1) 10 y/o girl with ITP and profuse nosebleeds; 2) 14 y/o boy with TTP and skin hemorrhage; 3) 8 y/o girl with SLE-induced thrombocytopenia and hematuria; 4) 1500 g preterm infant on a ventilator; 5) 4 y/o boy with Evans syndrome and bloody stool
4) 1500 g preterm infant on a ventilator
6 month old girl presents with pseudomonas skin infection. ANC is noted to be 40000. Evaluation of neutrophil function reveals an inability of the neutrophils to adhere to endothelium. Which of the following disorders accounts for the phenotype? 1) Chediak-Higashi syndrome; 2) chronic granulomatous disease; 3) specific granule deficiency; 4) leukocyte adhesion deficiency, type 1
4) leukocyte adhesion deficiency, type 1
All clotting factors are produced in the liver and some are also produced in other locations. Which of the following coagulation factor is found in megakaryocytes? 1) factor VIII; 2) factor XI; 3) factor IX; 4) factor II
b) factor XI
A 17 y/o immigrant from Poland develops DVT after being kicked playing soccer. The most likely hereditable predisposition contributing to his thrombosis is: 1) lupus anticoagulant syndrome; 2) antithrombin III deficiency; 3) activated protein C resistance (Factor V Leiden); 4) prothrombin (Factor II) deficiency; 5) protein C deficiency
3) activated protein C resistance (Factor V Leiden)
A 6 wk old Pakistani male is referred for an abnormal "Hgb F only" on newborn screen. The infant appears healthy. On exam, spleen tip is palpable 1-2 cm. Labs show Hgb 9.5 g/dL. Hgb electrophoresis confirms Hgb F only. Mom's Hgb electrophoresis shows Hgb A with 6.1% Hgb A2. Father is not available for testing. The most likely diagnosis in this child is: 1) beta thal major; 2) beta thal minor; 3) two gene deletion alpha thal; 4) three gene deletion alpha thal; 5) four gene deletion alpha thal
1) beta thal major
Cryoprecipitate is derived from a unit of FFP that has been thawed at 6-8 degrees Celsius. Cryoprecipitate is rich in factors I, VIII, XIII, and VWF. In USA, cryoprecipitate is a good choice for treatment of bleeding in patients with: 1) severe hemophilia A; 2) mild hemophilia B; 3) Type 1 VWD; 4) Hypofibrinogenemia; 5) Hypoprothrombinemia
4) Hypofibrinogenemia
In the eariest stages of iron deficiency, which of the following test results becomes abnormal first? 1) hemoglobin; 2) MCV; 3) ferritin; 4) serum iron; 5) RDW
3) ferritin
Thrombin is one of the key proteins in the coagulation cascade and has both procoagulant and anticoagulant properties. Which one of the following is an important anticoagulation function of thrombin? 1) activation of factors V and VIII; 2) factor XIII activation; 3) stimulation of thrombin activatable fibrinolytic inhibitor (TAFI); 4) forms a complex with thrombomodulin (TM)
4) forms a complex with thrombomodulin (TM)
5) factor VIII
3) alpha chain variant with a point mutation at the stop codon
3) mitomycin C/DEB chromosome fragility assay
Compared to normal erythrocytes, spherocytes have:1) increased osmotic fragility at all saline concentrations; 2) increased osmotic fragility at 0.5% saline; 3) decreased osmotic fragility at 0.9% saline; 4) increased levels of 2,3 DPG; 5) increased number of Howell-Jolly bodies
2) increased osmotic fragility at 0.5% saline
Type 2N VWD is distinguished from type 2M VWD by the following lab testing: 1) abnormal multimeric pattern; 2) markedly reduced FVIII activity in comparison to the VWF Ag/RCo activity; 3) inappropriately low VWF:RCo activity when compared to VWF:Ag; 4) abnormal response to low dose ristocetin
2) markedly reduced FVIII activity in comparison to the VWF Ag/RCo activity
A 2800 gram term newborn male has presumed sepsis and a purpuric rash. Protein C is 6%, protein S is 40%, and ATIII is 60%. To discern whether this is acquired rather than hereditary protein C deficiency, you should: 1) order molecular studies for Factor V Leiden; 2) measure prothrombin level; 3) infuse recombinant activated protein C; 4) check protein C levels on parents; 5) give vitamin K
4) check protein C levels on parents
In the deoxygenated state, the mutation in sickle hemoglobin primarily affects which of the following interactions? 1) alpha globin to beta globin; 2) beta globin to beta globin; 3) Hgb tetramer to Hgb tetramer; 4) Hema ring to Hgb; 5) RBC to RBC
3) Hgb tetramer to Hgb tetramer
A newborn infant presents with omphalitis and an ANC of 200. He receives 10 mcg/kg G-CSF with normalization of ANC. Three years later a mutation in his G-CSF receptor is found. Over the next few years, the patient is at increased risk of developing: 1) severe aplastic anemia; 2) AML; 3) oral cancer; 4) 1 and 3; 5) normal ANC without G-CSF
2) AML