Hematopathology Practice Questions

Q: Which of the following is the most common myeloproliferative disorder?

Essential Thrombocythemia. ***Essential Thrombocythemia*** - **Essential thrombocythemia (ET)** is the **most common myeloproliferative neoplasm**, with an incidence of approximately 1.5-2.4 per 100,000 per year. - It is characterized by **persistent thrombocytosis** (platelet count >450,000/μL) and megakaryocytic proliferation [1]. - Commonly associated with **JAK2 V617F mutation** (~55-60%), **CALR mutations** (~25-30%), and **MPL mutations** (~3-5%) [2]. *Polycythemia rubra vera* - **Polycythemia vera (PV)** is the **second most common** classic MPN, with an incidence of approximately 0.8-2.3 per 100,000 per year. - Characterized by increased red blood cell mass, often with leukocytosis and thrombocytosis [1]. - Strongly associated with **JAK2 V617F mutation** (present in >95% of cases) [2][3]. *CML* - **Chronic myeloid leukemia (CML)** has similar incidence to PV (approximately 1-2 per 100,000 per year). - Defined by the presence of the **Philadelphia chromosome (BCR-ABL1 fusion gene)** [2]. - Treated distinctly with tyrosine kinase inhibitors (TKIs). *Myelofibrosis* - **Primary myelofibrosis (PMF)** is the **least common** of the classic MPNs, with an incidence of approximately 0.3-1.5 per 100,000 per year. - Characterized by bone marrow fibrosis, extramedullary hematopoiesis, and splenomegaly [3]. - Associated with **JAK2, CALR, or MPL mutations** [2]. **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. 627-628. [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. 624. [3] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 614-615.

Q: Dohle bodies are characteristically seen in which hereditary condition?

May-Hegglin anomaly. ***May-Hegglin anomaly*** - **Dohle bodies** are characteristic inclusions in neutrophils, formed by aggregates of **rough endoplasmic reticulum**, and are a defining feature of **May-Hegglin anomaly**. - This condition also presents with **macrothrombocytopenia** and a tendency for bleeding, along with the presence of these distinctive inclusions. *Infections* - While infections can cause **toxic granulation** and sometimes **Dohle bodies** in neutrophils, they are not considered a hereditary condition. - The Dohle bodies seen in infections are typically transient and not associated with macrothrombocytopenia. *Burns* - Similar to infections, severe burns can induce **toxic changes** in neutrophils, including the formation of Dohle bodies. - However, burns are an acquired condition and not a hereditary disorder, nor are they typically associated with the other features of May-Hegglin anomaly. *Myelodysplastic syndrome* - This is a clonal hematopoietic stem cell disorder characterized by **ineffective hematopoiesis** and **dysplastic changes** in myeloid cells. - While various abnormalities can be seen in neutrophils in MDS, **Dohle bodies** as a characteristic hereditary feature are not typically associated with it; instead, other dysplastic features like hypogranulation or pseudo-Pelger-Huët anomaly are more common.

Q: Which of the following is not considered a poor prognostic factor for Hodgkin's lymphoma?

Young age. ***Young age*** - Young age is generally associated with a **better prognosis** in Hodgkin's lymphoma, as this population often responds well to treatment [1][3]. - Patients are likely to have **fewer comorbid conditions**, which contributes to improved survival rates. *Extranodal metastasis* - Extranodal involvement is a **poor prognostic factor** indicating more advanced disease and increased treatment resistance [1]. - It is commonly associated with a **higher stage of disease**, leading to less favorable outcomes. *Lymphocyte depletion* - Lymphocyte depletion subtype of Hodgkin's lymphoma is linked to a **worse prognosis** due to its aggressive nature and poor response to therapies [2]. - It presents with fewer lymphocytes, indicating a more challenging disease course and **lower survival rates** [2]. *Involvement of stomach* - Gastric involvement in Hodgkin's lymphoma is also considered a **poor prognostic factor**, suggesting advanced disease. - It often correlates with **systemic symptoms** and may incur a higher risk of complications and treatment failures. **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. 616-618. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Diseases Of The Urinary And Male Genital Tracts, pp. 559-560. [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, p. 618.

Q: Hemophilia B is a deficiency of which factor?

IX. ***IX*** - Hemophilia B, also known as **Christmas disease**, is caused by a deficiency in **Factor IX** [1]. - This condition is an **X-linked recessive disorder** that impairs the blood's ability to form clots, leading to prolonged bleeding [1]. *XII* - Deficiency in **Factor XII** (Hageman factor) is usually **asymptomatic** and does not lead to a bleeding disorder. - Individuals with Factor XII deficiency often have a **prolonged aPTT** but no clinical bleeding. *VIII* - A deficiency in **Factor VIII** causes **Hemophilia A**, which is the more common form of hemophilia [1]. - Hemophilia A also presents with bleeding symptoms, but it is distinct from Hemophilia B due to the specific factor involved [1]. *X* - Deficiency in **Factor X** (Stuart-Prower factor) is a rare **autosomal recessive** bleeding disorder. - Factor X deficiency affects both the intrinsic and extrinsic coagulation pathways, leading to prolongation of both **PT and aPTT**.

Q: Fanconi's anemia is a:

Genetic anemia. ***Genetic anemia*** - Fanconi's anemia is a rare, **inherited disorder** characterized by bone marrow failure, physical abnormalities, and an increased risk of cancer [1]. - It results from mutations in genes involved in **DNA repair**, leading to chromosomal instability and defective hematopoietic stem cell function. *Anemia due to iron deficiency* - **Iron deficiency anemia** occurs when there is insufficient iron to produce adequate hemoglobin, often due to poor diet, malabsorption, or blood loss. - It is not associated with the genetic mutations or bone marrow failure seen in Fanconi's anemia. *Anemia due to autoimmune response* - **Autoimmune hemolytic anemia** involves the immune system mistakenly attacking and destroying red blood cells, which is a different mechanism from Fanconi's anemia. - Conditions like **Lupus** or **autoimmune lymphoproliferative syndrome** are examples of diseases causing autoimmune anemias. *Anemia due to red blood cell destruction* - Anemia due to red blood cell destruction, or **hemolytic anemia**, can be caused by various factors including genetic defects (e.g., sickle cell anemia), infections, or certain medications [2]. - While Fanconi's anemia can eventually lead to pancytopenia and affect red blood cells, its primary cause is **bone marrow failure** due to genetic defects in DNA repair, rather than direct hemolysis. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 595-596. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, p. 638.

Q: What is the cause of the bleeding disorder in patients with Hemophilia A?

Deficiency of factor VIII in the coagulation cascade. ***Lack of reaction accelerator during activation of factor X in coagulation cascade*** - Hemophilia A is primarily due to a **deficiency of Factor VIII**, which acts as a cofactor for Factor X activation in the intrinsic pathway of the coagulation cascade [2]. - This lack of Factor VIII leads to **impaired fibrin clot formation**, resulting in prolonged bleeding [1]. *Neutralization of antithrombin III* - Antithrombin III is involved in the **inhibition of thrombin** and other serine proteases, not directly related to hemophilia A. - Neutralization of antithrombin III could lead to **increased clotting** rather than bleeding. *Release of Thromboxane A2* - Thromboxane A2 is primarily involved in **platelet aggregation and vasoconstriction**, which does not directly relate to the bleeding disorder of hemophilia A. - Its release is more relevant in conditions leading to **thrombotic events**, rather than bleeding disorders caused by factor deficiencies. *Lack of platelet aggregation* - Hemophilia A is specifically due to a deficiency in coagulation factors, not platelet dysfunction. - While platelet aggregation can affect hemostasis, it is not the underlying issue in hemophilia A, which causes prolonged bleeding times due to coagulation factor deficiencies.

Q: Test used for factor VIII deficiency identification is:

APTT. ***APTT*** - Activated Partial Thromboplastin Time (APTT) is specifically used to identify deficiencies in the **intrinsic pathway**, including **factor VIII deficiency** [1]. - A prolonged APTT indicates a defect in this pathway, which is essential for diagnosing **hemophilia A**, linked to factor VIII [1][2]. *FDP* - Fibrin degradation products (FDP) are used to assess **fibrinolytic activity**, not specific for factor VIII deficiency. - While elevated in various conditions, they do not provide specific information about the intrinsic pathway or factor levels. *D dimer* - D-dimer is primarily used to rule out **thrombotic disorders** such as **deep vein thrombosis** or **pulmonary embolism**. - It does not assess **coagulation factors** or deficiencies like factor VIII, thus not appropriate for this diagnosis. *PT* - Prothrombin Time (PT) evaluates the **extrinsic pathway** of coagulation, primarily involving **factors I, II, V, VII, and X** [1]. - Factor VIII deficiency will not typically affect the PT, making it unsuitable for identifying issues related to the intrinsic pathway [1]. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Hemodynamic Disorders, Thromboembolic Disease, and Shock, pp. 128-130. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 669-670.

Q: Relative polycythemia can be caused by all of the following conditions, except:

High altitude. ***High altitude*** - High altitude results in **hypoxia**, which stimulates **erythropoietin** production and increases the **absolute number of red blood cells**, leading to **absolute polycythemia**, not relative polycythemia [1]. - In absolute polycythemia, the **red cell mass** is genuinely increased, whereas in relative polycythemia, it is the **plasma volume** that decreases, making the red cell count appear high [1]. *Dehydration* - Dehydration causes a **decrease in plasma volume**, concentrating the remaining blood components. - This effectively increases the **hematocrit** and **red blood cell count** relative to the reduced plasma, leading to **relative polycythemia**. *Gaisbock syndrome* - **Gaisbock syndrome**, also known as **stress erythrocytosis** or **pseudo-polycythemia**, is characterized by a **normal red cell mass** but a **reduced plasma volume**. - This reduction in plasma volume leads to an elevated **hematocrit**, falling under the definition of **relative polycythemia**. *Dengue haemorrhagic fever* - In severe dengue haemorrhagic fever, there is significant **plasma leakage**, which leads to a decrease in **intravascular plasma volume**. - This plasma leakage results in **hemoconcentration**, causing an apparent increase in red blood cell count and hematocrit, thus representing **relative polycythemia**.

Question 1

Which of the following is the most common myeloproliferative disorder?

Accepted Answer

Essential Thrombocythemia

Answer

Polycythemia rubra vera

Answer

CML

Answer

Myelofibrosis

Question 2

Dohle bodies are characteristically seen in which hereditary condition?

Accepted Answer

May-Hegglin anomaly

Answer

Infections

Answer

Burns

Answer

Myelodysplastic syndrome

Question 3

Myeloid sarcoma, which is a tumor mass consisting of myeloid blasts occurring at anatomic sites other than the bone marrow, is often characterized by chromosome aberrations. Which of the following is NOT typically associated with myeloid sarcoma?

Accepted Answer

t (15;17)

Answer

Monosomy 7

Answer

Trisomy 8

Answer

inv (16)

Question 4

Which of the following is not considered a poor prognostic factor for Hodgkin's lymphoma?

Accepted Answer

Young age

Answer

Lymphocyte depletion

Answer

Extranodal metastasis

Answer

Involvement of stomach

Question 5

Which of the following statements on lymphoma is not true?

Accepted Answer

In general, follicular (nodular) NHL has worse prognosis compared to diffuse NHL.

Answer

HD more often tends to remain localized to a single group of lymph nodes and spreads by contiguity.

Answer

Several types of non-Hodgkin's lymphoma (NHL) may have a leukemic phase.

Answer

A single classification system for Hodgkin's disease (HD) is almost universally accepted.

Question 6

Hemophilia B is a deficiency of which factor?

Accepted Answer

IX

Answer

XII

Answer

VIII

Answer

X

Question 7

Fanconi's anemia is a:

Accepted Answer

Genetic anemia

Answer

Anemia due to iron deficiency

Answer

Anemia due to autoimmune response

Answer

Anemia due to red blood cell destruction

Question 8

What is the cause of the bleeding disorder in patients with Hemophilia A?

Accepted Answer

Deficiency of factor VIII in the coagulation cascade

Answer

Lack of platelet aggregation

Answer

Neutralization of antithrombin III

Answer

Release of Thromboxane A2

Question 9

Test used for factor VIII deficiency identification is:

Accepted Answer

APTT

Answer

PT

Answer

D dimer

Answer

FDP

Question 10

Relative polycythemia can be caused by all of the following conditions, except:

Accepted Answer

High altitude

Answer

Gaisbock syndrome

Answer

Dengue haemorrhagic fever

Answer

Dehydration

Hematopathology — MCQs

Hematopathology — MCQs

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