Lymphomas — MCQs
What is the primary basis for the Working Formulation in the classification of non-Hodgkin's lymphoma?
Which type of Hodgkin's lymphoma is characterized by the presence of Reed-Sternberg cells with a lacunar appearance?
Which marker is commonly associated with positivity in follicular lymphoma?
Which of the following is not included in the International Prognostic Index (IPI) for lymphoma?
Most common hematological malignancy associated with Rheumatoid Arthritis (RA)?
Development of Lymphoma in Sjogren's syndrome is suggested by all of the following except
CHOP is used in the treatment of?
Which one of the following is not associated with a high reticulocyte count?
Hemolysis is seen in all except:
What is true about sickle cell anemia?
Lymphomas Indian Medical PG Practice Questions and MCQs
Question 1: What is the primary basis for the Working Formulation in the classification of non-Hodgkin's lymphoma?
- A. Morphology of cells (Correct Answer)
- B. Cell surface markers
- C. Survival characteristic of cells
- D. Cellular genetics
Explanation: ***Morphology of cells*** - The **Working Formulation** primarily classified non-Hodgkin's lymphomas based on the **histological appearance** of the malignant cells, such as cell size, nuclear features, and growth patterns. - This classification aimed to group lymphomas with similar prognoses, broadly categorizing them into low, intermediate, and high-grade based on their **cytological features**. *Cell surface markers* - While cell surface markers (immunophenotyping) are crucial in modern lymphoma classification (e.g., WHO classification), they were not the **primary basis** for the Working Formulation. - Immunophenotyping identifies the lineage and differentiation stage of lymphoid cells (e.g., B-cell, T-cell) but became widely integrated into lymphoma classification later. *Survival characteristic of cells* - The Working Formulation did indirectly consider survival by grouping lymphomas with similar prognoses, but **survival characteristics** themselves were not the primary *basis* for classifying each specific lymphoma type. - Prognosis was an outcome derived from the morphological classification, not the initial classifying factor. *Cellular genetics* - **Cellular genetics**, including chromosomal translocations and gene mutations, are fundamental to current World Health Organization (WHO) classifications of lymphoma. - However, comprehensive genetic analysis was not readily available or the primary method for classifying lymphomas when the Working Formulation was developed.
Question 2: Which type of Hodgkin's lymphoma is characterized by the presence of Reed-Sternberg cells with a lacunar appearance?
- A. Nodular sclerosis (Correct Answer)
- B. Lymphocyte predominance
- C. Lymphocyte depletion
- D. Mixed cellularity
Explanation: ***Nodular sclerosis*** - Lacunar-type Reed-Sternberg cells are **characteristic** of nodular sclerosis Hodgkin's lymphoma [1], often associated with **mediastinal lymphadenopathy**. - This subtype shows a **fibrous septation** pattern with lacunar cells [1] frequently seen in histopathological examination. *Lymphocyte depletion* - Characterized by a **poor prognosis** with a predominance of **non-neoplastic lymphocytes**, lacking typical Reed-Sternberg cells. - Reed-Sternberg cells, when present, do not typically display the **lacunar type** morphology. *Lymphocyte predominance* - This subtype has a **different cellular composition** with lymphocytes being the dominant component and fewer Reed-Sternberg cells. - Reed-Sternberg cells in this type are not of the **lacunar morphology** seen in nodular sclerosis. *Mixed cellularity* - Contains a variety of cells including **eosinophils** and histiocytes but predominantly classical Reed-Sternberg cells. - It typically does not feature **lacunar-type** cells, which are specific to nodular sclerosis. **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. 616.
Question 3: Which marker is commonly associated with positivity in follicular lymphoma?
- A. Bcl-1
- B. Bcl-6
- C. Bcl-2 (Correct Answer)
- D. None of the options
Explanation: ***Bcl-2*** - **Follicular lymphoma** is characterized by the overexpression of the **Bcl-2 protein**, which inhibits apoptosis, leading to the survival of malignant B cells [1][3]. - The **Bcl-2 gene** is often involved in the **t(14;18)** chromosomal translocation, which is a hallmark of this lymphoma [1][3][4]. *Bcl-6* - Although **Bcl-6** can be expressed in some lymphomas, it is primarily associated with **diffuse large B-cell lymphoma**, not follicular lymphoma. - **Bcl-6** is involved in **germinal center formation** and its positivity does not indicate follicular lymphoma specifically. *Bcl-1* - **Bcl-1** (also known as **CCND1**) is primarily associated with **mantle cell lymphoma** and is not a characteristic marker for follicular lymphoma. - It is linked to the **t(11;14)** translocation, which is distinct from the genetic alterations seen in follicular lymphoma. *None of the above* - This option is incorrect as **Bcl-2 positivity** is definitive for follicular lymphoma [2]. - The presence of other markers like **Bcl-6** or **Bcl-1** does not negate the expression of Bcl-2 in this lymphoma type. **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. 602-604. [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. 604. [3] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Diseases Of The Urinary And Male Genital Tracts, pp. 561-562. [4] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Neoplasia, pp. 310-311.
Question 4: Which of the following is not included in the International Prognostic Index (IPI) for lymphoma?
- A. Stage of disease
- B. LDH
- C. Hemoglobin (Correct Answer)
- D. Number of extralymphatic sites involved
Explanation: ***Hemoglobin*** - **Hemoglobin** level is not one of the five factors included in the original **International Prognostic Index (IPI)** for lymphoma. - While anemia can be a feature of lymphoma, it is not explicitly used as a prognosticator within the IPI. *LDH* - **Lactate dehydrogenase (LDH)** is a critical component of the IPI, with elevated levels indicating a worse prognosis due to higher tumor burden and cellular turnover [1]. - Higher LDH reflects increased metabolic activity of rapidly proliferating lymphoma cells [1]. *Stage of disease* - The **Ann Arbor clinical stage** (III or IV versus I or II) is a key factor in the IPI, as advanced stage signifies more widespread disease [1]. - Higher disease burden and dissemination generally correlate with a poorer prognosis. *Number of extralymphatic sites involved* - The presence of **more than one extralymphatic site** of involvement is an important prognostic factor in the IPI, indicating aggressive disease. - Involvement outside the lymphatic system suggests more systemic dissemination and a less favorable outcome.
Question 5: Most common hematological malignancy associated with Rheumatoid Arthritis (RA)?
- A. Diffuse large B cell lymphoma
- B. Chronic lymphocytic leukemia
- C. T-cell prolymphocytic leukemia
- D. Large granular lymphocytic leukemia (LGLL) (Correct Answer)
Explanation: ***Large granular lymphocytic leukemia (LGLL)*** - **LGLL** is the most common hematological malignancy strongly associated with **rheumatoid arthritis (RA)**, often presenting with features such as **neutropenia** and splenomegaly. - Approximately 80% of patients with LGLL have a **T-cell phenotype**, and a significant subset experiences **autoimmune diseases**, with RA being the most frequent. *Diffuse large B cell lymphoma* - While patients with **RA** have an increased risk of **lymphoma**, **diffuse large B-cell lymphoma (DLBCL)** is a more aggressive type but not the most common hematologic malignancy directly associated with the disease itself in terms of prevalence [3]. - Inflammatory conditions like **RA** can contribute to chronic immune stimulation, increasing the risk of certain lymphomas, but LGLL holds a more direct and prevalent association [1]. *Chronic lymphocytic leukemia* - **Chronic lymphocytic leukemia (CLL)** is a lymphoproliferative disorder of **B lymphocytes**, but it does not have a particularly strong or common association with **RA** compared to LGLL [2]. - The elevated risk of hematological malignancies in RA patients typically points more towards lymphoproliferative disorders driven by specific immune dysregulations characteristic of RA. *T-cell prolymphocytic leukemia* - **T-cell prolymphocytic leukemia (T-PLL)** is a rare and aggressive **T-cell leukemia** that generally presents with a high white blood cell count and splenomegaly, but it is not commonly linked with **RA**. - Its clinical presentation and biology are distinct from the more indolent leukemias like LGLL that are often seen in conjunction with autoimmune conditions.
Question 6: Development of Lymphoma in Sjogren's syndrome is suggested by all of the following except
- A. Leukopenia
- B. Cryoglobulinemia
- C. Persistent parotid gland enlargement
- D. High C4 complement levels (Correct Answer)
Explanation: High C4 complement levels - **High C4 complement levels** are not typically associated with an increased risk or development of lymphoma in Sjögren's syndrome. In fact, **low C4** (due to consumption) is more commonly linked to complications like vasculitis or cryoglobulinemia, which can sometimes precede lymphoma. - Decreased complement levels, particularly **C4**, are often seen in Sjögren's patients with **lymphoproliferative disorders** or **cryoglobulinemia**, making elevated levels an unlikely indicator of lymphoma. Leukopenia - **Leukopenia** (low white blood cell count) can be a significant indicator of developing **lymphoma** in patients with Sjögren's syndrome. It may reflect bone marrow infiltration or a systemic effect of the underlying disease. - Persistent or worsening **leukopenia** should prompt further investigation for lymphoproliferative disorders. Cryoglobulinemia - **Cryoglobulinemia** is a well-established risk factor and a paraneoplastic manifestation strongly associated with the development of **B-cell non-Hodgkin lymphoma** in Sjögren's syndrome [1]. - The presence of **cryoglobulins** indicates abnormal B-cell activity, which can evolve into overt lymphoma. Persistent parotid gland enlargement - **Persistent parotid gland enlargement** in a Sjögren's patient is a critical warning sign for potential **lymphoma**, especially **mucosa-associated lymphoid tissue (MALT) lymphoma** [1]. - This symptom warrants a **biopsy** to rule out malignancy, as it often signifies increased lymphoid proliferation within the salivary glands.
Question 7: CHOP is used in the treatment of?
- A. NHL (Correct Answer)
- B. Head and neck cancer
- C. Ca Stomach
- D. Ca Lung
Explanation: ***NHL*** - **CHOP** is the **gold standard first-line chemotherapy regimen** for most types of **Non-Hodgkin Lymphoma**, particularly **diffuse large B-cell lymphoma (DLBCL)** [1]. - The regimen combines **cyclophosphamide** (alkylating agent), **hydroxydaunorubicin/doxorubicin** (anthracycline), **oncovin/vincristine** (vinca alkaloid), and **prednisone** (corticosteroid) for optimal efficacy [1]. *Head and neck cancer* - Treatment primarily involves **platinum-based regimens** such as **cisplatin or carboplatin** combined with **5-fluorouracil** or **taxanes**. - **CHOP is not a standard chemotherapy regimen** for head and neck malignancies, which are solid tumors requiring different therapeutic approaches. *Ca Stomach* - Gastric cancer chemotherapy typically uses regimens like **FOLFOX** (fluorouracil, leucovorin, oxaliplatin) or **FLOT** (fluorouracil, leucovorin, oxaliplatin, docetaxel). - **CHOP is not used for gastric cancer** treatment, as it requires **platinum-based or fluoropyrimidine-based combinations**. *Ca Lung* - Lung cancer treatment involves **platinum-based doublets** such as **cisplatin/carboplatin** combined with **pemetrexed, paclitaxel, or gemcitabine** [2]. - **CHOP is not used for lung cancer** as it is specifically designed for **hematological malignancies**, not solid tumors like lung cancer.
Question 8: Which one of the following is not associated with a high reticulocyte count?
- A. Acute bleed
- B. Hemolytic anemia
- C. Megaloblastic anemia (Correct Answer)
- D. Response to treatment in nutritional deficiency anemia
Explanation: **Explanation:** The **reticulocyte count** is a measure of the bone marrow's ability to produce new red blood cells (erythropoiesis) in response to anemia. **Why Megaloblastic Anemia is the correct answer:** Megaloblastic anemia (caused by Vitamin B12 or Folate deficiency) is characterized by **ineffective erythropoiesis** [1]. Although the bone marrow is hypercellular, the RBC precursors undergo intramedullary destruction due to defective DNA synthesis. Consequently, the bone marrow cannot release mature cells or reticulocytes into the peripheral blood, leading to a **low reticulocyte count** (reticulocytopenia) [1]. **Analysis of incorrect options:** * **Acute Bleed:** Following a sudden loss of blood, the healthy bone marrow responds to erythropoietin stimulation by rapidly increasing RBC production, leading to reticulocytosis within 3–5 days. * **Hemolytic Anemia:** In hemolysis, RBCs are destroyed prematurely in the periphery. Since the bone marrow is functional, it compensates by increasing production, resulting in a high reticulocyte count. * **Response to treatment:** When a patient with nutritional deficiency (Iron, B12, or Folate) receives the missing nutrient, the "block" in erythropoiesis is removed [1]. This leads to a predictable "reticulocyte crunch" or peak, usually seen within 5–10 days of starting therapy. **NEET-PG High-Yield Pearls:** 1. **Corrected Reticulocyte Count (CRC):** In anemia, always use CRC [% Retic × (Patient Hct / Normal Hct)] to assess marrow response. A CRC >2% suggests hemolysis or blood loss; <2% suggests impaired production. 2. **Reticulocyte Production Index (RPI):** The most accurate measure, correcting for "shift reticulocytes." RPI >3 indicates an adequate marrow response. 3. **Megaloblastic Anemia Hallmark:** Look for hypersegmented neutrophils (>5 lobes) on peripheral smear and increased LDH due to ineffective erythropoiesis .
Question 9: Hemolysis is seen in all except:
- A. Hemophilia (Correct Answer)
- B. Thalassemia
- C. Sickle cell anemia
- D. Methotrexate therapy
Explanation: **Explanation:** The core concept of this question lies in distinguishing between **hemolytic disorders** (premature destruction of RBCs) and **coagulation disorders** (defects in the clotting cascade). **1. Why Hemophilia is the correct answer:** Hemophilia (A or B) is an X-linked recessive **coagulation disorder** caused by a deficiency of Factor VIII or IX, respectively [1]. It is characterized by a failure in the secondary hemostasis pathway, leading to deep tissue bleeding and hemarthrosis. It does **not** involve the destruction of red blood cells; therefore, hemolysis is not a feature of this disease. **2. Why the other options are incorrect:** * **Thalassemia:** This is a quantitative defect in globin chain synthesis [2]. The imbalance of chains leads to the formation of unstable tetramers that precipitate (Heinz bodies), causing intramedullary and peripheral hemolysis. * **Sickle Cell Anemia:** This is a qualitative defect (HbS). Under deoxygenated conditions, HbS polymerizes, causing RBCs to "sickle" [2]. These rigid cells are trapped and destroyed in the splenic sinusoids (extravascular hemolysis). * **Methotrexate Therapy:** While primarily known for causing megaloblastic anemia (folate antagonism), Methotrexate can induce **oxidative stress** and has been associated with drug-induced immune hemolytic anemia (DIIHA) in specific clinical contexts. **Clinical Pearls for NEET-PG:** * **Hallmark of Hemolysis:** Elevated indirect bilirubin, increased LDH, and decreased **Haptoglobin** (most sensitive marker for intravascular hemolysis). * **Hemophilia Triad:** Prolonged aPTT, normal PT, and normal bleeding time. * **High-Yield Fact:** Thalassemia and Sickle Cell Anemia are both causes of **extravascular hemolysis**, often presenting with splenomegaly (until autosplenectomy occurs in Sickle Cell).
Question 10: What is true about sickle cell anemia?
- A. Leukopenia
- B. Decreased ESR (Correct Answer)
- C. Microcardia
- D. Ringed sideroblasts
Explanation: **Explanation:** **Correct Option: B. Decreased ESR** The Erythrocyte Sedimentation Rate (ESR) depends on the formation of **rouleaux** (stacks of RBCs). Rouleaux formation requires normal, flexible, biconcave discs. In Sickle Cell Anemia (SCA), the RBCs are abnormally shaped (sickled) and rigid. These cells cannot stack effectively, which significantly hinders rouleaux formation. Consequently, the RBCs remain suspended longer, leading to a characteristically **low or near-zero ESR**. **Analysis of Incorrect Options:** * **A. Leukopenia:** SCA is typically associated with **leukocytosis** (elevated WBC count), not leukopenia. This occurs due to chronic inflammation, demargination of neutrophils during crises, and premature release from the bone marrow. * **C. Microcardia:** Patients with SCA often develop **cardiomegaly** (enlarged heart). This is a compensatory mechanism for chronic severe anemia (hyperdynamic circulation) and potential iron overload from frequent transfusions. * **D. Ringed sideroblasts:** These are the hallmark of **Sideroblastic Anemia**, characterized by iron accumulation in the mitochondria of erythroid precursors. They are not a feature of SCA, which is a hemoglobinopathy [1]. **High-Yield Clinical Pearls for NEET-PG:** * **ESR Exceptions:** ESR is also decreased in Polycythemia Vera, Spherocytosis, and Afibrinogenemia. * **Peripheral Smear:** Look for **Sickle cells** (drepanocytes) and **Howell-Jolly bodies** (indicating autosplenectomy [1]). * **Diagnosis:** **Hb Electrophoresis** is the gold standard (shows HbS; absence of HbA [1]). * **Management:** **Hydroxyurea** is used to increase HbF levels, reducing the frequency of painful crises [1].
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