28 Lymphomas

Nina Wagner-Johnston and Dilan Patel

QUESTIONS

Each of the numbered items below is followed by lettered answers. Select the ONE lettered answer that is BEST in each case unless instructed otherwise.

Question 28.1 An 8-year-old child is brought to clinic by his mother due to a jaw lump that has been increasing in size over the past month. The mother states that the lump is preventing her child from eating. Physical examination reveals a child in moderate distress with a left jaw mass that displaces teeth and is impinging on the trachea. Biopsy reveals sheets of atypical lymphocytes with areas of necrosis and hemorrhage. Ki-67 is 99%. Flow cytometry shows B-cell markers as well as CD10 and BCL-6. CD5, BCL-2, and TdT are absent. Which of the following is the most common translocation in this malignancy?

A. t(1;14)

B. t(2;8)

C. t(8;14)

D. t(8;22)

Question 28.2 Which of the following characterize/s a difference between endemic and sporadic Burkitt lymphoma in children?

A. Endemic disease occurs more commonly in Africa and is associated with EBV infection.

B. Common sites of endemic disease are the mediastinal and hilar lymph nodes, which often leads to rapid respiratory compromise.

C. Dysregulation of both c-myc and BCL-2 are required for tumor cell growth.

D. Sporadic Burkitt lymphoma occurs in nonmalaria endemic areas, with common areas of development including the abdomen.

Question 28.3 Which one of the following syndromes is associated with a higher risk of adult lymphoma as opposed to childhood lymphoma?

A. Ataxia-telangiectasia

B. HIV/AIDS

C. Wiskott–Aldrich syndrome

D. X-linked lymphoproliferative syndrome

Question 28.4 A 35-year-old Caucasian male presents to his primary doctor due to 2 months of gradually worsening fevers, chills, and night sweats that soak through multiple pillows. He recalls having lost some weight over the past months, involuntarily, but is most bothered by an itchy rash on his abdomen that has recently started to ulcerate. Physical examination reveals painless lymphadenopathy. Biopsy shows pleomorphic large cells with prominent nucleoli. Immunophenotyping shows cells positive for CD30 and ALK, and negative for B-cell markers. Which of the following is TRUE regarding this condition?

A. ALK-positive cases are more common in adults.

B. ALK protein is detected in a minority of cases.

C. Cells are often derived from mature, activated T cells.

D. Prognosis in ALK-positive cases is worse than ALK-negative cases.

Question 28.5 A 55-year-old African-American man presents to his PCP with abdominal pain that has been gradually increasing over 3 months. He notes that he has been urinating less than normal. Furthermore, he complains of persistent fevers and drenching night sweats. His wife notes that he looks thinner than before. The patient is less active than his baseline but is able to perform light housework without difficulty. Physical examination reveals nontender adenopathy in the bilateral cervical and left axillary regions. PET/CT shows FDG-avid bilateral cervical, left axillary, and mediastinal lymphadenopathy as well as a 7-cm retroperitoneal mass resulting in bilateral hydronephrosis. CBC is within normal limits. LDH is 210 IU/L (upper limit of normal 250 IU/L). Bone marrow biopsy demonstrates no evidence of disease. Which of the following best describes the patient’s International Prognostic Index (IPI) score?

A. 0

B. 1

C. 2

D. 3

Question 28.6 Human herpesvirus-8 is implicated in which of the following diseases?

A. Burkitt lymphoma

B. GCB DLBCL

C. Monocytoid B-cell lymphoma

D. Primary effusion lymphoma

Question 28.7 Which of the following is/are mature T-cell neoplasms?

A. Anaplastic large-cell lymphoma

B. Hairy cell leukemia

C. Mycosis fungoides

D. Primary effusion lymphoma

Question 28.8 Which of the following B-cell lymphomas may have CD10 positivity and the t(14;18) translocation? (Select two correct responses)

A. Diffuse large B-cell lymphoma

B. Follicular lymphoma

C. Lymphoplasmacytic lymphoma

D. Mantle cell lymphoma

Question 28.9 A 66-year-old Caucasian male with HIV and CD4 count of 53 is being worked up by his oncologist for a new diagnosis of diffuse large B-cell lymphoma, which involves diffuse lymphadenopathy, the paranasal sinuses, testes, and bone marrow based on initial studies. What is the next best step in management?

A. Initiation of testicular radiation

B. Lumbar puncture

C. Orchiectomy

D. Upper and lower endoscopy

Question 28.10 A 71-year-old man presents to clinic with abdominal pain. Review of systems is also positive for fatigue and easy bleeding from the nose and gums. Physical examination reveals splenomegaly. Pertinent labs include hemoglobin of 12 g/dL, platelets of 16,000, LDH of 400 IU/L (upper limit of normal 250 IU/L), and albumin of 3.6 g/dL. Peripheral smear reveals “villous lymphocytes.” Flow cytometry is positive for CD19, CD20, and CD22 and negative for CD5, CD25, and CD103. Which of the following are possible steps in initial management? (Select three correct responses)

A. Autologous stem cell transplantation

B. Chemotherapy with alkylating agents

C. Splenectomy

D. Single agent rituximab

Question 28.11 Which of the following findings portend a shorter survival/worse prognosis for patients with splenic marginal zone lymphoma? (Select three correct responses)

A. Albumin <3.5 g/dL

B. Hemoglobin <12 g/dL

C. LDH >normal

D. Presence of villous lymphocytes

Question 28.12 A 69-year-old Caucasian man presents to his primary care doctor for persistent nausea associated with nonbloody, nonbilious emesis, loss of appetite, and 30-lb weight loss over approximately 1 year. He also recalls having occasional dark stools. Physical examination reveals splenomegaly. PET/CT reveals FDG avidity of intra-abdominal lymph nodes as well as uptake in the stomach and small and large bowel. Upper and lower endoscopy reveals a nonbleeding gastric ulcer and diffuse polyps in the colon. Biopsies are performed. Cells express surface IgM and IgD along with CD5, CD19, and CD20. Cytogenetics reveals t(11;14). Which of the following statement(s) is/are accurate regarding the next step in management?

A. Consolidation with autologous stem cell transplantation in first remission is an approach often considered for fit patients.

B. Treatment with ibrutinib in the upfront setting is limited because of its association with peripheral neuropathy.

C. Front-line therapy for elderly patients with good performance status includes bendamustine and rituximab.

D. Hyper-CVAD with rituximab may be administered to patients with more aggressive disease.

Question 28.13 A 46-year-old Caucasian man presents to clinic due to drenching night sweats associated with persistent, high-grade fevers and 40-lb weight loss over the past 6 months. Physical examination reveals lymphadenopathy of the cervical and axillary nodes as well as hepatospenomegaly. PET/CT reveals avid uptake throughout the mediastinum, stomach, and small bowel, with multiple nodes measuring up to 2 cm. FISH reveals BCL-1/IgH fusion gene product. Additional studies show decreased expression of p21, p27, and p53. Which of the following statement(s) regarding the illness is/are CORRECT?

A. Cells arise from antigen-naive B-cells of the inner mantle zone.

B. Most patients present in early-stage disease.

C. The condition is more common in females.

D. Tumor cells strongly express surface IgG and IgD as well as CD19 and CD20.

Question 28.14 A 26-year-old Caucasian man with no past medical history presents to the emergency department with right knee pain that occurred suddenly while playing volleyball. He is found to have a pathologic fracture of the right distal femur. Further imaging with PET/CT reveals localized uptake of the right femur and tibia. Flow cytometry shows that cells are CD19, CD20, and CD22 positive. Immunohistochemistry also reveals that cells are CD10 positive and negative for BCL-6 and MUM-1. Which of the following statement(s) regarding disease subtype is/are accurate?

A. Ig gene shows intraclonal homogeneity.

B. Nuclear factor kappa beta signaling is the primary driver of proliferation and metastases.

C. Survival is worse compared to other subtypes.

D. The subtype is often associated with t(14;18).

Question 28.15 A 63-year-old African-American woman with a past medical history of HIV presents to the emergency room with shortness of breath. She notes that it has been worsening over the past 3 to 4 months, but became unbearable over the past week. Review of systems is positive for involuntary weight loss of 30 lb over 4 months and pedal edema. Physical examination reveals pitting edema to the knees bilaterally. PET/CT scan shows diffuse adenopathy of the mediastinum and the pelvis, with the largest nodes measuring 2.5 cm. No extranodal disease is appreciated. Excisional biopsy reveals proliferating large and small lymphocytes. Ki-67 is 85%. Flow cytometry reveals cells that are CD19, CD20, and CD22 positive and negative for CD10 and BCL-6. Which of the following statements regarding management is accurate?

A. R-CHOP chemotherapy is appropriate.

B. ABVD chemotherapy is preferred as first-line management.

C. Allogeneic stem cell transplant is the treatment of choice if the patient were to relapse with chemosensitive disease.

D. Rituximab with CHOP confers greater benefits in patients who lack BCL-6 expression, based on the GELA R-CHOP trial.

Question 28.16 A 55-year-old Chinese man presents to his primary care physician due to chronic sinus pain that has been present for 5 months. He also complains of epistaxis, fevers, drenching night sweats, and 30-lb weight loss. Physical examination is pertinent for temporal wasting and tenderness to palpation around the paranasal sinuses as well as cervical adenopathy. Labs reveal LDH of 425 IU/L (upper limit of normal 250 IU/L). CT of the sinuses reveals an infiltrating mass in the midline nasal sinus. Immunophenotype reveals atypical cells that are positive for CD2, CD56, and cytoplasmic CD3, and negative for CD4, CD8, and surface CD3. EBV is detected. The cytoplasmic granule proteins, granzyme B and TIA-1, are present. Cytogenetics reveals del(6)(q21;q25). Which of the following statement(s) regarding treatment is/are CORRECT?

A. Allogeneic stem cell transplantation is a standard first-line therapy in fit patients.

B. EBV-related hemophagocytic syndrome is often fatal and treated with front-line radiation.

C. Localized disease is initially managed with concurrent chemotherapy and radiation

D. Patients with disseminated disease that is chemotherapy sensitive are effectively treated with a combination of R-CHOP and radiotherapy

Question 28.17 A 43-year-old woman presents to a dermatologist with a diffuse skin rash, described as red and itchy with associated thickening of the palms and soles that has been slowly progressing for the past 4 years. Physical examination reveals erythematous plaques on 20% of the body surface area. FDG PET reveals absence of extracutaneous disease. Skin biopsy reveals Pautrier microabscesses. Immunophenotyping reveals expression of CD2, CD3, CD5, and CD7. What is the next best step in management?

A. EPOCH chemotherapy combined with pentostatin and fludarabine with interferon alpha

B. Extracorporeal photochemotherapy

C. Locally applied denileukin diftitox with or without vorinostat

D. Topical carmustine or mechlorethamine, bexarotene, or ultraviolet B with or without interferon alpha

Question 28.18 Which of the following accurately describes an aspect of primary CNS lymphoma (PCNSL)?

A. AIDS patients tend to have large, invasive unifocal masses that rapidly progress without HAART therapy.

B. Incidence has decreased fivefold from 1985 to 1997 due to advances in treatment of immunosuppressed patients.

C. Many masses are periventricular, allowing tumor cells access to cerebrospinal fluid.

D. Presents most commonly in the occipital lobe, manifesting as vision changes and gait abnormalities.

Question 28.19 Which of the following statement(s) regarding the presentation of primary CNS lymphoma is/are CORRECT?

A. Approximately 20% of patients have ocular involvement at the time of presentation, particularly of the optic nerve, vitreous, retina, and choroid.

B. Most patients present in stage I, explaining the good prognosis of the condition.

C. Primary leptomeningeal lymphoma in the absence of a brain mass accounts for 40% of cases, with symptoms including leg weakness and incontinence/retention.

D. Studies have shown that 10% of patients can have spread of disease outside of the CNS, mostly to the bone marrow and lymph nodes.

Question 28.20 A 71-year-old Caucasian man is brought to his primary care physician by his wife, who says that he has been exhibiting increasingly aggressive behavior over the past 5 months, manifesting as irritability and outbursts of anger, both of which are outside of his norm. She also says that he has had two motor vehicle accidents in the same time period, due to not noticing traffic lights. Neurologic examination reveals decreased ability to follow commands, 20/200 vision in both eyes, and left-sided weakness. Brain MRI shows a 4 × 5 cm solitary, nonhemorrhagic mass in the right frontal lobe. Lumbar puncture reveals predominance of clonal lymphocytes. Biopsy indicates cells positive for BCL-2, BCL-6, and MUM1 as well as CD19, CD20, and CD22. What is the next best step in management?

A. High-dose methotrexate and rituximab, with or without Ara-C and temozolomide.

B. R-CHOP chemotherapy with intrathecal methotrexate.

C. Six weeks of concurrent chemotherapy with methotrexate and dexamethasone combined with focal radiation therapy.

D. Systemic adriamycin and etoposide with high-dose dexamethasone.

Question 28.21 Which of the following statements best characterizes nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) in contrast to classic Hodgkin lymphoma (cHL)?

A. Approximately 80% of patients with NLPHL have stage I to II disease at the time of diagnosis.

B. Bone marrow involvement is frequent with stage III to IV disease.

C. Disease presentation manifests predominantly in thoracic nodes before spreading to the mediastinum.

D. NLPHL cells express CD30 and CD45 as well as B-cell antigens.

Question 28.22 A 24-year-old G1P0 woman presents to her physician with fever, neck pain, fatigue, and 10-lb weight loss during the first trimester of pregnancy. She is concerned about the health of her fetus. Physical examination reveals nontender cervical adenopathy, leading to a biopsy, which reveals cells that are positive for CD15 and CD30 and negative for CD3, CD7, CD20, and CD45. Morphology is pertinent for Reed–Sternberg cells in a background of inflammatory cells. Which of the following accurately describe(s) an aspect of care for such patients?

A. BEACOPP chemotherapy is preferred to ABVD for treatment during the first trimester due to the lack of antimetabolites in the regimen.

B. MRI scanning is preferred for staging because it is nonteratogenic.

C. Long-term survival of treated pregnant women is inferior to that of nonpregnant women with the disease.

D. Radiation therapy with abdominal shielding can be safely used for bulky disease above the diaphragm.

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