Hematopathology Practice Questions

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**.

Q: Gandy-Gamma nodules are typically seen in chronic venous congestion of

Spleen. ***Spleen*** - **Gandy-Gamma nodules** (also called **Gamna-Gandy bodies**) are characteristic findings in the spleen, resulting from **chronic passive congestion**. - These nodules are composed of **fibrous tissue**, **hemosiderin**, and **calcium deposits**, reflecting old microhemorrhages and fibrosis due to chronic congestion. - They appear as **rust-brown nodules** on the splenic capsule, typically seen in conditions causing splenic congestion such as **portal hypertension** or **congestive splenomegaly**. *Lungs* - Chronic venous congestion in the lungs typically leads to **pulmonary hypertension** and **hemosiderin-laden macrophages** (heart failure cells). - It does not involve the formation of Gandy-Gamma nodules. *Kidneys* - Chronic venous congestion of the kidneys can lead to **renal dysfunction** and morphological changes like **glomerular sclerosis** or **tubulointerstitial fibrosis**. - Gandy-Gamma nodules are not associated with renal pathology. *Liver* - Chronic venous congestion of the liver, often due to **right-sided heart failure**, results in **nutmeg liver** appearance. - This involves **centrilobular necrosis** and congestion, but not Gandy-Gamma nodules.

Q: In the context of extranodal Hodgkin's lymphoma, which of the following sites is most commonly involved?

Bone. ***Bone*** - Among the options listed, **bone is the most frequently involved extranodal site** in Hodgkin's lymphoma, particularly in patients with advanced stage disease. - **Note:** The most common extranodal sites overall in Hodgkin's lymphoma are the **spleen**, **liver**, and **bone marrow** [1]. However, among the specific sites listed in this question, bone involvement is most frequent. - Osseous involvement often manifests as **osteolytic or osteoblastic lesions** visible on imaging and may cause bone pain or pathological fractures. *Thymus* - While the **mediastinum** (including thymus) is a common **nodal site** for Hodgkin's lymphoma (especially nodular sclerosis subtype), extensive thymic involvement as a true **extranodal site** is less common than bone [1]. - Primary thymic lymphomas are more characteristic of **T-cell lymphoblastic lymphoma** rather than Hodgkin's lymphoma. *Thyroid* - **Thyroid involvement** in Hodgkin's lymphoma is rare, typically occurring as secondary extension from adjacent cervical lymph nodes. - Primary thyroid lymphoma is more commonly **non-Hodgkin lymphoma** (particularly MALT lymphoma or DLBCL) associated with Hashimoto's thyroiditis. *Small bowel* - **Gastrointestinal involvement**, including small bowel, is a rare manifestation of Hodgkin's lymphoma. - GI tract lymphomas are predominantly **non-Hodgkin lymphomas** (especially MALT lymphoma, DLBCL, and enteropathy-associated T-cell lymphoma). **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. 557-559.

Q: Working Formulation in classification of NHL is based on:

Morphology of cells. ***Morphology of cells*** - The staging of non-Hodgkin lymphoma (NHL) is primarily based on the **morphological characteristics** of the tumor cells, which helps in classifying the type of lymphoma [1]. - Accurate morphological assessment, including cell size, shape, and arrangement, aids in determining **prognosis** and guiding treatment decisions [1]. *Cells of origin* - While the **origin of the cells** is important, it is not the primary factor in staging NHL. - Cell origin is more relevant for **classification** rather than staging, which focuses on tumor morphology. *None of the above* - This option implies that the correct basis for staging is not listed, which is incorrect since morphology is the appropriate criterion. - Furthermore, choosing this isregards valid and clear factors that do influence staging in NHL. *Survival characteristics of cells* - Survival characteristics primarily relate to **prognosis** and how well a patient might do with treatment, not staging. - Staging involves **anatomical and histopathological** evaluations rather than just survival metrics of the cells. **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. 596-598.

Q: Which immunoglobulin is primarily associated with lymphoplasmacytoid lymphoma?

IgM. ***IgM*** - Lymphoplasmacytoid lymphomas are often associated with **elevated serum IgM** levels, indicative of monoclonal gammopathy [1]. - These lymphomas primarily involve **plasma cells** that secrete IgM, leading to conditions such as **Waldenström macroglobulinemia** [2]. *IgA* - IgA is primarily involved in **mucosal immunity** and is not typically associated with lymphoplasmacytoid lymphomas. - These lymphomas do not usually show a **dominance of IgA secreting cells** compared to the IgM type [1]. *IgG* - While IgG is important in immune responses, it is not the hallmark of lymphoplasmacytoid lymphomas, which are characterized by **IgM secretion** [1]. - These lymphomas do not present with **significant IgG monoclonal gammopathy** like other plasma cell disorders. *IgE* - IgE is predominantly associated with **allergic reactions** and parasitic infections, and has no known association with lymphoplasmacytoid lymphomas. - These lymphomas are even less related to **IgE production**, which plays a minimal role in lymphoid malignancies. **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. 609-610. [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. 606-607.

Q: Which marker is used to differentiate a thymoma from acute lymphoblastic leukemia (ALL)?

Cytokeratin. ***Cytokeratin*** - **Thymomas** are epithelial tumors and express **cytokeratin**, which is a marker for epithelial cells, while **ALL** (Acute Lymphoblastic Leukemia) does not express this marker. - The presence of **cytokeratin** indicates a **thymic origin**, differentiating it from lymphoid neoplasms like **ALL**. *CD1a* - This marker is primarily associated with **Hodgkin's lymphoma** and some **T-cell neoplasms**, not thymomas [2]. - The lack of expression in **ALL** makes it an unsuitable differentiator for thymomas. *Tdt* - **Tdt** (Terminal deoxynucleotidyl transferase) is a marker typically found in lymphoid progenitor cells, especially in **ALL**. - Its presence would not indicate a **thymoma**, which does not express **Tdt**. *CD3* - While **CD3** is a marker of **T-cells** [2], it is not specific for thymomas, which can be **CD3-positive**, but it is also seen in various **lymphoid proliferations** including **ALL** [1]. - Therefore, it cannot be definitively used to distinguish between a thymoma and **ALL**. **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. 599-600. [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. 598.

Question 1

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 2

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

Accepted Answer

HbA

Answer

HbF

Answer

HbS

Answer

HbA2

Question 3

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 4

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 5

Ratio of fat cells to hematopoietic cells in adult bone marrow

Accepted Answer

1:1

Answer

2:1

Answer

6:1

Answer

4:1

Question 6

Gandy-Gamma nodules are typically seen in chronic venous congestion of

Accepted Answer

Spleen

Answer

Lungs

Answer

Kidneys

Answer

Liver

Question 7

In the context of extranodal Hodgkin's lymphoma, which of the following sites is most commonly involved?

Accepted Answer

Bone

Answer

Thyroid

Answer

Small bowel

Answer

Thymus

Question 8

Working Formulation in classification of NHL is based on:

Accepted Answer

Morphology of cells

Answer

Survival characteristics of cells

Answer

Cells of origin

Answer

None of the options

Question 9

Which immunoglobulin is primarily associated with lymphoplasmacytoid lymphoma?

Accepted Answer

IgM

Answer

IgG

Answer

IgA

Answer

IgE

Question 10

Which marker is used to differentiate a thymoma from acute lymphoblastic leukemia (ALL)?

Accepted Answer

Cytokeratin

Answer

CD1a

Answer

CD3

Answer

TdT

Hematopathology — MCQs

Hematopathology — MCQs

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