Hematopathology Practice Questions

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: 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: 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: Plasma cell dyscrasias include all the following except:

Systemic lupus erythematosus. ***Systemic lupus erythematosus*** - A multi-system autoimmune condition characterized by a range of symptoms, rather than a dyscrasia affecting plasma cells. - Does not involve the **monoclonal proliferation** of plasma cells that define plasma cell dyscrasias [1]. *Heavy chain disease* - A type of plasma cell dyscrasia involving the **production of abnormal heavy chains** of immunoglobulins without light chains [1]. - Associated with symptoms like **lymphadenopathy** and bone pain due to its hematological nature. *Waldenström's macroglobulinemia* - A type of lymphoma characterized by the overproduction of **IgM** antibodies, signifying a plasma cell disorder [1]. - Symptoms include **hyperviscosity syndrome**, leading to blurred vision and neurologic issues [1,3]. *Monoclonal gammopathy* - Refers to a condition characterized by the presence of a monoclonal protein in the serum, indicative of plasma cell proliferation [1,2]. - Can progress to more serious conditions such as **multiple myeloma** or related disorders [2,4]. **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. 606-607. [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. 608-609. [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. 609-610. [4] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 618-619.

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.

Q: Which of the following conditions is caused by the deletion of all four alpha globin genes?

Hb Bart's. ***Hb Bart's*** - This condition, also known as **hydrops fetalis**, results from the **deletion of all four alpha globin genes**, leading to a complete absence of alpha globin chains [1]. - The excess gamma globin chains in the fetus form **tetramers (gamma4)**, which have a very high affinity for oxygen and do not release it to tissues, causing severe tissue hypoxia and usually intrauterine death [3]. *Beta thalassemia major* - This condition involves a reduced or absent production of **beta globin chains**, not alpha globin chains [2]. - It leads to an excess of **alpha globin chains**, which precipitate in red blood cells, causing severe anemia and ineffective erythropoiesis. *HbH* - This condition is caused by the **deletion of three out of four alpha globin genes**, leaving only one functional alpha globin gene [1], [2]. - The excess beta globin chains in adults form **tetramers (beta4)**, known as HbH, which are unstable and precipitate in red blood cells, leading to hemolytic anemia. *alpha thalassemia trait* - This refers to the deletion of **one or two alpha globin genes**, resulting in a mild reduction in alpha globin chain synthesis [1]. - Individuals with one gene deletion are often asymptomatic, while those with **two gene deletions** (alpha thalassemia minor) may have mild anemia with microcytosis but are generally asymptomatic [1], [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. 649-650. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 600-601. [3] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of Infancy and Childhood, pp. 470-472.

Q: In acute myeloid leukemia, Auer rods are numerous in which?

M3 (Acute promyelocytic leukemia). ***M3*** - In acute myeloid leukemia (AML), **M3 subtype (promyelocytic leukemia)** is characterized by a high number of **Auer rods** in the leukemic cells [1][2]. - These *Auer rods* play a significant role in the diagnosis of this specific type of AML and are often associated with a **hypergranular promyelocyte** morphology [1]. *M5* - M5 is known as **acute monocytic leukemia**, which primarily features **monoblasts** and lacks Auer rods. - The predominant findings are **extramedullary infiltration** and a higher incidence of **gum hypertrophy**. *M2* - M2 refers to **acute myeloblastic leukemia** that does demonstrate some Auer rods, but not in as significant numbers as seen in M3 [2]. - This subtype is characterized by both **myeloblasts** and **maturation** into more differentiated cells, leading to varied morphology. *M4* - M4 is defined as **acute myelomonocytic leukemia**, which may have **myeloid and monocytic blasts** but usually has fewer Auer rods compared to M3. - It often presents with features of both **myeloid and monocytic lineages**, which differ from the Auer rod prominence in M3. **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. 621-622. [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. 620.

Q: Ratio of fat cells to hematopoietic cells in adult bone marrow

1:1. ***1:1*** - In a **healthy adult bone marrow**, the ratio of **fat cells (adipocytes)** to **hematopoietic cells (including RBC precursors)** is approximately 1:1. - This balance ensures adequate space for effective hematopoiesis while providing a stored energy reserve. *2:1* - A 2:1 ratio would imply a significantly higher proportion of **fat cells**, which is not typical for **healthy adult bone marrow**. - Such a ratio might be seen in conditions like **aplastic anemia** or in older individuals where **hematopoiesis** declines. *4:1* - A 4:1 ratio suggests a substantial **replacement of active marrow** with **adipose tissue**. - This extreme shift would severely compromise **hematopoietic function** and is indicative of severe **marrow hypoplasia** or **aplasia**. *6:1* - A 6:1 ratio of fat cells to hematopoietic cells would represent a highly **atrophic or aplastic marrow**. - This would be incompatible with normal **hematopoiesis** and would result in severe **cytopenias**.

Question 1

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 2

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

Accepted Answer

Hemochromatosis

Answer

Hemosiderosis

Answer

Cholelithiasis

Answer

None of the options

Question 3

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 4

Specific stain for myeloblasts is

Accepted Answer

Myeloperoxidase

Answer

Sudan black B

Answer

Periodic acid-Schiff (PAS)

Answer

Leukocyte alkaline phosphatase (LAP)

Question 5

Plasma cell dyscrasias include all the following except:

Accepted Answer

Systemic lupus erythematosus

Answer

Monoclonal gammopathy

Answer

Waldenström's macroglobulinemia

Answer

Heavy chain disease

Question 6

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

Accepted Answer

HbA

Answer

HbF

Answer

HbS

Answer

HbA2

Question 7

An 18-year-old man recently diagnosed with beta-thalassemia minor has a hemoglobin level of 10.5 g/dL and feels well. Which of the following findings is characteristic of this condition?

Accepted Answer

an increased amount of fetal hemoglobin (HbF) and hemoglobin A2 (HbA2)

Answer

increased osmotic fragility of red blood cells

Answer

normal bone marrow iron stores

Answer

increased macroglobulins in the serum

Question 8

Which of the following conditions is caused by the deletion of all four alpha globin genes?

Accepted Answer

Hb Bart's

Answer

Beta thalassemia major

Answer

HbH

Answer

alpha thalassemia trait

Question 9

In acute myeloid leukemia, Auer rods are numerous in which?

Accepted Answer

M3 (Acute promyelocytic leukemia)

Answer

M2 (Myeloblastic leukemia)

Answer

M4 (Acute myelomonocytic leukemia)

Answer

M5 (Acute monocytic leukemia)

Question 10

Ratio of fat cells to hematopoietic cells in adult bone marrow

Accepted Answer

1:1

Answer

2:1

Answer

6:1

Answer

4:1

Hematopathology — MCQs

Hematopathology — MCQs

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