FRCPath Haem Part 1 EMQs-Oncology 39 and 40
- amirhayat2527
- 1 day ago
- 3 min read
Theme: Diagnosis and Initial Management of Rare Aggressive T-cell Lymphomas
Options
A. CHOP chemotherapy followed by consideration of autologous HSCT consolidation
B. Intensive non-cross-resistant chemotherapy (e.g. NCRI/SNLG protocol) followed by BEAM-conditioned auto-HSCT
C. Intensive non-anthracycline-based chemotherapy such as IVAC/ICE with early referral for allo-HSCT
D. Immediate allogeneic HSCT without induction chemotherapy
E. Brentuximab vedotin + CHP
F. Watchful waiting with nutritional optimisation only
G. Alemtuzumab monotherapy
H. R-CHOP chemotherapyI. Surgical resection alone
J. High-dose methotrexate-based CNS-directed therapy
Questions
1.
A 52-year-old man presents with 6 months of weight loss, abdominal pain, chronic diarrhoea and severe hypoalbuminaemia. He has a long history of poorly controlled coeliac disease and poor adherence to a gluten-free diet. CT abdomen demonstrates jejunal wall thickening with mesenteric lymphadenopathy. Emergency laparotomy is performed following bowel perforation, and histology shows pleomorphic medium-to-large atypical lymphoid cells with marked inflammatory background. Immunophenotype: CD3+, CD7+, CD30 variable+, CD4–, CD8–, CD56–. EBER negative.
He is cachectic with ECOG PS 3 and requires TPN.
What is the most appropriate initial treatment?
2.
A 48-year-old woman with previously undiagnosed coeliac disease presents with recurrent abdominal pain, fevers, severe weight loss and small bowel obstruction. Resection specimen confirms enteropathy-associated T-cell lymphoma. Following nutritional rehabilitation and improvement in performance status, she is now ECOG 1 and PET shows residual metabolically active nodal disease.
She is otherwise fit with preserved cardiac and renal function.
What is the preferred treatment strategy?
3.
A 39-year-old Asian man presents with abdominal pain, acute intestinal perforation and B symptoms. Histology from ileal resection shows monomorphic medium-sized lymphoid infiltrate with marked epitheliotropism. Immunophenotype: CD3+, CD8+, CD56+, MATK+, TCRγδ+, CD30 negative. There is no history of coeliac disease.
Diagnosis is confirmed as MEITL.
He is young and transplant eligible.
What is the most appropriate management?
4.
A 27-year-old man presents with fever, drenching night sweats and marked hepatosplenomegaly without lymphadenopathy. FBC shows Hb 92 g/L, platelets 58 ×109/L, WBC 3.1 ×109/L. Bone marrow biopsy shows sinusoidal infiltration by CD3+, CD2+, CD56+, TCRγδ+ lymphoid cells, CD4–, CD8–. Cytogenetics demonstrate isochromosome 7q.
Diagnosis is hepatosplenic T-cell lymphoma.
What is the best initial management?
5.
A 31-year-old woman with longstanding azathioprine exposure for Crohn disease develops fevers, hepatosplenomegaly and pancytopenia. PET shows diffuse marrow and splenic uptake but no significant nodal disease. Liver biopsy confirms hepatosplenic T-cell lymphoma.
She achieves partial remission after induction therapy.
What should be the next major management step?
6.
A 67-year-old frail man presents with perforated jejunal lymphoma on a background of refractory coeliac disease. Histology confirms EATL. He is severely malnourished, albumin 19 g/L, ECOG PS 4, and remains dependent on parenteral nutrition following surgery.
What is the most appropriate first-line approach?
7.
A 44-year-old man is diagnosed with MEITL after resection for bowel obstruction. He has recovered well post-operatively and is fit for intensive therapy. His consultant is considering standard CHOP because of familiarity and lower toxicity.
According to guideline-based management, which approach is preferred?
8.
A 29-year-old man with HSTL receives CHOP chemotherapy as initial therapy in a peripheral hospital before specialist review. Interim PET shows progressive splenic disease and worsening cytopenias.
What would have been the preferred first-line regimen?
9.
A 55-year-old woman with EATL receives 6 cycles of CHOP because of poor nutritional status at diagnosis. She achieves complete metabolic remission and is now significantly fitter.
For disease consolidation in an eligible patient, what should be considered?
10.
A 34-year-old man with newly diagnosed hepatosplenic T-cell lymphoma is reviewed in a tertiary lymphoma MDT. He is responding to ICE chemotherapy. No sibling donor is available, but unrelated donor search is underway.
What is the recommended transplantation strategy?
Answers with Explanations
Q1-A
Frail EATL patients with poor PS and major nutritional deficits should receive initial CHOP rather than highly intensive regimens. Guideline specifically recommends CHOP particularly in impaired PS/nutritional compromise.
Q2-B
Fit EATL patients should be considered for intensification using non-cross-resistant regimens (e.g. NCRI/SNLG) followed by BEAM-conditioned autologous HSCT consolidation.
Q3-B
MEITL is managed similarly to EATL in transplant-eligible patients—intensive first-line therapy followed by auto-HSCT is preferred over standard CHOP alone.
Q4-C
HSTL requires intensive non-anthracycline regimens such as IVAC or ICE, not CHOP. Early transplant planning is essential due to aggressive disease biology.
Q5-C
Patients with HSTL who are potentially eligible should be referred early for allogeneic HSCT and consolidation allo-HSCT should be pursued if donor identified.