A 45-year-old man from coastal Japan presents with generalised lymphadenopathy, skin nodules, lytic bone lesions, and hypercalcemia. Peripheral blood shows atypical lymphoid cells with deeply lobulated nuclei resembling clover leaves. Cells are CD4+, CD7 negative, and strongly CD25 positive with clonal integration of a retrovirus. Which virus and what is the typical first-line approach for the acute leukaemic subtype?

• A Epstein-Barr virus; rituximab-based immunochemotherapy
• B HTLV-2; allogeneic stem cell transplant in all patients
• C Human herpesvirus 8; ganciclovir with antiretroviral therapy
• D Human T-cell leukemia virus type 1; zidovudine plus interferon-alpha, or intensive combination chemotherapy in eligible patients ✓

Explanation

Adult T-cell leukemia/lymphoma is caused by HTLV-1, endemic in southwestern Japan, the Caribbean basin, and parts of Central Africa and South America. The neoplastic cells are CD4-positive T cells with cloverleaf nuclei and intense CD25 expression. Acute subtype features hypercalcemia, skin lesions, lytic bones, and rapid progression. Zidovudine combined with interferon-alpha improves survival in leukaemic presentations, and intensive chemotherapy such as VCAP-AMP-VECP is used in fit patients. EBV drives endemic Burkitt lymphoma, not this entity.

Reference: Robbins and Cotran Pathologic Basis of Disease, 10th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.