FRCPath Haem Part 1 EMQs-Oncology 33 and 34

Theme: Management of Mature T-cell Leukaemia’s

Options (Each option may be used once, more than once, or not at all)

A. Watch-and-wait (observation only)

B. Intravenous alemtuzumab

C. Alemtuzumab + purine analogue

D. Alemtuzumab re-treatment

E. Allogeneic HSCT

F. Autologous HSCT

G. Methotrexate (low-dose weekly)

H. Ciclosporin

I. Cyclophosphamide

J. Supportive therapy (G-CSF ± erythropoietin ± short steroids)

K. Alemtuzumab (for refractory LGLL)

L. Purine analogue (e.g. pentostatin) for refractory disease

Scenarios

1.

A 68-year-old man is incidentally found to have lymphocytosis (WBC 45 ×10⁹/L) during routine testing. Flow cytometry confirms T-PLL. He is asymptomatic with no organomegaly, cytopenias, or B symptoms.

What is the most appropriate management?

2.

A 61-year-old woman presents with rapidly rising lymphocytosis, splenomegaly, and B symptoms. Diagnosis of T-PLL is confirmed. She is otherwise fit with no major comorbidities.

Best initial therapy?

3.

A 63-year-old man with T-PLL receives IV alemtuzumab. After 6 weeks, there is only minimal reduction in lymphocyte count, and bulky abdominal lymphadenopathy persists.

Next best step?

4.

A 55-year-old patient with T-PLL achieves complete remission after alemtuzumab. He is fit with a matched sibling donor available.

Best consolidation strategy?

5.

A 70-year-old patient with T-PLL achieves remission with alemtuzumab but is deemed high-risk for transplant-related mortality due to cardiac comorbidities.

Best consolidation option?

6.

A 66-year-old patient previously treated with alemtuzumab for T-PLL relapses after 9 months. Flow cytometry confirms persistent CD52 expression.

Best next management?

7.

A 72-year-old woman with LGLL is found incidentally to have neutrophils 1.2 ×10⁹/L, Hb 120 g/L, platelets normal. She has no infections or symptoms.

Best management?

8.

A 69-year-old man with LGLL presents with severe neutropenia (0.3 ×10⁹/L) and recurrent bacterial infections. He also has longstanding rheumatoid arthritis.

Best first-line therapy?

9.

A 64-year-old patient with LGLL has transfusion-dependent anaemia and neutropenia. He is started on methotrexate but shows no response after 5 months.

Best next step?

10.

A 67-year-old patient with LGLL has failed methotrexate, ciclosporin, and cyclophosphamide. He remains transfusion-dependent with severe neutropenia.

Best next management?