Hematopathology Practice Questions

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: ESR is increased in

Multiple myeloma. ***Multiple myeloma*** [1] - **Erythrocyte sedimentation rate (ESR)** is commonly elevated due to inflammatory cytokines and the presence of abnormal proteins affecting blood viscosity [1]. - Patients often present with **bone pain** and can have **hypercalcemia**, which further contributes to an increased ESR [2]. *Thalassemia* - Typically presents with **microcytic anemia** but does not significantly elevate the ESR [3]. - The chronic hemolysis in thalassemia often results in a normal or slightly raised ESR, not markedly increased as seen in malignancies. *Polycythemia vera* - Characterized by excessive red blood cell production, leading to increased blood viscosity, but usually results in a **low or normal ESR**. - The inflammatory response is minimal, and the only changes in ESR are due to viscous blood rather than true inflammation. *Sickle cell anemia* - While patients may experience vasocclusive crises leading to inflammation, the overall ESR is generally normal or only mildly elevated. - The primary issue in sickle cell anemia is the **sickle-shaped red blood cells**, not significant changes in ESR levels [3].

Q: Aplastic anemia in hereditary spherocytosis is precipitated by.

Parvo virus. ***Parvo virus*** - **Parvovirus B19** specifically infects and destroys erythroid precursors in the bone marrow, leading to a temporary cessation of red blood cell production [1]. - In individuals with **hereditary spherocytosis**, who already have shortened red blood cell lifespans, this transient aplasia can trigger a severe **aplastic crisis** due to the inability to compensate for ongoing hemolysis [1]. *HIV* - While HIV can affect bone marrow function over time, it typically causes **myelosuppression** and pancytopenia through various mechanisms, rather than an acute, severe **aplastic crisis** predominantly affecting erythroid lineage, as seen in hereditary spherocytosis. - HIV infection is more commonly associated with **chronic anemia of inflammation** or **opportunistic infections** affecting marrow, not a direct precipitant of acute aplastic anemia in this context. *Adenovirus* - Adenoviruses are more commonly associated with **respiratory**, **gastrointestinal**, and **ocular infections**. - They are not a recognized cause of **aplastic crisis** or severe erythroid aplasia in patients with hereditary spherocytosis. *Influenza virus* - Influenza virus primarily causes **respiratory tract infections** and can lead to systemic symptoms and complications like pneumonia. - It is not implicated in causing **pure red cell aplasia** or an **aplastic crisis** in the setting of hereditary spherocytosis.

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.

Q: A 65-year-old patient presented to the medicine OPD with weakness and fatigue for 6 months, accompanied by mild abdominal discomfort. On examination, moderate splenomegaly was noted. Laboratory findings revealed severe normocytic normochromic anemia, neutropenia with monocytopenia, and thrombocytopenia. Bone marrow aspiration was performed to investigate the cause of decreased cell counts, but the tap was dry. Therefore, a bone marrow biopsy was conducted. Which of the following CD markers is NOT typically positive in hairy cell leukemia?

CD117. ***CD117*** - CD117 (c-Kit) is a transmembrane receptor tyrosine kinase typically expressed on hematopoietic stem cells, mast cells, and gastrointestinal stromal tumors (GIST). - It is generally **negative in hairy cell leukemia**, making it a useful marker to differentiate HCL from other lymphoproliferative disorders. - This is the correct answer as the question asks which marker is NOT typically positive in HCL. *CD11c* - CD11c is a beta-2 integrin subunit that is **strong and consistently expressed** on the surface of hairy cells. - It is a key diagnostic marker for hairy cell leukemia due to its high expression. *CD103* - CD103 (alpha-E integrin) is another **highly characteristic marker for hairy cell leukemia**, showing strong expression on hairy cells. - Its presence helps confirm the diagnosis and distinguish HCL from other B-cell lymphomas. *CD25* - CD25 (alpha chain of the IL-2 receptor) is also **typically expressed** on hairy cells, although its expression can be more variable or less intense than CD11c and CD103 [1]. - Along with CD11c and CD103, CD25 contributes to the characteristic immunophenotype of hairy cell leukemia [1]. *Clinical Presentation and Bone Marrow* - Hairy cell leukemia typically presents with splenomegaly and pancytopenia. Because the leukemic cells are enmeshed in an extracellular matrix of reticulin fibrils, they usually cannot be aspirated, resulting in a "dry tap" [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. 612.

Q: Among the following AML subtypes, non-specific esterase (NSE) staining is typically NEGATIVE in which one?

Acute Promyelocytic Leukemia (M3). ***Acute Promyelocytic Leukemia (M3)*** - Non-specific esterase (NSE) is **negative** in Acute Promyelocytic Leukemia (M3) because NSE primarily stains cells of the **monocytic lineage**. - M3 is characterized by abnormal **promyelocytes** with heavy granulation, which are granulocytic precursors without monocytic differentiation. - M3 shows strong positivity for **myeloperoxidase (MPO)** instead, which is the characteristic marker for granulocytic lineage. *Acute Myelomonocytic Leukemia (M4)* - NSE staining is **positive** in M4 because this subtype has both myeloid and **monocytic components**. - The monocytic component (≥20% of non-erythroid cells) stains positively with NSE, which helps differentiate it from pure myeloid leukemias. - NSE positivity (inhibited by sodium fluoride) is a key diagnostic feature alongside myeloperoxidase positivity. *Acute Erythroleukemia (M6)* - NSE is typically **negative** in the predominant erythroid component of M6. - The diagnosis of M6 relies on the presence of ≥50% erythroid precursors (which are PAS positive) and ≥20% myeloblasts among non-erythroid cells. - NSE is not a characteristic marker for erythroleukemia. *Acute Monocytic Leukemia (M5)* - NSE staining is characteristically **strongly positive** in M5, which primarily consists of **monoblasts and promonocytes**. - This strong NSE positivity (inhibited by sodium fluoride) is a defining diagnostic feature demonstrating pure monocytic differentiation. - M5 typically shows weak or negative myeloperoxidase, helping distinguish it from other AML subtypes.

Q: What is the most common type of lymphoma observed in post-transplant patients?

B cell. ***B cell*** - The vast majority of **post-transplant lymphoproliferative disorders (PTLD)**, which are common in transplant patients, arise from **B lymphocytes** [1]. - This is primarily due to the **immunosuppression** compromising T-cell control over **Epstein-Barr virus (EBV)**, which then drives B-cell proliferation [1]. *T cell* - While T-cell PTLD can occur, it is significantly **less common** than B-cell PTLD in the post-transplant setting. - T-cell lymphomas generally have **different epidemiological and pathogenetic profiles** compared to B-cell PTLD. *Null cell* - **Null cell lymphomas** are rare and do not express typical T-cell or B-cell markers. - They are not considered a common form of PTLD in transplant recipients. *NK cell* - **Natural killer (NK) cell lymphomas** are also rare and represent a small fraction of all lymphomas. - They are not typically associated with the common mechanisms or incidence of PTLD in transplant patients. **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. 595-596.

Q: Cells seen in cutaneous T-cell lymphoma are called?

Sezary cells. ***Sezary cells*** - Characteristic of cutaneous T-cell lymphoma, particularly in **Sezary syndrome**, where malignant T-cells circulate in the blood [1,2]. - These cells exhibit **abnormal morphology**, including significant **pleomorphism** and a **cerebriform nucleus** [1,2]. *Dohle bodies* - Dohle bodies are indicative of **cytoplasmic inclusions** in neutrophils, often seen in infections or inflammatory conditions. - They are **not associated** with cutaneous T-cell lymphoma and do not represent malignant lymphoid cells. *Barr bodies* - Barr bodies are **inactivated X chromosomes**, typically present in female cells, and are unrelated to any form of lymphoma. - They do not indicate the presence of malignant **T-cells** or any form of cutaneous T-cell malignancy. *Councilman bodies* - Councilman bodies are **apoptotic hepatocytes** seen in liver pathology, particularly in viral hepatitis or toxic injury. - They bear no relation to cutaneous T-cell lymphoma or to any **lymphoid** malignancies. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Diseases Of The Urinary And Male Genital Tracts, pp. 564-565. [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. 613-614.

Question 1

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 2

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 3

ESR is increased in

Accepted Answer

Multiple myeloma

Answer

Polycythemia

Answer

Thalassemia

Answer

Sickle cell disease

Question 4

Aplastic anemia in hereditary spherocytosis is precipitated by.

Accepted Answer

Parvo virus

Answer

HIV

Answer

Adenovirus

Answer

Influenza virus

Question 5

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

Question 6

A 65-year-old patient presented to the medicine OPD with weakness and fatigue for 6 months, accompanied by mild abdominal discomfort. On examination, moderate splenomegaly was noted. Laboratory findings revealed severe normocytic normochromic anemia, neutropenia with monocytopenia, and thrombocytopenia. Bone marrow aspiration was performed to investigate the cause of decreased cell counts, but the tap was dry. Therefore, a bone marrow biopsy was conducted. Which of the following CD markers is NOT typically positive in hairy cell leukemia?

Accepted Answer

CD117

Answer

CD11c

Answer

CD103

Answer

CD25

Question 7

Among the following AML subtypes, non-specific esterase (NSE) staining is typically NEGATIVE in which one?

Accepted Answer

Acute Promyelocytic Leukemia (M3)

Answer

Acute Erythroleukemia (M6)

Answer

Acute Myelomonocytic Leukemia (M4)

Answer

Acute Monocytic Leukemia (M5)

Question 8

A 45-year-old woman presents with marked splenomegaly, a leukocyte count of 300,000/μL, and a differential count showing a predominance of myelocytes, metamyelocytes, bands, and segmented neutrophils, along with increased basophils and platelets, without anemia. Leukocyte alkaline phosphatase is decreased. Which of the following describes a major characteristic of chronic myeloid leukemia?

Accepted Answer

Translocation of chromosome 9 and 22 (Philadelphia chromosome)

Answer

Expansion of mature B lymphocytes in lymph nodes

Answer

Low levels of immunoglobulins (hypogammaglobulinemia)

Answer

Presence of hairy cells with filamentous projections

Question 9

What is the most common type of lymphoma observed in post-transplant patients?

Accepted Answer

B cell

Answer

T cell

Answer

Null cell

Answer

NK cell

Question 10

Cells seen in cutaneous T-cell lymphoma are called?

Accepted Answer

Sezary cells

Answer

Barr bodies

Answer

Dohle bodies

Answer

Councilman bodies

Hematopathology — MCQs

Hematopathology — MCQs

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