Hematological Malignancies — MCQs

10 questions

A boy presents with fever, night sweats, and neck swelling. The biopsy of swelling showed a starry sky appearance. What is the most likely genetic abnormality seen in this case?

A 20-year-old male presented with fatigue, weakness, and jaundice. What is the most likely diagnosis?

Which of the following types of leukemia is administered prophylactic methotrexate for CNS prophylaxis –

In acute myeloid leukemia, Auer rods are numerous in which?

Multiple myeloma is characterized by which of the following?

Among the following AML subtypes, non-specific esterase (NSE) staining is typically NEGATIVE in which one?

The most common cause of malignant adrenal mass is:

Which of the following is true regarding carcinoid tumor?

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

Q10

A 40-year-old patient presents with complains of low back pain and development of pallor. During workup very high levels of urinary beta2 microglobulin was noted. X-ray skull was performed. Bone marrow aspiration shows >10% plasma cells and serum electrophoresis shows M protein >30 g/L. All are useful for management of this patient except?

Hematological Malignancies Indian Medical PG Practice Questions and MCQs

Question 1: A boy presents with fever, night sweats, and neck swelling. The biopsy of swelling showed a starry sky appearance. What is the most likely genetic abnormality seen in this case?

A. RAS
B. BCR-ABL
C. p53
D. MYC (Correct Answer)

Explanation: ***MYC*** - The clinical presentation of **fever, night sweats, and neck swelling** in a child, coupled with a **starry sky appearance** on biopsy, is highly suggestive of **Burkitt lymphoma** [2, 3]. - **Burkitt lymphoma** is characterized by a **translocation involving the *MYC* gene** on chromosome 8, most commonly t(8;14), which leads to its overexpression and uncontrolled cell proliferation [1]. *RAS* - Mutations in the **RAS family of genes (HRAS, KRAS, NRAS)** are commonly found in a wide variety of cancers, including **leukemias, pancreatic cancer, and colorectal cancer**. - While *RAS* mutations drive proliferation, they are **not the primary genetic driver** of Burkitt lymphoma, nor are they linked to the characteristic starry sky appearance. *BCR-ABL* - The **BCR-ABL fusion gene**, resulting from the **Philadelphia chromosome (t(9;22))**, is the defining genetic abnormality of **chronic myeloid leukemia (CML)**. - CML presents with different symptoms and a distinct peripheral blood and bone marrow morphology, **not the "starry sky" appearance** seen in Burkitt lymphoma. *p53* - The **p53 tumor suppressor gene** is frequently mutated or inactivated in over half of all human cancers, leading to a loss of cell cycle control and apoptosis. - While **p53 mutations can occur in aggressive lymphomas**, including Burkitt lymphoma, the **primary and characteristic genetic abnormality** associated with Burkitt lymphoma and its presentation is the *MYC* translocation, not solely *p53* mutations. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Neoplasia, pp. 324-325. [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. 605-606. [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. 606.

Question 2: A 20-year-old male presented with fatigue, weakness, and jaundice. What is the most likely diagnosis?

A. Acute lymphoblastic leukemia
B. Chronic myeloid leukemia
C. Chronic lymphocytic leukemia
D. Acute myeloid leukemia (Correct Answer)

Explanation: ***Acute myeloid leukemia*** - Presents with **fatigue** and **weakness** due to bone marrow infiltration and resultant cytopenias, typical in this age group [1]. - Often shows **myeloblasts** on peripheral blood smear, confirming the diagnosis [2]. *Chronic myeloid leukemia* - Usually occurs in **older adults** and characterized by **elevated white blood cell counts** with a predominance of mature neutrophils. - Symptoms like fatigue may arise, but there are distinct **Philadelphia chromosome** findings and typically a **longer symptom duration**. *Acute lymphoblastic leukemia* - More common in **younger children** and often associated with **lymphadenopathy** and **thrombocytopenia**, rather than fatigue alone. - Characteristically shows **lymphoblasts** in the blood, which are not mentioned in this patient's presentation. *Chronic lymphocytic leukemia* - Typically presents in adults over **50 years** and is characterized by **lymphocytosis** and often asymptomatic in early stages. - Fatigue may occur but lacks the acute presentation and findings seen in **acute leukemias**. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 607-608. [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. 621-622.

Question 3: Which of the following types of leukemia is administered prophylactic methotrexate for CNS prophylaxis –

A. AML
B. CLL
C. CML
D. ALL (Correct Answer)

Explanation: ***ALL*** - **Acute Lymphoblastic Leukemia (ALL)** has a high propensity for **central nervous system (CNS) involvement**, necessitating prophylactic intrathecal methotrexate to prevent CNS relapse [1]. - Prophylactic treatment of the CNS is a standard component of ALL treatment protocols due to the risk of leukemic cell infiltration into the brain and spinal cord [1]. *AML* - **Acute Myeloid Leukemia (AML)** has a lower incidence of CNS involvement compared to ALL, so prophylactic CNS treatment is generally not routine unless specific risk factors are present. - While CNS involvement can occur in AML, it is more commonly treated with systemic chemotherapy that has CNS penetration or intrathecal treatment only when CNS disease is confirmed. *CLL* - **Chronic Lymphocytic Leukemia (CLL)** rarely involves the CNS, and prophylactic CNS treatment is not part of standard management. - When CNS involvement does occur in CLL, it is an aggressive, late-stage complication and typically requires specific, targeted therapy rather than prophylaxis. *CML* - **Chronic Myeloid Leukemia (CML)** has an extremely low risk of CNS involvement, especially in the chronic phase, and therefore, prophylactic CNS treatment is not administered. - CNS involvement in CML is usually seen during a blast crisis and is rare, making prophylaxis unnecessary.

Question 4: In acute myeloid leukemia, Auer rods are numerous in which?

A. M2 (Myeloblastic leukemia)
B. M4 (Acute myelomonocytic leukemia)
C. M5 (Acute monocytic leukemia)
D. M3 (Acute promyelocytic leukemia) (Correct Answer)

Explanation: ***M3*** - In acute myeloid leukemia (AML), **M3 subtype (promyelocytic leukemia)** is characterized by a high number of **Auer rods** in the leukemic cells [1][2]. - These *Auer rods* play a significant role in the diagnosis of this specific type of AML and are often associated with a **hypergranular promyelocyte** morphology [1]. *M5* - M5 is known as **acute monocytic leukemia**, which primarily features **monoblasts** and lacks Auer rods. - The predominant findings are **extramedullary infiltration** and a higher incidence of **gum hypertrophy**. *M2* - M2 refers to **acute myeloblastic leukemia** that does demonstrate some Auer rods, but not in as significant numbers as seen in M3 [2]. - This subtype is characterized by both **myeloblasts** and **maturation** into more differentiated cells, leading to varied morphology. *M4* - M4 is defined as **acute myelomonocytic leukemia**, which may have **myeloid and monocytic blasts** but usually has fewer Auer rods compared to M3. - It often presents with features of both **myeloid and monocytic lineages**, which differ from the Auer rod prominence in M3. **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. 621-622. [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. 620.

Question 5: Multiple myeloma is characterized by which of the following?

A. Monoclonal gammopathy (Correct Answer)
B. Bence Jones proteins
C. Presence of light chains
D. Hypergammaglobulinemia

Explanation: ***Monoclonal gammopathy*** - **Multiple myeloma** is defined by the proliferation of a **single clone of plasma cells** that produce a characteristic **monoclonal immunoglobulin** (M-protein) detected in serum or urine [1]. - This **monoclonal expansion** leads to the accumulation of abnormal, identical **immunoglobulins** or their fragments [2]. *Presence of light chains* - While the presence of **monoclonal free light chains** (either kappa or lambda) is typical in myeloma, this option describes only a component and not the overarching characteristic that defines the disease [2]. - Not all light chain presence indicates myeloma; a **monoclonal proliferation** of these light chains is what is significant. *Bence Jones proteins* - **Bence Jones proteins** are **monoclonal light chains** excreted in the urine, a common finding in multiple myeloma [2]. - However, like the presence of light chains, this is a **consequence** or **manifestation** of the underlying monoclonal gammopathy, not the defining characteristic itself. *Hypergammaglobulinemia* - This term refers to an **elevated total level of immunoglobulins** in the blood, which can be **polyclonal** (diverse antibodies) or **monoclonal** in nature. - In multiple myeloma, the elevation is specifically due to a **monoclonal immunoglobulin**, making "monoclonal gammopathy" a more precise and defining term [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. 606-609. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 616-617.

Question 6: Among the following AML subtypes, non-specific esterase (NSE) staining is typically NEGATIVE in which one?

A. Acute Erythroleukemia (M6)
B. Acute Promyelocytic Leukemia (M3) (Correct Answer)
C. Acute Myelomonocytic Leukemia (M4)
D. Acute Monocytic Leukemia (M5)

Explanation: ***Acute Promyelocytic Leukemia (M3)*** - Non-specific esterase (NSE) is **negative** in Acute Promyelocytic Leukemia (M3) because NSE primarily stains cells of the **monocytic lineage**. - M3 is characterized by abnormal **promyelocytes** with heavy granulation, which are granulocytic precursors without monocytic differentiation. - M3 shows strong positivity for **myeloperoxidase (MPO)** instead, which is the characteristic marker for granulocytic lineage. *Acute Myelomonocytic Leukemia (M4)* - NSE staining is **positive** in M4 because this subtype has both myeloid and **monocytic components**. - The monocytic component (≥20% of non-erythroid cells) stains positively with NSE, which helps differentiate it from pure myeloid leukemias. - NSE positivity (inhibited by sodium fluoride) is a key diagnostic feature alongside myeloperoxidase positivity. *Acute Erythroleukemia (M6)* - NSE is typically **negative** in the predominant erythroid component of M6. - The diagnosis of M6 relies on the presence of ≥50% erythroid precursors (which are PAS positive) and ≥20% myeloblasts among non-erythroid cells. - NSE is not a characteristic marker for erythroleukemia. *Acute Monocytic Leukemia (M5)* - NSE staining is characteristically **strongly positive** in M5, which primarily consists of **monoblasts and promonocytes**. - This strong NSE positivity (inhibited by sodium fluoride) is a defining diagnostic feature demonstrating pure monocytic differentiation. - M5 typically shows weak or negative myeloperoxidase, helping distinguish it from other AML subtypes.

Question 7: The most common cause of malignant adrenal mass is:

A. Adrenocoical carcinoma
B. Malignant phaeochromocytoma
C. Lymphoma
D. Metastasis from another solid tissue tumor (Correct Answer)

Explanation: ***Metastasis from another solid tissue tumor*** - The **most common cause of malignant adrenal masses** is metastasis, often originating from tumors in the **lung, breast, or kidney**. - These metastatic lesions typically present as **bilateral adrenal masses**, which differ from primary adrenal tumors. *Adrenocortical carcinoma* - Although it is a malignant tumor of the adrenal cortex, it is ***less common*** than metastases. - Adrenocortical carcinoma usually presents as a **unilateral adrenal mass** and is often associated with **endocrine symptoms** due to hormone overproduction. *Lymphoma* - Lymphoma can involve the adrenal glands, but it is not as frequent as **metastatic disease**. - It typically results in **enlargement of the adrenal glands** rather than a distinct adrenal mass, which helps differentiate it from other malignancies. *Malignant phaeochromocytoma* - This is a rare neuroendocrine tumor arising from the adrenal medulla and is not common as a cause of adrenal masses. - Phaeochromocytomas are often associated with **hypertension** due to catecholamine secretion, which does not typically apply to most malignant adrenal masses.

Question 8: Which of the following is true regarding carcinoid tumor?

A. Associated with serotonin production
B. Potentially malignant tumor
C. Neuroendocrine tumor (Correct Answer)
D. Most common site is lung

Explanation: ### Most common site is lung - Carcinoid tumors are more commonly found in the **gastrointestinal tract**, specifically the appendix and ileum, rather than the lungs [1]. - This statement is false as they can occur in the lungs but are not the most common site overall. ### Potentially malignant tumor - Carcinoid tumors can be classified as **malignant,** especially if they show aggressive behavior or metastasis. - Many carcinoid tumors, particularly those in the gastrointestinal tract, can be **non-functional** and less aggressive [1]. ### Neuroendocrine tumor - Carcinoid tumors are indeed a type of **neuroendocrine tumor**, arising from **neuroendocrine cells**. - This classification emphasizes their origin and potential for secretion of hormones like **serotonin**. ### Associated with serotonin production - Many carcinoid tumors produce **serotonin**, leading to symptoms like **carcinoid syndrome** when they metastasize, particularly to the liver [1]. - This statement is true, indicating their involvement in neuroendocrine secretions.

Question 9: 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 10: A 40-year-old patient presents with complains of low back pain and development of pallor. During workup very high levels of urinary beta2 microglobulin was noted. X-ray skull was performed. Bone marrow aspiration shows >10% plasma cells and serum electrophoresis shows M protein >30 g/L. All are useful for management of this patient except?

A. Lenalidomide
B. Bortezomib
C. Melphalan (Correct Answer)
D. Autologous transplantation

Explanation: ***Melphalan*** - While melphalan is used in **multiple myeloma**, its high-dose application is primarily a conditioning regimen for **autologous stem cell transplantation**, rather than a standalone maintenance or initial therapy, especially in younger patients who are transplant-eligible. - Furthermore, alkylating agents like melphalan are associated with an increased risk of **secondary malignancies**, making their long-term use less favorable in transplant-eligible patients when newer, less toxic agents are available. *Lenalidomide* - **Lenalidomide** is an immunomodulatory drug (IMiD) that is highly effective in the treatment of multiple myeloma, often used in maintenance therapy post-transplant or as part of initial induction regimens. - It works by modulating the immune system and has direct anti-myeloma activity, making it a valuable tool in the management of this patient [1]. *Bortezomib* - **Bortezomib** is a proteasome inhibitor that is a cornerstone of initial induction therapy for transplant-eligible and transplant-ineligible multiple myeloma patients [1]. - It effectively inhibits the break down of cellular proteins, leading to apoptosis of myeloma cells, and is crucial for achieving deep responses. *Autologous transplantation* - Given the patient's age (40 years old) and likely fitness, **autologous stem cell transplantation** is a standard and highly effective treatment option for multiple myeloma, aimed at achieving deep and durable remissions [1]. - It involves harvesting the patient's own stem cells, then administering high-dose chemotherapy (often melphalan) to eradicate myeloma cells, followed by reinfusion of the stored stem cells to rescue hematopoiesis [1].

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