American Society of Hematology (ASH) ITE Practice Test

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• Historically, which test was used to screen for hereditary spherocytosis?
• HbH disease typical findings include which combination?
• Chronic granulomatous disease can be linked to which genetic/phenotype?
• Which of the following laboratory findings is typical of red blood cell membrane disorders?
• What is the typical bleeding pattern in secondary hemostatic defects?
• Which variant is noted to have no hemolysis?
• The PIGA gene influences the synthesis of what cell surface structure?
• Which statement about Protein S and Protein C is true?
• In sickle cell disease, which complications are more frequent compared with HbSS?
• In early iron deficiency anemia, which laboratory finding is typically increased?
• Two first-line indications for warfarin include:
• Which of the following clinical features is commonly associated with red blood cell membrane disorders?
• Cold agglutinin disease (CAD) can be associated with which statement regarding underlying conditions?
• What is the inheritance pattern of G6PD deficiency?
• HbH tetramers are composed of which chains?
• Which infection has been reported to trigger G6PD oxidative stress?
• Pyruvate kinase deficiency is included as an iron-loading anemia due to ineffective erythropoiesis.
• Warm autoimmune hemolytic anemia (wAIHA) labs typically show:
• Which intrinsic pathway factor deficiency is most often associated with bleeding and may present later in life?
• What is the principal hematologic risk driving mortality in PNH?
• Sildenafil has been observed to do what in sickle cell disease?
• Dysfibrinogenemia is inherited in which pattern?
• Within the iron-loading anemia group, Pyruvate kinase deficiency is an example of which type of disorder?
• Which immunoglobulin class is more strongly associated with risk in antiphospholipid antibodies?
• HbA2 is composed of which globin chains?
• Which statement best describes type IIN von Willebrand disease?
• Which regimen is used to eradicate Factor VIII inhibitors?
• Approximately how much iron is stored in the body of a typical adult male?
• In valve-related severe hemolysis, which urinary finding is expected?
• Which therapy has been used off-label to treat hyperhemolysis crisis in SCD?
• In intrinsic pathway defects, how does proximity to the common pathway affect bleeding severity?
• Which of the following decreases hepcidin levels?
• How is ddAVP dosed for von Willebrand disease?
• The main driver mutation for hereditary hemochromatosis is:
• What can cause falsely elevated assays of Protein C, Protein S, or Antithrombin activity?
• Which statement about Factor XII deficiency is correct?
• Which findings are consistent with depleted iron stores?
• Which G6PD variants are most often associated with severe disease?
• Which condition is an example of an iron-loading anemia associated with ineffective erythropoiesis?
• In type IIN von Willebrand disease, which laboratory pattern best describes the condition?
• Compared with sickle cell anemia (SS), SC disease is generally which?
• PCC reverses VKA-related coagulopathy. Which statement is true?
• What does triple positive mean in antiphospholipid antibody testing?
• Which infection is associated with a boxed warning for Eculizumab?
• Which components belong to the natural anticoagulant pathways?
• Fondaparinux action is to:
• Which test is used to screen for paroxysmal nocturnal hemoglobinuria clones?
• In mixing studies, progressive prolongation with incubation indicates which phenomenon?
• How are von Willebrand factor multimers formed?
• At approximately what age does HbA become the major hemoglobin species in humans?
• Factor IX dosing: 1 U/kg raises the level by approximately what percent?
• Megaloblastic anemia is described as an iron-loading anemia in the notes?
• Who should get voxelotor?
• In PNH, which of the following is a consequence affecting the bone marrow?
• Which statement about clearance of unfractionated heparin, LMWH, and fondaparinux is true?
• For myelonecrosis due to an underlying condition, which statement is true?
• What is another name for serous atrophy?
• What is the normal percentage range of HbA2 on electrophoresis?
• True or false: Megaloblastic anemia is described among iron-loading anemias in the notes.
• What is the role of antihuman globulin in the direct antiglobulin test?
• On electrophoresis, which hemoglobin migrates furthest toward the anode?
• A heme-related manifestation of zinc deficiency is which of the following?
• Where is hemolysis most prominent in warm AIHA?
• Adhesion is the main driver for which sickle cell disease complications?
• Two gene deletions with the same phenotype (alpha, alpha/ -ve, -ve or alpha, -ve/ alpha, -ve) typically present as
• What is the major mechanism of death in PNH?
• In von Willebrand disease subtypes IIA and IIB, how does vWF ristocetin cofactor activity (vWF:RCo) typically compare with FVIII and vWF antigen?
• Acute megaloblastosis can occur after recreational use of which substance?
• Which statement best describes labs during a G6PD hemolytic episode?
• Which statement best describes the effect of Factor V Leiden status on first and recurrent VTE risk?
• Which of the following is a recognized cause of acute chest syndrome in sickle cell disease?
• Altuvio’s fusion protein consists of D'D3 domains of von Willebrand factor fused to which component?
• Electrophoresis pattern for sickle cell trait typically shows which distribution?
• Which statement best describes PTT and PT testing?
• Primary clearance routes: Argatroban is cleared by the liver; Bivalirudin is cleared by the kidneys. Which option reflects this?
• What is a standard von Willebrand panel composed of?
• Which statement best describes smear differences between warm AIHA and CAD?
• What reaction is catalyzed by glucose-6-phosphate dehydrogenase (G6PD)?
• Which are the intrinsic pathway contact factors?
• Which of the following is a recognized sign of copper deficiency?
• Thalassemia trait laboratory characteristics typically include which combination?
• What is the typical bleeding pattern in primary hemostatic defects?
• Compared with controls, the relative risk of thrombosis is approximately 2.8-fold for Protein C, Protein S, or Antithrombin deficiency and about 2-fold for antiphospholipid antibodies (APLA).
• What does von Willebrand factor ristocetin cofactor (RCoF) activity refer to?
• How does voxelotor work?
• Which statement describes the effect of antithrombin in the presence of heparin?
• Which hematologic adverse effect is specifically noted for Deferiprone in the notes?
• Which agent can trigger release of Weibel-Palade bodies to increase von Willebrand factor release?
• Which statement about PAI-1 is correct?
• What is a key risk associated with abrupt discontinuation of voxelotor?
• Unstable alpha4 tetramers lead to which pathophysiology?
• Warfarin MOA is best described as:
• What is the laboratory pattern in Type I von Willebrand disease?
• For voxelotor, the eligible Hb range and clinical history criterion include which of the following?
• Which option is commonly considered a second-line option after glucocorticoids for warm AIHA?
• Splenic sequestration crisis is characterized by which presentation?
• Which therapy reduces vaso-occlusive crises by targeting the P-selectin adhesion pathway?
• Hyperhemolysis crisis is best described as which of the following?
• A patient who is heterozygous for Factor V Leiden and heterozygous for the Prothrombin G20210A mutation has what level of risk for recurrent VTE compared to non-carriers?
• Which statement best describes the relationship between iron-loading anemias and ineffective erythropoiesis?
• Two antibodies causing immune hemolytic anemia missed by DAT are:
• Which statement best describes the function of von Willebrand factor at a vascular injury site?
• The four iron-loading anemia examples are Thalassemia syndromes, Megaloblastic anemia, PK deficiency, and MDS. Which of the following is NOT listed among them?
• Which statement about DAT testing is true?
• Which is a key management option for hereditary spherocytosis?
• Myelodysplastic syndrome is included among iron-loading anemias due to ineffective erythropoiesis. Which of the following best describes this relationship?
• CD59 deficiency in PNH results in increased susceptibility to which process?
• Which electrophoresis finding is pathognomonic for thalassemia trait?
• What is the role of CD59 in PNH?
• In first VTE risk assessment, which findings predict higher risk?
• The bone marrow changes described as vacuoles in RBC precursors and ringed sideroblasts are most characteristic of copper deficiency and can resemble which disorder?
• Which are inhibitors of plasmin and plasminogen?
• In valve-related hemolysis, which statement is true?
• Argatroban pharmacokinetics include a half-life of about 1 hour and clearance primarily by the liver. Which option captures this?
• Which dietary change is recommended for hemochromatosis?
• Porcine FVIII cross-reactivity with human inhibitors is:
• What does the Direct Antiglobulin Test detect?
• What is the maintenance FIX dose for major surgery to keep above 50%?
• Hgb electrophoresis in thalassemia trait shows which pattern?
• If both PT and PTT are prolonged, which factors are common culprits?
• Which conditions are associated with acquired Factor XIII inhibitors?
• Which amino acid substitution constitutes the HbS abnormality in hemoglobin?
• Desmopressin is contraindicated due to risk of worsening thrombocytopathy in which vWD type?
• During an acute PNH attack, which management is most commonly employed?
• Which products can bypass inhibitors in hemophilia patients?
• For which scenario are vWF concentrates most appropriately dosed with redosing every 12–24 hours?
• Which distribution best describes hemoglobin A and S in sickle cell trait?
• In hereditary spherocytosis, what happens to spherocytes in the spleen?
• Which genotype represents sickle cell disease (SCD)?
• Which of the following can cause acquired Protein S deficiency?
• In PNH, direct antiglobulin test (DAT) results are typically which?
• The HFE protein's role is to sense iron stores by interacting with which receptor?
• In healthy adults, HbF constitutes less than what percent?
• Which statement correctly describes the relative severities of G6PD variants?
• In the management of acute stroke in sickle cell disease, what is the goal of exchange transfusion?
• Which of the following is listed as an iron-loading anemia?
• Hereditary spherocytosis commonly involves mutations in which membrane protein?
• What is a typical dosing strategy for vWF concentrates during major surgery?
• By age 40, approximately what percentage of individuals with SS have a venous thromboembolism?
• HbH disease results from deletion of how many alpha-globin genes?
• Therapeutic options for acquired von Willebrand disease include which of the following?
• What are the three main tests for antiphospholipid antibodies (APLA) testing?
• Altuvio is a fusion protein composed of D'D3 domains of von Willebrand factor fused to which FVIII component?
• Which two tests measure heparin effect, and which is considered better?
• When is G6PD testing most reliable?
• In CAD, the primary mechanism of hemolysis is:
• Which medication is a known oxidative stressor in G6PD deficiency?
• Hydroxyurea therapy should be considered for which sickle cell genotypes?
• Polychromasia on a smear indicates which hematologic condition?
• In von Willebrand disease type 2B, ristocetin-induced platelet aggregation is characterized by?
• Which test demonstrates rapid clot lysis in Factor XIII deficiency or inhibitors?
• Which diagnostic test is used to diagnose paroxysmal nocturnal hemoglobinuria (PNH)?
• Which statement best describes type III von Willebrand disease?
• How long is typically required to replete iron stores with oral therapy?
• What is the standard cutoff for severe hemophilia in percent of pooled normal plasma?
• Loxoscelism refers to a potentially fatal condition from bite of which spider?
• What is the endpoint of primary hemostasis?
• Major vs minor hemorrhage dosing goals for FVIII/IX: which statement is correct?
• Which statement best describes Factor VII deficiency?
• Spectrin and protein 4.1 can be implicated in which conditions?
• What is the primary purpose of chronic transfusion therapy in sickle cell disease?
• For dental extractions in von Willebrand disease, what is the typical vWF concentrate dose?
• Beta-thalassemia major is associated with which clinical features?
• Which of the following is a less common mutation associated with hemochromatosis?
• Factor XII deficiency is characterized by which of the following?
• Which of the following is a common hematologic abnormality in SS anemia aside from the anemia itself?
• In sickle/Beta-plus thalassemia, what is the typical composition of HbS and HbA?
• Which coagulation factors are vitamin K dependent?
• Which red blood cell morphology is typical of late iron deficiency anemia?
• Which antiplatelet agent has a very short half-life of less than 1 hour?
• Which of the following is an indication for exchange transfusion in sickle cell disease?
• FVIII dosing: 1 U/kg raises the level by approximately what percent?
• The notes indicate that both Deferiprone and Deferasirox cause which of the following?
• Which agent increases the oxygen affinity of hemoglobin, thereby reducing sickling?
• Which molecule is the master regulator of iron homeostasis, acting as a negative regulator of iron export?
• How does VEXAS typically present on laboratory testing?
• Cold agglutinin disease (CAD) labs typically show:
• In major surgery, what is the maintenance FVIII dose and timing to maintain levels never below 50%?
• Where is von Willebrand factor synthesized and what is its function?
• The clinical criteria for VEXAS syndrome include which manifestations?
• Factor XI deficiency has higher prevalence in which population?
• During major surgery or trauma in a patient with dysfibrinogenemia, which replacement strategy is recommended to achieve a fibrinogen level > 100 mg/dL?
• Serous atrophy can occur in which conditions?
• Regarding the H63D polymorphism in HFE, which statement is true?
• Which transporter moves Fe2+ across the apical membrane of enterocytes in the duodenum?
• Crizanlizumab is a monoclonal antibody directed against which target?
• In intravascular hemolysis, which laboratory finding is typically observed?
• Which genetic mutation is associated with increased risk for thrombosis?
• Which enzyme activates plasminogen to plasmin?
• Altuvio is the brand name for which novel FVIII product?
• Which statement best describes how vitamin K antagonists prevent coagulation?
• What cellular change is seen in PKD deficiency due to ATP depletion?
• Alpha-2 antiplasmin functions to:
• What is a ddAVP challenge in the management of von Willebrand disease?
• When might simple transfusion be used in SCD?
• Screening test for hereditary spherocytosis?
• Crizanlizumab's FDA-approved use includes reduction of vaso-occlusive crises in which patients?
• Flow cytometry with EMA dye detects what in hereditary spherocytosis?
• What is the expected Hb increase at 8-12 weeks on Mitapivat?
• HbH disease is associated with which red blood cell changes?
• Which factor does not change PT or PTT but can lead to bleeding in rare patients?
• What is the mechanism of action of heparin?
• ddAVP is considered for mild hemophilia A except in which situations?
• Which statement best describes the pathophysiology behind spherocytes in hereditary spherocytosis?
• Main way to reverse antiplatelet agents in acute bleeding?
• Peripheral blood smear finding typical of hereditary spherocytosis?
• On iron staining, which pattern is characteristic of primary hemochromatosis?
• Which situations can cause acquired Antithrombin deficiency?
• In patients with inhibitors and a titer below 5 Bethesda units, which strategy is used to bypass the inhibitor?
• What is the mechanism of action of Eculizumab?
• Which condition most strongly increases hepcidin levels?
• Fondaparinux reversal in bleeding can be achieved with:
• FLAER is used in PNH testing to bind to which structure to help detect PNH?
• Which scenarios can cause false-positive lupus anticoagulant results?
• If MCV is low in SS anemia, which coexisting conditions should be considered?
• Which option correctly lists the four strong thrombophilias?
• In interpreting FVIII results, which statement is true regarding laboratory variation?
• What is the mechanism of action of argatroban?
• In a patient on warfarin with severe bleed, 4-factor PCC dosing for INR 2-4 is:
• What is the mechanism of action of Sutimlimab?
• Hemophilia is inherited in which pattern?
• Two most common causes of spherocytes are which of the following?
• In PNH, deficiency of which surface proteins results from the PIGA mutation?
• Iron-loading anemias are predominantly associated with which mechanism?
• Which bilirubin pattern is typically seen in red blood cell membrane disorders?
• Myelodysplastic syndrome is included among iron-loading anemias due to ineffective erythropoiesis.
• One alpha gene deletion genotype (alpha,alpha/alpha,-ve) typically presents as
• Which gene is mutated in pyruvate kinase deficiency and what is the inheritance pattern?
• Which gene mutation underlies paroxysmal nocturnal hemoglobinuria (PNH)?
• What is the treatment of hepatic sequestration crisis?
• Desmopressin (DDAVP) is useful in which von Willebrand disease patients?
• CD55 is also known as which protein?
• What is the primary action of plasmin?
• In heterozygous Factor V Leiden, the relative risk for a first clot is about:
• Beta-thalassemia major is associated with which electrophoresis finding?
• Which product is absolutely contraindicated with emicizumab due to thrombotic complications?
• In VEXAS syndrome, what does the 'V' stand for?
• Which FVIII mimetic is used for congenital hemophilia A with/without inhibitors?
• In valve-related hemolysis, which statement is true?
• Which statement best describes the action of Activated Protein C on the coagulation cofactors Va and VIIIa?
• Splenic sequestration crisis is more commonly observed in which population?
• Homozygous Factor V Leiden increases risk for first VTE event by approximately how much?
• Which medication is used for chronic PNH by inhibiting C5?
• Lupus anticoagulant testing during acute thrombosis may yield positive results requiring what action?
• Zinc supplementation can contribute to microcytic anemia primarily by causing deficiency of which trace element due to competitive absorption?
• Zinc is commonly found in which products?
• Sutimlimab targets which component of the complement pathway?
• Which statement best describes the relationship between hemoglobin oxygen affinity and anemia/polycythemia as described in the material?
• Which genotype may be considered for hydroxyurea therapy if there are recurrent painful episodes?
• Compared with HbSS, which statement about SC disease is true?
• Fe2+ is oxidized to Fe3+ in order to bind to which protein for circulation?
• In dysfibrinogenemia, which statement best describes fibrinogen levels and function?
• Which statement regarding Factor XII deficiency is true?
• Blister cells are another name for which red cell abnormality?
• Name a common medication that can cause cobalamin deficiency.
• Iron loading in certain anemias is most closely linked to which pathophysiologic process?
• In SC disease, the presence of HbC leads to what hematologic pattern?
• Which of the following is an inappropriate time to transfuse in sickle cell disease?
• RiaSTAP fibrinogen concentrate is approved for which condition?
• What is the transfusion goal Hb for sickle cell patients undergoing general anesthesia?
• Is the MTHFR gene mutation a risk factor for VTE or pregnancy complications?
• Which statement best describes antithrombin's mechanism when bound to heparin?
• In early iron deficiency, what happens to reticulocyte hemoglobin content (CHr)?
• Treatment of serous atrophy / myelonecrosis includes which statement?
• First-line treatment in the algorithm for warm AIHA?
• Which staining finding is associated with G6PD deficiency during an episode?
• What does vWF ristocetin cofactor activity measure?
• What is a common management approach for Factor XI deficiency prior to minor procedures?
• Crizanlizumab is best described as which type of therapeutic agent?
• What characterizes secondary hemostasis?
• Emergent reversal of warfarin is fastest with which option?
• Bivalirudin MOA:
• Which molecule primarily initiates platelet activation in hemostasis?
• Elimination of warfarin occurs primarily through which organ?
• Which bone marrow finding is associated with copper deficiency?
• Which tests may be ordered to characterize type 2 von Willebrand disease?
• Which condition can cause reduced iron absorption due to malabsorption?
• In a patient with an acquired Factor XIII inhibitor, which laboratory finding is typically observed?
• Which disorder is characteristically associated with microcytosis?
• In mixing studies, full correction after mixing indicates which of the following explanations?
• When transfusing blood to a patient with CGD due to Mcleod phenotype, which type of blood should be used?
• Which peripheral smear finding is commonly seen during a G6PD hemolytic episode?
• What causes reticulocytes to stain differently on Wright-Giemsa stains?
• Which chronic PNH therapy is administered subcutaneously?
• Which treatment approach is appropriate for Factor VII deficiency?
• What is the best testing approach for suspected Protein C, Protein S, or Antithrombin deficiencies?
• What laboratory finding is used as a diagnostic criterion for hereditary hemochromatosis?
• An older man with macrocytic anemia and systemic inflammatory symptoms should prompt consideration of which syndrome?
• Electrophoresis pattern in S/B+ thalassemia is best described by which composition?
• Which medication can be used for PKD deficiency?
• Which is a third-line option for persistent warm AIHA?
• Which medication is particularly helpful for mucosal bleeding in hemophilia?
• What supplement should be given to all patients with chronic hemolytic disorders?
• In iron deficiency anemia, which pattern best describes ferritin, transferrin saturation, and TIBC?
• L-Glutamine is indicated for SCD in which scenario, and what is the dosing?
• Recurrent VTE risk for FVL: heterozygous 1.5x; homozygous higher
• FLAER stands for which of the following?
• Which mixing study result indicates lupus anticoagulant?
• In normal adults, what percentage does HbA constitute?
• What is the reversal agent for unfractionated heparin and low molecular weight heparin?
• What is the treatment goal for factor levels in severe bleeds in hemophilia?
• Which intraoperative management is recommended for sickle cell patients undergoing general anesthesia?
• Which statement best distinguishes Bo thalassemia from B+ thalassemia?
• Which statement correctly differentiates sickle cell trait from S/B+ thalassemia?
• Which laboratory pattern is typical of dysfibrinogenemia?
• Which statement best describes type II N von Willebrand disease?
• What is the storage form of iron within cells?
• Antidote to factor Xa inhibitors?
• Which mechanism contributes to thrombosis risk in hemolytic disorders?
• Indication for intravenous iron therapy includes:
• Emicizumab is used for what purpose in hemophilia?
• Which of the following can cause acquired Protein C deficiency?
• Start with this test if concerned for hereditary spherocytosis; which combination increases sensitivity?
• Globin gene loci: which statement is true?
• What is the role of CD55 in PNH?
• Which statement is true about the shared adverse effects of Deferiprone and Deferasirox?
• What testing practice should be avoided when evaluating for hereditary Protein C, Protein S, or Antithrombin deficiencies?
• What are Weibel-Palade bodies?
• Fondaparinux reversal options in bleeding can be achieved with:
• Which statement about FVIII results is true?
• Free hemoglobin released from lysed red blood cells scavenges nitric oxide; this contributes to which complication in sickle cell disease?
• HbF is composed of which globin chains?
• If Protein S antigen is normal but functional deficiency is suspected, which test can be informative?
• Khorana score of 3 is associated with approximately what annual risk of thrombosis?
• PNH is best described as which of the following?
• What is the approximate daily insensible iron loss in an adult?
• In hereditary hemochromatosis, hyperferritinemia is defined as ferritin exceeding which value?
• Which agent is a C3 inhibitor used for chronic PNH?
• What is the best preventive measure for acute chest syndrome in sickle cell disease?