Anemia (Iron Deficiency, Hemolytic, Sickle Cell, Thalassemia) MCQs

Medicine · 88 free questions with answers & explanations.

  1. A 30-year-old woman from West Africa presents with episodes of severe bony pain (hand-foot syndrome), recurrent fever, and jaundice since childhood. Hemoglobin is 7.5 g/dL, MCV 82 fL, reticulocyte count 12%, peripheral smear shows sickle-shaped cells and Howell-Jolly bodies. Which of the following treatments has been shown to reduce the frequency of painful crises in sickle cell disease?
  2. A 55-year-old woman presents with fatigue and pallor. CBC shows Hb 8.2 g/dL, MCV 68 fL, MCH 20 pg, RDW 18%. Serum ferritin is 6 ng/mL, serum iron 38 μg/dL, TIBC 480 μg/dL (elevated). Peripheral smear shows hypochromic microcytic cells with pencil cells. Which is the single most important next step before initiating iron therapy?
  3. A 32-year-old African-American woman with sickle cell disease presents with acute onset of severe left hip pain, fever, and inability to bear weight. WBC is 18,000/μL. MRI hip shows areas of low T1 and high T2 signal in the femoral head without contrast enhancement. Which complication is most likely?
  4. A 28-year-old woman of Mediterranean origin is found to have Hb 9.2 g/dL, MCV 62 fL, MCH 18 pg. HPLC shows HbA2 5.8%, HbF 3.2%, HbA 91%. Iron studies are normal. Her husband has HbA2 3.8% on HPLC. What is the risk to their offspring of having transfusion-dependent thalassemia (beta-thalassemia major)?
  5. A 22-year-old man with sickle cell disease (HbSS) presents with acute chest syndrome (ACS): fever, chest pain, new pulmonary infiltrate, and oxygen saturation 89% on room air. Which therapeutic intervention has been shown to reduce the severity and frequency of ACS episodes when used as chronic disease-modifying therapy?
  6. A 32-year-old woman of Mediterranean origin has haemolytic anaemia with haemoglobin 9.2 g/dL. Blood film shows target cells, hypochromic microcytic red cells, and no spherocytes. HPLC shows HbA 72%, HbA2 5.8%, HbF 1.5%. Her husband is found to have HbA 93%, HbA2 5.5%, HbF 1.5%. What is the risk to their offspring of having transfusion-dependent thalassaemia?
  7. In autoimmune haemolytic anaemia (AIHA), warm AIHA is caused by IgG antibodies reactive at 37°C, primarily cleared by splenic macrophages. Cold AIHA (cold agglutinin disease) is caused by IgM antibodies active at 4°C. Which test distinguishes warm from cold AIHA on direct antiglobulin test (DAT)?
  8. Paroxysmal nocturnal haemoglobinuria (PNH) results from a somatic PIG-A mutation in a haemopoietic stem cell, leading to deficiency of GPI-anchored proteins. Which two proteins are most critical for complement regulation, and their absence leads to intravascular haemolysis?
  9. In sickle cell disease, hydroxyurea reduces HbS polymerisation by increasing HbF. The molecular mechanism by which HbF inhibits sickling is:
  10. A 28-year-old woman with haemolytic anaemia, splenomegaly, and pigment gallstones has a direct Coombs test (DAT) negative result. Peripheral smear shows spherocytes. Osmotic fragility test is markedly increased. The MOST likely diagnosis and its inheritance pattern is:
  11. A 22-year-old Afro-Caribbean man with known sickle cell disease presents with acute onset severe pain in the chest, fever, hypoxia (SpO₂ 89%), and new pulmonary infiltrate on CXR. The MOST urgent specific therapeutic intervention (beyond analgesia, O₂, and IV fluids) is:
  12. In beta-thalassaemia major, the primary mechanism of anaemia is:
  13. A 28-year-old woman with sickle cell disease (HbSS) presents with a painful vaso-occlusive crisis. Her Hb is 7.8 g/dL (baseline 8.2). She is on hydroxyurea. Based on the HOPE trial (2019, NEJM), which newer agent approved for prevention of vaso-occlusive episodes in sickle cell disease targets P-selectin to reduce sickling-related inflammation?
  14. A 45-year-old woman on methyldopa for hypertension develops fatigue and jaundice. CBC shows Hb 8.2 g/dL, MCV 102 fL, reticulocyte count 9.8%, indirect bilirubin 3.4 mg/dL. Direct Coombs test is strongly positive (IgG warm antibodies). The mechanism by which methyldopa induces hemolytic anemia is:
  15. A 30-year-old woman presents with severe hemolytic anemia (Hb 5.8 g/dL), thrombocytopenia, renal failure, and confusion after a diarrheal illness. Peripheral smear shows schistocytes. ADAMTS13 activity is 80% (normal). Stool cultures grow Shiga toxin-producing E. coli (STEC, O157:H7). What is the definitive treatment?
  16. In beta-thalassemia major, the major cause of morbidity in adequately transfused patients (Hb maintained >9–10 g/dL) is iron overload from transfusion hemosiderosis. Which organ is MOST critically monitored using T2* cardiac MRI?
  17. A 40-year-old man with sickle cell anaemia (HbSS) presents with severe acute chest syndrome: SpO₂ 88%, bilateral infiltrates, fever, chest pain, and Hb 5.8 g/dL (baseline 8 g/dL). Which is the most critical immediate intervention?
  18. A 25-year-old woman of Mediterranean origin has a haemoglobin of 9 g/dL, MCV 62 fL, MCH 19 pg, and Hb electrophoresis showing HbA2 5.8%, HbF 3.2%, HbA 91%. Iron studies are normal. Which is the diagnosis and what additional test confirms carrier status in her partner for genetic counselling?
  19. A 35-year-old woman with homozygous sickle cell disease (HbSS) has been admitted 4 times in the past year for vaso-occlusive crises. Her hydroxyurea therapy was stopped 6 months ago due to leukopenia. Her sibling is an HLA-matched donor. According to ASH 2020 SCD guidelines, the most appropriate escalation of therapy is:
  20. A 28-year-old woman presents with episodic dark urine, especially in the morning, and jaundice. CBC: Hb 8.1 g/dL, reticulocyte count 8%, LDH 850 U/L, haptoglobin undetectable. Direct Coombs test is negative. Flow cytometry shows absence of CD55 and CD59 on erythrocytes (>50% affected). The first-line treatment for this patient to prevent thrombosis and hemolysis is:
  21. A 35-year-old African-American man presents with severe back and chest pain after a long-haul flight. His known sickle cell disease is usually well-controlled. Hemoglobin drops from 8.5 to 6.2 g/dL. LDH rises to 980 U/L, indirect bilirubin increases, and he develops oxygen saturation of 88%. CXR shows new bilateral infiltrates. The diagnosis and immediate intervention priority is:
  22. A 4-year-old boy of Mediterranean descent has hemoglobin 5.8 g/dL, MCV 58 fL, MCH 16 pg. Peripheral smear shows target cells, anisopoikilocytosis, and nucleated red cells. HPLC shows HbA absent, HbF 93%, HbA2 5.2%. The diagnosis is:
  23. A 28-year-old Nigerian man with known sickle cell anaemia (HbSS) presents with acute severe chest pain, fever (38.9°C), worsening hypoxia (SpO2 88% on room air), and a new bilateral pulmonary infiltrate on CXR. His haematocrit is 18% (baseline 24%). What is the diagnosis and most critical immediate therapeutic intervention?
  24. A 40-year-old woman of Mediterranean descent with beta-thalassaemia trait (HbA2 5.8%, mild microcytosis, Hb 11.2 g/dL) is referred for counselling. Her husband also has beta-thalassaemia trait confirmed on HPLC. What is the risk that their child will have beta-thalassaemia major, and what prenatal diagnosis option is available at 11–14 weeks of gestation?
  25. A 25-year-old woman from West Africa has haemoglobin electrophoresis showing HbS 80%, HbF 18%, HbA 0%, HbA2 2%. She presents in a sickle cell crisis. Concerning hydroxyurea therapy for sickle cell disease, which statement is MOST accurate?
  26. A 35-year-old man has autoimmune haemolytic anaemia (AIHA) with Hb 7.2 g/dL, reticulocytosis, positive direct antiglobulin test (DAT/Coombs) with IgG warm antibody. He has failed prednisolone 1 mg/kg for 6 weeks (Hb stable at 7–7.5 g/dL). According to current ASH 2021 AIHA guidelines, the recommended second-line therapy is:
  27. A 42-year-old woman presents with fatigue, haemoglobinuria in the morning, pancytopaenia, and a positive flow cytometry showing GPI-anchor deficient RBCs (PNH clone 38%). Which complication is the leading cause of death in paroxysmal nocturnal haemoglobinuria (PNH)?
  28. Hydroxyurea is the standard disease-modifying therapy for sickle cell disease (SCD). Which primary mechanism explains its therapeutic benefit in SCD?
  29. A 35-year-old woman presents with hemolytic anemia, thrombocytopenia, and renal failure following a gastroenteritis. Blood film shows schistocytes. Serum LDH is markedly elevated. ADAMTS13 activity returns at 75% (normal). Stool culture grows E. coli O157:H7. The diagnosis is Shiga toxin-associated HUS. Unlike TTP, ADAMTS13 in typical HUS is:
  30. A 22-year-old man with sickle cell disease (HbSS) presents with acute severe chest pain, fever 38.8°C, and oxygen saturation 91% on room air. CXR shows new bilateral lower zone infiltrates. This is acute chest syndrome (ACS). In addition to oxygen, analgesics, and antibiotics, which intervention has the most immediate impact on severity if the patient continues to deteriorate?
  31. A 30-year-old man with beta-thalassemia major on regular blood transfusion has serum ferritin of 4,500 ng/mL and liver iron concentration (LIC) of 18 mg/g dry weight on MRI. The PREFERRED oral iron chelation agent with the most extensive evidence and convenient once-daily oral dosing is:
  32. A 25-year-old woman of Mediterranean descent has Hb 7.2 g/dL, MCV 62 fL, target cells on peripheral smear, and HbA2 of 5.8% on HPLC. Serum iron and ferritin are normal. What is the diagnosis?
  33. A 22-year-old man with sickle cell disease presents with severe chest pain, fever, tachycardia, and worsening hypoxia with new pulmonary infiltrate on CXR. His SpO2 has dropped from 98% to 88% over 12 hours. What is this complication and the most important specific treatment?
  34. A 25-year-old woman with sickle cell disease (HbSS) presents with severe bone pain and fever. She is managed with IV fluids, opioid analgesia and antibiotics. Over the next 48 hours she develops progressive hypoxia (SpO₂ 84% on room air), bilateral pulmonary infiltrates and falling Hb from 8.5 to 5.8 g/dL. This complication is best managed with:
  35. A 45-year-old man is found to have Hb 7.2 g/dL, MCV 68 fL. Serum iron 8 µmol/L, TIBC 72 µmol/L (elevated), serum ferritin 6 ng/mL. He has no overt GI bleeding, melena or haematuria. Which investigation is mandatory as the next step in a middle-aged male with unexplained iron deficiency anaemia?
  36. A 30-year-old man with known sickle cell disease (HbSS) presents with sudden onset right upper quadrant pain, jaundice, worsening anemia (Hb dropped from 8 to 5 g/dL), and a tender rapidly enlarging liver. Reticulocyte count is 18%. The most likely diagnosis is:
  37. A 25-year-old woman from the Mediterranean region has microcytic hypochromic anemia (Hb 8.5 g/dL, MCV 62 fL). Serum iron and TIBC are normal. Serum ferritin is normal. HbA2 is 5.8% on HPLC. Which diagnosis is confirmed?
  38. A 22-year-old woman presents with recurrent jaundice, splenomegaly, and dark urine. Peripheral blood smear shows spherocytes. Direct Coombs test is negative. Osmotic fragility is increased. Her father has similar history. Which enzyme assay is MOST useful to confirm hereditary spherocytosis if clinical and laboratory features are classic but osmotic fragility is borderline?
  39. A 25-year-old woman of Mediterranean origin presents with mild anaemia (Hb 10 g/dL), splenomegaly, and jaundice. Her MCV is 65 fL and MCH is 20 pg. HPLC shows HbA2 of 5.8% (normal < 3.5%). Serum ferritin is 120 ng/mL. The diagnosis and the key counselling point are:
  40. A 30-year-old man with known sickle cell disease presents with severe acute chest pain, fever, and new bilateral pulmonary infiltrates on CXR. SpO2 is 90% on room air. WBC is 22,000/µL. The diagnosis and most critical acute intervention are:
  41. A 40-year-old man presents with episodic haemoglobinuria (dark urine especially in the morning), anaemia (Hb 8.5 g/dL), thrombocytopenia, and a recent history of Budd-Chiari syndrome. Flow cytometry of peripheral blood shows a deficiency of CD55 and CD59 on red blood cells and granulocytes. The drug that has transformed outcomes in this condition by blocking complement C5 is:
  42. A 28-year-old woman presents with fatigue and pallor. CBC shows Hb 7.2 g/dL, MCV 62 fL, MCH 19 pg, RBC 5.8 million/μL. Peripheral smear shows target cells and microcytes. Serum ferritin is 68 μg/L (normal 15–150). Hb electrophoresis shows HbA2 5.8%, HbF 3.2%, HbA 91%. What is the MOST likely diagnosis?
  43. A 22-year-old man with known sickle cell disease presents with sudden onset left hip pain and limitation of movement, which has been worsening over the past 3 months. Plain X-ray shows flattening of the femoral head. Which is the MOST likely long-term complication responsible and the underlying mechanism?
  44. A 55-year-old woman presents with fatigue, pallor, and macrocytosis (MCV 118 fL). Serum vitamin B12 is 88 pg/mL (normal 200–900). Gastric parietal cell antibodies are positive. Bone marrow shows hypersegmented neutrophils and megaloblastic changes. Which laboratory parameter confirms impaired B12 metabolism at the cellular level even when total B12 is borderline low?
  45. A 22-year-old man with sickle cell anaemia presents with severe chest pain, fever, tachypnoea, and new bilateral pulmonary infiltrates. SpO₂ is 88% on room air. What is this acute complication and what is its most critical immediate intervention?
  46. A 19-year-old woman with thalassaemia major on regular transfusions has serum ferritin of 3800 ng/mL and hepatic iron concentration of 9 mg/g dry weight on MRI T2* (normal <1.8). She has poor compliance with deferoxamine injections. Which oral iron chelator is first-line for this level of iron overload?
  47. A 32-year-old man with sickle cell disease presents with priapism lasting 5 hours. Initial management includes hydration and analgesia. After 4 hours with no resolution (total duration now 9 hours), according to ASH 2020 sickle cell disease guidelines, the next specific intervention is:
  48. A 4-year-old girl of Mediterranean descent presents with severe pallor and hepatosplenomegaly. Peripheral smear shows target cells, nucleated RBCs, and hypochromia. Hb electrophoresis shows HbF 92%, HbA2 5%, no HbA. The diagnosis is:
  49. A 30-year-old man presents with episodic dark urine, haemolytic anaemia, and thrombocytopenia. He reports the haemolytic episodes are worse in the morning. Flow cytometry of peripheral blood shows absent CD55 and CD59 on red blood cells and granulocytes. The treatment that addresses the underlying complement dysregulation is:
  50. A 28-year-old woman has haemoglobin 7.2 g/dL, MCV 68 fL, serum ferritin 8 ng/mL, TIBC 450 µg/dL. She is prescribed oral ferrous sulphate 325 mg TDS. After 4 weeks, haemoglobin rises to 8.8 g/dL (increase of 1.6 g/dL). For how long should iron therapy be continued after haemoglobin normalises?
  51. A 22-year-old man with sickle cell disease presents with acute severe chest pain, fever, tachypnoea, and new bilateral infiltrates on chest X-ray with SpO₂ 88% on room air. Which is the single most important immediate intervention for this acute chest syndrome episode?
  52. A 25-year-old woman with sickle cell disease (HbSS) has 4 vaso-occlusive crises per year and is not on disease-modifying therapy. Which agent, approved by FDA for adults with SCA to reduce vaso-occlusive crises, works by inhibiting P-selectin?
  53. A 30-year-old man presents with haemolytic anaemia (Hb 7.2 g/dL), jaundice, and episodic dark urine after infections and medications. G6PD assay during crisis shows borderline low value. What is the most appropriate next step to confirm G6PD deficiency?
  54. A 30-year-old woman with sickle cell anaemia presents with acute severe pain in her chest, back, and limbs. Temperature is 38.8°C, SpO2 90%, CXR shows new bilateral pulmonary infiltrates, and WBC is 18,000/μL. This is her third episode with a pulmonary infiltrate in 2 years. What is the most critical immediate intervention and long-term disease-modifying therapy?
  55. A 5-year-old boy presents with severe anaemia (Hb 5.2 g/dL), microcytosis, target cells, hepatosplenomegaly, and a family history of blood transfusions. HbA2 is 3.8% and HbF is 65%. The most likely diagnosis is:
  56. A 24-year-old African American man presents with episodic bone pain crises, dactylitis in childhood, and a hemoglobin of 7.8 g/dL. Peripheral smear shows sickle cells. Hemoglobin electrophoresis shows HbS 90%, HbF 8%, HbA2 2.4%. He has had 4 pain crises requiring hospitalization in the past year. According to current ASH guidelines, which disease-modifying therapy should be offered?
  57. A 68-year-old man with CKD stage 4 (eGFR 19 mL/min) has hemoglobin 8.4 g/dL, serum ferritin 95 ng/mL, and transferrin saturation 18%. Reticulocyte count is low. Erythropoietin level is inappropriately low for the degree of anemia. KDIGO 2023 guidelines recommend initiating erythropoiesis-stimulating agent (ESA) therapy when:
  58. A 42-year-old woman presents with anemia (Hb 7.2 g/dL), indirect hyperbilirubinemia, splenomegaly, and spherocytes on peripheral smear. Direct Coombs test is positive (IgG-coated RBCs). Cold agglutinin titer is negative. Which condition most likely underlies this warm autoimmune hemolytic anemia?
  59. A 35-year-old woman of Mediterranean origin is found to have haemoglobin 9.2 g/dL, MCV 62 fL, low MCH, target cells, and HbA2 4.8% on HPLC. Serum ferritin is 18 ng/mL. Which is the correct interpretation?
  60. A 42-year-old man presents with haemolytic anaemia, Coombs-negative (direct antiglobulin test negative), episodes of dark urine especially in the morning, and flow cytometry showing absence of CD55 and CD59 on red cell surface. What complication is he at highest risk of, and what is the current treatment?
  61. A 28-year-old man with sickle cell disease presents with severe acute chest syndrome (bilateral infiltrates, oxygen saturation 88% on room air, fever, and chest pain). His haemoglobin is 6.2 g/dL and WBC 22,000. Which is the most urgent disease-specific intervention?
  62. A 24-year-old woman has haemolytic anaemia. Direct antiglobulin (Coombs) test is negative. Blood film shows fragmented red cells (schistocytes), thrombocytopenia, elevated LDH, low haptoglobin, and haemoglobinuria. Serum creatinine is normal. ADAMTS13 activity is 8% (severely deficient). What is the diagnosis and first-line treatment?
  63. A 20-year-old man from Nigeria has sickle cell anaemia (HbSS). He presents with bilateral hip pain for 3 months, worsening on weight-bearing. X-ray shows subchondral lucency and 'crescent sign' in the femoral head. MRI confirms the diagnosis. What is the most likely diagnosis and the mainstay of medical therapy for sickle cell disease that reduces vaso-occlusive crises?
  64. A 35-year-old man presents with episodic painless dark urine, fatigue, and venous thrombosis of hepatic veins (Budd-Chiari syndrome). CBC shows anaemia, reticulocytosis, and pancytopenia. Flow cytometry of peripheral blood erythrocytes shows absence of CD55 and CD59. The MOST appropriate treatment for haematological control is:
  65. A 24-year-old woman with sickle cell anaemia (HbSS) is initiated on hydroxyurea. The PRIMARY mechanism by which hydroxyurea reduces sickle cell crises is:
  66. A 28-year-old woman from the Mediterranean has moderate anaemia (Hb 8.1 g/dL), target cells, microcytosis, splenomegaly, and Hb electrophoresis showing HbA 70%, HbA₂ 5.8%, HbF 3%. Which is the diagnosis and what does the elevated HbA₂ indicate?
  67. A 22-year-old woman from Gujarat has microcytic hypochromic anaemia (Hb 8.2 g/dL, MCV 62 fL). Serum iron is 12 µmol/L, TIBC 28 µmol/L (low-normal), ferritin 55 ng/mL (normal). Haemoglobin electrophoresis: HbA2 2.5%, HbF 4%, HbA 93.5%. HPLC shows a borderline HbA2. The MOST important next diagnostic test to confirm a diagnosis relevant to genetic counselling is:
  68. A 30-year-old man with homozygous sickle cell disease presents with acute sickle cell crisis. He was recently started on hydroxyurea 15 mg/kg/day, which is titrated up. Hydroxyurea reduces vaso-occlusive episodes primarily by which mechanism?
  69. A 22-year-old woman with sickle cell disease (HbSS) develops sudden severe chest pain, dyspnea, fever (38.5°C), WBC 18,000, SpO2 88% on room air, and a new bilateral infiltrate on CXR. This is her third episode of acute chest syndrome (ACS) in 2 years. What is the most important long-term disease-modifying intervention to prevent recurrent ACS?
  70. A 45-year-old man presents with a hemoglobin of 7.2 g/dL, MCV 72 fL, raised LDH, reduced haptoglobin, raised indirect bilirubin, peripheral smear showing schistocytes (3%), and a direct Coombs test that is negative. ADAMTS13 activity is <5%. Platelets are 28,000/µL. Creatinine 2.8 mg/dL. What is the immediate treatment?
  71. A 35-year-old woman has haemolytic anaemia, thrombocytopenia, and repeated episodes of dark urine at night. Flow cytometry of peripheral blood shows >50% loss of CD55 and CD59 on red blood cells and granulocytes. The diagnosis is paroxysmal nocturnal haemoglobinuria (PNH). What is the MECHANISM of haemolysis and current standard of care?
  72. A 22-year-old patient with homozygous sickle cell disease (HbSS) is on hydroxyurea but has two acute painful crises requiring hospitalisation in the past 12 months. Which newer therapy, approved by FDA in 2019, works by activating pyruvate kinase in red blood cells to reduce HbS polymerisation by increasing 2,3-BPG consumption?
  73. In a patient with sickle cell disease (SCD), hydroxyurea reduces sickle crisis frequency by which mechanism?
  74. Paroxysmal nocturnal haemoglobinuria (PNH) results from a somatic mutation in PIGA gene causing absence of which cell surface anchor proteins that protect RBCs from complement?
  75. In thalassaemia intermedia (non-transfusion-dependent thalassaemia, NTDT), a major complication unique to this group compared to transfusion-dependent thalassaemia major is:
  76. A 24-year-old woman with sickle cell anaemia (HbSS) has had three painful crises in the past year. She is not on any disease-modifying therapy. Her current haemoglobin is 7.1 g/dL and fetal haemoglobin (HbF) is 4%. According to the ASH 2020 guidelines, which intervention is recommended as first-line disease-modifying therapy to reduce vaso-occlusive crises?
  77. A 30-year-old man is found to have a microcytic, hypochromic anaemia with haemoglobin 9.2 g/dL. Serum ferritin is 560 µg/L and transferrin saturation is 72%. Peripheral smear shows target cells. HPLC reveals HbA2 3.8%, HbF 2.1%, HbA 94.1%. Family history is positive for anaemia. What is the most likely diagnosis?
  78. A 28-year-old woman with sickle cell disease (HbSS) has frequent vaso-occlusive crises (4 in the past year) and a recent acute chest syndrome episode. She is not on hydroxyurea. A comprehensive disease-modifying strategy is being planned. The HOPE trial specifically evaluated which agent for adults with sickle cell disease experiencing ≥ 2 vaso-occlusive crises/year?
  79. A 35-year-old woman has autoimmune haemolytic anaemia (AIHA). Direct Coombs test is strongly positive with warm IgG antibodies, Hb 6.2 g/dL, reticulocyte count 12%, elevated indirect bilirubin, and spherocytes on blood film. She fails prednisolone 1 mg/kg/day after 4 weeks. The CARDINAL trial evaluated which agent for warm AIHA and showed clinically meaningful durable responses?
  80. A 30-year-old man with sickle cell disease presents with priapism lasting 4 hours. Which immediate management is MOST appropriate?
  81. In paroxysmal nocturnal hemoglobinuria (PNH), the deficiency of GPI-anchored proteins CD55 and CD59 leads to complement-mediated hemolysis. Eculizumab targets which complement component?
  82. A 35-year-old man with sickle cell disease presents with severe bone pain (vaso-occlusive crisis). He has been on hydroxyurea 35 mg/kg/day for 18 months with suboptimal response (HbF 8%, still having 4 crises/year). What is the mechanism by which hydroxyurea reduces sickling, and what alternative newer therapy has demonstrated reduction in vaso-occlusive crises through P-selectin inhibition?
  83. A 22-year-old woman is found to have hypochromic microcytic anaemia (Hb 8.2 g/dL, MCV 62 fL). Serum ferritin is 85 ng/mL, serum iron 90 µg/dL, TIBC 280 µg/dL. Red cell distribution width (RDW) is 13.2% (normal). Peripheral smear shows target cells and basophilic stippling. HPLC (haemoglobin electrophoresis) shows HbA 93%, HbA2 2.1%, HbF 1.4%. What is the most likely diagnosis?
  84. A 30-year-old African man presents with painful priapism and right hypochondrial pain during a febrile illness. Peripheral smear shows sickle cells. Haematocrit is 22%. He is on hydroxyurea. Which is the MOST appropriate ACUTE management for his vaso-occlusive crisis?
  85. A 22-year-old woman has a haemolytic anaemia with negative Coombs test. Peripheral smear shows 'bite cells' and 'blister cells'. Heinz body preparation is positive. G6PD level is normal (measured during an acute haemolytic episode). Which is the MOST likely explanation for the normal G6PD level?
  86. Beta-thalassaemia major patient on regular transfusions presents with bronze skin, elevated ferritin (4200 ng/mL), and cardiac T2* on MRI of 9 ms (normal >20 ms). Which is the MOST appropriate chelation therapy?
  87. A 30-year-old woman with sickle cell disease presents with acute severe chest syndrome — fever, pleuritic chest pain, new pulmonary infiltrate, and SpO2 88%. What is the MOST appropriate treatment?
  88. A 40-year-old woman has microangiopathic haemolytic anaemia (MAHA), thrombocytopenia (platelet 18,000), normal PT/aPTT, elevated LDH, and elevated creatinine 2.6 mg/dL. Peripheral smear shows schistocytes. ADAMTS13 activity is 8% (normal >67%). Which diagnosis is confirmed and what is the FIRST treatment?
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