Hematopathology Practice Questions

Q: A 5-year-old boy presented to the emergency room with vomiting and abdominal distension. Abdominal imaging showed a mass in the ileocecal region. Histopathological examination of a biopsy from the region showed a diffuse infiltrate of lymphoid cells admixed with macrophages, giving a "starry-sky" appearance. Which of the following translocations is least likely to be seen in Burkitt lymphoma?

t(11;14). ***t(8;22)*** - This translocation is commonly associated with **Burkitt lymphoma**, which presents with a **starry-sky appearance** on histopathology due to the high proliferation of lymphoid cells and macrophages [1]. - It often occurs in the **abdominal region** and is characterized by rapidly growing tumors typically seen in pediatrics, aligning with the case of the 5-year-old boy. *t(11;14)* - Primarily associated with **Mantle cell lymphoma**, which typically presents in older adults, and not usually in the pediatric population. - This translocation involves the **BCL-1 gene**, leading to overexpression of cyclin D1, and does not match the histological features described. *t(2;8)* - This translocation is linked to **Lymphoblastic lymphoma** and some variants of **Burkitt lymphoma**, but it is less common compared to t(8;14) and t(8;22). - It involves the **MYC gene**, but the specific clinical and histological features described do not fit this option. *t(8;14)* - While this translocation is associated with Burkitt lymphoma, it involves the **myc oncogene and immunoglobulin heavy chain locus**, affecting a different presentation profile [2]. - It usually manifests with **extranodal masses**, particularly in the jaw or abdomen, but does not align specifically with the features outlined in this case. **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. 606. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Neoplasia, pp. 324-325.

Q: Which of the following is not a myeloproliferative disorder?

Acute myeloid leukemia. ***Acute myeloid leukemia*** - *Acute myeloid leukemia (AML)* is a **myeloid neoplasm** characterized by the rapid proliferation of myeloid cells and is classified as an acute leukemia, not a myeloproliferative disorder. - It involves **highly abnormal cells** that impede normal blood cell production, contrasting with chronic myeloproliferative disorders which have a more gradual progression. *Essential thrombocytosis* - This is a true **myeloproliferative disorder** characterized by an **increase in platelet count** and is due to the increased production of megakaryocytes in the bone marrow [1]. - Patients can present with thrombotic or hemorrhagic complications, supporting its classification as a myeloproliferative neoplasm. *Chronic myeloid leukemia* - Chronic myeloid leukemia (CML) is another type of **myeloproliferative disorder**, arising from a genetic mutation leading to excessive production of myeloid cells. - It is associated with the **Philadelphia chromosome** and typically presents in a chronic phase with variable leukocytosis. *Polycythemia vera* - Polycythemia vera is a **myeloproliferative neoplasm** characterized by hyperproduction of red blood cells, often accompanied by leukocytosis and thrombocytosis [1]. - It is associated with mutations in the **JAK2 gene**, leading to increased erythropoiesis and elevation of hemoglobin levels, confirming its classification [1]. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 614-615.

Q: Which is the most common mutation in hereditary spherocytosis?

Ankyrin (ANK1). ***Ankyrin*** - The most common mutation in hereditary spherocytosis is in the **ankyrin gene**, which disrupts the spectrin-actin cytoskeleton structure in red blood cells [1]. - This leads to **decreased membrane stability**, causing the spherocytes to be more prone to hemolysis [1]. *Band 3* - Mutations in the **Band 3 protein** can also occur in hereditary spherocytosis but are less common compared to ankyrin mutations [1]. - Band 3 primarily affects **chloride-bicarbonate exchange** but does not represent the most prevalent mutation in this condition. *Glycophorin* - Glycophorin mutations are typically associated with **hereditary elliptocytosis**, not with hereditary spherocytosis. - This oes not relate to the pathophysiology of spherocytosis, which involves cytoskeletal proteins. *Spectrin* - While **spectrin** is crucial for maintaining the cell membrane's integrity, mutations here are not the most frequently observed in hereditary spherocytosis cases [1]. - Spectrin mutations can cause red blood cell shape abnormalities but are overshadowed by ankyrin-related mutations in terms of prevalence. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 640-641.

Q: A 15-year-old female presented to the emergency department with a history of recurrent epistaxis, hematuria, and hematochezia. There was a history of profuse bleeding from the umbilical stump at birth. Previous investigations revealed normal prothrombin time, activated partial thromboplastin time, thrombin time, and fibrinogen levels. Her platelet counts as well as platelet function tests were normal, but the urea clot lysis test was positive. Which clotting factor is most likely to be deficient?

Factor XIII. ***Factor XIII*** - A positive **urea clot lysis test** indicates deficiency of factor XIII, which is responsible for stabilizing the **fibrin clot**. - **Recurrent bleeding** from various sites, including the umbilical stump, with normal standard coagulation tests (PT, aPTT, TT, fibrinogen, platelet count, and function) is characteristic of factor XIII deficiency. *Factor X* - Deficiency of factor X would lead to abnormal **PT and aPTT**, as it is involved in both the intrinsic and extrinsic pathways, which is not the case here [1]. - Patients typically present with **severe bleeding manifestations**, and routine coagulation tests would be prolonged. *Factor XI* - Factor XI deficiency primarily causes an abnormally **prolonged aPTT**, as it is part of the intrinsic pathway, which is normal in this patient [1]. - Bleeding symptoms can be variable, but it does not specifically present with a **positive urea clot lysis test**. *Factor XII* - Factor XII deficiency causes a markedly prolonged **aPTT** [1] but is usually **asymptomatic** and not associated with significant bleeding. - It would not result in a **positive urea clot lysis test** or the severe bleeding symptoms described.

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: Hemolytic anemia may be characterized by all of the following except which of the following?

Increased plasma haptoglobin level. ***Increased plasma haptoglobin level*** - **Haptoglobin** binds to free hemoglobin released during **hemolysis** and is then cleared from the circulation [1]. Therefore, in hemolytic anemia, the plasma haptoglobin level would be **decreased**, not increased. - An increased haptoglobin level typically indicates an **inflammatory response** or is observed in conditions without significant intravascular hemolysis. *Hyperbilirubinemia* - **Increased breakdown of red blood cells** releases large amounts of **heme**, which is metabolized into **unconjugated bilirubin** [2]. - This leads to an overload of the liver's capacity to conjugate bilirubin, resulting in **unconjugated hyperbilirubinemia** [2]. *Reticulocytosis* - The body responds to the loss of red blood cells through hemolysis by **increasing production of new red blood cells** in the bone marrow. - This increased production leads to a higher-than-normal percentage of immature red blood cells, known as **reticulocytes**, in the peripheral blood. *Hemoglobinuria* - When **intravascular hemolysis** occurs, free hemoglobin is released into the bloodstream and exceeds the binding capacity of haptoglobin [1]. - This excess unbound hemoglobin is filtered by the kidneys and excreted in the urine, leading to **dark-colored urine** due to hemoglobin.

Q: What is the estimated amount of iron delivered from the transfusion of 2 units of packed red cells?

500mg. ***500mg*** - Each unit of **packed red blood cells (PRBCs)** contains approximately **250 mg of iron**. - Therefore, two units would deliver approximately **500 mg of iron** to the recipient. *1g* - This amount would typically be derived from four units of PRBCs, which is double the number of units specified in the question. - This level of iron is usually associated with more significant transfusion burden, as seen in patients with chronic transfusions. *100mg* - This amount represents less than half the iron content of a single unit of PRBCs, making it an underestimation. - One unit of PRBCs contains approximately 200-250 mg of iron, so 100 mg is insufficient. *250mg* - This is the approximate iron content of a single unit of PRBCs, not the two units mentioned in the question. - The question specifically asks for the iron content from two units of PRBCs, requiring a cumulative calculation.

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.

Question 1

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 2

A 5-year-old boy presented to the emergency room with vomiting and abdominal distension. Abdominal imaging showed a mass in the ileocecal region. Histopathological examination of a biopsy from the region showed a diffuse infiltrate of lymphoid cells admixed with macrophages, giving a "starry-sky" appearance. Which of the following translocations is least likely to be seen in Burkitt lymphoma?

Accepted Answer

t(11;14)

Answer

t(8;14)

Answer

t(2;8)

Answer

t(8;22)

Question 3

Which of the following is not a myeloproliferative disorder?

Accepted Answer

Acute myeloid leukemia

Answer

Chronic myeloid leukemia

Answer

Essential thrombocytosis

Answer

Polycythemia vera

Question 4

Which is the most common mutation in hereditary spherocytosis?

Accepted Answer

Ankyrin (ANK1)

Answer

Glycophorin

Answer

Spectrin

Answer

Band 3 (SLC4A1)

Question 5

A 15-year-old female presented to the emergency department with a history of recurrent epistaxis, hematuria, and hematochezia. There was a history of profuse bleeding from the umbilical stump at birth. Previous investigations revealed normal prothrombin time, activated partial thromboplastin time, thrombin time, and fibrinogen levels. Her platelet counts as well as platelet function tests were normal, but the urea clot lysis test was positive. Which clotting factor is most likely to be deficient?

Accepted Answer

Factor XIII

Answer

Factor XII

Answer

Factor X

Answer

Factor XI

Question 6

'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 7

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 8

Hemolytic anemia may be characterized by all of the following except which of the following?

Accepted Answer

Increased plasma haptoglobin level

Answer

Hyperbilirubinemia

Answer

Reticulocytosis

Answer

Hemoglobinuria

Question 9

What is the estimated amount of iron delivered from the transfusion of 2 units of packed red cells?

Accepted Answer

500mg

Answer

1g

Answer

100mg

Answer

250mg

Question 10

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)

Hematopathology — MCQs

Hematopathology — MCQs

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