Hematopathology Practice Questions

Q: In which subtype of Hodgkin's disease are popcorn cells predominantly found?

Lymphocyte predominant subtype. ***Lymphocyte predominant*** - Popcorn cells, or **"L&H" (Lymphocytic and Histiocytic) cells**, are characteristic of lymphocyte predominant Hodgkin's disease, indicating a **rich lymphocytic background** [1]. - This subtype is more commonly seen in younger patients and is associated with a better prognosis relative to other types of Hodgkin's lymphoma [1]. *Mixed cellularity* - This subtype is characterized by a **varied cellular composition** including numerous Reed-Sternberg cells but does not specifically feature popcorn cells [2]. - It tends to have a higher number of **eosinophils** and **plasma cells** rather than lymphocytes predominating [2]. *Lymphocyte depletion* - Lymphocyte depletion is marked by a **deficient lymphocytic component** and an abundance of Reed-Sternberg cells, lacking the characteristic popcorn cells. - This variant is often more aggressive and typically has a poorer prognosis. *Nodular sclerosis* - Nodular sclerosis is recognized by the presence of **collagen bands** separating lymphoid nodules and also does not include popcorn cells. - This form is the most common variant of Hodgkin's lymphoma and often presents with **mediastinal masses**. **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. 618. [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. 616-618.

Q: What is the defect in Glanzmann's thrombasthenia?

GpIIb-IIIa. ***Gp1Ib-IlIa*** - Glanzmann's thrombasthenia is characterized by a defect in the **Gp1b-IlIa complex**, which is essential for platelet aggregation. - Patients with this condition exhibit **prolonged bleeding time** and poor clot formation due to ineffective platelet-fibrinogen binding. *GpIIIa-I lb* - This option refers to a component of the GpIIb-IIIa complex, which is primarily involved in **platelet aggregation** and is not the defect in Glanzmann's thrombasthenia. - Defects in GpIIb-IIIa lead to conditions such as **Glanzmann's thrombasthenia** [1], but specifically, the Gp1b-IlIa complex is implicated. *Gpfilb-I la* - This designation does not correspond correctly to either a known receptor complex related to platelet function or to Glanzmann's thrombasthenia. - Mislabeling of the glanzmann's complex results in confusion as it does not specify the **correct interaction involved in platelet aggregation**. *GpIla-Illb* - This combination refers to components that are not involved in Glanzmann's thrombasthenia, which specifically involves the **Gp1b-IlIa interaction**. - GpIla-Illb complex is part of the **integrin family**, but its defect does not lead to the bleeding problems seen in Glanzmann's thrombasthenia [1]. **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: A child presents with intermittent jaundice and splenomegaly. There is a history of similar complaints in the elder brother. Peripheral smear shows the following finding. What is the most appropriate investigation for this condition?

Osmotic fragility test. ***Osmotic fragility test*** - The image shows **spherocytes** (dense, small red cells lacking central pallor), a hallmark of **hereditary spherocytosis**. The clinical picture of intermittent jaundice, splenomegaly, and a family history further supports this diagnosis. - The **osmotic fragility test** measures the red blood cell's susceptibility to hemolysis in hypotonic solutions and is the most appropriate test to confirm hereditary spherocytosis. *Coombs test* - The Coombs test (direct antiglobulin test) detects **antibodies on the surface of red blood cells** or in the serum. It is primarily used to diagnose **autoimmune hemolytic anemia**. - Hereditary spherocytosis is a membrane defect, not an immune-mediated condition, so the Coombs test would typically be negative. *G6PD deficiency testing* - **Glucose-6-phosphate dehydrogenase (G6PD) deficiency** leads to episodic hemolytic anemia triggered by oxidative stress (e.g., fava beans, certain drugs, infections). - While it causes episodic hemolysis and jaundice, the peripheral smear typically shows **Heinz bodies** and **bite cells** during hemolytic episodes, not spherocytes, and without specific triggers, it doesn't fit the clinical picture as well as hereditary spherocytosis. *Flow cytometry for PNH* - **Flow cytometry** is used to diagnose **paroxysmal nocturnal hemoglobinuria (PNH)** by detecting the absence of GPI-anchored proteins (e.g., CD55, CD59) on blood cells. - PNH is characterized by dark urine (hemoglobinuria), thrombosis, and bone marrow failure, and its peripheral smear does not typically show spherocytes.

Q: A child presents with recurrent chest infections and abdominal pain. There is a history of 1 blood transfusion in the past. On examination, he had icterus and mild splenomegaly. Electrophoresis shows increased HbA2, HbF, and S spike. What is the likely diagnosis?

Sickle Beta Thalassemia. ***Sickle Beta Thalassemia*** - The combination of **sickle cell disease manifestations** (recurrent chest infections, abdominal pain, icterus, splenomegaly) with **electrophoresis showing increased HbA2, elevated HbF, and S spike** is diagnostic of **Sickle Beta Thalassemia**. - **Increased HbA2 (>3.5%)** is the key distinguishing feature that differentiates this from pure sickle cell disease. It indicates co-inheritance of a **beta-thalassemia gene** along with the **sickle cell gene**. - Clinical presentation is similar to sickle cell disease with **vaso-occlusive crises**, **acute chest syndrome**, hemolytic anemia, and organomegaly. - The severity depends on the type: S/β⁰-thalassemia (no HbA production) is clinically more severe and similar to SS disease, while S/β⁺-thalassemia (reduced HbA) tends to be milder. *Sickle cell disease* - Pure sickle cell disease (HbSS) presents with similar clinical features: recurrent chest infections, abdominal pain, hemolysis, and splenomegaly. - However, electrophoresis would show **normal or only slightly elevated HbA2 (2-3%)**, not the increased HbA2 mentioned in this case. - The presence of significantly increased HbA2 rules out pure sickle cell disease. *HbC disease* - Patients with HbC disease typically have **mild chronic hemolytic anemia** and **splenomegaly** but usually lack severe vaso-occlusive crises. - Electrophoresis would show primarily **HbC**, not an S spike. - The clinical picture is much milder than described in this case. *Aplastic anemia* - Characterized by **pancytopenia** due to bone marrow failure, leading to fatigue, infections, and bleeding tendency. - Does not involve hemolysis, icterus, or abnormal hemoglobin variants on electrophoresis. - The electrophoresis findings completely exclude this diagnosis.

Q: All trans retinoic acid is used in the treatment of acute promyelocytic leukemia (APL) associated with which genetic mutation?

PML-RARA fusion gene. ***PML-RARA fusion gene*** - The **PML-RARA fusion gene** is the hallmark genetic abnormality in **acute promyelocytic leukemia (APL)**, which results from a **t(15;17) chromosomal translocation** [1]. - **All-trans retinoic acid (ATRA)** targets the PML-RARA fusion protein, inducing differentiation of the immature promyelocytes and promoting remission in APL patients. *BCR-ABL fusion gene* - The **BCR-ABL fusion gene** is associated with **chronic myeloid leukemia (CML)**, resulting from the **Philadelphia chromosome (t(9;22))** [2]. - While a target for therapy, it is treated with **tyrosine kinase inhibitors (TKIs)**, not ATRA [2]. *CMYC gene alteration* - **CMYC alterations**, such as translocations, are characteristic of **Burkitt lymphoma** and some cases of diffuse large B-cell lymphoma [2]. - These alterations are not specifically associated with APL or sensitive to ATRA therapy. *CEBPA mutation* - **CEBPA mutations** are found in a subset of patients with **acute myeloid leukemia (AML)**, particularly those with a normal karyotype. - While relevant to AML prognosis and treatment stratification, these mutations do not define APL or respond to ATRA.

Q: Which marker is commonly associated with positivity in follicular lymphoma?

Bcl-2. ***Bcl-2*** - **Follicular lymphoma** is characterized by the overexpression of the **Bcl-2 protein**, which inhibits apoptosis, leading to the survival of malignant B cells [1][3]. - The **Bcl-2 gene** is often involved in the **t(14;18)** chromosomal translocation, which is a hallmark of this lymphoma [1][3][4]. *Bcl-6* - Although **Bcl-6** can be expressed in some lymphomas, it is primarily associated with **diffuse large B-cell lymphoma**, not follicular lymphoma. - **Bcl-6** is involved in **germinal center formation** and its positivity does not indicate follicular lymphoma specifically. *Bcl-1* - **Bcl-1** (also known as **CCND1**) is primarily associated with **mantle cell lymphoma** and is not a characteristic marker for follicular lymphoma. - It is linked to the **t(11;14)** translocation, which is distinct from the genetic alterations seen in follicular lymphoma. *None of the above* - This option is incorrect as **Bcl-2 positivity** is definitive for follicular lymphoma [2]. - The presence of other markers like **Bcl-6** or **Bcl-1** does not negate the expression of Bcl-2 in this lymphoma type. **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. 602-604. [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, p. 604. [3] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Diseases Of The Urinary And Male Genital Tracts, pp. 561-562. [4] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Neoplasia, pp. 310-311.

Q: Which disease is associated with the CD59 marker?

PNH. ***PNH*** - The **CD59 marker** is associated with **Paroxysmal Nocturnal Hemoglobinuria (PNH)**, a condition characterized by the loss of glycosylphosphatidylinositol (GPI) anchored proteins [1]. - It protects red blood cells from **complement-mediated lysis**, and its absence leads to hemolysis and thrombosis in patients with PNH [1,4]. *PTEN* - The **PTEN gene** is a tumor suppressor associated with various cancers and is not related to CD59. - It is primarily involved in the **regulation of the Akt signaling pathway**, not in complement regulation. *BRR* - **BRR (Birt-Hogg-Dubé syndrome)** is linked to folliculin and does not involve CD59. - This genetic condition is characterized by **skin tumors** and renal tumors, unrelated to the complement system. *Cowden syndrome* - **Cowden syndrome** is associated with mutations in the PTEN gene, relating to **hamartomas** and breast cancer risk, not CD59. - It affects multiple systems but does not involve **complement regulatory proteins** like CD59. **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.

Q: TRALI occurs within how many hours of transfusion?

6 hours. ***6 hours*** - **Transfusion-related acute lung injury (TRALI)** is defined as new acute lung injury occurring during or within **6 hours** after the completion of a blood transfusion [1]. - It is a severe and potentially life-threatening transfusion reaction characterized by **acute respiratory distress**, **hypoxemia**, and **bilateral pulmonary infiltrates** on chest imaging [1]. *48 hours* - While other transfusion reactions or complications may manifest within 48 hours, TRALI has a more **acute onset**, typically within the first 6 hours. - A pulmonary event occurring between 6 and 48 hours post-transfusion might be considered **delayed TRALI** or another diagnosis like **transfusion-associated circulatory overload (TACO)**, but the classic definition refers to the 6-hour window. *72 hours* - Reactions occurring 72 hours after transfusion are generally considered **delayed transfusion reactions**, which include conditions like **delayed hemolytic transfusion reactions** or **post-transfusion purpura**. - This timeframe is too long for the typical presentation of TRALI, which is characterized by rapid onset. *12 hours* - Although 12 hours falls within an acute window, the most commonly accepted and diagnostically crucial timeframe for TRALI is **within 6 hours** of transfusion. - A reaction occurring between 6 and 12 hours would still be considered suspiciously TRALI, but the strict definition emphasizes the earlier onset.

Q: Whole blood is used as a sample for which test?

Complete Blood Count (CBC). **Complete Blood Count (CBC)** - A **CBC** directly analyzes the cellular components of blood (red blood cells, white blood cells, and platelets) which are found suspended in **whole blood**. [1] - It provides information on cell counts, sizes, and hemoglobin concentration, requiring the complete, unaltered blood mixture for accurate results. [3] *Interferon Gamma Release Assay (IGRA)* - IGRAs typically use **whole blood** in specialized collection tubes, but they are not measuring components *within* the whole blood itself. - Instead, they detect **interferon-gamma release** from T-cells stimulated by specific antigens, a cellular immune response assessed in a stimulated blood sample. *HIV ELISA* - **ELISA** (Enzyme-Linked Immunosorbent Assay) for HIV primarily detects **antibodies** or **antigens** in the blood. - For this, **plasma** or **serum** (the liquid components of blood after cells have been removed) is typically preferred over whole blood. *Blood Culture for Bacterial Infections* - **Blood cultures** require whole blood to be inoculated directly into specific culture media. [2] - The goal is to grow and identify **live bacteria** circulating in the bloodstream, making whole blood essential, but it is incorrect when compared to the direct cellular analysis of a CBC.

Q: In which type of Hodgkin's lymphoma are classical Reed-Sternberg cells most characteristically observed?

Mixed cellularity Hodgkin. ***Lymphocyte predominance*** - The **Hodgkin's lymphoma (HL) lymphocyte predominance** variant characteristically displays a predominance of lymphocytes in the cellular makeup [1]. - This subtype is often associated with a better prognosis and fewer symptoms than other types of HL [1]. *Lymphocyte depleted* - This subtype features a significant decrease in lymphocytes, leading to a **higher proportion of Reed-Sternberg cells** [3]. - It typically presents with a more aggressive clinical course, which contrasts with lymphocyte predominance [3]. *Mixed cellularity hodgkin* - Mixed cellularity shows a variety of cell types, including a significant number of **Reed-Sternberg cells**, but does not demonstrate **lymphocyte predominance** [2]. - This subtype is generally found in older patients and associated with advanced disease, unlike lymphocyte predominance [2]. *Nodular sclerosis* - Nodular sclerosis subtype is characterized by **collagen bands** and a particular architecture that is distinct from lymphocyte predominance [2]. - It primarily affects younger patients and can often involve mediastinal lymph nodes; however, it does not have the features of lymphocyte predominance [2]. **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. 618. [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. 616-618. [3] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Diseases Of The Urinary And Male Genital Tracts, pp. 559-560.

Question 1

In which subtype of Hodgkin's disease are popcorn cells predominantly found?

Accepted Answer

Lymphocyte predominant subtype

Answer

Nodular sclerosis subtype

Answer

Mixed cellularity subtype

Answer

Lymphocyte depletion subtype

Question 2

What is the defect in Glanzmann's thrombasthenia?

Accepted Answer

GpIIb-IIIa

Answer

GpIIIa-Ib

Answer

GpIIa-IIIb

Answer

GpIIb-Ia

Question 3

A child presents with intermittent jaundice and splenomegaly. There is a history of similar complaints in the elder brother. Peripheral smear shows the following finding. What is the most appropriate investigation for this condition?

Accepted Answer

Osmotic fragility test

Answer

Coombs test

Answer

G6PD deficiency testing

Answer

Flow cytometry for PNH

Question 4

A child presents with recurrent chest infections and abdominal pain. There is a history of 1    blood transfusion in the past. On examination, he had icterus and mild splenomegaly.    Electrophoresis shows increased HbA2, HbF, and S spike. What is the likely diagnosis?

Accepted Answer

Sickle Beta Thalassemia

Answer

HbC disease

Answer

Sickle cell disease

Answer

Aplastic anemia

Question 5

All trans retinoic acid is used in the treatment of acute promyelocytic leukemia (APL) associated with which genetic mutation?

Accepted Answer

PML-RARA fusion gene

Answer

BCR-ABL fusion gene

Answer

CMYC gene alteration

Answer

CEBPA mutation

Question 6

Which marker is commonly associated with positivity in follicular lymphoma?

Accepted Answer

Bcl-2

Answer

Bcl-1

Answer

Bcl-6

Answer

None of the options

Question 7

Which disease is associated with the CD59 marker?

Accepted Answer

PNH

Answer

Cowden syndrome

Answer

Bannayan-Riley-Ruvalcaba syndrome

Answer

PTEN hamartoma tumor syndrome

Question 8

TRALI occurs within how many hours of transfusion?

Accepted Answer

6 hours

Answer

48 hours

Answer

72 hours

Answer

12 hours

Question 9

Whole blood is used as a sample for which test?

Accepted Answer

Complete Blood Count (CBC)

Answer

Interferon Gamma Release Assay (IGRA)

Answer

HIV ELISA

Answer

Blood Culture for Bacterial Infections

Question 10

In which type of Hodgkin's lymphoma are classical Reed-Sternberg cells most characteristically observed?

Accepted Answer

Mixed cellularity Hodgkin

Answer

Lymphocyte depleted

Answer

Nodular sclerosis

Answer

Lymphocyte predominance

Hematopathology — MCQs

Hematopathology — MCQs

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