Hematopathology Practice Questions

Q: All of the following are seen in polycythemia rubra vera except :

Decreased LAP Score. ***Decreased LAP Score*** - **LAP (Leukocyte Alkaline Phosphatase) score** is typically **increased or normal** in polycythemia vera. - A decreased LAP score is characteristic of **chronic myeloid leukemia (CML)**, which must be differentiated from polycythemia vera [2]. *Increased platelets* - **Thrombocytosis** (increased platelets) is a common feature of **polycythemia vera**, often contributing to vascular complications [1], [2]. - The unregulated proliferation of all myeloid cell lines, including megakaryocytes, leads to this increase [1], [3]. *Increased Vit B12 binding capacity (>9000 micromols/dL)* - Polycythemia vera often leads to **increased vitamin B12 levels** and **binding capacity** due to increased production of **transcobalamin I** by proliferating granulocytes. - While not a direct diagnostic criterion, it is a frequent finding supportive of the diagnosis. *Leucocytosis* - **Leukocytosis** (increased white blood cell count), particularly granulocytosis, is a common feature of polycythemia vera [1], [2]. - It results from the **clonal proliferation** of myeloid stem cells, leading to an overproduction of all myeloid lineage cells [1]. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 614-615. [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. 626-627. [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, pp. 627-628.

Q: Which of the following is NOT a genetic risk factor for leukemia?

Hemophilia A. ***Hemophilia A*** - Hemophilia A is an **X-linked recessive bleeding disorder** caused by deficiency of **Factor VIII** (clotting factor) [1]. - While it is a genetic condition, it is **NOT associated with increased risk of leukemia**. - Hemophilia A affects the coagulation cascade and causes bleeding tendency, but does not involve genomic instability or DNA repair defects that predispose to malignancy [1], [2]. *Incorrect: Ataxia telangiectasia* - This is an **autosomal recessive disorder** caused by mutations in the **ATM gene**, which is involved in DNA repair. - Individuals with ataxia telangiectasia have an increased risk of developing **acute lymphoblastic leukemia (ALL)** and lymphomas due to impaired DNA repair mechanisms and chromosomal instability. *Incorrect: Down syndrome* - Also known as **Trisomy 21**, this chromosomal disorder is the most common genetic condition associated with an increased risk of leukemia. - Individuals with Down syndrome have a **10- to 20-fold higher risk** of developing **acute myeloid leukemia (AML)** and **acute lymphoblastic leukemia (ALL)**, particularly a unique form called **transient myeloproliferative disorder (TMD)** in infancy, which can progress to AML. *Incorrect: None of the options* - This is incorrect because **Hemophilia A** is indeed NOT a genetic risk factor for leukemia, making it the correct answer to this "NOT" question. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 670-671. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 622-623.

Q: Hemolysis is predominantly intravascular in which of the following conditions?

Paroxysmal nocturnal hemoglobinuria. ***Paroxysmal nocturnal hemoglobinuria*** - **PNH** is characterized by a defect in the **PIG-A gene**, leading to a deficiency of **GPI-anchored proteins** like CD55 and CD59 on red blood cells [1]. - This deficiency makes the red blood cells susceptible to complement-mediated lysis, predominantly occurring **intravascularly** [1]. *Warm autoimmune hemolytic anemia* - This condition involves **IgG autoantibodies** binding to red blood cells, which are then primarily removed by **macrophages in the spleen** and liver (extravascular hemolysis) [2]. - The presence of **spherocytes** and a positive direct **antiglobulin test (DAT)** are characteristic [2]. *Cold autoimmune hemolytic anemia* - Involves **IgM autoantibodies** that bind to red blood cells at colder temperatures, often causing agglutination in the peripheral circulation. - While some complement activation and lysis can occur intravascularly, the primary mechanism involves **macrophages in the liver** clearing antibody-coated red cells (extravascular), or red cell destruction in the cooler acral areas. *Spherocytosis* - This is a condition of abnormal red blood cell shape due to defects in **cytoskeletal proteins** (e.g., spectrin, ankyrin), making them less deformable. - These rigid spherocytes are primarily trapped and destroyed by the **phagocytic cells in the spleen**, indicating an **extravascular hemolytic process** [2]. **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. 602-603.

Q: Why is citrate phosphate dextrose (CPD) better than acid citrate dextrose (ACD) for storage of blood?

Contains phosphate and dextrose. ***Contains phosphate and dextrose*** - CPD contains **phosphate**, which acts as a buffer and helps maintain crucial 2,3-bisphosphoglycerate (2,3-BPG) levels in red blood cells, improving oxygen delivery capacity. - The presence of **dextrose** provides a substrate for glycolysis, which is essential for ATP production and red blood cell viability during storage. - This combination allows CPD to extend blood storage life to approximately **35 days** compared to ACD's **21 days**. *Maintains pH stability during storage* - Both ACD and CPD help maintain pH stability due to their **citrate** content, which acts as an anticoagulant and buffer. - However, CPD's phosphate component offers superior buffering capacity, but pH maintenance alone is not the primary distinguishing advantage. - This is a shared characteristic of both solutions, not the key reason CPD is preferred. *Prevents hemolysis in stored blood* - Both CPD and ACD prevent hemolysis by chelating **calcium**, which prevents coagulation and maintains red blood cell integrity. - While both solutions successfully prevent hemolysis, this is not the distinguishing feature that makes CPD superior. - The primary advantage of CPD lies in its better support of red blood cell metabolism and viability through phosphate and dextrose. *Reduces metabolic activity in stored blood* - This is **incorrect** - the purpose of anticoagulant solutions is to preserve blood components, not to reduce metabolic activity. - The dextrose in CPD is provided precisely to **fuel essential metabolic activity** (glycolysis) to sustain red blood cells during storage. - While refrigeration at 1-6°C slows metabolism, CPD actively supports rather than reduces the metabolic processes necessary for RBC survival.

Q: Glanzmann thrombasthenia is due to defect in:-

Gp IIb/IIIa. ***Gp IIb/IIIa*** - Glanzmann thrombasthenia is a **rare, inherited bleeding disorder** characterized by a defect or deficiency in the **glycoprotein IIb/IIIa (Gp IIb/IIIa) complex** on the platelet surface [1]. - This complex is crucial for platelet aggregation as it acts as the receptor for **fibrinogen**, which links activated platelets together [1]. *Gp VI* - **Glycoprotein VI (Gp VI)** is a collagen receptor on platelets, important for initial **platelet adhesion and activation** at sites of vascular injury. - Defects in Gp VI are associated with milder bleeding disorders, not Glanzmann thrombasthenia. *Thromboxane A2* - **Thromboxane A2 (TXA2)** is a potent **vasoconstrictor** and **platelet aggregator** synthesized by platelets. - Disorders in TXA2 synthesis or response, such as aspirin-induced platelet dysfunction, cause bleeding but are biochemically distinct from Glanzmann thrombasthenia. *Gp Ia/IIa* - The **glycoprotein Ia/IIa (Gp Ia/IIa) complex** (also known as integrin ̡2̢1) is another **collagen receptor** on platelets, mediating platelet adhesion to collagen. - Defects in Gp Ia/IIa lead to a different type of mild bleeding disorder, affecting initial adhesion rather than aggregation. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 668-669.

Q: Which of the following is true about factor V leiden mutation?

Factor V becomes resistant to cleavage by protein C. **Factor V becomes resistant to cleavage by protein C** - The **Factor V Leiden mutation** involves a substitution in the Factor V gene, rendering the **activated Factor V (FVa)** resistant to inactivation by **activated protein C (APC)** [1]. - This resistance leads to prolonged activity of FVa, which is a cofactor in the formation of thrombin, thereby increasing the risk of **thrombosis** [1]. *Increased bleeding tendency* - The Factor V Leiden mutation leads to a **hypercoagulable state**, which means an **increased tendency to form blood clots**, not an increased bleeding tendency [1]. - Bleeding disorders are typically associated with deficiencies or dysfunctions in clotting factors or platelets that impair clot formation. *Deficiency of factor V* - The Factor V Leiden mutation is a **gain-of-function mutation** that results in a modified, more active Factor V, not a deficiency of Factor V [1]. - Deficiency of Factor V (known as **parahemophilia**) is a rare bleeding disorder. *Arginine to glycine substitution* - The Factor V Leiden mutation specifically involves a single nucleotide polymorphism leading to an **arginine to glutamine substitution at position 506** (Arg506Gln) in the Factor V protein [1]. - This specific amino acid change alters the cleavage site for activated protein C, making it less effective at inactivating Factor V [1]. **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.

Q: Tumor cells in chronic lymphocytic leukemia or small lymphoblastic lymphoma (CLL/SLL) arise from which of the following?

Mature B cell. ***Mature B cell*** - CLL/SLL is characterized by the accumulation of **monoclonal B lymphocytes** that are morphologically mature but functionally incompetent. [1] - These malignant cells exhibit a mature immunophenotype, expressing **CD5, CD19, CD20 (dim), and CD23**, indicating their origin from a post-germinal center or memory B cell lineage. [1] *Naive B cell* - Naive B cells are typically found circulating in the blood and lymph nodes; while they are B cells, the specific immunophenotype and genomic features of CLL/SLL cells point to a more differentiated origin. - Although CLL/SLL cells express some markers of naive B cells, their overall profile, particularly the expression of **CD23**, is more consistent with a mature or activated B cell stage. [1] *Centrocytes of germinal center* - Malignancies arising from centrocytes of germinal centers often include **follicular lymphoma**, which presents with distinct clinical and immunophenotypic features from CLL/SLL. - Centrocytes are typically involved in somatic hypermutation and class switching, processes relevant to later stage B cell development, but not the direct origin of CLL/SLL. *Progenitor B-cell* - Progenitor B-cells are early developmental stages of B cells, and their malignant transformation leads to conditions like **B-cell acute lymphoblastic leukemia (B-ALL)**. [1] - B-ALL presents with immature blast cells and a different immunophenotype (e.g., lack of CD23), distinct from the mature lymphoid cells seen in CLL/SLL. [1] **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. 596-598.

Q: Which of the following anticoagulant preservatives can be used to store blood, so that it can be kept for 35 days?

Citrate phosphate dextrose-adenine (CPD-A). ***Citrate phosphate dextrose-adenine (CPD-A)*** - **CPD-A** is the most common anticoagulant preservative used for whole blood storage, allowing it to be stored for up to **35 days**. - The addition of **adenine** provides a substrate for red blood cell metabolism, helping to maintain **ATP levels** and extend viability. *Acid citrate dextrose (ACD)* - **ACD** was an earlier anticoagulant solution that allowed blood to be stored for **21 days**. - While effective, it does not support red blood cell viability for as long as CPD-A due to the lack of **adenine**. *CP2D - citrate phosphate double dextrose* - **CP2D** is similar to CPD but contains **double the amount of dextrose**, which can slightly improve red blood cell viability. - However, CP2D typically allows for blood storage of **21 days**, not the 35 days provided by CPD-A. *CPD Citrate phosphate dextrose* - **CPD** was an advancement over ACD, allowing blood to be stored for **21 days**. - While it improved upon ACD, it does not contain **adenine**, which is crucial for extending storage to **35 days**.

Question 1

All of the following are seen in polycythemia rubra vera except :

Accepted Answer

Decreased LAP Score

Answer

Increased platelets

Answer

Increased Vit B12 binding capacity (>9000 micromols/dL)

Answer

Leucocytosis

Question 2

Which of the following is NOT a genetic risk factor for leukemia?

Accepted Answer

Hemophilia A

Answer

Ataxia telangiectasia

Answer

Down's syndrome

Answer

None of the options

Question 3

Bleeding time assesses

Accepted Answer

Function of platelets

Answer

Fibrinogen Level

Answer

Extrinsic clotting pathway

Answer

Intrinsic clotting pathway

Question 4

Which of the following is positive in Follicular lymphoma?

Accepted Answer

Bcl-2 protein

Answer

Cyclin D1 (Bcl-1)

Answer

Bcl-10 signaling protein

Answer

Bcl-6 transcription factor

Question 5

Hemolysis is predominantly intravascular in which of the following conditions?

Accepted Answer

Paroxysmal nocturnal hemoglobinuria

Answer

Warm autoimmune hemolytic anemia

Answer

Cold autoimmune hemolytic anemia

Answer

Spherocytosis

Question 6

Why is citrate phosphate dextrose (CPD) better than acid citrate dextrose (ACD) for storage of blood?

Accepted Answer

Contains phosphate and dextrose

Answer

Maintains pH stability during storage

Answer

Prevents hemolysis in stored blood

Answer

Reduces metabolic activity in stored blood

Question 7

Glanzmann thrombasthenia is due to defect in:-

Accepted Answer

Gp IIb/IIIa

Answer

Gp VI

Answer

Thromboxane A2

Answer

Gp Ia/IIa

Question 8

Which of the following is true about factor V leiden mutation?

Accepted Answer

Factor V becomes resistant to cleavage by protein C

Answer

Increased bleeding tendency

Answer

Deficiency of factor V

Answer

Arginine to glutamine substitution

Question 9

Tumor cells in chronic lymphocytic leukemia or small lymphoblastic lymphoma (CLL/SLL) arise from which of the following?

Accepted Answer

Mature B cell

Answer

Naive B cell

Answer

Centrocytes of germinal center

Answer

Progenitor B-cell

Question 10

Which of the following anticoagulant preservatives can be used to store blood, so that it can be kept for 35 days?

Accepted Answer

Citrate phosphate dextrose-adenine (CPD-A)

Answer

Acid citrate dextrose (ACD)

Answer

CP2D - citrate phosphate double dextrose

Answer

CPD Citrate phosphate dextrose

Hematopathology — MCQs

Hematopathology — MCQs

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