Lymphomas and Lymphoid Neoplasms — MCQs
A cervical lymph node biopsy shows 'starry sky' pattern. Which immunophenotype would best support Burkitt lymphoma?
Tumor cells in chronic lymphocytic leukemia or small lymphoblastic lymphoma (CLL/SLL) arise from which of the following?
Which protein is a key marker of Reed-Sternberg cells in Hodgkin's lymphoma?
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?
The subtype of Hodgkin's disease which is histogenetically distinct from all the other subtypes is:
Which of the following is positive in Follicular lymphoma?
ALL L3 morphology is a malignancy arising from which cell lineage –
In the context of extranodal Hodgkin's lymphoma, which of the following sites is most commonly involved?
Starry sky appearance is seen in which of the following?
Which of the following statements is/are incorrect about Hodgkin's lymphoma?
Lymphomas and Lymphoid Neoplasms Indian Medical PG Practice Questions and MCQs
Question 1: A cervical lymph node biopsy shows 'starry sky' pattern. Which immunophenotype would best support Burkitt lymphoma?
- A. CD30+/ALK+
- B. CD15+/CD30+
- C. CD5+/cyclin D1+
- D. CD10+/c-myc+ (Correct Answer)
Explanation: ***CD10+/c-myc+*** - **Burkitt lymphoma** is characterized by the **t(8;14) translocation**, leading to **c-myc overexpression**, which is a key diagnostic marker. - **CD10** positivity is typical for **Burkitt lymphoma**, as it is a marker of germinal center B-cells from which this lymphoma originates [1]. *CD30+/ALK+* - This immunophenotype is characteristic of **anaplastic large cell lymphoma (ALCL)**, which presents with different histological features and clinical behavior. - ALK-positive ALCL is often seen in younger patients and has a distinct genetic alteration involving the **ALK gene**. *CD15+/CD30+* - This immunophenotype is classic for **classical Hodgkin lymphoma**, particularly the **nodular sclerosis** and **mixed cellularity** subtypes. - Hodgkin lymphoma involves large, atypical **Reed-Sternberg cells** surrounded by inflammatory cells, which is distinct from the "starry sky" pattern [1]. *CD5+/cyclin D1+* - This immunophenotype is highly suggestive of **mantle cell lymphoma**, which is associated with the **t(11;14) translocation** leading to **cyclin D1 overexpression**. - Mantle cell lymphoma typically has a diffuse or nodular growth pattern and does not exhibit the "starry sky" morphology. **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. 606.
Question 2: Tumor cells in chronic lymphocytic leukemia or small lymphoblastic lymphoma (CLL/SLL) arise from which of the following?
- A. Naive B cell
- B. Mature B cell (Correct Answer)
- C. Centrocytes of germinal center
- D. Progenitor B-cell
Explanation: ***Mature B cell*** - CLL/SLL is characterized by the accumulation of **monoclonal B lymphocytes** that are morphologically mature but functionally incompetent. [1] - These malignant cells exhibit a mature immunophenotype, expressing **CD5, CD19, CD20 (dim), and CD23**, indicating their origin from a post-germinal center or memory B cell lineage. [1] *Naive B cell* - Naive B cells are typically found circulating in the blood and lymph nodes; while they are B cells, the specific immunophenotype and genomic features of CLL/SLL cells point to a more differentiated origin. - Although CLL/SLL cells express some markers of naive B cells, their overall profile, particularly the expression of **CD23**, is more consistent with a mature or activated B cell stage. [1] *Centrocytes of germinal center* - Malignancies arising from centrocytes of germinal centers often include **follicular lymphoma**, which presents with distinct clinical and immunophenotypic features from CLL/SLL. - Centrocytes are typically involved in somatic hypermutation and class switching, processes relevant to later stage B cell development, but not the direct origin of CLL/SLL. *Progenitor B-cell* - Progenitor B-cells are early developmental stages of B cells, and their malignant transformation leads to conditions like **B-cell acute lymphoblastic leukemia (B-ALL)**. [1] - B-ALL presents with immature blast cells and a different immunophenotype (e.g., lack of CD23), distinct from the mature lymphoid cells seen in CLL/SLL. [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. 596-598.
Question 3: Which protein is a key marker of Reed-Sternberg cells in Hodgkin's lymphoma?
- A. CD19
- B. CD20
- C. CD15 (Correct Answer)
- D. CD45
Explanation: ***CD15*** - **CD15** (along with **CD30**) is a characteristic **immunophenotypic marker** of **Reed-Sternberg cells** in **classical Hodgkin's lymphoma** [1]. - The classic immunophenotype is **CD30+/CD15+/CD45-/CD20-** (CD20 may be weakly positive in some cases). - **CD30 is the most sensitive marker** (positive in >95% of cases), while **CD15 is positive in 75-85%** of classical Hodgkin's lymphoma cases. - Both markers together aid in the definitive diagnosis and differentiation of Hodgkin's lymphoma from other lymphomas. *CD19* - **CD19** is an important **B-cell marker** expressed on normal B lymphocytes and in most B-cell non-Hodgkin lymphomas, but it is typically **negative** in Reed-Sternberg cells [1]. - Its presence usually points away from Hodgkin's lymphoma and towards a B-cell lineage malignancy. *CD20* - **CD20** is another key **B-cell marker** found on the surface of normal B lymphocytes and **most B-cell non-Hodgkin lymphomas**. - Reed-Sternberg cells usually **lack CD20 expression** (or show only weak positivity in 20-40% of cases), helping to distinguish Hodgkin's lymphoma from B-cell non-Hodgkin lymphomas [1]. *CD45* - **CD45** (also known as leukocyte common antigen, LCA) is expressed on nearly all **hematopoietic cells**, including most lymphomas. - However, **Reed-Sternberg cells are typically negative or only weakly positive for CD45**, which is a significant diagnostic feature that helps differentiate Hodgkin's lymphoma from many non-Hodgkin lymphomas [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. 614-618.
Question 4: 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 5: The subtype of Hodgkin's disease which is histogenetically distinct from all the other subtypes is:
- A. Lymphocyte predominant (Correct Answer)
- B. Nodular sclerosis
- C. Mixed cellularity
- D. Lymphocyte depleted
Explanation: ***Lymphocyte predominant*** - This subtype is characterized by the presence of **popcorn cells** (a variant of Reed-Sternberg cells) and is histogenically distinct from other forms of Hodgkin's lymphoma [1]. - It typically features a **predominance of lymphocytes**, contrasting with the other subtypes that may show mixed cellularity or different cell backgrounds [1]. *Lymphocyte depleted* - This subtype has a high number of **Reed-Sternberg cells** and is often associated with **immunocompromised states**, making it less distinct than lymphocyte predominant [2]. - It typically presents with a poor prognosis and is characterized by **depletion** of lymphocytes rather than predominance [2]. *Nodular sclerosis* - This is one of the most common subtypes of Hodgkin's lymphoma, featuring **fibrosis** and often affecting mediastinal lymph nodes. - While distinct, it shares histopathological features with other standard forms of Hodgkin's lymphoma, thus not being **histogenically distinct**. *Mixed cellularity* - This subtype presents a heterogeneous mix of cells, including **Reed-Sternberg cells** and leukocytes, and is the most frequent subtype after nodular sclerosis [2]. - It is associated with lower lymphocyte predominance, thus lacking the unique histological characteristics found in lymphocyte predominant Hodgkin's lymphoma. **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. 618. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Diseases Of The Urinary And Male Genital Tracts, pp. 559-560.
Question 6: Which of the following is positive in Follicular lymphoma?
- A. Cyclin D1 (Bcl-1)
- B. Bcl-2 protein (Correct Answer)
- C. Bcl-10 signaling protein
- D. Bcl-6 transcription factor
Explanation: ***Bcl-2 protein*** - The characteristic **t(14;18) chromosomal translocation** in follicular lymphoma leads to the **overexpression of the Bcl-2 protein**, which promotes cell survival by inhibiting apoptosis [1], [4]. - This constitutive activation makes the tumor cells resistant to programmed cell death, contributing to their accumulation [2]. - **Bcl-2 positivity is highly specific for follicular lymphoma** among lymphomas, making it the most diagnostically useful marker [3]. *Cyclin D1 (Bcl-1)* - **Cyclin D1** overexpression is characteristic of **Mantle Cell Lymphoma**, typically due to a **t(11;14) translocation**. - It plays a role in cell cycle progression rather than directly inhibiting apoptosis in the same manner as Bcl-2. *Bcl-10 signaling protein* - **Bcl-10** is involved in **NF-ΙB activation**, particularly in certain types of **MALT lymphoma** and other lymphoid neoplasms. - It is not a primary diagnostic marker for follicular lymphoma. *Bcl-6 transcription factor* - **Bcl-6** is a germinal center marker and is **positive in most follicular lymphomas** since they are germinal center-derived B-cell neoplasms. - However, Bcl-6 is also expressed in other germinal center-derived lymphomas like **DLBCL of germinal center subtype**, making it **less specific** than Bcl-2 [3], [5]. - While both Bcl-2 and Bcl-6 can be positive in follicular lymphoma, **Bcl-2 overexpression due to t(14;18) is the defining molecular hallmark** and most diagnostically specific feature [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. 602-604. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Neoplasia, pp. 310-311. [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. 604. [4] 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. [5] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Diseases Of The Urinary And Male Genital Tracts, pp. 563-564.
Question 7: ALL L3 morphology is a malignancy arising from which cell lineage –
- A. Mature B cell (Correct Answer)
- B. Mixed B cell & T cell
- C. Immature T cell
- D. Precursor B cell
Explanation: ***Mature B cell*** - ALL L3, also known as **Burkitt-type ALL**, arises from a **mature B-cell lineage**. [1] - This is characterized by specific immunophenotypic markers (e.g., strong expression of **surface immunoglobulin** and **CD20**) and frequent chromosomal translocations involving the **MYC oncogene**, typically t(8;14). [1] *Mixed B cell & T cell* - This refers to **mixed phenotype acute leukemia (MPAL)**, which exhibits features of both B-cell and T-cell lineages. - MPAL is distinct from ALL L3, which is definitively B-cell in origin based on morphology and immunophenotype. *Immature T cell* - Malignancies arising from immature T cells are classified as **T-cell ALL**, which have a different morphology (L1 or L2) and distinct immunophenotype (e.g., expression of **cytoplasmic CD3**, CD1a, CD2, CD5, CD7). [1] - T-cell ALL is characterized by **T-cell receptor gene rearrangements** and typically presents with a mediastinal mass, unlike ALL L3. [1] *Precursor B cell* - Most cases of ALL (ALL L1 and L2) arise from **precursor B cells**, also known as B-lymphoblastic leukemia. [2] - These are characterized by lack of surface immunoglobulin expression and generally express **CD10, CD19, CD22**, and **TdT**, contrasting with the mature B-cell immunophenotype of ALL L3. [2] **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. 606. [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. 596-598.
Question 8: In the context of extranodal Hodgkin's lymphoma, which of the following sites is most commonly involved?
- A. Thyroid
- B. Small bowel
- C. Thymus
- D. Bone (Correct Answer)
Explanation: ***Bone*** - Among the options listed, **bone is the most frequently involved extranodal site** in Hodgkin's lymphoma, particularly in patients with advanced stage disease. - **Note:** The most common extranodal sites overall in Hodgkin's lymphoma are the **spleen**, **liver**, and **bone marrow** [1]. However, among the specific sites listed in this question, bone involvement is most frequent. - Osseous involvement often manifests as **osteolytic or osteoblastic lesions** visible on imaging and may cause bone pain or pathological fractures. *Thymus* - While the **mediastinum** (including thymus) is a common **nodal site** for Hodgkin's lymphoma (especially nodular sclerosis subtype), extensive thymic involvement as a true **extranodal site** is less common than bone [1]. - Primary thymic lymphomas are more characteristic of **T-cell lymphoblastic lymphoma** rather than Hodgkin's lymphoma. *Thyroid* - **Thyroid involvement** in Hodgkin's lymphoma is rare, typically occurring as secondary extension from adjacent cervical lymph nodes. - Primary thyroid lymphoma is more commonly **non-Hodgkin lymphoma** (particularly MALT lymphoma or DLBCL) associated with Hashimoto's thyroiditis. *Small bowel* - **Gastrointestinal involvement**, including small bowel, is a rare manifestation of Hodgkin's lymphoma. - GI tract lymphomas are predominantly **non-Hodgkin lymphomas** (especially MALT lymphoma, DLBCL, and enteropathy-associated T-cell lymphoma). **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Diseases Of The Urinary And Male Genital Tracts, pp. 557-559.
Question 9: Starry sky appearance is seen in which of the following?
- A. Burkitt's Lymphoma (Correct Answer)
- B. Diffuse Large B Cell Lymphoma
- C. Anaplastic Large Cell Lymphoma (ALCL)
- D. Follicular Lymphoma
Explanation: ***Burkitts lymphoma*** - The **starry sky appearance** is a characteristic histopathological finding due to interspersed macrophages containing **phagocytosed apoptotic cells** and necrotic debris in Burkitt's lymphoma [1]. - It is associated with **MYC gene translocation** and presents typically in children or young adults, commonly affecting the **jaw or abdomen**. *CIL* - CIL (chronic inflammatory leukocytosis) does not exhibit a **starry sky appearance**; it typically reflects a reactive process without specific histological features. - The histology is more characterized by **increased white blood cell counts** rather than tissue architecture alterations seen in lymphomas. *Diffuse large B cell lymphoma* - While this lymphoma can show **varied morphology**, it does not have a **starry sky appearance** as a defining feature, rather presenting with **large atypical B-cells**. - The histological appearance is generally of a **diffuse infiltrate**, which lacks the classic starry sky histology. *ALCL* - Anaplastic large cell lymphoma (ALCL) is characterized by **large, pleomorphic cells** but does not show a starry sky appearance. - The histological pattern primarily focuses on **large anaplastic lymphoid cells** rather than the scattered macrophages seen in Burkitt's lymphoma. **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. 606.
Question 10: Which of the following statements is/are incorrect about Hodgkin's lymphoma?
- A. Nodular sclerosis is most common and involves mediastinum
- B. RS cells are B cell derived
- C. CD 15, 30 positivity in RS cell
- D. Waldeyer's ring is involved (Correct Answer)
Explanation: ***Waldeyer's ring is involved*** - **Hodgkin's lymphoma** rarely involves **extranodal sites** like the Waldeyer's ring, which is more commonly affected by **Non-Hodgkin's lymphoma** [3]. - Its typical presentation involves **lymph nodes** in a contiguous manner, rather than lymphoid tissue in the head and neck [3],[4]. *Nodular sclerosis is most common and involves mediastinum* - This statement is **correct**. **Nodular sclerosis** is the **most common subtype** of Hodgkin's lymphoma, often presenting with **mediastinal lymphadenopathy** [1],[2]. - It affects young adults and is characterized by collagen banding and lacunar cells [2]. *RS cells are B cell derived* - This statement is **correct**. The characteristic **Reed-Sternberg (RS) cells** in Hodgkin's lymphoma are derived from **transformed germinal center B lymphocytes** [3]. - These cells are large, often multinucleated, and are essential for diagnosis [3]. *CD 15, 30 positivity in RS cell* - This statement is also **correct**. **Reed-Sternberg cells** typically express the surface markers **CD15 and CD30**, which are crucial for immunohistochemical diagnosis. - The absence of CD45 and other B-cell markers further helps in differentiating Hodgkin's lymphoma from Non-Hodgkin's lymphomas. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Diseases Of The Urinary And Male Genital Tracts, pp. 558-559. [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. 616-618. [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. 614-616. [4] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Diseases Of The Urinary And Male Genital Tracts, pp. 557-558.
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