Hematology — MCQs
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An 8-year-old boy with requirement of multiple blood transfusions. X-ray skull was performed. All are true about the condition shown except:
A 12-year-old child presents with acute kidney injury after a bout of dysentery. Not seen is:
A 7-year-old boy presents with fever and weight loss. On examination he had pallor with lymphadenopathy. Peripheral smear is shown below. Diagnosis is: (AIIMS May 2017)
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
Which of the following does not require a lumbar puncture in children?
A previously healthy child has sudden onset of red spots on body. There is a history of a preceding viral infection 1-4 weeks before the onset.
Normal reticulocyte count at birth is
Which of the following is the most common hematologic malignancy associated with Neurofibromatosis-1 (NF-1) in a child?
False about Shwachman-Diamond syndrome
Which of the following is most specific for congenital Rubella syndrome?
Hematology Indian Medical PG Practice Questions and MCQs
Question 171: An 8-year-old boy with requirement of multiple blood transfusions. X-ray skull was performed. All are true about the condition shown except:
- A. Squaring of metacarpal bones
- B. Erythroid hyperplasia in liver and spleen
- C. Iron chelation leads to bronze diabetes (Correct Answer)
- D. Splenectomy if transfusion requirement increases by 50% over last year
Explanation: ***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.
Question 172: A 12-year-old child presents with acute kidney injury after a bout of dysentery. Not seen is:
- A. MAHA
- B. Schistocytes
- C. Normal serum haptoglobin (Correct Answer)
- D. Thrombocytopenia
Explanation: ***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.
Question 173: A 7-year-old boy presents with fever and weight loss. On examination he had pallor with lymphadenopathy. Peripheral smear is shown below. Diagnosis is: (AIIMS May 2017)
- A. ALL (Correct Answer)
- B. AML
- C. Aplastic anemia
- D. Juvenile myelomonocytic leukemia
Explanation: ***ALL*** - The peripheral smear shows numerous **blasts** with **scanty cytoplasm**, **prominent nucleoli**, and a **high nuclear-to-cytoplasmic ratio**, which is characteristic of **acute lymphoblastic leukemia (ALL)**. - Clinical features such as **fever**, **weight loss**, **pallor**, and **lymphadenopathy** in a 7-year-old child are classic presentations of ALL, the most common childhood leukemia. *AML* - While AML also presents with acute symptoms and blasts, the blasts in AML typically have **more abundant cytoplasm**, and may contain **Auer rods**, which are not clearly visible here. - The **morphology of the blasts** in the image, with their uniformly high N/C ratio and immature appearance, points away from typical AML. *Aplastic anemia* - Aplastic anemia is characterized by **pancytopenia** in the peripheral blood and **hypocellular bone marrow**, meaning a significant reduction in all blood cell lines, and **lacks the presence of blasts**. - The image clearly displays a proliferation of immature cells (blasts), which is contrary to the pathology of aplastic anemia. *Juvenile myelomonocytic leukemia* - JMML is a rare disorder characterized by **monocytosis**, **splenomegaly**, and typically presents in very young children (median age 2 years), often with **rashes** and **adenopathy**. - While there is some overlap in symptoms, the predominant cell type in the smear does not suggest a significant monocytic component, and the clinical picture in a 7-year-old child is less typical for JMML without other defining features.
Question 174: 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
- A. Anemia of chronic disease
- B. Iron deficiency anemia
- C. Lead poisoning
- D. Beta thalassemia trait (Correct Answer)
Explanation: ***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 175: Which of the following does not require a lumbar puncture in children?
- A. HL (Correct Answer)
- B. AML
- C. NHL
- D. ALL
Explanation: ***HL*** - While central nervous system (CNS) involvement is possible in Hodgkin lymphoma (HL), it is **rare** and does not routinely warrant a **lumbar puncture** for initial staging or surveillance in asymptomatic children. - HL primarily affects **lymph nodes** and the **spleen**, with CNS spread being an uncommon complication that typically presents with specific neurological symptoms. *AML* - **Acute myeloid leukemia (AML)** has a significant risk of **CNS involvement**, requiring a **lumbar puncture** for diagnostic staging and administration of intrathecal chemotherapy. - CNS prophylaxis and treatment are crucial in AML to prevent and manage **leptomeningeal disease**. *NHL* - **Non-Hodgkin lymphoma (NHL)**, particularly aggressive subtypes like Burkitt lymphoma or lymphoblastic lymphoma, has a **high propensity for CNS spread**. - A **lumbar puncture** is essential for staging to detect CNS involvement and guide the need for intrathecal chemotherapy or radiation. *ALL* - **Acute lymphoblastic leukemia (ALL)** carries a well-documented **high risk of CNS infiltration**, necessitating routine **lumbar punctures** at diagnosis for CNS staging and throughout treatment for intrathecal chemotherapy. - CNS prophylaxis is a cornerstone of ALL treatment to prevent **leptomeningeal relapse**.
Question 176: A previously healthy child has sudden onset of red spots on body. There is a history of a preceding viral infection 1-4 weeks before the onset.
- A. Dengue fever
- B. Hemophilia A
- C. Idiopathic thrombocytopenic purpura (Correct Answer)
- D. Thrombotic thrombocytopenic purpura
Explanation: ***Idiopathic thrombocytopenic purpura (ITP)*** - This presentation, especially in a previously healthy child with a preceding viral infection 1-4 weeks prior, is highly characteristic of **acute ITP**, leading to **purpuric rash** (red spots). - The preceding viral infection often triggers an autoimmune response causing destruction of **platelets**, resulting in **thrombocytopenia**. *Dengue fever* - Dengue fever typically presents with **acute onset of fever**, **headache**, **myalgia**, and a rash that appears 3-4 days after fever onset, often with a shorter incubation period than 1-4 weeks. - While it can cause petechiae due to **thrombocytopenia**, the symptom constellation does not perfectly align with the scenario, particularly the sudden onset of spots without mention of fever or other acute symptoms. *Hemophilia A* - **Hemophilia A** is a **hereditary bleeding disorder** causing deficits in **Factor VIII**, leading to spontaneous bleeding into joints and muscles, and prolonged bleeding after trauma. - It does not present as sudden onset red spots (petechiae/purpura) following a viral infection but rather as larger **hematomas** or **hemarthroses**, and it's a chronic condition, not typically triggered by recent infection. *Thrombotic thrombocytopenic purpura (TTP)* - TTP is characterized by the **pentad of symptoms**: **fever**, **neurological symptoms**, **renal dysfunction**, **microangiopathic hemolytic anemia**, and **thrombocytopenia**. - While it involves thrombocytopenia and can cause purpura, the patient's presentation lacks the other severe systemic features typically associated with TTP, and it's less commonly triggered by a simple viral infection in children.
Question 177: Normal reticulocyte count at birth is
- A. 2-6% (Correct Answer)
- B. 1-2%
- C. 6-10%
- D. 30-40%
Explanation: ***2-6%*** - At birth, the normal **reticulocyte count** is elevated primarily due to the physiological stress of **adapting to extrauterine life** and increased erythropoiesis. - This higher range reflects the body's need for a rapid turnover of red blood cells to meet oxygen demands after birth. *1-2%* - This range is considered a **normal reticulocyte count** for **older children and adults**, indicating a steady state of red blood cell production. - It does not account for the **physiological erythropoietic surge** observed in healthy neonates. *6-10%* - While higher than adult levels, a range of **6-10%** in a neonate would still be considered unusually high and might suggest **pathological hemolysis** or significant **blood loss**. - Without other supporting clinical signs, this level is typically higher than the **physiological norm**. *30-40%* - A reticulocyte count of **30-40%** at birth is **extremely elevated** and is highly indicative of a severe underlying condition such as **hemolytic disease of the newborn** or significant **neonatal hemorrhage**. - This level is far beyond the **normal physiological response** and requires urgent investigation.
Question 178: Which of the following is the most common hematologic malignancy associated with Neurofibromatosis-1 (NF-1) in a child?
- A. Chronic Myeloid Leukemia (CML)
- B. Acute Lymphoblastic Leukemia (ALL)
- C. Acute Myeloid Leukemia (AML)
- D. Juvenile Myelomonocytic Leukemia (JMML) (Correct Answer)
Explanation: ***Juvenile Myelomonocytic Leukemia (JMML)*** - **JMML** is a myelodysplastic/myeloproliferative neoplasm that is strongly associated with **NF-1** in children, particularly due to mutations in the *NF1* gene. - Children with **NF-1** have a significantly increased risk of developing **JMML** compared to the general pediatric population. *Chronic Myeloid Leukemia (CML)* - While CML can occur in children, it is typically associated with the **Philadelphia chromosome (BCR-ABL1 fusion gene)** and not a common tumor directly linked to NF-1. - **CML** is relatively rare in childhood compared to other leukemias and is not a characteristic complication of NF-1. *Acute Lymphoblastic Leukemia (ALL)* - **ALL** is the most common childhood cancer overall, but its association with **NF-1** is not as specific or strong as that of **JMML**. - While children with NF-1 may have a slightly increased risk of certain cancers, **ALL** is not the *most common* tumor directly linked to NF-1. *Acute Myeloid Leukemia (AML)* - **AML** has a weak association with **NF-1**, particularly specific subtypes, but it is less frequent and less specifically linked than **JMML**. - The direct genetic pathway involving the **NF1 gene** mutation in the development of **AML** is not as clearly defined as it is for **JMML**.
Question 179: False about Shwachman-Diamond syndrome
- A. Bone marrow dysfunction
- B. Exocrine pancreatic insufficiency
- C. Leucocytosis (Correct Answer)
- D. Short stature
Explanation: ***Leucocytosis*** - **Leucocytosis** (an increase in white blood cells) is generally **not** a feature of Shwachman-Diamond syndrome (SDS); rather, patients typically experience **neutropenia** (low neutrophils) due to bone marrow dysfunction. - This persistent or intermittent neutropenia is a hallmark of the immune deficiency seen in SDS, making leucocytosis an incorrect finding. *Bone marrow dysfunction* - **Bone marrow dysfunction** is a defining characteristic of Shwachman-Diamond syndrome, leading to various **cytopenias**, most notably **neutropenia**. - This dysfunction can also manifest as anemia or thrombocytopenia, contributing to the overall morbidity of the disease. *Exocrine pancreatic insufficiency* - **Exocrine pancreatic insufficiency** is a primary clinical feature of Shwachman-Diamond syndrome, leading to **malabsorption** and **failure to thrive**. - This insufficiency is due to abnormal pancreatic development and is distinct from the more severe pancreatic involvement seen in cystic fibrosis. *Short stature* - **Short stature** is a common finding in children with Shwachman-Diamond syndrome, often resulting from a combination of **growth plate abnormalities** and **malnutrition** due to pancreatic insufficiency. - It is considered a key **skeletal manifestation** of the disease, along with metaphyseal chondrodysplasia.
Question 180: Which of the following is most specific for congenital Rubella syndrome?
- A. Blueberry muffin rash is seen
- B. Triad of CRS are cataract, cardiac defects, sensorineural deafness (Correct Answer)
- C. Infection is most serious in the first trimester of pregnancy
- D. Virus can be isolated up to 12 months after birth
Explanation: ***Triad of CRS are cataract, cardiac defects, sensorineural deafness*** - The **classic Gregg triad** of **cataracts**, **cardiac defects** (especially patent ductus arteriosus and pulmonary artery stenosis), and **sensorineural deafness** is the **most specific and pathognomonic** feature of **congenital Rubella syndrome**. - While individual components can occur in other conditions, the **combination of this triad** is highly specific for CRS and distinguishes it from other congenital infections. - This triad was first described by **Norman Gregg** in 1941 and remains the hallmark diagnostic feature of congenital rubella syndrome. *Blueberry muffin rash is seen* - The **blueberry muffin rash** (dermal erythropoiesis) presents as purpuric lesions or small dark blue papules and can be seen in congenital rubella syndrome. - However, this finding is **NOT specific to rubella** and occurs in multiple congenital infections including **CMV, toxoplasmosis, parvovirus B19**, and can also be seen in neonatal malignancies like neuroblastoma. - While characteristic, it is less specific than the Gregg triad for diagnosing CRS. *Infection is most serious in the first trimester of pregnancy* - Maternal rubella infection during the **first trimester** carries the highest risk (up to 85% if infected before 12 weeks) of severe multi-organ abnormalities due to rapid organogenesis. - While true, this describes the **timing and severity** of infection rather than a specific clinical feature that distinguishes rubella from other congenital infections. - Many congenital infections (CMV, toxoplasmosis, HSV) are also more severe when acquired in early pregnancy. *Virus can be isolated up to 12 months after birth* - Infants with **congenital Rubella syndrome** can shed virus in bodily fluids (urine, nasopharyngeal secretions) for **12 months or longer** after birth. - This prolonged viral shedding is important for **infection control** and isolation precautions but is a virological characteristic rather than a specific diagnostic clinical feature. - Other congenital infections (CMV) can also demonstrate prolonged viral shedding in infants.
Practice by Chapter
Anemias in Children
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Hemoglobinopathies
Practice Questions
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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