Hematology — MCQs
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A 3-year-old presents with a history of pallor, irritability, and fatigue. Physical examination reveals mucosal pallor and a systolic murmur. Laboratory results show hemoglobin of 6.5 g/dL, mean corpuscular volume of 55 fL, and reticulocyte count of 1%. Analyze and determine the likely cause of these findings.
A 5-year-old boy with sickle cell disease presents with acute chest syndrome. His SpO2 is 88% on room air, with elevated WBC and LDH levels, and the chest X-ray shows new infiltrates. Assess and choose the best combination of treatments.
A child with frequent nosebleeds and a family history of similar symptoms is being evaluated for a possible bleeding disorder. Which test is most critical for diagnosing a likely inherited condition?
A 5-year-old girl presents with pallor, fatigue, and a new heart murmur. Laboratory tests reveal hemolytic anemia and reticulocytosis. What is the most likely diagnosis?
What is the first-line treatment for a child under 2 years of age with iron deficiency anemia?
A 5-year-old boy presents with abdominal pain, bloody diarrhea, and lethargy. Laboratory findings reveal microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. What is the most likely diagnosis?
A 12-year-old girl presents with fatigue, petechiae, and a nosebleed that won’t stop. Physical examination reveals pallor, splenomegaly, and ecchymoses. Laboratory results show hemoglobin of 7.5 g/dL, platelets of 20,000/µL, and white blood cells of 1,000/µL. What is the most likely diagnosis and initial management?
Which of the following is a typical hemoglobin level for term infants?
What is the mean hemoglobin level in a 1-year-old child (in g/dl)?
A child presents with recurrent chest infections and abdominal pain. There is a history of 1 blood transfusion in the past. On examination, he had icterus and mild splenomegaly. Electrophoresis shows increased HbA2, HbF, and S spike. What is the likely diagnosis?
Hematology Indian Medical PG Practice Questions and MCQs
Question 181: A 3-year-old presents with a history of pallor, irritability, and fatigue. Physical examination reveals mucosal pallor and a systolic murmur. Laboratory results show hemoglobin of 6.5 g/dL, mean corpuscular volume of 55 fL, and reticulocyte count of 1%. Analyze and determine the likely cause of these findings.
- A. Iron deficiency anemia; treatment with iron supplements (Correct Answer)
- B. β-thalassemia minor; regular monitoring
- C. Sickle cell anemia; supportive care
- D. G6PD deficiency; management of oxidative stress
Explanation: ***Iron deficiency anemia; treatment with iron supplements*** - The patient presents with **pallor**, **fatigue**, and **irritability**, classical symptoms of anemia. The **hemoglobin of 6.5 g/dL** confirms severe anemia, and the **MCV of 55 fL** indicates microcytic anemia. - A **reticulocyte count of 1%** suggests an inadequate red blood cell production response to the anemia, which is consistent with **iron deficiency** as the bone marrow lacks the necessary iron for hemoglobin synthesis and erythropoiesis. This is the **most common cause of microcytic anemia in children**, especially in the 1-3 year age group. - The **low reticulocyte count** is key—it indicates a **production defect** rather than hemolysis, pointing directly to nutritional deficiency (iron) or chronic disease. **Iron supplementation** (oral ferrous sulfate 3-6 mg/kg/day of elemental iron) is the appropriate treatment. *β-thalassemia minor; regular monitoring* - While **β-thalassemia minor** can cause **microcytic anemia** (low MCV), patients are usually asymptomatic or have mild anemia (Hb rarely as low as 6.5 g/dL) and often have a normal or elevated reticulocyte count, unlike this patient's low count. - The severity of symptoms and the very low hemoglobin level make β-thalassemia minor less likely as the primary cause here. Additionally, thalassemia minor typically shows **elevated RBC count** with disproportionately low MCV. *Sickle cell anemia; supportive care* - **Sickle cell anemia** typically causes **normocytic** or **macrocytic anemia** with a characteristically high **reticulocyte count** (often 5-15%) due to chronic hemolysis, which contradicts the presented microcytic anemia and low reticulocyte count. - Children with sickle cell anemia also experience vaso-occlusive crises and pain, which are not mentioned in this presentation. *G6PD deficiency; management of oxidative stress* - **G6PD deficiency** leads to episodic **hemolytic anemia** triggered by oxidative stressors, resulting in an acute drop in hemoglobin and an **elevated reticulocyte count** (3-10% or higher during hemolytic episodes), not chronic microcytic anemia with a low reticulocyte count. - The red blood cell morphology would typically show **Heinz bodies** and **bite cells** during hemolytic episodes, which are not suggested by the lab results provided. The anemia in G6PD deficiency is typically **normocytic**, not microcytic.
Question 182: A 5-year-old boy with sickle cell disease presents with acute chest syndrome. His SpO2 is 88% on room air, with elevated WBC and LDH levels, and the chest X-ray shows new infiltrates. Assess and choose the best combination of treatments.
- A. Oxygen, antibiotics, blood transfusion (Correct Answer)
- B. Bronchodilators, corticosteroids, IV fluids
- C. Exchange transfusion, anticoagulation
- D. Hydroxyurea, IV fluids, pain management
Explanation: ***Oxygen, antibiotics, blood transfusion*** - **Oxygen** is crucial for treating **hypoxemia** (SpO2 88%) in acute chest syndrome (ACS) to improve tissue oxygenation and reduce sickling. - **Broad-spectrum antibiotics** are essential due to the high likelihood of bacterial infection as a trigger for ACS, given the **elevated WBC** and risk of atypical organisms. Exchange transfusion (or simple transfusion) may be required in severe cases or to rapidly increase oxygen-carrying capacity. *Bronchodilators, corticosteroids, IV fluids* - **Bronchodilators** and **corticosteroids** are typically used for **asthma** or other obstructive airway diseases, which are not the primary pathology in ACS, although a component of bronchospasm can exist. - While **IV fluids** are important for hydration in sickle cell disease, this combination does not address the core issues of infection, sickling, and severe hypoxemia. *Exchange transfusion, anticoagulation* - **Exchange transfusion** is an important treatment for severe ACS, but it is often combined with **oxygen and antibiotics** as initial stabilizers, not used in isolation. - **Anticoagulation** is generally not indicated in the routine management of ACS unless there is a confirmed thrombotic event, as the pathophysiology is primarily vaso-occlusion and inflammation, not thrombosis requiring anticoagulation. *Hydroxyurea, IV fluids, pain management* - **Hydroxyurea** is a long-term preventative medication for sickle cell disease, not an acute treatment for ACS. - **IV fluids** and **pain management** are supportive measures, but they do not directly address the severe hypoxemia, infection, or the acute sickling crisis in the lungs, which require more aggressive intervention.
Question 183: A child with frequent nosebleeds and a family history of similar symptoms is being evaluated for a possible bleeding disorder. Which test is most critical for diagnosing a likely inherited condition?
- A. Complete blood count
- B. Platelet function tests
- C. Genetic testing for clotting factor mutations
- D. Coagulation profile (Correct Answer)
Explanation: ***Coagulation profile*** - A **coagulation profile**, including **prothrombin time (PT)**, **activated partial thromboplastin time (aPTT)**, and **fibrinogen levels**, is critical for evaluating the **extrinsic, intrinsic, and common pathways** of coagulation. - This helps identify potential deficiencies in **clotting factors**, which are often the cause of **inherited bleeding disorders** like **hemophilia** or **von Willebrand disease**. *Complete blood count* - A **CBC** provides information about **red blood cells**, **white blood cells**, and **platelet count**. - While it can reveal **anemia** from chronic bleeding or **thrombocytopenia**, it does not assess the **function of clotting factors** or the overall coagulation cascade. *Platelet function tests* - **Platelet function tests** (e.g., **platelet aggregation studies**) evaluate how well platelets adhere and aggregate to form a primary plug. - While important for disorders like **Glanzmann thrombasthenia** or **Bernard-Soulier syndrome**, a more general assessment of the **coagulation cascade** (coagulation profile) is typically the initial critical step in diagnosing the spectrum of inherited bleeding disorders when the type is unknown. *Genetic testing for clotting factor mutations* - **Genetic testing** is highly specific and confirms the exact **genetic mutation** responsible for an inherited bleeding disorder. - However, it is usually performed **after abnormalities are identified** in a **coagulation profile** or other initial screening tests, as it guides which specific genes to test for.
Question 184: A 5-year-old girl presents with pallor, fatigue, and a new heart murmur. Laboratory tests reveal hemolytic anemia and reticulocytosis. What is the most likely diagnosis?
- A. Iron deficiency anemia
- B. Sickle cell disease
- C. G6PD deficiency
- D. Hereditary spherocytosis (Correct Answer)
Explanation: ***Hereditary spherocytosis*** - **Pallor**, **fatigue**, and a new **heart murmur** suggesting anemia, coupled with laboratory findings of **hemolytic anemia** and **reticulocytosis**, are hallmark signs. - The presence of **spherocytes** on a peripheral blood smear and a positive **osmotic fragility test** would further confirm this diagnosis. *Iron deficiency anemia* - While it causes **pallor** and **fatigue**, it is characterized by **microcytic, hypochromic** red blood cells, not hemolytic anemia or reticulocytosis. - **Reticulocytosis** typically occurs only after iron supplementation as the bone marrow begins to produce new red blood cells. *Sickle cell disease* - Presents with significant **pain crises**, **vaso-occlusive events**, and organ damage, not just pallor and a murmur. - Characterized by **sickled red blood cells** on a peripheral smear, and a **hemoglobin electrophoresis** would confirm the presence of **HbS**. *G6PD deficiency* - An X-linked recessive disorder causing episodic **hemolytic anemia** when exposed to certain **oxidative stressors** like fava beans or certain medications. - While it causes hemolytic anemia and reticulocytosis, the episodic nature and trigger exposure are usually key distinguishing features, and there's no mention of a trigger here.
Question 185: What is the first-line treatment for a child under 2 years of age with iron deficiency anemia?
- A. Oral iron supplementation (Correct Answer)
- B. Intravenous iron therapy
- C. Red blood cell transfusion
- D. Dietary modification alone
Explanation: ***Oral iron supplementation*** - **Oral iron supplementation** is the **first-line treatment** for iron deficiency anemia in children under 2 years of age due to its effectiveness, safety, and ease of administration. - The goal is to replenish **iron stores** and restore adequate hemoglobin levels through consistent daily dosing. *Intravenous iron therapy* - **Intravenous iron therapy** is typically reserved for children who cannot tolerate oral iron, have severe malabsorption, or have significant ongoing blood loss. - It is not the initial treatment given the availability of effective and less invasive oral options. *Red blood cell transfusion* - **Red blood cell transfusions** are indicated for severe, symptomatic anemia, often with **hemodynamic instability** or critical organ compromise, not as a primary treatment for uncomplicated iron deficiency. - Transfusions carry risks such as **transfusion reactions** and **iron overload**, making them a last resort. *Dietary modification alone* - While **dietary modification** is crucial for preventing recurrence and supporting iron repletion, it is usually insufficient as the sole treatment for established iron deficiency anemia. - **Therapeutic doses of iron** are needed to correct existing deficiencies, which diet alone cannot provide quickly enough.
Question 186: A 5-year-old boy presents with abdominal pain, bloody diarrhea, and lethargy. Laboratory findings reveal microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. What is the most likely diagnosis?
- A. Hemolytic uremic syndrome (Correct Answer)
- B. Henoch-Schönlein purpura
- C. Intussusception
- D. Crohn's disease
Explanation: ***Hemolytic uremic syndrome (HUS)*** - The classic triad of **microangiopathic hemolytic anemia**, **thrombocytopenia**, and **acute kidney injury** is pathognomonic for **hemolytic uremic syndrome (HUS)**, especially in a child with bloody diarrhea. - This condition is most commonly triggered by **Shiga toxin-producing E. coli (STEC)** infection, which causes widespread endothelial damage and microvascular thrombosis. - The typical presentation includes a prodrome of bloody diarrhea (often from contaminated food), followed by the development of the triad within days. *Henoch-Schönlein purpura* - Characterized by palpable **purpura**, **arthralgia**, **abdominal pain**, and **renal involvement** (often IgA-mediated glomerulonephritis). - Importantly, **platelet counts are typically normal or elevated** in HSP, which distinguishes it from HUS. - While it can cause bloody diarrhea due to GI vasculitis, the microangiopathic hemolytic anemia and thrombocytopenia seen in HUS are absent. *Intussusception* - Presents with sudden onset, intermittent, severe **abdominal pain** (often described as colicky), **vomiting**, and **currant jelly stools** (blood and mucus). - Peak incidence is 6-36 months of age, typically in well-appearing infants between episodes. - Does not cause **microangiopathic hemolytic anemia**, **thrombocytopenia**, or **acute kidney injury** as seen in this case. *Crohn's disease* - A chronic inflammatory bowel disease presenting with **abdominal pain**, **diarrhea** (sometimes bloody), **weight loss**, and **fatigue**, often with a more insidious onset. - Does not cause the acute triad of **microangiopathic hemolytic anemia**, **thrombocytopenia**, and **acute kidney injury** that defines HUS. - Would be unusual to present acutely at age 5 without preceding chronic symptoms.
Question 187: A 12-year-old girl presents with fatigue, petechiae, and a nosebleed that won’t stop. Physical examination reveals pallor, splenomegaly, and ecchymoses. Laboratory results show hemoglobin of 7.5 g/dL, platelets of 20,000/µL, and white blood cells of 1,000/µL. What is the most likely diagnosis and initial management?
- A. Immune thrombocytopenic purpura (ITP) - corticosteroids and platelet transfusions
- B. Aplastic anemia - supportive care with blood transfusions and platelet transfusions
- C. Disseminated intravascular coagulation (DIC) - treat underlying cause and supportive coagulation management
- D. Acute leukemia - urgent hematology consultation and chemotherapy (Correct Answer)
Explanation: ***Aplastic anemia; bone marrow biopsy + immunosuppressive therapy*** - The patient presents with **pancytopenia**, characterized by low **hemoglobin, low platelets**, and low **white blood cells**, indicating possible aplastic anemia [2]. - **Bone marrow biopsy** is essential for diagnosis [2] and **immunosuppressive therapy** is the first-line treatment for severe cases [1]. *ITP; IVIG + corticosteroids* - Immune Thrombocytopenic Purpura (ITP) usually presents with **isolated thrombocytopenia** without significant anemia or leukopenia. - Initial management involves **IVIG and corticosteroids**, not suitable given the severe cytopenias seen in this patient. *Leukemia; immediate chemotherapy* - While the low blood counts can suggest hematological malignancy, the **absence of blasts** or specific symptoms related to leukemia (like lymphadenopathy) makes this less likely. - Immediate chemotherapy is not appropriate without a confirmed diagnosis and is not the first step in suspected acute leukemia. *DIC; FFP + cryoprecipitate* - Disseminated Intravascular Coagulation (DIC) would typically present with **coagulation abnormalities** alongside thrombocytopenia [3], often with signs of systemic disease such as sepsis. - Management involves treating the underlying cause and not just transfusing **FFP** or **cryoprecipitate**, which is not indicated in this scenario. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, p. 662. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 662-663. [3] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 665-666.
Question 188: Which of the following is a typical hemoglobin level for term infants?
- A. 8 gm/dL
- B. 12 gm/dL
- C. 16 gm/dL (Correct Answer)
- D. 18 gm/dL
Explanation: ***16 gm/dL*** - Term infants typically have **higher hemoglobin** levels at birth due to higher fetal erythropoiesis and late cord clamping. - The **normal range** for term neonates at birth is **14-20 gm/dL**, with an **average/typical value of 16-17 gm/dL**. - A hemoglobin level of **16 gm/dL** represents the **most typical** value for a healthy full-term neonate in the first few days of life. *8 gm/dL* - A hemoglobin level of **8 gm/dL** is significantly below the normal range for a term infant and would indicate **severe anemia**, requiring investigation and treatment. - This level would likely be associated with clinical signs of anemia such as **pallor**, **lethargy**, and **tachycardia**. *12 gm/dL* - While 12 gm/dL is a normal hemoglobin level for an older child or adult, it is **lower than expected** for a healthy term neonate. - A value of **12 gm/dL** might suggest mild anemia in a newborn or could be considered at the lower end of the normal range, perhaps seen a few weeks post-birth as physiological anemia develops. *18 gm/dL* - A hemoglobin level of **18 gm/dL** is within the **upper end of the normal range** for a term infant at birth, but is **higher than the typical average** of 16-17 gm/dL. - While not abnormal, it is less representative of the "typical" neonate compared to 16 gm/dL. - Levels significantly higher than this (e.g., above 20-22 gm/dL) could suggest **polycythemia**, which can lead to hyperviscosity syndrome.
Question 189: What is the mean hemoglobin level in a 1-year-old child (in g/dl)?
- A. 18.5 g/dl
- B. 12 g/dl (Correct Answer)
- C. 16.5 g/dl
- D. 14 g/dl
Explanation: ***12 g/dl*** - The mean **hemoglobin level** in a 1-year-old child is typically around **12 g/dL**. - This value represents the average, though the normal range can vary slightly. *18.5 g/dl* - A hemoglobin level of **18.5 g/dL** would be considered very high and is outside the normal range for a 1-year-old. - Such a high level could indicate conditions like **polycythemia**. *16.5 g/dl* - A value of **16.5 g/dL** is higher than the typical mean for a 1-year-old, usually seen in newborns or early infancy. - Hemoglobin levels naturally decrease after birth before stabilizing. *14 g/dl* - While closer to the normal range, **14 g/dL** is still slightly above the mean for a 1-year-old child. - This level might be seen in older infants but is not the average for a child of one year.
Question 190: A child presents with recurrent chest infections and abdominal pain. There is a history of 1 blood transfusion in the past. On examination, he had icterus and mild splenomegaly. Electrophoresis shows increased HbA2, HbF, and S spike. What is the likely diagnosis?
- A. HbC disease
- B. Sickle cell disease
- C. Aplastic anemia
- D. Sickle Beta Thalassemia (Correct Answer)
Explanation: ***Sickle Beta Thalassemia*** - The combination of **sickle cell disease manifestations** (recurrent chest infections, abdominal pain, icterus, splenomegaly) with **electrophoresis showing increased HbA2, elevated HbF, and S spike** is diagnostic of **Sickle Beta Thalassemia**. - **Increased HbA2 (>3.5%)** is the key distinguishing feature that differentiates this from pure sickle cell disease. It indicates co-inheritance of a **beta-thalassemia gene** along with the **sickle cell gene**. - Clinical presentation is similar to sickle cell disease with **vaso-occlusive crises**, **acute chest syndrome**, hemolytic anemia, and organomegaly. - The severity depends on the type: S/β⁰-thalassemia (no HbA production) is clinically more severe and similar to SS disease, while S/β⁺-thalassemia (reduced HbA) tends to be milder. *Sickle cell disease* - Pure sickle cell disease (HbSS) presents with similar clinical features: recurrent chest infections, abdominal pain, hemolysis, and splenomegaly. - However, electrophoresis would show **normal or only slightly elevated HbA2 (2-3%)**, not the increased HbA2 mentioned in this case. - The presence of significantly increased HbA2 rules out pure sickle cell disease. *HbC disease* - Patients with HbC disease typically have **mild chronic hemolytic anemia** and **splenomegaly** but usually lack severe vaso-occlusive crises. - Electrophoresis would show primarily **HbC**, not an S spike. - The clinical picture is much milder than described in this case. *Aplastic anemia* - Characterized by **pancytopenia** due to bone marrow failure, leading to fatigue, infections, and bleeding tendency. - Does not involve hemolysis, icterus, or abnormal hemoglobin variants on electrophoresis. - The electrophoresis findings completely exclude this diagnosis.
Practice by Chapter
Anemias in Children
Practice Questions
Hemoglobinopathies
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Hemolytic Anemias
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Nutritional Anemias
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Thrombocytopenia
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Bleeding Disorders
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Thrombotic Disorders
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White Blood Cell Disorders
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Bone Marrow Failure Syndromes
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Blood Component Therapy
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Hemophilia and Von Willebrand Disease
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Evaluation of Bleeding Tendencies
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