Hematopathology — MCQs
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All of the following are features of juvenile CML except which of the following?
In which condition is Hemoglobin F typically elevated?
Helmet cells are characteristic of anemia of?
What is the chromosomal translocation associated with Acute Myeloid Leukemia M3?
Which acquired condition is most commonly associated with spherocytes?
What type of anaemia is primarily associated with leukaemia?
Which of the following best describes the shape of Birbeck granules?
Which type of immunoglobulin is primarily associated with cold agglutinin disease?
What do Döhle bodies represent in neutrophils?
Which of the following is not typically seen in Disseminated Intravascular Coagulation (DIC)?
Hematopathology Indian Medical PG Practice Questions and MCQs
Question 1401: All of the following are features of juvenile CML except which of the following?
- A. Fetal Hb is increased
- B. Lymphadenopathy
- C. Thrombocytopenia
- D. Philadelphia chromosome is positive (Correct Answer)
Explanation: ***Philadelphia chromosome is positive*** - **Juvenile Chronic Myeloid Leukemia (JCML)**, now known as **Chronic Myelomonocytic Leukemia (CMML)** of childhood, is characterized by the **absence** of the **Philadelphia chromosome (Ph chromosome)**. - The Ph chromosome, a t(9;22)(q34;q11) translocation forming the **BCR-ABL1 fusion gene**, is the hallmark of adult Chronic Myeloid Leukemia (CML), but not JCML. *Thrombocytopenia* - **Thrombocytopenia** (low platelet count) is a common feature in JCML due to ineffective hematopoiesis and bone marrow infiltration. - This contrasts with adult CML, where **thrombocytosis** (high platelet count) is more characteristic of the chronic phase. *Fetal Hb is increased* - An **increased level of fetal hemoglobin (HbF)** is a characteristic laboratory finding in children with JCML. - This elevation is related to the dysregulated hematopoiesis and is a useful diagnostic marker. *Lymphadenopathy* - **Lymphadenopathy** (enlarged lymph nodes) is a frequent clinical manifestation in JCML, reflecting the widespread infiltration of monocytic cells. - This is part of the systemic involvement seen in this aggressive myeloproliferative disorder.
Question 1402: In which condition is Hemoglobin F typically elevated?
- A. Congenital red cell aplasia
- B. Hereditary spherocytosis
- C. Sickle cell disease
- D. Beta-thalassemia (Correct Answer)
Explanation: ***Beta-thalassemia*** - In **beta-thalassemia**, there is reduced or absent synthesis of beta-globin chains, leading to an excess of alpha-globin chains. - To compensate, the body markedly increases production of fetal hemoglobin (**HbF**, α2γ2), which can constitute **50-90% of total hemoglobin** in beta-thalassemia major. - This compensatory mechanism helps alleviate the severity of anemia and ineffective erythropoiesis, making elevated HbF a **defining laboratory feature** of beta-thalassemia. *Hereditary spherocytosis* - This condition is characterized by defects in **erythrocyte membrane proteins** (spectrin, ankyrin, band 3), leading to fragile, spherical red blood cells. - While it causes **hemolytic anemia**, it does not involve elevated HbF levels; the defect is structural, not related to globin chain synthesis. *Congenital red cell aplasia* - This condition (e.g., **Diamond-Blackfan anemia**) involves a primary defect in early **erythroid progenitor cells**, leading to severe anemia. - While some patients may have elevated HbF, it is not a consistent or defining feature; the main problem is the lack of adequate red blood cell production, not a compensatory shift in globin synthesis. *Sickle cell disease* - In **sickle cell disease**, there is an abnormal beta-globin chain leading to the production of **hemoglobin S (HbS)**. - While HbF is indeed elevated in most patients (typically **2-15%**, compared to <1% in normal adults), the elevation is **less marked and consistent** compared to beta-thalassemia. - The primary defect is the presence of HbS, and while HbF elevation provides some clinical benefit (it inhibits HbS polymerization), beta-thalassemia remains the condition most characteristically associated with high HbF levels.
Question 1403: Helmet cells are characteristic of anemia of?
- A. Hemolytic uremic syndrome (HUS) (Correct Answer)
- B. Disseminated intravascular coagulation (DIC)
- C. Thrombotic thrombocytopenic purpura (TTP)
- D. Autoimmune hemolytic anemia (AIHA)
Explanation: ***Hemolytic uremic syndrome*** - Helmet cells are **fragmented red blood cells** associated with **microangiopathic hemolytic anemia** [1], commonly seen in hemolytic uremic syndrome. - This condition frequently results in **thrombocytopenia** and acute renal failure. *Acanthocytosis* - Acanthocytosis is characterized by **spiky red blood cells** (acanthocytes) rather than helmet cells. - It is commonly associated with **liver disease** and **abetalipoproteinemia**, not hemolytic anemia. *Polysplenia* - Polysplenia is a condition involving multiple spleens but does not typically relate to the **formation of helmet cells**. - It may cause **asplenic complications**, but anemia characteristics do not include helmet cells. *Spherocytosis* - Spherocytosis involves the presence of **spherical red blood cells** rather than fragmented (helmet) cells. - It is associated with **hereditary conditions** like hereditary spherocytosis, which leads to increased hemolysis but not typically to helmet cells. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 596-597.
Question 1404: What is the chromosomal translocation associated with Acute Myeloid Leukemia M3?
- A. t(18;21)
- B. t(15;17) (Correct Answer)
- C. t(9;11)
- D. t(8;21)
Explanation: ***T (15,17)*** [1][2][3] - This translocation pertains to **Acute Promyelocytic Leukemia (APL)**, associated with the fusion gene **PML-RARA** [1][3]. - APL is characterized by **promyelocytes** with heavy granulation and a clinical presentation that includes coagulopathy [2][3]. *T (8, 21)* - This translocation is associated with **AML M2**, involving the **RUNX1-RUNX1T1** fusion gene [3]. - It does not correlate with the classic features of AML M3, which is specifically characterized by T (15,17). *T (9,11)* - Primarily seen in **AML M5**, this translocation is not related to the pathophysiology of AML M3. - The fusion commonly observed here is **MLL-AFF1**, which affects different types of leukemias, not APL. *T (18,21)* - This translocation does not have a significant association with any specific type of acute myeloid leukemia. - Unlike T (15,17), it is not linked to the classic features or unique clinical presentation seen in AML M3. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of White Blood Cells, Lymph Nodes, Spleen, and Thymus, pp. 620-621. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of White Blood Cells, Lymph Nodes, Spleen, and Thymus, pp. 621-622. [3] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of White Blood Cells, Lymph Nodes, Spleen, and Thymus, p. 620.
Question 1405: Which acquired condition is most commonly associated with spherocytes?
- A. Autoimmune hemolytic anemia (AIHA) (Correct Answer)
- B. Vitamin B12 deficiency anemia
- C. Aplastic anemia
- D. None of the options
Explanation: ***Autoimmune haemolytic anemia*** - This condition is characterized by the **production of antibodies** against the patient's own red blood cells, leading to **destruction** and the formation of spherocytes [1]. - The presence of ***maximum spherocytes*** in blood smears is a hallmark of this disorder due to **extravascular hemolysis** [1]. *Aplastic anemia* - Aplastic anemia is mainly due to **failure of hematopoiesis**, resulting in pancytopenia rather than spherocyte formation. - It typically presents with **normocytic** or **macrocytic anemia**, not spherocytes, as the bone marrow is not producing enough red blood cells [2]. *Vit B12 deficiency* - Vit B12 deficiency usually causes **macrocytic anemia** characterized by **megaloblastic changes** in the bone marrow and peripheral blood, not the formation of spherocytes. - The primary indicators would be **hypersegmented neutrophils** and large, ovalocytes rather than spherocytes. *None* - This option incorrectly implies that no condition causes maximum spherocytes, while autoimmune hemolytic anemia is the known condition for this finding. - Spherocytes are a specific sign in hemolytic processes and are definitely seen in conditions like **autoimmune hemolytic anemia**. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 602-603. [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.
Question 1406: What type of anaemia is primarily associated with leukaemia?
- A. Aplastic anaemia
- B. Iron deficiency anaemia
- C. Megaloblastic anaemia
- D. Myelophthisic anaemia (Correct Answer)
Explanation: ***Myelophthisic anaemia*** - This condition arises from the **displacement of normal hematopoietic tissue** in the bone marrow by abnormal cells, like those seen in leukaemia, leading to **extramedullary hematopoiesis**. - Marrow infiltration causes **pancytopenia** and often results in the presence of **immature granulocytes** and **nucleated red blood cells** in the peripheral blood (leukoerythroblastosis). *Iron deficiency anaemia* - This type of anaemia is caused by insufficient iron for **hemoglobin synthesis**, often due to chronic blood loss or inadequate dietary intake. - While leukaemia patients can develop iron deficiency due to bleeding, it is not the **primary type of anaemia** directly resulting from the marrow infiltration by leukaemic cells. *Megaloblastic anaemia* - Characterized by the production of abnormally large, immature red blood cells, primarily due to **vitamin B12** or **folate deficiency**. - There is no direct causal link between leukaemia and the development of megaloblastic anaemia as a **primary haemato-pathological mechanism**. *Aplastic anaemia* - Characterized by **pancytopenia** due to bone marrow failure with hypocellular marrow, not marrow infiltration. - While both leukaemia and aplastic anaemia can present with cytopenias, aplastic anaemia shows a **hypocellular marrow** whereas leukaemia shows a **hypercellular marrow** with infiltration by malignant cells.
Question 1407: Which of the following best describes the shape of Birbeck granules?
- A. Resembling a tennis racket (Correct Answer)
- B. Resembling a hockey stick
- C. Resembling a bat
- D. Resembling a ball
Explanation: ***Tennis racket*** [1] - Birbeck granules are distinctive for their **tennis racket shape**, consisting of an elongated area and a bulbous end [1]. - They are typically found in **Langerhans cell histiocytosis** and are associated with **CD1a+** staining [1]. *Bat* - The term "bat" does not accurately describe the structure or morphology of Birbeck granules. - Unlike bats, Birbeck granules have a **specific elongated shape** with a bulbous tip rather than bat wings. *Hockey stick* - This shape implies a straight handle and a flat blade, which does not represent the **widely tapered ends** of Birbeck granules. - The **characteristic morphology** does not align with any hockey stick features. *Ball* - The term "ball" indicates a spherical shape, which is not fitting for Birbeck granules, as they are **elongated** and striated. - Birbeck granules are defined specifically by their **tapered structure**, contrasting markedly with a circular form. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of White Blood Cells, Lymph Nodes, Spleen, and Thymus, p. 630.
Question 1408: Which type of immunoglobulin is primarily associated with cold agglutinin disease?
- A. IgG
- B. IgM (Correct Answer)
- C. IgA
- D. IgD
Explanation: ***IgM*** - Cold agglutinins are primarily composed of **IgM antibodies** [1] which are produced in response to certain infections, notably Mycoplasma pneumoniae. - They can cause **cold agglutinin disease**, leading to hemolytic anemia when the blood is exposed to cooler temperatures [2]. *IgG* - While IgG is the most abundant antibody in blood, it is not responsible for cold agglutination. - IgG typically functions in **immune memory** and neutralization rather than agglutination at cold temperatures. *IgD* - IgD is mainly found on the surface of **B cells** and has a role in initiating B cell activation. - It plays a minimal role in the antibody response and does not participate in cold agglutination. *IgA* - IgA is primarily involved in mucosal immunity, found in secretions like saliva and tears. - It does not function as a cold agglutinin; hence, it is not associated with cold temperature-related agglutination. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. (Basic Pathology) introduces the student to key general principles of pathology, both as a medical science and as a clinical activity with a vital role in patient care. Part 2 (Disease Mechanisms) provides fundamental knowledge about the cellular and molecular processes involved in diseases, providing the rationale for their treatment. Part 3 (Systematic Pathology) deals in detail with specific diseases, with emphasis on the clinically important aspects., pp. 154-155. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 651-652.
Question 1409: What do Döhle bodies represent in neutrophils?
- A. Presence of dilated endoplasmic reticulum in neutrophils (Correct Answer)
- B. Increased mitochondria in neutrophils
- C. Golgi apparatus proliferation in neutrophils
- D. Lysosomal activity in neutrophils
Explanation: ***Presence of dilated endoplasmic reticulum in neutrophils*** - **Döhle bodies** are inclusions found in the cytoplasm of neutrophils, classic hallmarks of **severe infection** or inflammatory conditions. - They represent remnants of dilated **rough endoplasmic reticulum**, which appears as pale-blue, irregular aggregates on stained blood smears. *Increased mitochondria in neutrophils* - An increase in mitochondria is not a characteristic feature associated with Döhle bodies. - While mitochondria are essential for cellular energy production, their proliferation does not form visible inclusions known as Döhle bodies. *Golgi apparatus proliferation in neutrophils* - Proliferation of the Golgi apparatus is not typically observed as a Döhle body. - The Golgi apparatus is involved in protein modification and packaging, but its changes do not manifest as these specific inclusions. *Lysosomal activity in neutrophils* - Lysosomal activity involves the breakdown of cellular debris and pathogens and is not directly related to the formation of Döhle bodies. - Although lysosomes are abundant in neutrophils, their activity does not result in the presence of Döhle bodies.
Question 1410: Which of the following is not typically seen in Disseminated Intravascular Coagulation (DIC)?
- A. Thrombocytopenia
- B. PT elevation
- C. Fibrinogen decreased
- D. Normal aPTT (Correct Answer)
Explanation: ***Normal APTT*** - In Disseminated Intravascular Coagulation (**DIC**), **APTT** is typically **prolonged** due to consumption of clotting factors [1]. - The presence of normal APTT indicates that coagulation pathways are not significantly affected, which is contrary to what is seen in DIC. *Fibrinogen decreased* - **Decreased fibrinogen levels** are common in DIC, reflecting its consumption during the coagulation process [1]. - This depletion is linked to the increased clotting and is a hallmark of DIC, making this statement false in the context of the question. *Thrombocytopenia* - **Thrombocytopenia** occurs in DIC as platelets are consumed during the formation of microclots [1]. - A significant drop in platelet count is a key feature of DIC, therefore this statement does not align with the "except" clause. *PT elevation* - Prothrombin Time (**PT**) is usually **elevated** in DIC due to the consumption of clotting factors [1]. - This reflects the ongoing activation of the coagulation cascade, supporting the exclusion in the question context. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 625-626.
Practice by Chapter
Anemias: Classification and Approach
Practice Questions
Hemolytic Anemias
Practice Questions
Myeloproliferative Neoplasms
Practice Questions
Myelodysplastic Syndromes
Practice Questions
Acute Leukemias
Practice Questions
Chronic Leukemias
Practice Questions
Lymphomas and Lymphoid Neoplasms
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Plasma Cell Disorders
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Bleeding Disorders
Practice Questions
Thrombotic Disorders
Practice Questions
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