Hematology Practice Questions

Q: A 7-year-old boy has severe microcytic anemia due to beta-thalassemia major (homozygous). He requires frequent blood transfusions to prevent skeletal and developmental complications. Which of the following medications is also indicated in the treatment of patients requiring frequent blood transfusions?

Desferrioxamine. **Explanation:** The patient has **Beta-Thalassemia Major**, a condition characterized by ineffective erythropoiesis and severe anemia requiring lifelong regular blood transfusions. The primary complication of chronic transfusion therapy is **Secondary Hemochromatosis (Iron Overload)**. Since the human body lacks an active mechanism to excrete excess iron, each unit of blood (containing ~200mg of iron) contributes to systemic deposition, leading to multi-organ damage (heart, liver, and endocrine glands) [2]. **Why Desferrioxamine is correct:** **Desferrioxamine** is a high-affinity **iron-chelating agent**. It binds to free iron and ferritin-bound iron to form ferrioxamine, which is then excreted via the kidneys and bile. It is indicated when serum ferritin levels exceed 1,000 ng/mL or after the patient has received approximately 10–20 transfusions. **Why other options are incorrect:** * **Oral calcium supplements:** While patients with thalassemia may develop hypoparathyroidism or osteoporosis due to iron deposition, calcium is not a primary treatment for transfusion-related complications. * **Fresh frozen plasma (FFP):** FFP is used to replace clotting factors in coagulopathies; it has no role in treating thalassemia or iron overload. * **Penicillamine:** This is a chelating agent used primarily for **Copper** (Wilson’s disease) and sometimes in rheumatoid arthritis or cystinuria, not for iron overload [1]. **NEET-PG High-Yield Pearls:** * **Routes of Administration:** Desferrioxamine is given SC/IV (poor oral bioavailability). **Deferasirox** and **Deferiprone** are preferred oral alternatives. * **Side Effects:** Desferrioxamine can cause ototoxicity (high-frequency hearing loss) and retinal toxicity. * **Cause of Death:** The most common cause of death in inadequately chelated Thalassemia Major patients is **Heart Failure** (due to siderotic cardiomyopathy).

Q: A 7-year-old child is brought to the clinic with complaints of fatigue and poor concentration. The mother reports that the child has been eating non-food items such as chalk and soil for the past few months. A peripheral blood smear image shows microcytic, hypochromic red blood cells. Which of the following is the most likely diagnosis?

Iron deficiency anemia. ***Iron deficiency anemia*** - The patient's history of **pica** (craving and eating non-food items like chalk and soil) is a classic clinical sign of iron deficiency. - The peripheral blood smear confirms this diagnosis by showing **microcytic** (small) and **hypochromic** (pale) red blood cells, which result from impaired hemoglobin synthesis due to a lack of iron. *Vitamin B12 deficiency* - This condition causes **macrocytic anemia**, where red blood cells are larger than normal (high MCV), which is the opposite of the findings in this case. - Peripheral smear findings in B12 deficiency typically include **macro-ovalocytes** and **hypersegmented neutrophils**, neither of which is described or shown. *Thalassemia major* - Although thalassemia causes a **microcytic, hypochromic anemia**, it is a genetic disorder that typically presents in infancy with severe symptoms like failure to thrive and massive **hepatosplenomegaly**. - Pica is not a characteristic feature of thalassemia; it is strongly associated with iron deficiency anemia. *Lead poisoning* - Lead poisoning can cause microcytic anemia, but a key finding on the peripheral smear is **basophilic stippling**, which is not the prominent feature here. - While pica can be a risk factor for lead ingestion, pica itself is a more direct and classic symptom of underlying **iron deficiency**.

Q: A child with progressive pallor and bone pain has an elevated HbS based on the HPLC report. Which is the best treatment to manage hemolysis in this patient?

Voxelotor. ***Voxelotor*** - It is a **hemoglobin polymerization inhibitor** that stabilizes the oxygenated form of red blood cells, preventing **sickling** and subsequent hemolytic anemia, thereby directly managing the hemolysis. - It significantly improves **hemoglobin levels** and reduces markers of hemolysis, such as **indirect bilirubin** and **reticulocyte count**. *Hydroxyurea* - Its primary mechanism is inducing the production of **fetal hemoglobin (HbF)**, thereby diluting the concentration of HbS and *indirectly* reducing hemolysis over time. - Although crucial for managing **vaso-occlusive crises** (VOCs), it is not as direct an anti-hemolytic agent as Voxelotor. *Azacytidine* - This is a **DNA methyltransferase inhibitor**, primarily used in high-risk **Myelodysplastic Syndrome (MDS)** and Acute Myeloid Leukemia (AML). - While it can induce HbF like Hydroxyurea, it is not a standard or approved frontline treatment for the routine management of Sickle Cell Disease (SCD). *Bortezomib* - This drug is a **proteasome inhibitor** used exclusively in the treatment of **Multiple Myeloma** and certain related plasma cell dyscrasias. - It has no therapeutic role or clinical indication in the specific management of hemolysis or the underlying pathophysiology of SCD.

Q: A young boy presented with petechiae and his platelet count was 10,000/cu mm. Bone marrow aspirate revealed normal cellularity with megakaryocyte hyperplasia. Most appropriate initial therapy?

IVIG. ***IVIG*** - The presentation (young patient, **petechiae**, extremely low platelet count of 10,000/cu mm, and **megakaryocyte hyperplasia** in bone marrow) is classic for **Immune Thrombocytopenia (ITP)**. - Given the severe thrombocytopenia (platelets <20,000/cu mm) and active bleeding risk (petechiae), the initial treatment of choice is typically **IVIG (Intravenous Immunoglobulin)** or corticosteroids to rapidly increase the platelet count. *Aspirin* - **Aspirin** is an antiplatelet agent that **inhibits platelet aggregation**; it is strictly contraindicated in severe thrombocytopenia like ITP as it would increase the risk of hemorrhage. - It is used for pain relief or secondary prevention of cardiovascular events, not for treating low platelet counts. *Bone Marrow Transplant* - **Bone marrow transplant** is a radical and highly risky procedure reserved for refractory, life-threatening hematological malignancies or severe aplastic anemia, or failed therapy for ITP. - It is never the appropriate **initial therapy** for acute ITP, which is predominantly an autoimmune disorder of peripheral platelet destruction. *Methotrexate* - **Methotrexate** is an immunosuppressive drug used typically in chronic inflammatory conditions (like rheumatoid arthritis) or malignancies. - It is considered a second or third-line treatment option, or part of a more aggressive immunosuppression regimen, for ITP that has failed initial therapy (steroids/IVIG), not the immediate first choice.

Q: A 6-month-old child brought by parents for diffuse ecchymosis on extremities and trunk. Probable diagnosis is? (Recent NEET Pattern 2016-17)

Protein C deficiency. ***Protein C deficiency*** - A 6-month-old with **diffuse ecchymoses on extremities and trunk** is classic for **homozygous protein C deficiency** presenting as **purpura fulminans**. - Severe congenital protein C deficiency typically manifests in **early infancy** (within first few weeks to months) with widespread purpuric lesions, skin necrosis, and thrombotic complications. - The presentation includes **large ecchymotic patches** that can progress to necrosis, affecting extremities and trunk as described. - This is the **most likely diagnosis** for this age group and clinical presentation in the absence of other systemic features. *Disseminated intravascular coagulation* - DIC causes diffuse bleeding and purpura due to consumption coagulopathy and microthrombi formation. - However, DIC is a **secondary complication** requiring an underlying trigger (sepsis, trauma, malignancy) with acute systemic illness. - The child would typically present with **multiple bleeding sites**, systemic signs, and evidence of the underlying condition—not isolated ecchymosis. *A.P.L.A.S (Antiphospholipid Antibody Syndrome)* - Neonatal antiphospholipid syndrome from maternal antibodies presents in the **neonatal period** (first 28 days of life), not at 6 months. - APLAS primarily causes **thrombotic events** (stroke, renal vein thrombosis) rather than diffuse ecchymosis alone. - This diagnosis is **extremely rare** in infancy and would not be the probable diagnosis for isolated ecchymotic presentation at this age. *Dengue hemorrhagic fever* - Dengue requires **exposure in endemic areas** and presents with fever, headache, myalgia, and characteristic rash followed by hemorrhagic manifestations. - More common in **older children and adults**; the 6-month-old age and absence of fever/systemic symptoms make this unlikely. - The clinical picture describes ecchymosis without mention of fever or other dengue features.

Q: A 3-year-old child presents with sudden onset generalized petechiae and bruise on forehead. Sternal tenderness is absent and liver and spleen are not palpable. Bone marrow aspiration is normal. Probable cause is? (Recent NEET Pattern 2016-17)

Acute ITP. ***Acute ITP*** - A 3-year-old child presenting with **sudden onset petechiae and ecchymoses (bruise on forehead)**, without signs of systemic illness (no sternal tenderness, non-palpable liver/spleen), and a normal bone marrow is highly suggestive of **Idiopathic Thrombocytopenic Purpura (ITP)**. ITP is characterized by isolated thrombocytopenia due to autoimmune destruction of platelets. - The **normal bone marrow**, in particular, rules out conditions like leukemia and aplastic anemia, where bone marrow pathology would be evident. *Aplastic anemia* - **Aplastic anemia** would typically present with **pancytopenia** (affecting all blood cell lines), leading to symptoms like pallor, fatigue, infections, and bleeding. - A **bone marrow aspiration** in aplastic anemia would show a **hypocellular marrow** with a reduction in hematopoietic cells, which contradicts the "normal" finding in this case. *Acute leukemia* - **Acute leukemia** in children often presents with **bone pain (sternal tenderness)**, fever, fatigue, and organomegaly (enlarged liver and spleen) due to infiltration of leukemic cells. - A **bone marrow aspiration** would reveal a **hypercellular marrow** with a large proportion of blasts, which is not consistent with the "normal" bone marrow finding provided. *Dengue hemorrhagic fever* - **Dengue hemorrhagic fever** typically presents with symptoms like high fever, severe headache, muscle and joint pain, and often signs of bleeding (like petechiae and bruising) and can lead to severe thrombocytopenia. - However, it is usually preceded by a **fever** and other systemic symptoms, which are not mentioned in this sudden onset presentation in the given case scenario.

Q: An 8-year-old boy with requirement of multiple blood transfusions. X-ray skull was performed. All are true about the condition shown except:

Iron chelation leads to bronze diabetes. ***Iron chelation leads to bronze diabetes*** - **Bronze diabetes** is caused by **hemochromatosis** (iron overload), where excess iron deposits in the pancreas, leading to diabetes and skin bronzing. Iron chelation therapy is given to **prevent** or treat iron overload, therefore it would logically **prevent** or reverse bronze diabetes, not cause it. - While chronic blood transfusions can cause iron overload, leading to bronze diabetes, chelation therapy is the treatment to prevent this complication and associated organ damage. *Squaring of metacarpal bones* - This is a typical radiological finding in **thalassemia major**, caused by **expanded bone marrow** in response to chronic anemia. - The abnormal erythropoiesis leads to widening of the medullary cavity and thinning of the cortical bone, giving the metacarpals a characteristic rectangular or "squared" appearance. *Erythroid hyperplasia in liver and spleen* - In conditions like thalassemia, the body attempts to compensate for ineffective erythropoiesis by activating **extramedullary hematopoiesis** in organs like the liver and spleen. - This results in the proliferation of erythroid precursors (erythroid hyperplasia) in these organs, contributing to hepatosplenomegaly. *Splenectomy if transfusion requirement increases by 50% over last year* - **Splenectomy** is a consideration in patients with thalassemia who have significantly increased transfusion requirements, usually defined as a 30-50% increase, due to **hypersplenism**. - Hypersplenism leads to increased destruction of red blood cells and premature removal of transfused cells, necessitating frequent transfusions.

Q: A 12-year-old child presents with acute kidney injury after a bout of dysentery. Not seen is:

Normal serum haptoglobin. ***Normal serum haptoglobin*** - In hemolytic conditions like HUS (which classically follows dysentery in children), **haptoglobin** is consumed as it binds to free hemoglobin, leading to **decreased serum haptoglobin levels**, not normal levels. - The patient's presentation of acute kidney injury after dysentery is highly suggestive of **hemolytic uremic syndrome (HUS)**, a form of microangiopathic hemolytic anemia where red blood cells are destroyed. *MAHA* - **Microangiopathic hemolytic anemia (MAHA)** is central to the pathophysiology of HUS, where red blood cells are mechanically fragmented as they pass through narrowed and damaged microvessels. - The presence of **schistocytes** on a peripheral blood smear is a hallmark of MAHA. *Schistocytes* - **Schistocytes** (fragmented red blood cells) are a key laboratory finding in HUS, resulting from the shearing of red blood cells as they navigate obstructed microvasculature. - Their presence confirms the diagnosis of **microangiopathic hemolytic anemia**. *Thrombocytopenia* - **Thrombocytopenia** is a common feature of HUS, caused by the consumption of platelets within the microthrombi that form in the damaged microcirculation. - This platelet consumption contributes to the characteristic triad of HUS: hemolytic anemia, thrombocytopenia, and acute kidney injury.

Q: A 10-year-old boy presented with fatigue. Investigations revealed: Hemoglobin, 9 g/dL; MCV, 60 fL; MCH, 20 pg; and serum ferritin, 185 µg/L. The TLC was elevated and showed predominant lymphocytes and neutrophils. What is the likely diagnosis in this patient? **Normal values:** - Serum ferritin: 50-150 µg/L

Beta thalassemia trait. ***Beta thalassemia trait*** - The combination of **microcytic hypochromic anemia** (low MCV, MCH) with **normal to elevated ferritin** (185 µg/L is above the normal range of 50-150 µg/L) is highly suggestive of **beta thalassemia trait**, as iron stores are typically adequate or increased. - An elevated TLC with predominant lymphocytes and neutrophils is nonspecific but does not rule out thalassemia, as secondary infections or inflammatory processes can occur. *Anemia of chronic disease* - While anemia of chronic disease can cause **microcytic or normocytic anemia** and elevated ferritin (as ferritin is an acute phase reactant), the degree of **microcytosis** (MCV 60 fL) is more profound than typically seen in ACD. - ACD usually involves **inflammation or infection**, but the lab values provided more strongly point away from pure ACD. *Iron deficiency anemia* - **Iron deficiency anemia** is characterized by **microcytic hypochromic anemia** but would present with **low serum ferritin** levels, indicating depleted iron stores. - The patient's **elevated ferritin** (185 µg/L) rules out iron deficiency as the primary cause. *Lead poisoning* - **Lead poisoning** can cause **microcytic anemia** by interfering with heme synthesis, but it is typically associated with a **basophilic stippling** on peripheral blood smear and elevated blood lead levels. - It does not typically present with **elevated ferritin** as a classic feature, and the overall clinical picture is more consistent with a genetic hemoglobinopathy.

Question 1

A 7-year-old boy has severe microcytic anemia due to beta-thalassemia major (homozygous). He requires frequent blood transfusions to prevent skeletal and developmental complications. Which of the following medications is also indicated in the treatment of patients requiring frequent blood transfusions?

Accepted Answer

Desferrioxamine

Answer

Oral calcium supplements

Answer

Fresh frozen plasma (FFP)

Answer

Penicillamine

Question 2

A 7-year-old child is brought to the clinic with complaints of fatigue and poor concentration. The mother reports that the child has been eating non-food items such as chalk and soil for the past few months. A peripheral blood smear image shows microcytic, hypochromic red blood cells. Which of the following is the most likely diagnosis?

Accepted Answer

Iron deficiency anemia

Answer

Lead poisoning

Answer

Thalassemia major

Answer

Vitamin B12 deficiency

Question 3

A child with progressive pallor and bone pain has an elevated HbS based on the HPLC report. Which is the best treatment to manage hemolysis in this patient?

Accepted Answer

Voxelotor

Answer

Azacytidine

Answer

Hydroxyurea

Answer

Bortezomib

Question 4

A young boy presented with petechiae and his platelet count was 10,000/cu mm. Bone marrow aspirate revealed normal cellularity with megakaryocyte hyperplasia. Most appropriate initial therapy?

Accepted Answer

IVIG

Answer

Bone Marrow Transplant

Answer

Methotrexate

Answer

Aspirin

Question 5

A 1-year-old infant with the following lesion on face. CNS examination was normal. Blood counts show thrombocytopenia with P. smear suggestive of microangiopathic changes. Probable diagnosis is:

Accepted Answer

Kasabach-Merritt syndrome

Answer

Tuberous sclerosis

Answer

Thrombotic thrombocytopenic purpura

Answer

Sturge-Weber syndrome

Question 6

A 6-month-old child brought by parents for diffuse ecchymosis on extremities and trunk. Probable diagnosis is? (Recent NEET Pattern 2016-17)

Accepted Answer

Protein C deficiency

Answer

Disseminated intravascular coagulation

Answer

A.P.L.A.S

Answer

Dengue hemorrhagic fever

Question 7

A 3-year-old child presents with sudden onset generalized petechiae and bruise on forehead. Sternal tenderness is absent and liver and spleen are not palpable. Bone marrow aspiration is normal. Probable cause is? (Recent NEET Pattern 2016-17)

Accepted Answer

Acute ITP

Answer

Aplastic anemia

Answer

Acute leukemia

Answer

Dengue hemorrhagic fever

Question 8

An 8-year-old boy with requirement of multiple blood transfusions. X-ray skull was performed. All are true about the condition shown except:

Accepted Answer

Iron chelation leads to bronze diabetes

Answer

Squaring of metacarpal bones

Answer

Erythroid hyperplasia in liver and spleen

Answer

Splenectomy if transfusion requirement increases by 50% over last year

Question 9

A 12-year-old child presents with acute kidney injury after a bout of dysentery. Not seen is:

Accepted Answer

Normal serum haptoglobin

Answer

MAHA

Answer

Schistocytes

Answer

Thrombocytopenia

Question 10

A 10-year-old boy presented with fatigue. Investigations revealed: Hemoglobin, 9 g/dL; MCV, 60 fL; MCH, 20 pg; and serum ferritin, 185 µg/L. The TLC was elevated and showed predominant lymphocytes and neutrophils. What is the likely diagnosis in this patient?

**Normal values:**
- Serum ferritin: 50-150 µg/L

Accepted Answer

Beta thalassemia trait

Answer

Anemia of chronic disease

Answer

Iron deficiency anemia

Answer

Lead poisoning

Hematology — MCQs

Hematology — MCQs

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