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 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 1346: Flow cytometry is primarily used for analysis of which of the following cell types?

A. Lymphocytes (Correct Answer)
B. Platelets
C. Erythrocytes
D. Basophils

Explanation: ***Lymphocytes*** - Flow cytometry is excellently suited for **lymphocyte analysis** due to their distinct surface markers (CD antigens) that can be labeled with fluorescent antibodies. - It allows for the **identification and quantification of various lymphocyte subsets** (e.g., T cells, B cells, NK cells), crucial in diagnosing immunodeficiencies, autoimmune diseases, and hematologic malignancies. *Erythrocytes* - While flow cytometry can detect erythrocytes, their primary role is oxygen transport, and they **lack cell surface markers** commonly analyzed by flow cytometry for classification. - **Complete blood count (CBC)** is the standard method for erythrocyte quantification and morphological analysis. *Platelets* - Flow cytometry can be used to study platelet activation and surface markers, but it's not their **primary or routine analytical tool** for mere count or general assessment. - **Automated hematology analyzers** are routinely used for platelet counts and basic morphology. *Basophil* - Basophils are a type of granulocyte and can be identified by flow cytometry, but they are a **very small percentage of circulating leukocytes**, making them less commonly the *primary* target of a typical flow cytometry panel focused on overall leukocyte populations. - While they can be analyzed, lymphocytes offer a much **broader range of clinical utility** due to their diverse subpopulations and roles in immunity and disease.

Question 1347: 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 1348: 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 1349: Richter's syndrome refers to which of the following malignant transformations?

A. CLL evolving into aggressive lymphoma (Correct Answer)
B. Hairy cell leukemia evolving to AML
C. Blast crisis in CML
D. Splenic infiltration in NHL

Explanation: ***CLL evolving into aggressive lymphoma*** - **Richter's transformation** specifically describes the malignant transformation of Chronic Lymphocytic Leukemia (CLL) into a more aggressive form of B-cell non-Hodgkin lymphoma, most commonly **diffuse large B-cell lymphoma (DLBCL)**. - This transformation is characterized by a rapid clinical decline, palpable lymphadenopathy, and splenomegaly, with a distinct change in morphology and immunophenotype of the malignant cells. *Hairy cell leukemia evolving to AML* - Hairy cell leukemia (HCL) is a chronic lymphoproliferative disorder that rarely transforms into secondary malignancies; however, transformation into **acute myeloid leukemia (AML)** is not a hallmark of HCL and is not referred to as Richter's syndrome. - HCL is characterized by pancytopenia and distinctive hairy-looking lymphocytes, distinct from the progression pattern seen in Richter's. *Blast crisis in CML* - **Blast crisis** is the terminal phase of chronic myeloid leukemia (CML), where the number of myeloid blasts in the blood or bone marrow increases to >20%. - This is a progression of CML, a myeloproliferative neoplasm, and is distinct from the lymphoid transformation described by Richter's syndrome. *Splenic infiltration in NHL* - While non-Hodgkin lymphoma (NHL) can involve the spleen, **splenic infiltration** itself does not describe a malignant transformation syndrome like Richter's. - Richter's syndrome describes a transformation *from* CLL *to* a more aggressive lymphoma, not just a site of involvement for NHL.

Question 1350: Which condition is most commonly associated with basophilic leukocytosis?

A. Acute Myeloid Leukemia (AML)
B. Acute Lymphoblastic Leukemia (ALL)
C. Chronic Myeloid Leukemia (CML) (Correct Answer)
D. Chronic Lymphocytic Leukemia (CLL)

Explanation: ***Chronic Myeloid Leukemia (CML)*** - **Basophilic leukocytosis** is a characteristic feature of **CML**, resulting from the clonal expansion of myeloid stem cells. - The presence of **immature granulocytes**, including basophils, is key to diagnosing CML, particularly in the chronic phase. *Acute Myeloid Leukemia (AML)* - AML is characterized by an excessive proliferation of **myeloblasts** (immature myeloid cells) in the bone marrow and peripheral blood, not mature basophils. - While other myeloid lineages can be affected, basophilic leukocytosis is not a defining or common feature. *Acute Lymphoblastic Leukemia (ALL)* - ALL involves the uncontrolled proliferation of **lymphoblasts** (immature lymphoid cells). - This condition is specifically associated with the lymphoid lineage and does not typically cause an increase in myeloid cells like basophils. *Chronic Lymphocytic Leukemia (CLL)* - CLL is characterized by the accumulation of **mature, but dysfunctional, B lymphocytes**. - It involves the lymphoid lineage and does not lead to basophilic leukocytosis, which is a feature of myeloid disorders.

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

Practice Questions

Plasma Cell Disorders

Practice Questions

Bleeding Disorders

Practice Questions

Thrombotic Disorders

Practice Questions

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