Hematopathology Practice Questions

Q: The marker for B-lymphocyte is

CD19. ***CD19*** - **CD19** is a specific surface marker expressed on **B-lymphocytes**, crucial for their development and activation [1]. - It is used in diagnosing and monitoring B-cell neoplasms, making it a reliable **biomarker** for these cells [1]. *CD34* - **CD34** is a marker associated with **hematopoietic stem and progenitor cells**, not specifically limited to B-lymphocytes. - It is primarily found on **hematopoietic stem cells** and endothelial cells, indicating its roles in various cell types. *CD68* - **CD68** is predominantly a marker for **macrophages** and is not specific to B-lymphocytes. - It is involved in the identification of **activated macrophages** during inflammation and immune responses. *CD4* - **CD4** is primarily associated with **T-helper cells**, playing a critical role in the immune response and is not a marker for B-lymphocytes [1]. - Its presence indicates helper T-cell function rather than B-cell identification. **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: Multiple myeloma is characterized by which of the following?

Monoclonal gammopathy. ***Monoclonal gammopathy*** - **Multiple myeloma** is defined by the proliferation of a **single clone of plasma cells** that produce a characteristic **monoclonal immunoglobulin** (M-protein) detected in serum or urine [1]. - This **monoclonal expansion** leads to the accumulation of abnormal, identical **immunoglobulins** or their fragments [2]. *Presence of light chains* - While the presence of **monoclonal free light chains** (either kappa or lambda) is typical in myeloma, this option describes only a component and not the overarching characteristic that defines the disease [2]. - Not all light chain presence indicates myeloma; a **monoclonal proliferation** of these light chains is what is significant. *Bence Jones proteins* - **Bence Jones proteins** are **monoclonal light chains** excreted in the urine, a common finding in multiple myeloma [2]. - However, like the presence of light chains, this is a **consequence** or **manifestation** of the underlying monoclonal gammopathy, not the defining characteristic itself. *Hypergammaglobulinemia* - This term refers to an **elevated total level of immunoglobulins** in the blood, which can be **polyclonal** (diverse antibodies) or **monoclonal** in nature. - In multiple myeloma, the elevation is specifically due to a **monoclonal immunoglobulin**, making "monoclonal gammopathy" a more precise and defining term [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. 606-609. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 616-617.

Q: Earliest manifestation of megaloblastic anemia is

Hypersegmented neutrophils. ***Hypersegmented neutrophils*** - The earliest manifestation of megaloblastic anemia includes the presence of **hypersegmented neutrophils**, which have more than five lobes in their nuclei [1][2]. - This finding is indicative of impaired DNA synthesis often associated with **vitamin B12** or **folate deficiency**. *Basophilic stippling* - **Basophilic stippling** is more commonly linked to lead poisoning and certain alcohol-related disorders rather than megaloblastic anemia. - It reflects RNA aggregate remnants in red blood cells, which is not a primary feature of this type of anemia. *Cabot ring* - **Cabot rings** are seen in conditions like **pernicious anemia** but are not the **earliest manifestation**; they are infrequently encountered. - This abnormality is related to nuclear remnant material and lacks direct correlation to megaloblastic changes. *Macrocytosis* - **Macrocytosis** refers to the increased size of red blood cells and can be found in megaloblastic anemia but is not the **initial manifestation**. - It may develop later as the anemia progresses, whereas hypersegmented neutrophils appear much earlier. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 593-594. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, p. 654.

Q: In the context of myelodysplastic syndromes (MDS) and acute myelogenous leukemia (AML), which of the following cytogenetic abnormalities is associated with the worst prognosis?

Monosomy 7. ***Monosomy 7*** - Monosomy 7 is associated with **poor prognosis** in conditions like **acute myeloid leukemia (AML)**, often leading to a higher risk of treatment failure. - It implies more aggressive disease and often correlates with **poor response to therapy**. *8/21 translocation* - Generally indicates a more favorable prognosis [1], commonly seen in **Acute Lymphoblastic Leukemia (ALL)**. - Associated with **treatment responsiveness** and a higher chance of remission compared to other cytogenetic abnormalities. *Normal cytogenetics* - In many leukemias, **normal cytogenetics** suggest a relatively **better prognosis** [1] and a more favorable response to treatment. - It is often an indicator of **favorable disease characteristics** and lower risk of relapse. *Inversion 16* - Typically associated with **acute myeloid leukemia (AML)** and indicates a **better prognosis** [1] due to a favorable response to therapy. - Patients with this cytogenetic change generally exhibit **good overall outcomes** compared to other abnormalities. **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: What is the most characteristic morphological finding in G6PD deficiency?

Bite cells. ***Bite cells*** - **Bite cells** (or **degmacytes**) are red blood cells from which a portion of the cytoplasm has been "bitten off" by macrophages in the spleen [1]. - This occurs due to the removal of **Heinz bodies**, which are precipitated, denatured hemoglobin molecules formed as a result of oxidative stress in G6PD deficient red blood cells [1]. *Intravascular hemolysis* - While **intravascular hemolysis** certainly occurs in G6PD deficiency, causing symptoms like hemoglobinuria and jaundice, it is a clinical consequence and not a direct morphological finding observed on a peripheral blood smear [1]. - The primary defect in G6PD deficiency leads to oxidative damage to red blood cells, which subsequently undergo hemolysis. *Splenomegaly* - **Splenomegaly** can be present in G6PD deficiency due to the increased workload of the spleen in removing damaged red blood cells and Heinz bodies [1]. - However, it is an organ enlargement, a clinical sign, and not a cellular morphological finding characteristic of the red blood cells themselves. *Hemoglobinuria* - **Hemoglobinuria** is the presence of free hemoglobin in the urine, resulting from significant **intravascular hemolysis** [1]. - It is a clinical symptom of acute hemolytic episodes in G6PD deficiency rather than a characteristic morphological feature of the red blood cells themselves. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 642-643.

Q: A 2-year-old child presents with scattered lesions in the skull, and biopsy reveals Langerhans cells. The most commonly associated marker with this condition will be

CD 1a. ***CD1a*** - **CD1a** is a definitive cell surface marker for **Langerhans cells**, which are the pathological cells proliferating in **Langerhans cell histiocytosis (LCH)**. - The presence of **Langerhans cells** in a scattered skull lesion biopsy confirms the diagnosis, and **CD1a** staining is crucial for their identification. - **CD207 (Langerin)** is another highly specific marker for LCH, but CD1a remains the most commonly used diagnostic marker [1]. *CD3* - **CD3** is a key surface marker found on **T lymphocytes**, identifying them as part of the adaptive immune system. - While T-cells may be present in the inflammatory infiltrate of LCH lesions, **CD3** is not expressed by the pathological Langerhans cells themselves. *CD68* - **CD68** is a marker commonly used to identify **macrophages** and monocytes, which are phagocytic cells of the innate immune system. - Although Langerhans cells have some macrophage-like features and may show weak CD68 positivity, it is not as specific or defining a marker for LCH as **CD1a**. *CD57* - **CD57** is typically expressed on a subset of **natural killer (NK) cells** and some T-cell populations, particularly cytotoxic T cells. - It has no known specific association with Langerhans cells or the diagnosis of Langerhans cell histiocytosis. **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. 629-630.

Q: Disseminated intravascular coagulation (DIC) differs from thrombotic thrombocytopenic purpura. In this reference, DIC is most likely characterized by:

Low levels of coagulation factors. ***Decreased coagulation factor levels*** - DIC is characterized by an activation of the coagulation cascade, leading to increased consumption of **coagulation factors** and resulting in low levels [1]. - This process causes a paradoxical increased risk of bleeding despite a **consumption coagulopathy** scenario [1]. *Significant thrombocytopenia* - While thrombocytopenia can occur in DIC, it is not as pronounced as in **thrombotic thrombocytopenic purpura** (TTP), which features **severe thrombocytopenia** as its hallmark [2]. - DIC typically presents with **variable platelet counts**, often fluctuating based on the underlying cause. *A brisk reticulocytosis* - Reticulocytosis is common in hemolytic processes, but it is not a defining characteristic of DIC, which primarily involves dysfunction in the **coagulation cascade** rather than increased red blood cell production. - In contrast, TTP may show reticulocytosis due to hemolysis, but this does not apply directly to DIC. *Significant numbers of schistocytes* - Schistocytes are seen in microangiopathic hemolytic anemias, but **quantity and significance** vary; they may not be prominently present in DIC cases compared to TTP, which is distinguished by more pronounced schistocytes [2]. - DIC primarily leads to a **consumption coagulopathy**, whereas schistocytes more specifically indicate **mechanical hemolysis** [2].

Q: Specific stain for myeloblasts is

Myeloperoxidase. ***Myeloperoxidase*** - **Myeloperoxidase (MPO)** is an enzyme found in the primary granules of myeloblasts and other myeloid cells, making it a specific marker. - A positive MPO stain helps differentiate **acute myeloid leukemia (AML)** from **acute lymphoblastic leukemia (ALL)**. *Sudan black B* - **Sudan black B (SBB)** stains lipids within granules of myeloid and monocytoid cells, including myeloblasts. - While it is positive in myeloblasts, it is **less specific** than MPO as it can also stain monocytes, and its staining mechanism is different from enzyme activity. *Periodic acid-Schiff (PAS)* - **PAS stain** detects glycogen and other carbohydrates, typically showing a granular pattern in ALL and often negative or weakly positive in AML. - It is **not specific for myeloblasts** and is more characteristic of erythroblasts and lymphoblasts. *Leukocyte alkaline phosphatase (LAP)* - **LAP score** is used to distinguish between chronic myeloid leukemia (CML), which typically has a low LAP score, and other myeloproliferative disorders or leukemoid reactions, which have high scores. - It is **not used for the identification of myeloblasts** or for distinguishing AML from ALL.

Q: Which of the following conditions is not associated with hemolytic anemia?

Hemochromatosis. ***None of the above*** - Hemolytic anemia can lead to various complications, but this option indicates that all listed options are seen in the condition, which is incorrect. - Therefore, saying "None of the above" about this particular question is a misinterpretation of the options provided. *Cholelithiasis* - This is a common complication due to increased **bilirubin** levels from hemolysis, leading to the formation of **bilirubinate gallstones**. - It can frequently be seen in cases of hemolytic anemia, particularly if there's chronic hemolysis, such as in hereditary spherocytosis [1]. *Hemosiderosis* - This condition occurs when there is excess **iron deposition**, often due to repeated blood transfusions in conditions like thalassemia or sideroblastic anemia. - While patients with hemolytic anemia can develop it due to frequent transfusions, it is not an inherent feature of hemolytic anemia itself. Intravascular hemolysis is specifically associated with haemosiderinuria [2]. *Hemochromatosis* - Hemochromatosis is a hereditary condition causing excess iron absorption and storage, which is distinct from the iron overload seen in hemosiderosis. - Hemolytic anemia does not lead to hemochromatosis directly; hence, it is not a typical finding associated with hemolytic anemia. Other syndromes like hemolytic-uremic syndrome specifically characterize certain hemolytic presentations [3].

Q: Which of the following is not seen on hemoglobin electrophoresis in sickle cell anemia?

HbA. ***HbA*** - Sickle cell anemia is characterized by the production of **HbS** (sickle hemoglobin) instead of **HbA** [1]. - Patients with sickle cell anemia typically have a significant reduction or absence of **HbA**, as it is replaced by HbS [1]. *HbA2* - **HbA2** is usually present in normal individuals and may be slightly increased in sickle cell disease but is not absent. - It is made up of **2 alpha and 2 delta chains** and does not directly correlate with sickle cell pathology. *HbF* - **HbF** (fetal hemoglobin) can actually be elevated in sickle cell anemia, helping to reduce sickling episodes. - Patients may have some level of **HbF**, which provides a degree of protection against the effects of sickle hemoglobin. *HbS* - **HbS** is the primary hemoglobin present in sickle cell anemia, leading to characteristic sickling of red blood cells [1][2]. - It is essential for the diagnosis of sickle cell disease, and its presence is confirmed during hemoglobin electrophoresis [1]. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 598-599. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 599-600.

Question 1

The marker for B-lymphocyte is

Accepted Answer

CD19

Answer

CD34

Answer

CD4

Answer

CD68

Question 2

Multiple myeloma is characterized by which of the following?

Accepted Answer

Monoclonal gammopathy

Answer

Bence Jones proteins

Answer

Presence of light chains

Answer

Hypergammaglobulinemia

Question 3

Earliest manifestation of megaloblastic anemia is

Accepted Answer

Hypersegmented neutrophils

Answer

Macrocytosis

Answer

Basophilic stippling

Answer

Cabot ring

Question 4

In the context of myelodysplastic syndromes (MDS) and acute myelogenous leukemia (AML), which of the following cytogenetic abnormalities is associated with the worst prognosis?

Accepted Answer

Monosomy 7

Answer

inv(16)

Answer

Normal cytogenetics

Answer

t(8;21) translocation

Question 5

What is the most characteristic morphological finding in G6PD deficiency?

Accepted Answer

Bite cells

Answer

Intravascular hemolysis

Answer

Splenomegaly

Answer

Hemoglobinuria

Question 6

A 2-year-old child presents with scattered lesions in the skull, and biopsy reveals Langerhans cells. The most commonly associated marker with this condition will be

Accepted Answer

CD 1a

Answer

CD68

Answer

CD57

Answer

CD3

Question 7

Disseminated intravascular coagulation (DIC) differs from thrombotic thrombocytopenic purpura. In this reference, DIC is most likely characterized by:

Accepted Answer

Low levels of coagulation factors

Answer

Elevated reticulocyte count

Answer

Severe thrombocytopenia

Answer

Presence of schistocytes in the blood smear

Question 8

Specific stain for myeloblasts is

Accepted Answer

Myeloperoxidase

Answer

Sudan black B

Answer

Periodic acid-Schiff (PAS)

Answer

Leukocyte alkaline phosphatase (LAP)

Question 9

Which of the following conditions is not associated with hemolytic anemia?

Accepted Answer

Hemochromatosis

Answer

Hemosiderosis

Answer

Cholelithiasis

Answer

None of the options

Question 10

Which of the following is not seen on hemoglobin electrophoresis in sickle cell anemia?

Accepted Answer

HbA

Answer

HbF

Answer

HbS

Answer

HbA2

Hematopathology — MCQs

Hematopathology — MCQs

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