Myeloproliferative Neoplasms — MCQs
An asymptomatic patient on regular health checkup has platelet counts of 8,00,000/cu.mm. Next line of management is:
A bone marrow biopsy in a patient with suspected myelofibrosis shows atypical megakaryocyte proliferation. Which additional finding would confirm the diagnosis?
All are true about polycythemia vera except one.
Which of the following is commonly seen in Polycythemia Vera?
Which of the following is not a myeloproliferative disorder?
Chemotherapeutic Agent of Choice for the treatment of CML?
Which is not seen in Polycythemia Vera?
What is the typical bone marrow finding in myelofibrosis?
Haemoglobin F is raised in which condition?
Which of the following conditions is not associated with antiphospholipid syndrome?
Myeloproliferative Neoplasms Indian Medical PG Practice Questions and MCQs
Question 1: An asymptomatic patient on regular health checkup has platelet counts of 8,00,000/cu.mm. Next line of management is:
- A. Phlebotomy
- B. Plasmapheresis
- C. Bone marrow biopsy
- D. Follow up and observe (Correct Answer)
Explanation: ***Follow up and observe*** - This patient is **asymptomatic** despite a high platelet count (800,000/cu.mm), suggesting that the thrombocytosis may be **benign** or a **transient phenomenon**. - In an asymptomatic patient with isolated thrombocytosis, it is crucial to **repeat the complete blood count (CBC)** to rule out laboratory error or a temporary reactive process before proceeding with invasive or extensive investigations. *Phlebotomy* - **Phlebotomy** is primarily used to reduce red cell mass in conditions like **polycythemia vera**, not typically for high platelet counts. - While it can be considered in specific cases of **thrombocytosis with erythrocytosis**, it is not the initial management for isolated asymptomatic thrombocytosis. *Plasmapheresis* - **Plasmapheresis** involves removing plasma and is indicated for conditions like thrombotic thrombocytopenic purpura (TTP) or some autoimmune diseases [2]. - It is **not used to manage isolated thrombocytosis**, as it does not directly affect platelet production or removal in a sustained manner. *Bone marrow biopsy* - A **bone marrow biopsy** is an invasive procedure generally reserved for investigating suspected **myeloproliferative neoplasms (MPN)** or other hematological malignancies [1]. - In an **asymptomatic patient** with an initial high platelet count, it is premature and not the first line of management without prior re-evaluation of the platelet count and exclusion of reactive causes [1].
Question 2: A bone marrow biopsy in a patient with suspected myelofibrosis shows atypical megakaryocyte proliferation. Which additional finding would confirm the diagnosis?
- A. Increased reticulin fibrosis (Correct Answer)
- B. Sea-blue histiocytes
- C. Pseudo-Gaucher cells
- D. Crystal-storing histiocytes
Explanation: Detailed Analysis: ***Increased reticulin fibrosis*** - **Increased reticulin fibrosis** (grade 2-3) detected by reticulin staining is the **hallmark diagnostic feature** of myelofibrosis [2]. - Myelofibrosis is characterized by proliferation of atypical **megakaryocytes** that release growth factors (PDGF, TGF-̠) leading to reactive **reticulin and collagen deposition** [1], [2]. - Diagnosis requires both **atypical megakaryocytes** and **increased bone marrow fibrosis** on biopsy. *Sea-blue histiocytes* - These are lipid-laden macrophages seen in **Niemann-Pick disease**, **chronic myeloid leukemia**, and some storage disorders. - Not a diagnostic criterion for myelofibrosis. - Their presence is incidental and non-specific. *Pseudo-Gaucher cells* - These resemble Gaucher cells but are found in **chronic myeloid leukemia** and other myeloproliferative neoplasms. - They are macrophages with wrinkled-paper cytoplasm due to lipid accumulation. - Not specific for myelofibrosis diagnosis. *Crystal-storing histiocytes* - Rare finding associated with **monoclonal gammopathies** and **plasma cell dyscrasias**. - Histiocytes contain immunoglobulin crystals. - Not related to myelofibrosis pathogenesis or diagnosis. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 614-615. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 615-616.
Question 3: All are true about polycythemia vera except one.
- A. Increased erythropoietin (Correct Answer)
- B. Decreased erythropoietin
- C. Increased LAP Score
- D. Increased blood viscosity
Explanation: ***Increased erythropoietin*** - Polycythemia vera is characterized by **low erythropoietin levels** due to the autonomous proliferation of erythroid progenitor cells in the bone marrow [3]. - The disease is associated with a **JAK2 mutation**, which leads to erythrocytosis independent of erythropoietin stimulation [2,3]. *Increased LAP Score* - The LAP (leukocyte alkaline phosphatase) score is often **increased** in polycythemia vera due to increased leukocyte activity. - This test helps differentiate it from **essential thrombocythemia**, which typically has a normal or low LAP score. *Decreased erythropoietin* - Polycythemia vera generally exhibits **decreased erythropoietin levels** because of the negative feedback from increased red blood cell mass [3]. - Patients typically show a lack of normal response to hypoxia, which is observed in secondary causes of erythrocytosis [3]. *Increased ESR* - The erythrocyte sedimentation rate (ESR) can be **increased** in polycythemia vera due to elevated levels of multiple proteins in the blood. - This non-specific marker often reflects **inflammation** or other hematological conditions. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 614-615. [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. 626-627. [3] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 663-664.
Question 4: Which of the following is commonly seen in Polycythemia Vera?
- A. Hyperuricemia
- B. Prone for acute leukemia
- C. Spontaneous severe infection
- D. Thrombosis (Correct Answer)
Explanation: ***Spontaneous severe infection*** - In Polycythemia Vera, there is usually an **increased red blood cell mass** leading to complications like thrombosis, rather than a predisposition to severe infections. - Severe infections are not a typical feature, as the condition usually maintains **functional immunity** despite hyperviscosity. *Thrombosis* - Individuals with Polycythemia Vera have increased blood viscosity that results in a higher risk of **thrombosis**, which is a common complication [1]. - Events like **deep vein thrombosis (DVT)** or **cerebral venous sinus thrombosis** are often observed due to altered hemodynamics. *Hyperuricemia* - Hyperuricemia occurs due to increased cell turnover and breakdown of red cells in Polycythemia Vera, leading to elevated **uric acid levels** [1]. - Patients may experience **gout attacks** as a consequence of this elevated uric acid [1]. *Prone for acute leukemia* - While there is an increased risk of transformation to myeloid neoplasms, the risk for **acute leukemia** is not directly attributed to Polycythemia Vera in most cases. - It is more related to myelofibrosis or secondary conditions developing over time rather than a direct association.
Question 5: Which of the following is not a myeloproliferative disorder?
- A. Acute myeloid leukemia (Correct Answer)
- B. Chronic myeloid leukemia
- C. Essential thrombocytosis
- D. Polycythemia vera
Explanation: ***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.
Question 6: Chemotherapeutic Agent of Choice for the treatment of CML?
- A. Imatinib (Correct Answer)
- B. Vincristine
- C. Cyclophosphamide
- D. Methotrexate
Explanation: ***Imatinib*** - **Imatinib** is a **tyrosine kinase inhibitor** specifically targeting the **BCR-ABL fusion protein**, which is the hallmark of CML [1][2]. - It dramatically improved the prognosis of CML patients, making it the **first-line therapy** and agent of choice due to its high efficacy and relatively low toxicity compared to conventional chemotherapy [2]. *Vincristine* - **Vincristine** is a **vinca alkaloid** that inhibits microtubule formation, primarily used in acute leukemias and lymphomas. - It is not the agent of choice for CML due to its different mechanism of action and the availability of more targeted therapies for CML. *Cyclophosphamide* - **Cyclophosphamide** is an **alkylating agent** that causes DNA damage, used in various cancers and autoimmune diseases. - While it can be used in some hematologic malignancies, it is not the preferred or most effective treatment for CML, especially given the success of targeted therapies. *Methotrexate* - **Methotrexate** is an **antimetabolite** that interferes with DNA synthesis, commonly used in acute leukemias, lymphomas, and autoimmune conditions. - It is not considered the chemotherapeutic agent of choice for CML, as its mechanism of action is not specific to the BCR-ABL anomaly characteristic of CML.
Question 7: Which is not seen in Polycythemia Vera?
- A. Increased erythropoietin level (Correct Answer)
- B. Increased RBC count
- C. Ocular congestion
- D. Increased Vitamin B12 binding capacity
Explanation: ***Increased erythropoietin level*** - In polycythemia vera, there is typically a **decrease in erythropoietin levels** due to the autonomous production of red blood cells. - The overproduction of red cells occurs independently of erythropoietin stimulation, distinguishing this condition from secondary causes of erythrocytosis. *Ocular congestion* - Ocular congestion can occur due to **increased blood volume** and vascular stasis associated with polycythemia vera. - It is often a result of elevated hematocrit levels leading to **erythromelalgia** and **hyperviscosity symptoms**. *Increase RBC count* - A hallmark of polycythemia vera is a **significant increase in red blood cell (RBC) count**, which is diagnostic for the condition. - This increase in RBCs contributes to symptoms such as **headaches, dizziness**, and ruddy complexion. *Increased Vit B12 binding capacity* - Polycythemia vera is associated with **increased vitamin B12 levels** due to elevated levels of transcobalamin, resulting in high binding capacity. - This phenomenon helps differentiate polycythemia vera from other forms of polycythemia. [1]
Question 8: What is the typical bone marrow finding in myelofibrosis?
- A. Megaloblastic cells
- B. Microcytic cells
- C. Thrombocytosis
- D. Dry tap (hypocellular) (Correct Answer)
Explanation: ***Dry tap (hypocellular)*** - In myelofibrosis, the bone marrow is often **hypocellular** due to fibrosis [1][2], leading to a **dry tap** during aspiration. - The presence of **reticulin** and collagen deposition replaces normal hematopoietic cells [2], resulting in ineffective hematopoiesis. *Thrombocytosis* - Myelofibrosis typically leads to **thrombocytopenia**, not thrombocytosis, due to ineffective megakaryopoiesis and splenic sequestration. - Though elevated platelets can occur, they are generally a **secondary response** to the disease and not a hallmark finding. *Megaloblastic cells* - Megaloblastic changes are associated with **vitamin B12** or **folate deficiencies**, which do not occur in myelofibrosis. - In myelofibrosis, the predominant issue is **marrow fibrosis** [1][2], which does not lead to megaloblastosis. *Microcytic cells* - Microcytic cells are commonly linked to **iron deficiency anemia**, not myelofibrosis. - Myelofibrosis typically results in **variable red cell morphology** [1], but microcytic anemia is not a primary characteristic. **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.
Question 9: Haemoglobin F is raised in which condition?
- A. Hereditary persistence of fetal hemoglobin (HPFH)
- B. Beta-thalassemia major
- C. Sickle cell disease
- D. Juvenile chronic myeloid leukemia (Correct Answer)
Explanation: ***Juvenile chronic myeloid leukemia*** - This condition is characterized by a high proportion of **fetal hemoglobin (HbF)**, often exceeding 50%, alongside other typical myeloproliferative features. - The elevated HbF is a distinguishing feature of **juvenile CML** from adult CML, which typically presents with normal or only slightly elevated HbF levels. *Beta-thalassemia major* - While patients with **beta-thalassemia major** can have elevated HbF, it is typically in response to a severe deficiency in beta-globin chain production, leading to compensatory gamma-chain synthesis. - However, the primary genetic defect lies in the beta-globin genes, and the HbF increase is usually not as universally high or definitive as in HPFH or juvenile CML. *Sickle cell disease* - Patients with **sickle cell disease** can have variable levels of HbF, and higher levels are associated with a milder disease course [1]. - HbF acts as a protective factor by inhibiting hemoglobin S polymerization, but the presence of high HbF is not a diagnostic marker in the same way it is for HPFH or juvenile CML [1]. *Hereditary persistence of fetal hemoglobin (HPFH)* - This is a benign condition characterized by the **continued production of high levels of HbF into adulthood** due to genetic mutations that prevent the normal developmental switch from gamma-globin to beta-globin synthesis. - While it features significantly raised HbF, HPFH is typically **asymptomatic** and does not present with the myeloproliferative features seen in juvenile CML.
Question 10: Which of the following conditions is not associated with antiphospholipid syndrome?
- A. Venous thrombosis
- B. Neurological manifestations
- C. Thrombocytosis (Correct Answer)
- D. Recurrent foetal loss
Explanation: ***Thrombocytosis*** - **Antiphospholipid syndrome (APS)** is characterized by increased **thrombosis** (clotting), not an increase in platelet count (thrombocytosis). - Patients with APS may experience **thrombocytopenia** (low platelet count), which is distinct from thrombocytosis [1]. *Venous thrombosis* - **Venous thrombosis**, particularly deep vein thrombosis (DVT) and pulmonary embolism (PE), is a hallmark feature of APS [2]. - The presence of **antiphospholipid antibodies** promotes a procoagulant state, leading to clot formation in veins. *Recurrent foetal loss* - **Recurrent foetal loss**, including miscarriages and stillbirths, is a classic obstetric manifestation of APS. - The antibodies interfere with placental function and blood supply, leading to pregnancy complications. *Neurological manifestations* - Various **neurological manifestations** are associated with APS, including stroke, transient ischemic attacks (TIAs), and cognitive dysfunction. - These are often due to **microthrombosis** in the cerebral vasculature or direct antibody effects.
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