Hematology Practice Questions

Q: 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.

Oxygen, antibiotics, blood transfusion. ***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.

Q: 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?

Coagulation profile. ***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.

Q: 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?

Hereditary spherocytosis. ***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.

Q: What is the first-line treatment for a child under 2 years of age with iron deficiency anemia?

Oral iron supplementation. ***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.

Q: 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?

Hemolytic uremic syndrome. ***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.

Q: What is the mean hemoglobin level in a 1-year-old child (in g/dl)?

12 g/dl. ***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.

Q: Which of the following is a typical hemoglobin level for term infants?

16 gm/dL. ***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.

Q: 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?

Sickle Beta Thalassemia. ***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.

Question 1

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.

Accepted Answer

Oxygen, antibiotics, blood transfusion

Answer

Bronchodilators, corticosteroids, IV fluids

Answer

Exchange transfusion, anticoagulation

Answer

Hydroxyurea, IV fluids, pain management

Question 2

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.

Accepted Answer

Iron deficiency anemia; treatment with iron supplements

Answer

β-thalassemia minor; regular monitoring

Answer

Sickle cell anemia; supportive care

Answer

G6PD deficiency; management of oxidative stress

Question 3

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?

Accepted Answer

Coagulation profile

Answer

Complete blood count

Answer

Platelet function tests

Answer

Genetic testing for clotting factor mutations

Question 4

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?

Accepted Answer

Hereditary spherocytosis

Answer

Iron deficiency anemia

Answer

Sickle cell disease

Answer

G6PD deficiency

Question 5

What is the first-line treatment for a child under 2 years of age with iron deficiency anemia?

Accepted Answer

Oral iron supplementation

Answer

Intravenous iron therapy

Answer

Red blood cell transfusion

Answer

Dietary modification alone

Question 6

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?

Accepted Answer

Hemolytic uremic syndrome

Answer

Henoch-Schönlein purpura

Answer

Intussusception

Answer

Crohn's disease

Question 7

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?

Accepted Answer

Acute leukemia - urgent hematology consultation and chemotherapy

Answer

Immune thrombocytopenic purpura (ITP) - corticosteroids and platelet transfusions

Answer

Aplastic anemia - supportive care with blood transfusions and platelet transfusions

Answer

Disseminated intravascular coagulation (DIC) - treat underlying cause and supportive coagulation management

Question 8

What is the mean hemoglobin level in a 1-year-old child (in g/dl)?

Accepted Answer

12 g/dl

Answer

18.5 g/dl

Answer

16.5 g/dl

Answer

14 g/dl

Question 9

Which of the following is a typical hemoglobin level for term infants?

Accepted Answer

16 gm/dL

Answer

8 gm/dL

Answer

12 gm/dL

Answer

18 gm/dL

Question 10

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?

Accepted Answer

Sickle Beta Thalassemia

Answer

HbC disease

Answer

Sickle cell disease

Answer

Aplastic anemia

Hematology — MCQs

Hematology — MCQs

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