Hematopathology — MCQs

Hematopathology Indian Medical PG Practice Questions and MCQs

Question 1341: A blood grouping test shows clumping with Anti-A serum, clumping with Anti-B serum, and no clumping in the control. What blood group does this indicate?

A. A
B. B
C. O
D. AB (Correct Answer)

Explanation: ***AB*** - The results show **clumping with both Anti-A and Anti-B serum**, indicating the presence of both A and B antigens on the red blood cells. - The absence of clumping in the control confirms that the **agglutination with Anti-A and Anti-B is due to specific antigen-antibody reactions**, not nonspecific agglutination. - Blood group AB individuals have both A and B antigens on their RBCs and no anti-A or anti-B antibodies in their serum. *A* - Blood group A would show **clumping with Anti-A serum only** and no clumping with Anti-B serum. - This is incorrect because the sample shows clumping with both antisera. *B* - Blood group B would show **clumping with Anti-B serum only** and no clumping with Anti-A serum. - This is incorrect because the sample shows clumping with both antisera. *O* - Blood group O would show **no clumping with either Anti-A or Anti-B serum**, as it lacks both A and B antigens. - This is incorrect because the sample clearly shows clumping with both Anti-A and Anti-B sera.

Question 1342: Which disease is characterized by the absence of the CD59 protein?

A. Atypical Hemolytic Uremic Syndrome (aHUS)
B. Hereditary Spherocytosis
C. Paroxysmal Nocturnal Hemoglobinuria (PNH) (Correct Answer)
D. Autoimmune Hemolytic Anemia

Explanation: ***PNH*** - CD59 is a **glycoprotein** that inhibits the formation of the membrane attack complex of the complement system, which is deficient in **Paroxysmal Nocturnal Hemoglobinuria (PNH)** [1][2]. - The presence of CD59 deficiency leads to **hemolysis** and manifestations like **dark urine**, especially in the morning [1]. *BRR* - Refers to **Bloom-Richardson Grade** in breast cancer pathology, not related to CD59. - It focuses more on **histological features** rather than specific **markers** like CD59. *PTEN* - PTEN is a **tumor suppressor gene** associated with various cancers, particularly in **Cowden syndrome** and **PTEN hamartoma syndrome**. - It does not relate to the **CD59 deficiency** found in PNH. *Cowden syndrome* - Cowden syndrome is linked to mutations in the **PTEN gene**, leading to numerous hamartomas and an increased risk for breast and thyroid cancers. - It does not exhibit any relationship with **CD59**, which is specifically associated with PNH. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 650-651. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 601-602.

Question 1343: Which of the following statements about sideroblastic anemia is correct?

A. All of the options are false
B. Severity of the disease is influenced by ALA synthase activity
C. Prussian blue staining reveals ringed sideroblasts (Correct Answer)
D. Pyridoxine supplementation is the definitive treatment for all types of sideroblastic anemia

Explanation: ***Prussian blue staining reveals ringed sideroblasts*** - Sideroblastic anemia is characterized by the presence of **ringed sideroblasts** in the bone marrow, which are erythroblasts with iron granules accumulating in a ring around the nucleus. - These iron deposits are visible with **Prussian blue staining**, confirming the diagnosis. *Severity of the disease is influenced by ALA synthase activity* - While defects in **heme synthesis** can cause sideroblastic anemia, the severity is not primarily or solely determined by **ALA synthase activity**. - Sideroblastic anemias involve diverse genetic and acquired causes, affecting various enzyme steps and pathways, not just specifically ALA synthase. *All of the options are false* - This statement is incorrect because the presence of **ringed sideroblasts** seen with Prussian blue staining is a hallmark diagnostic feature of sideroblastic anemia, making that option true. - This option would only be true if all other statements were false, which is not the case here. *Pyridoxine supplementation is the definitive treatment for all types of sideroblastic anemia* - **Pyridoxine (vitamin B6)** is a cofactor for some enzymes in the heme synthesis pathway, and about one-third of congenital sideroblastic anemias respond to it. - However, it is not a definitive treatment for **all types of sideroblastic anemia**, especially acquired forms or those with specific genetic mutations not responsive to pyridoxine.

Question 1344: Which of the following genes is most commonly mutated in Juvenile myelomonocytic leukemia (JMML)?

A. PTPN11 (Correct Answer)
B. KRAS
C. PTEN
D. APC

Explanation: ***PTPN11*** - **PTPN11** encodes the SHP2 protein, a non-receptor protein tyrosine phosphatase that plays a crucial role in the **RAS-MAPK signaling pathway**. - **Germline or somatic mutations** in PTPN11 are found in approximately **35% of JMML cases**, making it the **most commonly mutated gene** in this disorder. - These gain-of-function mutations lead to constitutive activation of RAS signaling, driving the myeloproliferative phenotype characteristic of JMML. - PTPN11 mutations are also associated with **Noonan syndrome**, and patients with Noonan syndrome have an increased risk of developing JMML. *KRAS* - **KRAS** is a proto-oncogene encoding a GTPase in the RAS-MAPK pathway. Mutations cause constitutive activation and uncontrolled cell proliferation. - KRAS mutations are found in approximately **15-20% of JMML cases**, making it the **second most common** genetic alteration in this disease. - While definitely associated with JMML, KRAS mutations are **less frequent than PTPN11 mutations**. *PTEN* - **PTEN** is a tumor suppressor gene regulating the PI3K-AKT pathway, involved in various cancers including Cowden syndrome and endometrial cancer. - PTEN mutations are **not associated with JMML** pathogenesis. *APC* - The **APC gene** is a tumor suppressor in the Wnt signaling pathway, critical for colon epithelial regulation. - APC mutations cause **familial adenomatous polyposis (FAP)** and colorectal cancer, but are **not implicated in JMML**.

Question 1345: Which of the following is considered a hypercoagulable factor?

A. Protein C
B. Protein S
C. Factor V Leiden (Correct Answer)
D. Antithrombin III

Explanation: ***Factor V Leiden*** - Factor V Leiden is a **mutation** of the Factor V gene, leading to a hypercoagulable state due to **increased resistance** to activated protein C [1]. - This condition substantially raises the risk of **venous thromboembolism**, making it a critical factor in thrombotic events [1]. *Protein S* - Protein S functions as a **cofactor** for activated protein C, promoting inactivation of coagulation factors and thus **decreasing** clot formation. - Deficiency in Protein S can lead to thrombosis, but it is not inherently a **hypercoagulable factor**. *Protein C* - Protein C is activated by the thrombin-thrombomodulin complex, which helps to **regulate coagulation** and **prevent clotting**. - It is protective against thrombosis, and its deficiency can predispose to clot formation, but it does not act as a hypercoagulable factor itself. *Antithrombin III* - Antithrombin III is a **natural anticoagulant** that inhibits thrombin and other proteases of the coagulation cascade, thus preventing excessive clotting. - Its deficiency also leads to hypercoagulability, but it is not classified as a hypercoagulable factor. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Hemodynamic Disorders, Thromboembolic Disease, and Shock, pp. 133-134.

Question 1346: TEL-AML1 fusion is associated with?

A. CLL
B. CML
C. AML
D. ALL (Correct Answer)

Explanation: ***ALL*** - The **TEL-AML1 fusion gene** (also known as ETV6-RUNX1) is a common chromosomal abnormality found in approximately 25% of cases of **B-cell precursor acute lymphoblastic leukemia (ALL)**, particularly in children. - This fusion creates an abnormal protein that interferes with normal blood cell development, leading to the uncontrolled proliferation of immature lymphocytes characteristic of ALL. *CLL* - **Chronic lymphocytic leukemia (CLL)** is a cancer of mature lymphocytes, specifically B cells, and is not associated with the TEL-AML1 fusion. - Typical genetic abnormalities in CLL include deletions or mutations in chromosomes 13q, 17p, 11q, and trisomy 12. *CML* - **Chronic myeloid leukemia (CML)** is characterized by the presence of the **Philadelphia chromosome (BCR-ABL1 fusion gene)**, which results from a translocation between chromosomes 9 and 22. - CML is a myeloproliferative neoplasm affecting myeloid stem cells, distinct from the lymphoid origin of ALL. *AML* - **Acute myeloid leukemia (AML)** involves the myeloid lineage and is associated with various chromosomal translocations and mutations, but **TEL-AML1 fusion is not characteristic of AML**. - Common genetic alterations in AML include t(8;21), inv(16), t(15;17) (PML-RARA), and mutations in FLT3, NPM1, or CEBPA.

Question 1347: Which oncogene is associated with Burkitt's lymphoma?

A. C-MYC (Correct Answer)
B. BCL-1 (IgH)
C. BCL-2 (IgH)
D. Anaplastic Lymphoma Kinase (ALK)

Explanation: ***C-MYC*** - Burkitt's lymphoma is associated with the translocation involving the **C-MYC gene**, which drives cell proliferation [1]. - This oncogene is typically involved in the **t(8;14)** translocation, linking C-MYC to the immunoglobulin heavy chain locus [1]. *ALK* - The **ALK gene** is primarily associated with anaplastic large cell lymphoma and is not linked to Burkitt's lymphoma. - ALK rearrangements lead to mutations that result in uncontrolled growth in other lymphomas, but not in Burkitt's. *BCL-1, IgH* - **BCL-1** (also known as CCND1) is associated with mantle cell lymphoma and is linked to the **t(11;14)** translocation, not Burkitt's. - It primarily involves cyclin D1 overexpression in the tumor pathogenesis distinct from Burkitt's behavior. *BCL-2, IgH* - The **BCL-2 gene** is commonly associated with follicular lymphoma and contributes to anti-apoptotic functions, unrelated to Burkitt's. - Burkitt's typically shows rapid proliferation and high apoptosis, unlike the mechanisms driven by BCL-2. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Neoplasia, pp. 324-325.

Question 1348: Left shift in Arneth's count is seen in?

A. Septicemia (Correct Answer)
B. Megaloblastic anemia
C. Liver disease
D. TB

Explanation: ***Septicemia*** - A **left shift** indicates a higher proportion of **immature neutrophils (bands or metamyelocytes)** in the blood, which is a hallmark of the body rapidly producing white blood cells to fight severe bacterial infections like **septicemia**. - This response is due to overwhelming bacterial load and systemic inflammation, prompting the bone marrow to release less mature neutrophils into circulation. *Megaloblastic anemia* - Characterized by **hypersegmented neutrophils** (a right shift), not a left shift, due to impaired DNA synthesis and delayed nuclear maturation. - The primary defect is in red blood cell production, although white blood cell morphology can also be affected. *Liver disease* - Liver disease does not typically cause a left shift in the Arneth count; instead, it can lead to various hematological abnormalities, including **anemia**, **thrombocytopenia**, or altered coagulation factors. - Neutrophil counts might fluctuate, but a sustained left shift is not a characteristic feature. *TB* - Tuberculosis usually causes a mild to moderate **neutrophilic leukocytosis** and sometimes a **monocytosis**, but a significant **left shift** is not a characteristic feature unless there is a severe acute exacerbation or secondary bacterial infection. - Chronic infections like TB are more often associated with changes like monocytosis or lymphocytosis.

Question 1349: Which of the following statements about thrombotic thrombocytopenic purpura is false?

A. Microangiopathic hemolytic anemia
B. Thrombocytopenia
C. Normal complement level
D. Grossly abnormal coagulation tests (Correct Answer)

Explanation: ***Grossly abnormal coagulation tests*** - In thrombotic thrombocytopenic purpura (TTP), coagulation tests typically remain **normal**, which is a distinguishing factor from other conditions like disseminated intravascular coagulation (DIC) [1]. - The primary pathology in TTP is due to a deficiency in **ADAMTS13**, leading to the formation of large von Willebrand factor multimers without significant **coagulation abnormalities** [2]. *Normal complement level* - TTP is not associated with **complement system abnormalities**; often, complement levels are normal, unlike conditions such as **atypical hemolytic uremic syndrome**. - Increased activation or consumption of complement is not a typical feature in TTP, making this statement false. *Micro angiopathic hemolytic anemia* - Microangiopathic hemolytic anemia is a hallmark of TTP, resulting from the **shearing of erythrocytes** in the small vasculature due to thrombi formation [1,2]. - The presence of **schistocytes** on a peripheral blood smear is commonly observed in this condition, indicating this statement is true. *Thrombocytopenia* - TTP is characterized by severe **thrombocytopenia** resulting from the consumption of platelets during the formation of microthrombi [1,2]. - This condition often leads to **purpura** and increased bleeding tendencies due to low platelet counts, confirming this option is true. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 667-668. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, pp. 947-948.

Question 1350: Heinz bodies are removed by?

A. Macrophages (Correct Answer)
B. Lymphocytes
C. Neutrophils
D. Fibroblasts

Explanation: ***Macrophages*** - Macrophages, particularly those in the **spleen**, are responsible for pitting out Heinz bodies from red blood cells. - This process is part of the **extravascular hemolysis** that occurs when red blood cells containing Heinz bodies are prematurely destroyed. *Lymphocytes* - Lymphocytes are primary cells of the **adaptive immune system**, involved in recognizing and targeting specific pathogens or abnormal cells. - They do not play a role in the removal of intracellular inclusions like Heinz bodies from red blood cells. *Neutrophils* - Neutrophils are a type of **phagocyte** and a crucial component of the **innate immune system**, primarily involved in fighting bacterial and fungal infections. - Their function is mainly in acute inflammation and phagocytosing microbes, not in the removal of inclusions from red blood cells. *Fibroblasts* - Fibroblasts are responsible for producing the **extracellular matrix** and **collagen**, playing a critical role in wound healing and tissue repair. - They are not immune cells and are not involved in the removal of cellular debris or inclusions like Heinz bodies.

Practice by Chapter

Anemias: Classification and Approach

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Hemolytic Anemias

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Myeloproliferative Neoplasms

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Myelodysplastic Syndromes

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Acute Leukemias

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Chronic Leukemias

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Lymphomas and Lymphoid Neoplasms

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Plasma Cell Disorders

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Bleeding Disorders

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Thrombotic Disorders

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