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Primary Effusion Lymphoma-like ATL Developing during Hemodialysis.
Miharu Masaki1, Yuki Okushi2, Atsushi Saito2
1Department of Cardiology, Kanazawa Medical University, Japan.
This study details a rare case of primary effusion lymphoma-like adult T-cell leukemia/lymphoma (PEL-like ATL) in a patient undergoing hemodialysis. Treatment with brentuximab vedotin showed a positive response, highlighting the need for new targeted therapies.
Area of Science:
- Hematology
- Oncology
- Virology
Background:
- Adult T-cell leukemia/lymphoma (ATL) is a lymphoproliferative malignancy associated with human T-cell leukemia virus type 1 (HTLV-1).
- Primary effusion lymphoma (PEL) is an aggressive non-Hodgkin lymphoma, typically seen in advanced HIV infection.
Observation:
- A 77-year-old male on hemodialysis presented with fever and ascites.
- Laboratory findings included elevated lactate dehydrogenase (LDH), calcium, soluble interleukin-2 receptor (sIL-2R), and positive HTLV-1 antibodies.
- Ascitic fluid contained lymphoma cells positive for HTLV-1 proviral DNA, with no neoplastic cells detected in peripheral blood or bone marrow, and no lymphadenopathy.
Findings:
- The patient was diagnosed with acute-type primary effusion lymphoma-like ATL (PEL-like ATL).
- Administration of brentuximab vedotin led to a decrease in serum LDH, sIL-2R, and atypical cells in ascites.
- This case demonstrates a unique presentation of PEL-like ATL in the context of chronic kidney disease and hemodialysis.
Implications:
- This case highlights the importance of considering rare ATL subtypes in patients with unexplained effusions, particularly those on hemodialysis.
- Brentuximab vedotin shows potential efficacy in managing PEL-like ATL.
- Further research into novel molecular-targeted therapies is crucial for improving outcomes in patients with PEL-like ATL.
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