Treatment of Acute or Lymphoma-Type Adult T-Cell Leukemia-Lymphoma with Bulky Lesions
This protocol addresses patients presenting with acute adult T-cell leukaemia-lymphoma (ATLL) with bulky lesions, or with lymphoma-type ATLL with bulky lesions. The presence of bulky disease in either subtype defines this clinical context and shapes the intensity of the recommended management approach.
The approach centres on intensive combination chemotherapy, alongside important prophylactic measures — including CNS prophylaxis and antimicrobial prophylaxis for opportunistic infections — indicated for all patients in this setting. The full regimen choices, sequencing, and individualised considerations are detailed in the complete protocol.
The primary aim is to achieve chemosensitive disease responding to first-line chemotherapy, enabling consolidation.
Patients with acute or lymphoma-type ATLL with bulky lesions may receive intensive combination ChT [e.g. CHOP, CHOEP or hyperfractionated cyclophosphamide-vincristine-doxorubicin-dexamethasone-MTX-cytarabine (hyper-CVAD)] with or without concurrent or sequential zidovudine-IFN-a (if tolerated) [III, C; not EMA or FDA approved].
CNS prophylaxis can be recommended for all patients with acute or lymphoma-type ATLL [III, B].
Antimicrobial prophylaxis for opportunistic infections can be recommended for all patients [II, B].
Chemosensitive patients can proceed to allo-HSCT [III, B].
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