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 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 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?
What is the mean hemoglobin level in a 1-year-old child (in g/dl)?
Which of the following is a typical hemoglobin level for term infants?
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 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 187: 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 188: 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 189: 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 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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