Hematopathology Practice Questions

Q: Which test quantifies fetal hemoglobin in maternal blood using acid elution?

Kleihauer-Betke test. ***Kleihauer-Betke test*** - This test uses **acid elution** to quantify **fetal hemoglobin (HbF)**, which is resistant to acid, allowing it to remain in red cells while adult hemoglobin (HbA) elutes out. - It's primarily used to detect and quantify **fetomaternal hemorrhage**, guiding the dose of Rh immune globulin [2]. *Hemoglobin electrophoresis* - This method separates different **hemoglobin types** based on their electrical charge, useful for diagnosing hemoglobinopathies like sickle cell disease or thalassemia [1]. - While it can identify HbF, it is not the primary method for quantifying small amounts of fetal blood in maternal circulation after acid elution. *Complete Blood Count* - Provides information on red blood cell count, hemoglobin concentration, hematocrit, and other indices, but does not specifically quantify **fetal hemoglobin**. - It's a general screen for anemia or other blood disorders, not designed for differential hemoglobin analysis. *Peripheral Blood Smear* - Allows for microscopic examination of blood cell morphology, identifying abnormal red cell shapes or inclusions and estimating the proportions of different cell types. - It does not quantify specific types of hemoglobin, nor does it involve acid elution to differentiate fetal from adult red cells. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 649-650. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of Infancy and Childhood, pp. 469-470.

Q: All are true about polycythemia vera except one.

Increased erythropoietin. ***Increased erythropoietin*** - Polycythemia vera is characterized by **low erythropoietin levels** due to the autonomous proliferation of erythroid progenitor cells in the bone marrow [3]. - The disease is associated with a **JAK2 mutation**, which leads to erythrocytosis independent of erythropoietin stimulation [2,3]. *Increased LAP Score* - The LAP (leukocyte alkaline phosphatase) score is often **increased** in polycythemia vera due to increased leukocyte activity. - This test helps differentiate it from **essential thrombocythemia**, which typically has a normal or low LAP score. *Decreased erythropoietin* - Polycythemia vera generally exhibits **decreased erythropoietin levels** because of the negative feedback from increased red blood cell mass [3]. - Patients typically show a lack of normal response to hypoxia, which is observed in secondary causes of erythrocytosis [3]. *Increased ESR* - The erythrocyte sedimentation rate (ESR) can be **increased** in polycythemia vera due to elevated levels of multiple proteins in the blood. - This non-specific marker often reflects **inflammation** or other hematological conditions. **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. Red Blood Cell and Bleeding Disorders, pp. 663-664.

Q: Classical markers for Hodgkin's disease are

CD 15 and CD 30. ***CD 15 and CD 30*** - These are **classical markers** specific for Reed-Sternberg cells, which are characteristic of Hodgkin's lymphoma [1][2]. - The presence of both markers is essential for **diagnosing Hodgkin's disease** and differentiating it from non-Hodgkin lymphomas [1]. *CD 20 and CD 30* - **CD 20** is primarily associated with B-cell non-Hodgkin lymphomas and is not a classical marker for Hodgkin's disease. - Hodgkin's lymphoma typically does not express **CD 20**, making this combination inappropriate for diagnosis. *CD 15 and CD 20* - While **CD 15** is a classical marker for Hodgkin's, **CD 20** is linked to B-lineage cells, which are not prominently present in Hodgkin's disease. - Diagnosis relies on the presence of **CD 30** alongside **CD 15**, highlighting the key difference. *CD 15 and CD 22* - Although **CD 15** is associated with Hodgkin's disease, **CD 22** is not a marker indicative of this lymphoma type. - **CD 22** is more relevant to B-cell malignancies; thus, it is not part of the classical markers for Hodgkin's lymphoma. **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. 614-616. [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. 616.

Q: Which of the following is not a B-cell marker?

CD 34. ***CD 19*** - CD 19 is a core B-cell marker crucial for B-cell development and differentiation in the immune response [1]. - It is universally present on all B-cell lineages, making it a reliable marker for identifying B-cells [1]. *CD 10* - CD 10, also known as the common acute lymphoblastic leukemia antigen (CALLA), is not exclusive to B-cells but can be found in **various cell types**, including early B-cells and T-cells [1]. - It is primarily used as a marker in certain leukemias rather than a definitive B-cell marker. *CD 34* - CD 34 is a marker for **hematopoietic stem cells** and progenitor cells, not specifically for B-cells. - While it can indicate early stages of B-cell lineages, it is not utilized as a definitive marker for B-cells themselves. *CD 20* - CD 20 is a well-established marker found on the surface of **mature B-cells** and is often targeted in therapies for B-cell malignancies [1]. - It plays a critical role in B-cell activation and differentiation, thus confirming it as a B-cell marker [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, p. 598.

Q: A 10-year-old boy presents with a palpable mass in the abdomen. On imaging, the para-aortic lymph nodes are found to be enlarged. A biopsy from the lymph node shows a starry sky appearance. What is the likely underlying abnormality?

cMYC gene translocation. ***Translocation involving cMYC gene*** - The **starry sky appearance** in a lymph node biopsy is often associated with **Burkitt lymphoma** [2], which is caused by a translocation involving the cMYC gene [1]. - This translocation leads to **overexpression of cMYC** [1], promoting rapid cellular proliferation characteristic of this aggressive lymphoma. *Translocation involving BCR-ABL genes* - This abnormality is associated with **Chronic Myeloid Leukemia (CML)**, not lymphoma, and does not present with a **starry sky appearance**. - In CML, the hallmark finding is the presence of **Philadelphia chromosome** and myeloid cell overproduction. *Rb tumor suppressor gene mutation* - Rb gene mutations are typically noted in **retinoblastoma** or osteosarcoma, not in lymphomas presenting with a starry sky pattern. - These tumors often have different clinical presentations and do not typically involve lymph nodes as a primary finding. *Tumor suppressor gene Tp53 mutation* - Mutations in the Tp53 gene are commonly involved in **various cancers**, including sarcomas and breast cancer, but are not linked to the **starry sky appearance** seen in lymphomas. - While critical for **cell cycle regulation**, Tp53 mutations do not directly correlate with the typical findings in Burkitt lymphoma. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Neoplasia, pp. 324-325. [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. 606.

Q: The most common translocation in acute promyelocytic leukemia (APML) is:

t(15;17). ***t (15:17)*** - The most common translocation in **acute promyelocytic leukemia (APML)** is the **t(15;17)**, which fuses the promyelocytic leukemia (PML) gene with the promyelocyte-retinoic acid receptor (RARA) gene [1]. - This genetic alteration is associated with a **specific clinical presentation** of APML, including bleeding diathesis and a high risk of coagulopathy [2]. *t (8:14)* - This translocation is commonly associated with **Burkitt lymphoma**, not APML. - It involves the **MYC gene**, leading to uncontrolled cell division, which is unrelated to the pathophysiology of APML. *t (8:21)* - Associated with **acute myeloid leukemia (AML)**, particularly the M2 subtype. - This translocation involves the **RUNX1 (AML1) gene** and does not pertain to APML's characteristic genetic changes. *t (9:22)* - Known as the **Philadelphia chromosome**, it is implicated in **chronic myeloid leukemia (CML)** and some types of ALL (acute lymphoblastic leukemia). - This translocation involves the **BCR-ABL fusion gene** which is distinctly different from the genetic abnormalities found in APML. **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. 620-622. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Neoplasia, p. 326.

Q: In which of the following conditions is an elevated leukocyte alkaline phosphatase (LAP) score typically observed?

Leukemoid reaction. ***Leukemoid reaction*** - A **leukemoid reaction** is characterized by a marked increase in the leukocyte count (usually >50,000/µL) due to a severe infection or inflammation, and is associated with a **high leukocyte alkaline phosphatase (LAP) score**. - This elevated LAP score helps differentiate it from **chronic myeloid leukemia (CML)**. *CML* - **Chronic myeloid leukemia (CML)** typically presents with a **low leukocyte alkaline phosphatase (LAP) score**, even with a high white blood cell count. - CML is a **myeloproliferative neoplasm** caused by the **Philadelphia chromosome (BCR-ABL1 fusion gene)**. *Eosinophilia* - **Eosinophilia** is an increase in the number of eosinophils, usually occurring in response to allergies, parasitic infections, or certain cancers. - It is not directly associated with changes in **alkaline phosphatase** levels. *Malaria* - **Malaria** is a parasitic infection that can cause various hematological changes, including anemia and thrombocytopenia. - It does not typically lead to an **elevated leukocyte alkaline phosphatase (LAP) score**.

Q: The size of the red blood cells is measured by?

Mean Corpuscular Volume (MCV). ***Mean Corpuscular Volume (MCV)*** - **MCV** is a direct measure of the average **volume** or size of individual **red blood cells**. - It is crucial for classifying anemias as **microcytic** (low MCV), **normocytic** (normal MCV), or **macrocytic** (high MCV). *Erythrocyte Sedimentation Rate (ESR)* - **ESR** measures the rate at which **red blood cells** settle in a tube in one hour, indicating the presence of **inflammation** or infection. - It is not a direct measure of **red blood cell size** but rather an indirect marker of systemic inflammation. *Mean Cell Hemoglobin (MCH)* - **MCH** indicates the average amount of **hemoglobin** in each individual **red blood cell**. - While related to size, it specifically quantifies the hemoglobin content, not the cell's volume or physical dimension. *Mean Corpuscular Hemoglobin Concentration (MCHC)* - **MCHC** measures the average concentration of **hemoglobin** within a given volume of **red blood cells**, reflecting the color intensity. - It helps determine if red blood cells are **hypochromic** (low MCHC) or normochromic, rather than directly measuring their size.

Q: Which genetic alteration is associated with a poor prognosis in acute myeloid leukemia?

Monosomy 7. ***Correct: Monosomy 7*** - **Monosomy 7** or **deletion 7q (del 7q)** are classified as **adverse/poor-risk cytogenetic abnormalities** in acute myeloid leukemia (AML) per the European LeukemiaNet (ELN) risk stratification - Consistently associated with **poor prognosis**, lower complete remission rates, and shorter overall survival - These alterations indicate **genomic instability** and often lead to **resistance to conventional chemotherapy** - Patients typically require consideration for **allogeneic stem cell transplantation** when eligible *Incorrect: Deletion of chromosome 5* - **Deletion 5q (del 5q)** or **monosomy 5** are also classified as adverse-risk abnormalities in AML - However, **monosomy 7** is more consistently cited as a marker for **very poor prognosis** in AML - Note: **Isolated 5q- syndrome** in myelodysplastic syndromes (MDS) has a better prognosis and responds to lenalidomide, but in AML context it indicates unfavorable outcome *Incorrect: t(8;21) translocation* - The **t(8;21)(q22;q22)** translocation is a **favorable-risk (core-binding factor AML)** genetic alteration [1] - Associated with **good prognosis** and high complete remission rates with standard chemotherapy [1] - Involves fusion of **RUNX1-RUNX1T1 genes** (previously AML1-ETO), creating a specific AML subtype with distinct morphology (often FAB M2 with abnormal eosinophils) [1] *Incorrect: Nucleophosmin mutation (NPM1)* - **NPM1 mutations** without concurrent **FLT3-ITD** (or with low FLT3-ITD allelic ratio) are classified as **favorable-risk** in AML [1] - Associated with **good response to intensive chemotherapy** and improved overall survival, particularly in younger patients with normal karyotype AML [1] - NPM1-mutated AML represents approximately 30% of adult AML cases [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, p. 620.

Question 1

Which test quantifies fetal hemoglobin in maternal blood using acid elution?

Accepted Answer

Kleihauer-Betke test

Answer

Complete Blood Count

Answer

Hemoglobin electrophoresis

Answer

Peripheral Blood Smear

Question 2

Which of the following statements is true about Russell bodies?

Accepted Answer

Eosinophilic inclusions found in plasma cells

Answer

Associated with multiple myeloma but not exclusive to it.

Answer

Not associated with any specific condition

Answer

Basophilic inclusions found in lymphocytes

Question 3

All are true about polycythemia vera except one.

Accepted Answer

Increased erythropoietin

Answer

Decreased erythropoietin

Answer

Increased LAP Score

Answer

Increased blood viscosity

Question 4

Classical markers for Hodgkin's disease are

Accepted Answer

CD 15 and CD 30

Answer

CD 15 and CD 20

Answer

CD 20 and CD 30

Answer

CD 15 and CD 22

Question 5

Which of the following is not a B-cell marker?

Accepted Answer

CD 34

Answer

CD 10

Answer

CD 20

Answer

CD 19

Question 6

A 10-year-old boy presents with a palpable mass in the abdomen. On imaging, the para-aortic lymph nodes are found to be enlarged. A biopsy from the lymph node shows a starry sky appearance. What is the likely underlying abnormality?

Accepted Answer

cMYC gene translocation

Answer

Mutation in the Rb tumor suppressor gene

Answer

BCR-ABL gene translocation

Answer

Mutation in the Tp53 tumor suppressor gene

Question 7

The most common translocation in acute promyelocytic leukemia (APML) is:

Accepted Answer

t(15;17)

Answer

t(8;21)

Answer

t(8;14)

Answer

t(9;22)

Question 8

In which of the following conditions is an elevated leukocyte alkaline phosphatase (LAP) score typically observed?

Accepted Answer

Leukemoid reaction

Answer

Eosinophilia

Answer

Malaria

Answer

CML

Question 9

The size of the red blood cells is measured by?

Accepted Answer

Mean Corpuscular Volume (MCV)

Answer

Mean Cell Hemoglobin (MCH)

Answer

Mean Corpuscular Hemoglobin Concentration (MCHC)

Answer

Erythrocyte Sedimentation Rate (ESR)

Question 10

Which genetic alteration is associated with a poor prognosis in acute myeloid leukemia?

Accepted Answer

Monosomy 7

Answer

Nucleophosmin mutation (NPM1)

Answer

Deletion of chromosome 5

Answer

T(8:21) translocation

Hematopathology — MCQs

Hematopathology — MCQs

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