Myeloproliferative Neoplasms — MCQs

10 questions

Excretory urography should be cautiously performed in

A 64-year-old woman comes to the physician for her routine health maintenance examination. She feels well. She had cervical cancer and received radiotherapy 8 years ago. Her vital signs are within normal limits. On percussion, the spleen size is 15 cm. Otherwise, the physical examination shows no abnormalities. The laboratory test results are as follows: Hemoglobin 10 g/dL Mean corpuscular volume 88 μm3 Leukocyte count 65,000/mm3 Platelet count 500,000/mm3 Two images of the peripheral blood smear are shown on the image. Which of the following is the most appropriate next step in management?

BCR-ABL fusion gene is MOST CHARACTERISTICALLY seen in?

In polycythemia vera all are true except

Before the advent of tyrosine kinase inhibitors, the most effective treatment of chronic myeloid leukemia was:

Which of the following is the most common hematologic malignancy associated with Neurofibromatosis-1 (NF-1) in a child?

All are true about polycythemia vera except one.

Which type of gout is seen in a patient who is on treatment of CML?

All of the following are seen in polycythemia rubra vera except :

Q10

Which of the following statements about Polycythemia vera is false?

Myeloproliferative Neoplasms Indian Medical PG Practice Questions and MCQs

Question 1: Excretory urography should be cautiously performed in

A. Bone metastases
B. Neuroblastoma
C. Leukemia
D. Multiple myeloma (Correct Answer)

Explanation: ***Multiple myeloma*** - Excretory urography (intravenous pyelography or IVP) involves the administration of **iodinated contrast media**, which can precipitate **Bence Jones proteins** in the renal tubules, leading to or worsening **acute kidney injury** in patients with multiple myeloma. - Patients with multiple myeloma often have **pre-existing renal dysfunction** (myeloma kidney) due to light chain deposition, making them highly susceptible to contrast-induced nephropathy. *Bone metastases* - While bone metastases can be painful and may require imaging, they do not directly contraindicate excretory urography; the primary concern with IVP is renal function. - The presence of bone lesions itself does not increase the risk of **contrast-induced nephropathy** in the same way that proteinuria from multiple myeloma does. *Neuroblastoma* - Neuroblastoma is a **childhood cancer** affecting the adrenal glands or sympathetic nervous system, and it is not typically associated with a specific risk for contrast-induced nephropathy from excretory urography. - The primary diagnostic imaging for neuroblastoma often involves ultrasound, CT, or MRI, and while contrast may be used, the specific renal risk seen in multiple myeloma is not present. *Leukemia* - While some forms of leukemia can affect the kidneys, particularly through infiltration, it does not typically pose the same specific risk for **contrast-induced nephropathy** as multiple myeloma. - The renal manifestations in leukemia are generally different from the **light chain proteinuria** seen in multiple myeloma, which directly interacts with iodinated contrast.

Question 2: A 64-year-old woman comes to the physician for her routine health maintenance examination. She feels well. She had cervical cancer and received radiotherapy 8 years ago. Her vital signs are within normal limits. On percussion, the spleen size is 15 cm. Otherwise, the physical examination shows no abnormalities. The laboratory test results are as follows: Hemoglobin 10 g/dL Mean corpuscular volume 88 μm3 Leukocyte count 65,000/mm3 Platelet count 500,000/mm3 Two images of the peripheral blood smear are shown on the image. Which of the following is the most appropriate next step in management?

A. Phlebotomy
B. Rituximab
C. Watchful waiting
D. Allogeneic stem cell transplantation
E. Dasatinib (Correct Answer)

Explanation: ***Dasatinib*** - The patient's presentation with **anemia**, **marked leukocytosis (65,000/mm³) with predominantly myeloid cells**, **thrombocytosis (500,000/mm³)**, and **splenomegaly (15 cm)** is highly suggestive of **Chronic Myeloid Leukemia (CML)**. The peripheral smear showing **myelocytes, metamyelocytes, and basophils** (indicated by arrows on the image) further supports this diagnosis. - **Dasatinib** is a second-generation **tyrosine kinase inhibitor (TKI)** that targets the **BCR-ABL fusion protein**, which is the hallmark of CML. TKIs are the first-line treatment for CML. *Phlebotomy* - This is primarily used for **polycythemia vera** to reduce red blood cell mass and hematocrit. - It is not indicated for CML, which is characterized by an overproduction of myeloid cells, not primarily red blood cells. *Rituximab* - **Rituximab** is a monoclonal antibody targeting the **CD20 antigen** on B-lymphocytes. - It is used in the treatment of **B-cell non-Hodgkin lymphoma** and some autoimmune diseases, not CML. *Watchful waiting* - Given the patient's clear signs of **CML (leukocytosis, thrombocytosis, splenomegaly, and characteristic peripheral smear)**, active treatment is indicated, not watchful waiting. - CML, if left untreated, progresses from the chronic phase to accelerated phase and then to **blast crisis**, which is rapidly fatal. *Allogeneic stem cell transplantation* - **Allogeneic stem cell transplantation** is a potentially curative treatment for CML. - However, it is typically reserved for patients who have **failed TKI therapy** or have high-risk features, as **TKIs are the initial first-line treatment** due to their effectiveness and lower toxicity compared to transplantation.

Question 3: BCR-ABL fusion gene is MOST CHARACTERISTICALLY seen in?

A. CML (Correct Answer)
B. AML
C. Chronic Lymphocytic Leukemia (CLL)
D. Acute Lymphoblastic Leukemia (ALL)

Explanation: ***CML*** - The **BCR-ABL gene mutation** is characteristic of **Chronic Myeloid Leukemia (CML)**, resulting from a translocation between chromosomes 9 and 22 [1]. - This mutation leads to the production of the **BCR-ABL fusion protein**, which promotes cell proliferation and inhibits apoptosis [1]. *AML* - Acute Myeloid Leukemia (AML) does not typically exhibit the **BCR-ABL fusion gene**; rather, it is associated with various other genetic mutations. - Key features of AML include **myeloblast proliferation** and it presents with different cytogenetic abnormalities like **FLT3 or NPM1 mutations**. *CLL* - Chronic Lymphocytic Leukemia (CLL) is characterized by the accumulation of **mature lymphocytes**, not the **BCR-ABL mutation**. - It is often associated with mutations such as **TP53** and **NOTCH1**, distinct from myeloid malignancies. *ALL* - Acute Lymphoblastic Leukemia (ALL) is primarily linked with **chromosomal translocations** involving **the TCF3** gene or others, but not specifically with **BCR-ABL**. - In ALL, **lymphoid progenitor cells** proliferate, whereas CML is primarily a **myeloid process** associated with the BCR-ABL gene [1]. **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. 624-625.

Question 4: In polycythemia vera all are true except

A. Increased leukocyte alkaline phosphatase
B. Thrombocytosis
C. Decreased levels of erythropoietin
D. High ESR (Correct Answer)

Explanation: ***High ESR*** - In **polycythemia vera**, the **increased red blood cell mass** leads to **hyperviscosity** and often a **decreased erythrocyte sedimentation rate (ESR)**, as the red blood cells settle more slowly due to hindrance. - A high ESR would be **atypical** for polycythemia vera and would suggest an underlying inflammatory process or another condition. *Increased leukocyte alkaline phosphatase* - **Leukocyte alkaline phosphatase (LAP) score** is typically **increased** in polycythemia vera, reflecting the **overproduction of mature neutrophils**. - This is a distinguishing feature from disorders like **chronic myeloid leukemia (CML)**, where the LAP score is low [3]. *Thrombocytosis* - **Thrombocytosis** (elevated platelet count) is a common finding in polycythemia vera due to the **panmyelosis** associated with the disease [1], [3]. - The **JAK2V617F mutation**, frequently present in PV, stimulates megakaryocyte production, leading to increased platelets [1]. *Decreased levels of erythropoietin* - In polycythemia vera, red blood cell production is **independent of erythropoietin**, leading to a **suppressed** or **very low erythropoietin level** in response to the high red cell mass [1], [2]. - This differentiates PV from **secondary polycythemia**, where erythropoietin levels are elevated [2]. **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. Red Blood Cell and Bleeding Disorders, pp. 663-664. [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. 626-627.

Question 5: Before the advent of tyrosine kinase inhibitors, the most effective treatment of chronic myeloid leukemia was:

A. Chemotherapy
B. Hydroxyurea and interferon
C. Haploidentical bone marrow transplant
D. Allogeneic bone marrow transplant (Correct Answer)

Explanation: ***Allogeneic bone marrow transplant*** - Before the advent of TKIs, **allogeneic hematopoietic stem cell transplantation (HSCT)** was the only curative treatment for CML [1]. - It involved replacing the patient's diseased bone marrow with healthy stem cells from a genetically matched donor, thereby eradicating the **Philadelphia chromosome-positive clone** [1]. *Haploidentical bone marrow transplant* - While a form of HSCT, **haploidentical transplants** were typically used as a backup option when a fully matched donor was unavailable due to higher risks of **graft-versus-host disease (GVHD)** and rejection. - It was not considered the most effective or preferred treatment before TKIs, being reserved for specific challenging cases. *Chemotherapy* - **Conventional chemotherapy** for CML, such as busulfan or hydroxyurea, primarily aimed at reducing the white blood cell count and controlling symptoms. - It was **palliative** and did not offer a cure or significantly prolong survival in the long term, unlike allogeneic HSCT [1]. *Hydroxyurea and interferon* - **Hydroxyurea** is a cytoreductive agent, and **interferon-alpha** was used to induce hematologic and cytogenetic responses in CML patients. - Although they provided some benefit in controlling the disease and improving survival compared to no treatment, they rarely achieved a cure and were associated with significant side effects, making them less effective than allogeneic HSCT [1].

Question 6: Which of the following is the most common hematologic malignancy associated with Neurofibromatosis-1 (NF-1) in a child?

A. Chronic Myeloid Leukemia (CML)
B. Acute Lymphoblastic Leukemia (ALL)
C. Acute Myeloid Leukemia (AML)
D. Juvenile Myelomonocytic Leukemia (JMML) (Correct Answer)

Explanation: ***Juvenile Myelomonocytic Leukemia (JMML)*** - **JMML** is a myelodysplastic/myeloproliferative neoplasm that is strongly associated with **NF-1** in children, particularly due to mutations in the *NF1* gene. - Children with **NF-1** have a significantly increased risk of developing **JMML** compared to the general pediatric population. *Chronic Myeloid Leukemia (CML)* - While CML can occur in children, it is typically associated with the **Philadelphia chromosome (BCR-ABL1 fusion gene)** and not a common tumor directly linked to NF-1. - **CML** is relatively rare in childhood compared to other leukemias and is not a characteristic complication of NF-1. *Acute Lymphoblastic Leukemia (ALL)* - **ALL** is the most common childhood cancer overall, but its association with **NF-1** is not as specific or strong as that of **JMML**. - While children with NF-1 may have a slightly increased risk of certain cancers, **ALL** is not the *most common* tumor directly linked to NF-1. *Acute Myeloid Leukemia (AML)* - **AML** has a weak association with **NF-1**, particularly specific subtypes, but it is less frequent and less specifically linked than **JMML**. - The direct genetic pathway involving the **NF1 gene** mutation in the development of **AML** is not as clearly defined as it is for **JMML**.

Question 7: 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 8: Which type of gout is seen in a patient who is on treatment of CML?

A. Pseudogout
B. Acute gout
C. Primary gout
D. Secondary gout (Correct Answer)

Explanation: ***Secondary gout*** - **Secondary gout** occurs when a high uric acid level is a consequence of another medical condition or its treatment, such as **chronic myelogenous leukemia (CML)** [1]. - The increased cell turnover in CML, especially during treatment, leads to a significant release of **purines**, which are then metabolized into **uric acid**, causing hyperuricemia and gout [1]. *Pseudogout* - **Pseudogout** is caused by the deposition of **calcium pyrophosphate dihydrate (CPPD)** crystals, not uric acid crystals, in the joints [2]. - While it can mimic gout attacks, it has a different underlying pathophysiology and is not directly linked to CML or its treatment. *Acute gout* - **Acute gout** describes the sudden onset of severe pain, swelling, and redness in a joint, which is a common presentation of any type of gout, whether primary or secondary. - This term refers to a **gout flare** rather than the underlying cause of the hyperuricemia. *Primary gout* - **Primary gout** occurs due to an intrinsic metabolic defect in purine metabolism or renal excretion of uric acid, without an identifiable underlying disease [1]. - In this scenario, the gout is secondary to CML and its treatment, making **primary gout** an incorrect classification [1].

Question 9: All of the following are seen in polycythemia rubra vera except :

A. Increased platelets
B. Increased Vit B12 binding capacity (>9000 micromols/dL)
C. Leucocytosis
D. Decreased LAP Score (Correct Answer)

Explanation: ***Decreased LAP Score*** - **LAP (Leukocyte Alkaline Phosphatase) score** is typically **increased or normal** in polycythemia vera. - A decreased LAP score is characteristic of **chronic myeloid leukemia (CML)**, which must be differentiated from polycythemia vera [2]. *Increased platelets* - **Thrombocytosis** (increased platelets) is a common feature of **polycythemia vera**, often contributing to vascular complications [1], [2]. - The unregulated proliferation of all myeloid cell lines, including megakaryocytes, leads to this increase [1], [3]. *Increased Vit B12 binding capacity (>9000 micromols/dL)* - Polycythemia vera often leads to **increased vitamin B12 levels** and **binding capacity** due to increased production of **transcobalamin I** by proliferating granulocytes. - While not a direct diagnostic criterion, it is a frequent finding supportive of the diagnosis. *Leucocytosis* - **Leukocytosis** (increased white blood cell count), particularly granulocytosis, is a common feature of polycythemia vera [1], [2]. - It results from the **clonal proliferation** of myeloid stem cells, leading to an overproduction of all myeloid lineage cells [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. [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. Diseases of White Blood Cells, Lymph Nodes, Spleen, and Thymus, pp. 627-628.

Question 10: Which of the following statements about Polycythemia vera is false?

A. Increased LAP score (Correct Answer)
B. Increased vitamin B12 levels
C. Leukocytosis is present
D. Increased platelet count

Explanation: ***Decrease LAP score*** - In polycythemia vera, the **LAP (leukocyte alkaline phosphatase) score** is typically increased, indicating more mature leukocytes. - A **decrease in LAP score** is not consistent with the disease, making this statement incorrect. *Increased platelets* - Polycythemia vera often results in **thrombocytosis**, characterized by increased platelet counts [1]. - This is a common feature of the disorder, reflecting overproduction of blood cells in the bone marrow. *Leucocytosis* - Patients with polycythemia vera frequently exhibit **leucocytosis**, or increased white blood cell counts, due to hypercellularity of the bone marrow [1]. - This is an important aspect of the disease, often seen alongside increases in red blood cells and platelets. *Increased vit B12* - An elevation in **vitamin B12** levels can occur in polycythemia vera, often due to increased binding proteins. - This is a well-recognized phenomenon associated with the increased cell turnover in this condition. **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. 626-627.

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