Hematopathology Practice Questions

Q: 'Hairy cell leukemia' is a neoplastic proliferation of which type of cells?

B lymphocytes. ***B lymphocytes*** - **Hairy cell leukemia** is a rare, chronic lymphoproliferative disorder characterized by the clonal proliferation of **mature B lymphocytes** [1]. - These malignant B cells have distinctive cytoplasmic projections, giving them a "hairy" appearance under a microscope [1]. *T cells* - While T-cell leukemias exist (e.g., T-cell prolymphocytic leukemia), **hairy cell leukemia** specifically originates from B lymphocytes, not T cells. - T-cell neoplasms have different clinical presentations and immunophenotypes [2]. *Myeloid cells* - Myeloid cells give rise to conditions like **acute myeloid leukemia** or **chronic myeloid leukemia**. - These are distinct from lymphoid malignancies like **hairy cell leukemia**, which involves lymphocytes. *Macrophages* - Macrophages are phagocytic cells involved in immune responses and do not typically proliferate in a neoplastic manner to cause **leukemia**. - **Hairy cell leukemia** is a lymphoid malignancy, not a disorder of macrophages. **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. [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. 596-598.

Q: Which of the following is a characteristic bone marrow finding in myelofibrosis?

Increased reticulin fibrosis. ***Leucoerythroblastosis*** - A hallmark finding in myelofibrosis is **leucoerythroblastosis**, characterized by the presence of immature white cells and nucleated red blood cells in the bloodstream [1][3]. - This reflects an **extramedullary hematopoiesis** due to the failure of normal marrow function and is commonly seen in myelofibrosis [1][2]. *Tear drop cells* - While **tear drop cells** (dacryocytes) can be associated with myelofibrosis, they are not exclusive findings and can appear in other conditions [1]. - They are indicative of **extramedullary hematopoiesis** but are not definitive for myelofibrosis specifically. *Leucocytopenia* - Myelofibrosis is associated with **neutrophilia** rather than leucocytopenia, which presents as low white blood cell counts. - The condition often leads to an increase in white blood cell counts due to reactive changes rather than a decrease. *All of the above* - This option is incorrect as it suggests that all previously mentioned findings are present in myelofibrosis. - Both **tear drop cells** and **leucocytopenia** are not definitive or accurate findings compared to **leucoerythroblastosis**. **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. 628-629. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 615-616. [3] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Heart, pp. 589-590.

Q: A baby's blood group was determined as O negative. Which of the following blood groups can the baby's mother or father not have?

AB Negative. ***AB Negative*** - A parent with **AB blood type** cannot have an O blood type child because an individual with AB blood type only has A and B alleles to pass on (i.e., *I A I B * genotype). - For a child to have **O blood type**, they must inherit the *i* allele from both parents (*ii* genotype), which is impossible if one parent is AB. *A Positive* - A parent with **A positive blood type** can have an O negative child if their genotype is *I A i* for A/B/O and *Rr* for Rh factor. - The child would inherit the *i* allele from this parent and the *r* allele for Rh, along with the same from the other parent. *B Positive* - Similar to A positive, a parent with **B positive blood type** can have an O negative child if their genotype is *I B i* and *Rr*. - The child would inherit the *i* allele from this parent and the *r* allele for Rh, along with the same from the other parent. *O positive* - A parent with **O positive blood type** can certainly have an O negative child; their genotype would be *ii* for A/B/O and *Rr* for Rh factor. - The child would inherit the *i* allele from this parent and the *r* allele for Rh.

Q: What is the primary basis for the Working Formulation in the classification of non-Hodgkin's lymphoma?

Morphology of cells. ***Morphology of cells*** - The **Working Formulation** primarily classified non-Hodgkin's lymphomas based on the **histological appearance** of the malignant cells, such as cell size, nuclear features, and growth patterns. - This classification aimed to group lymphomas with similar prognoses, broadly categorizing them into low, intermediate, and high-grade based on their **cytological features**. *Cell surface markers* - While cell surface markers (immunophenotyping) are crucial in modern lymphoma classification (e.g., WHO classification), they were not the **primary basis** for the Working Formulation. - Immunophenotyping identifies the lineage and differentiation stage of lymphoid cells (e.g., B-cell, T-cell) but became widely integrated into lymphoma classification later. *Survival characteristic of cells* - The Working Formulation did indirectly consider survival by grouping lymphomas with similar prognoses, but **survival characteristics** themselves were not the primary *basis* for classifying each specific lymphoma type. - Prognosis was an outcome derived from the morphological classification, not the initial classifying factor. *Cellular genetics* - **Cellular genetics**, including chromosomal translocations and gene mutations, are fundamental to current World Health Organization (WHO) classifications of lymphoma. - However, comprehensive genetic analysis was not readily available or the primary method for classifying lymphomas when the Working Formulation was developed.

Q: Neoplasm associated with Waldenström's macroglobulinemia is also known as:

Lymphoplasmacytic lymphoma. ***Lymphoplasmacytic lymphoma*** - **Waldenström's macroglobulinemia** is a type of **lymphoplasmacytic lymphoma (LPL)** that produces large amounts of **monoclonal IgM** protein [1]. - LPL is a **B-cell non-Hodgkin lymphoma** characterized by the presence of **lymphocytes, plasma cells, and plasmacytoid lymphocytes** in the bone marrow [1]. *Smoldering myeloma* - **Smoldering multiple myeloma (SMM)** is an **asymptomatic precursor** to multiple myeloma, characterized by high levels of **monoclonal protein (IgG or IgA)** and clonal plasma cells, but without end-organ damage. - It is distinct from Waldenström's macroglobulinemia, which involves **IgM paraprotein** and lymphoplasmacytic infiltration [1]. *Primary CNS lymphoma* - **Primary CNS lymphoma** is a rare and aggressive **non-Hodgkin lymphoma** that originates in the brain, spinal cord, or eyes, and is not typically associated with systemic **IgM paraproteinemia**. - Its clinical presentation involves **neurological symptoms** specific to CNS involvement, rather than symptoms of hyperviscosity or IgM-related coagulopathy. *MGUS (Monoclonal gammopathy of undetermined significance)* - **Monoclonal gammopathy of undetermined significance (MGUS)** is an asymptomatic condition with a **monoclonal protein (M-protein)** in the blood but no evidence of malignancy or end-organ damage. - While it can involve any immunoglobulin type, **IgM MGUS** is a precursor to Waldenström's macroglobulinemia, not the neoplasm itself. **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.

Q: Auer rods are a characteristic feature of which type of leukemia?

Acute myeloid leukemia (AML). ***Auer rods*** - Auer rods are **needle-shaped cytoplasmic inclusions** found in myeloid leukemias, particularly in acute myeloid leukemia (AML) [1]. - They are indicative of myeloid differentiation and are a classic **diagnostic feature** observed in bone marrow or peripheral blood smears [1]. *Intercytoplasmic granules* - While **intercytoplasmic granules** may appear in various leukemias, they are not specific or characteristic for Auer rods. - These granules do not serve as a **specific diagnostic marker** for any particular type of leukemia. *Eosinophils* - Eosinophils are associated with **allergic reactions** and parasitic infections, majorly presenting with **bilobed nuclei** and prominent granules. - They do not have **Auer rods**, making this option incorrect regarding the context of leukemia. *Leukemic cells* - While leukemic cells represent neoplastic white blood cells, they do not specifically refer to the **presence of Auer rods**. - Different types of leukemic cells have various **morphologies** and features that may not include Auer rods. **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: In which of the following conditions are Gamma-Gandy bodies typically not seen?

Chronic myeloid leukemia. ***Chronic myeloid leukemia*** - **Gamma-Gandy bodies** are fibrotic nodules in the spleen with **hemosiderin** and **calcium deposits**, typically resulting from **chronic passive congestion** or **repeated hemorrhages**. - **CML** causes massive **splenomegaly** due to **infiltration by leukemic cells** and extramedullary hematopoiesis, not the chronic vascular congestion or infarction pattern that produces Gamma-Gandy bodies. - While CML involves increased splenic iron turnover, it does NOT typically form the organized fibrotic nodules characteristic of Gamma-Gandy bodies. *Cirrhosis with portal hypertension* - **Portal hypertension** leads to **chronic passive congestion** of the spleen and splenomegaly. - This chronic congestion with elevated venous pressure and repeated microhemorrhages creates the ideal environment for **Gamma-Gandy body formation**. - This is one of the **classic associations** with Gamma-Gandy bodies. *Hemosiderosis* - While hemosiderosis involves **diffuse hemosiderin deposition** in tissues including the spleen, it represents generalized iron overload without the specific pathologic features of Gamma-Gandy bodies. - The organized **fibrotic nodules with calcium** that characterize Gamma-Gandy bodies are not a primary feature of hemosiderosis. - However, some sources consider hemosiderosis can be associated with these lesions in the context of chronic congestion. *Sickle Cell anemia* - **Repeated splenic infarctions** from vaso-occlusive crises lead to chronic injury, fibrosis, and hemosiderin deposition. - This creates the pathologic substrate for **Gamma-Gandy body formation**. - These bodies reflect chronic splenic damage in sickle cell disease, especially before autosplenectomy occurs.

Q: A 12-month-old girl of Punjabi parents developed pallor since 3 months of age. One unit of blood transfusion was done at 5 months of age. She now presents with pallor and hepatosplenomegaly. Her hemoglobin level is 3.8 g/dL, MCV is 68, and MCH is 19. A peripheral smear examination showed schistocytes, and bone marrow examination revealed erythroid hyperplasia. What is the diagnosis?

Beta-thalassemia (major). ***Beta-thalassemia (major)*** - The combination of **severe microcytic hypochromic anemia** (Hb 3.8 g/dL, MCV 68, MCH 19), early onset of pallor, **hepatosplenomegaly**, and **erythroid hyperplasia** with a need for transfusions is highly characteristic of **beta-thalassemia major** [1]. - The presence of **schistocytes** on peripheral smear indicates significant ineffective erythropoiesis and hemolysis, which is common in severe thalassemias due to the precipitation of unstable globin chains [1]. *Sickle cell disease* - This condition is characterized by **sickle-shaped red blood cells** and recurrent painful vaso-occlusive crises, which are not mentioned in the presentation, and the peripheral smear showed schistocytes, not sickled cells [1]. - While it can cause anemia, the **MCV is typically normal to high**, unlike the microcytosis seen here. *Alpha-thalassemia* - Severe forms like **Hb Barts hydrops fetalis** usually present *in utero* or at birth with massive edema and profound anemia, and are often fatal [1]. - Less severe forms might cause microcytic anemia, but **hepatosplenomegaly and severe transfusion dependence from 3 months of age** are more typical of beta-thalassemia major [1]. *Glucose-6-phosphate dehydrogenase deficiency* - This condition typically presents with **acute hemolytic anemia** triggered by certain drugs, infections, or fava beans, rather than chronic severe anemia and hepatosplenomegaly from early infancy. - Peripheral smear would show **bite cells** and **Heinz bodies** during a hemolytic episode, not schistocytes as the primary finding. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 644-650.

Question 1

'Hairy cell leukemia' is a neoplastic proliferation of which type of cells?

Accepted Answer

B lymphocytes

Answer

Myeloid cells

Answer

Macrophages

Answer

T cells

Question 2

Which of the following is a characteristic bone marrow finding in myelofibrosis?

Accepted Answer

Increased reticulin fibrosis

Answer

Absence of fibroblasts

Answer

Decreased megakaryocytes

Answer

Hypocellular marrow from onset

Question 3

A baby's blood group was determined as O negative. Which of the following blood groups can the baby's mother or father not have?

Accepted Answer

AB Negative

Answer

A Positive

Answer

B Positive

Answer

O positive

Question 4

What is the primary basis for the Working Formulation in the classification of non-Hodgkin's lymphoma?

Accepted Answer

Morphology of cells

Answer

Cell surface markers

Answer

Survival characteristic of cells

Answer

Cellular genetics

Question 5

Neoplasm associated with Waldenström's macroglobulinemia is also known as:

Accepted Answer

Lymphoplasmacytic lymphoma

Answer

Smoldering myeloma

Answer

Primary CNS lymphoma

Answer

MGUS (Monoclonal gammopathy of undetermined significance)

Question 6

Auer rods are a characteristic feature of which type of leukemia?

Accepted Answer

Acute myeloid leukemia (AML)

Answer

Chronic lymphocytic leukemia (CLL)

Answer

Myelodysplastic syndromes (MDS)

Answer

Acute lymphoblastic leukemia (ALL)

Question 7

Which of the following conditions is least likely to be associated with a high reticulocyte count?

Accepted Answer

Megaloblastic anemia (due to vitamin B12 or folate deficiency)

Answer

Acute bleed

Answer

Hemolytic anemia

Answer

Nutritional deficiency anemia under treatment

Question 8

In which of the following conditions are Gamma-Gandy bodies typically not seen?

Accepted Answer

Chronic myeloid leukemia

Answer

Cirrhosis with portal hypertension

Answer

Hemosiderosis

Answer

Sickle Cell anemia

Question 9

A 52-year-old man presents with back pain, fatigue, polyuria, and polydipsia. Imaging reveals lytic lesions in the lumbar vertebrae. Laboratory tests show hypoalbuminemia, mild anemia, and thrombocytopenia. Serum electrophoresis indicates a monoclonal immunoglobulin peak, and bone marrow aspiration reveals atypical plasma cells. Urinalysis shows significant proteinuria. What amyloid precursor protein is most likely to be deposited in this patient?

Accepted Answer

Immunoglobulin light chain protein

Answer

Amylin protein

Answer

Apolipoprotein serum amyloid A

Answer

Fibrinogen protein

Question 10

A 12-month-old girl of Punjabi parents developed pallor since 3 months of age. One unit of blood transfusion was done at 5 months of age. She now presents with pallor and hepatosplenomegaly. Her hemoglobin level is 3.8 g/dL, MCV is 68, and MCH is 19. A peripheral smear examination showed schistocytes, and bone marrow examination revealed erythroid hyperplasia. What is the diagnosis?

Accepted Answer

Beta-thalassemia (major)

Answer

Alpha-thalassemia

Answer

Sickle cell disease

Answer

Glucose-6-phosphate dehydrogenase deficiency

Hematopathology — MCQs

Hematopathology — MCQs

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