Lymphomas — MCQs
What is the primary basis for the Working Formulation in the classification of non-Hodgkin's lymphoma?
Which marker is commonly associated with positivity in follicular 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?
Hemolysis is seen in all except:
Which of the following is NOT typically seen in hereditary spherocytosis?
Howell-Jolly bodies are typically seen in which of the following conditions?
Which of the following is NOT a finding in later stages of sickle cell anemia?
In sickle cell anaemia, what is the typical percentage of normal adult haemoglobin (HbA)?
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 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 3: 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 4: 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 5: 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 6: 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 7: Which of the following is NOT typically seen in hereditary spherocytosis?
- A. Positive direct Coombs test (Correct Answer)
- B. Increased osmotic fragility
- C. Leg ulcers
- D. Splenomegaly
Explanation: **Explanation:** **Hereditary Spherocytosis (HS)** is an autosomal dominant inherited hemolytic anemia caused by defects in red blood cell (RBC) membrane proteins (most commonly **Ankyrin**, followed by Spectrin) [2]. These defects lead to a loss of membrane surface area, forcing the RBCs to assume a spherical shape [1]. **Why Option A is the correct answer:** The **Direct Coombs Test (DAT)** is used to detect antibodies or complement on the surface of RBCs. It is the hallmark of **Autoimmune Hemolytic Anemia (AIHA)**. Since HS is a genetic structural defect and not an immune-mediated process, the Coombs test is characteristically **negative**. A positive Coombs test in a patient with spherocytes strongly suggests AIHA rather than HS. **Analysis of Incorrect Options:** * **B. Increased Osmotic Fragility:** Spherocytes have a decreased surface-area-to-volume ratio, making them unable to expand in hypotonic solutions [1]. They lyse more easily than normal biconcave cells, which is the basis for the Osmotic Fragility Test (the classic screening test for HS). * **C. Leg Ulcers:** Chronic hemolysis can lead to decreased nitric oxide bioavailability and microvascular changes. While more common in Sickle Cell Anemia, leg ulcers are a recognized, albeit rare, complication of HS. * **D. Splenomegaly:** The spleen is the primary site of hemolysis in HS [3]. Spherocytes are trapped and destroyed in the splenic cords by macrophages. This chronic sequestration leads to compensatory hypertrophy of the spleen (splenomegaly). **NEET-PG High-Yield Pearls:** * **Gold Standard Test:** Eosin-5-maleimide (EMA) binding test via flow cytometry. * **MCHC:** Characteristically **increased** (>36 g/dL) due to mild cellular dehydration [1]. * **Peripheral Smear:** Spherocytes (small, dark cells lacking central pallor) and polychromasia (reticulocytosis). * **Treatment of Choice:** Splenectomy (usually deferred until after age 6 to reduce sepsis risk) [2]. * **Common Complication:** Pigment gallstones (calcium bilirubinate) due to chronic hyperbilirubinemia.
Question 8: Howell-Jolly bodies are typically seen in which of the following conditions?
- A. Liver disease
- B. Postsplenectomy (Correct Answer)
- C. Hemolysis
- D. Disseminated intravascular coagulation (DIC)
Explanation: **Explanation:** **Howell-Jolly bodies** are small, round, basophilic (purple-blue) nuclear remnants (clusters of DNA) found within erythrocytes. **Why Postsplenectomy is the correct answer:** Under normal physiological conditions, the spleen acts as a biological filter. As red blood cells (RBCs) pass through the splenic sinusoids, the "pitting" function of splenic macrophages identifies and removes these nuclear remnants without destroying the cell. When the spleen is absent (surgical splenectomy) or non-functional (functional asplenia, as seen in Sickle Cell Anemia), this filtration mechanism is lost. Consequently, these inclusions persist in the peripheral circulation and are visible on a peripheral blood smear. **Analysis of Incorrect Options:** * **Liver disease:** Typically associated with **Target cells** (due to increased membrane cholesterol) or **Acanthocytes** (Spur cells), but not specifically Howell-Jolly bodies. * **Hemolysis:** Often presents with **Schistocytes** (microangiopathic) or **Spherocytes** (hereditary/immune), depending on the etiology. * **DIC:** Characterized by microangiopathic hemolytic anemia (MAHA) where **Schistocytes** (fragmented RBCs) are the hallmark finding due to fibrin strand shearing. **NEET-PG High-Yield Pearls:** * **Pappenheimer bodies:** Iron-containing granules (siderocytes) seen in Sideroblastic anemia. * **Heinz bodies:** Denatured hemoglobin seen in G6PD deficiency (visualized with Supravital stains like Crystal Violet). * **Basophilic stippling:** Ribosomal precipitates seen in Lead poisoning and Thalassemia. * **Cabot rings:** Figure-of-eight inclusions (microtubule remnants) seen in Megaloblastic anemia. * **Other Postsplenectomy findings:** Target cells, Heinz bodies, and transient thrombocytosis/leukocytosis.
Question 9: Which of the following is NOT a finding in later stages of sickle cell anemia?
- A. Fish vertebrae
- B. Enlarged heart
- C. Splenomegaly (Correct Answer)
- D. Leukocytosis
Explanation: **Explanation:** The correct answer is **Splenomegaly**. **1. Why Splenomegaly is NOT found in later stages:** In the early stages of Sickle Cell Anemia (SCA), children often present with splenomegaly due to the sequestration of sickled red blood cells [1]. However, repeated episodes of vaso-occlusion and splenic infarction lead to progressive fibrosis and shrinkage of the organ. By adulthood (usually by age 5–8), the spleen becomes a small, shrunken, fibrotic remnant. This process is known as **Autosplenectomy** [1]. Therefore, a palpable spleen in an adult with SCA is highly unusual and should raise suspicion of other conditions like HbSC disease or Thalassemia. **2. Analysis of Incorrect Options:** * **Fish vertebrae:** Chronic marrow hyperplasia causes thinning of the trabeculae in the vertebrae. The pressure from the nucleus pulposus leads to biconcave indentations of the vertebral endplates, resembling the vertebrae of a fish (also called "H-shaped vertebrae"). * **Enlarged heart:** Chronic anemia leads to a hyperdynamic circulation [1]. Over time, this results in compensatory cardiomegaly and potentially high-output heart failure [1]. * **Leukocytosis:** Patients with SCA often have a baseline elevated white blood cell count (even in the absence of infection) due to the demargination of neutrophils caused by chronic inflammation and stress. **Clinical Pearls for NEET-PG:** * **Howell-Jolly Bodies:** Their presence on a peripheral smear is a hallmark of functional asplenia/autosplenectomy. * **Infection Risk:** Autosplenectomy increases susceptibility to encapsulated organisms (*S. pneumoniae, H. influenzae, N. meningitidis*). * **Radiology:** "Crew-cut appearance" on skull X-ray is another high-yield finding due to extramedullary hematopoiesis.
Question 10: In sickle cell anaemia, what is the typical percentage of normal adult haemoglobin (HbA)?
- A. 75 to 100% (Correct Answer)
- B. 10 to 20%
- C. 20 to 30%
- D. 50 to 60%
Explanation: In **Sickle Cell Anaemia (HbSS)**, the patient is homozygous for the sickle gene ($\beta^S\beta^S$). [1] This means there is a total absence of normal $\beta$-globin chain synthesis. Consequently, patients with HbSS produce **0% normal adult haemoglobin (HbA)**. ### **Detailed Explanation** 1. **Why 0% (Option A) is correct:** In HbSS, the genetic mutation replaces glutamic acid with valine at the 6th position of the $\beta$-globin chain. [3] Since both alleles are mutated, the body cannot produce any normal $\beta$ chains. [1] Therefore, the haemoglobin electrophoresis typically shows: * **HbS:** 80–95% * **HbF:** 5–15% (variable) [2] * **HbA2:** 2–4% * **HbA: 0%** 2. **Why other options are incorrect:** * **Options B, C, and D** represent levels of HbA that are never seen in homozygous sickle cell anaemia. If a patient has **40–50% HbA**, they likely have **Sickle Cell Trait (HbAS)**, which is the asymptomatic carrier state. If a patient has low but detectable HbA (e.g., 10–30%), they likely have **S-$\beta^+$ Thalassemia**. [3] ### **NEET-PG High-Yield Pearls** * **Sickle Cell Trait (HbAS):** HbA is always greater than HbS (approx. 60% HbA, 40% HbS). These patients are usually asymptomatic and protected against *P. falciparum* malaria. * **Diagnosis:** The gold standard for diagnosis is **Haemoglobin Electrophoresis** or **HPLC**. [1] * **Peripheral Smear:** Look for sickle cells and **Howell-Jolly bodies** (indicating functional asplenia). [4] * **Indication for Hydroxyurea:** It increases **HbF** levels, which inhibits the polymerization of HbS and reduces vaso-occlusive crises. [2]
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