Medical Care

Many agents have been used to treat angioimmunoblastic T-cell lymphoma (AITL), but none has proved universally or consistently effective. The dosing regimens of these treatments have not been definitively set.

Corticosteroids have long been first-line agents for AITL.^[50]Prednisone has been used alone or in combination with cyclophosphamide, vincristine, or both. The combination of cyclophosphamide, daunorubicin, Oncovin (vincristine), and prednisone (CHOP) has been used before or after prednisone and with or without interferon-alfa as consolidation. In retrospective analyses, CHOP and CHOP-based regimens have produced complete remission rates of about 60%.^{[51, 52]}

In 2018, the US Food and Drug Administration approved brentuximab vedotin for treatment of adult patients with previously untreated systemic anaplastic large cell lymphoma or other CD30-expressing peripheral T-cell lymphomas (PTCLs), including AITL and PTCL not otherwise specified, in combination with cyclophosphamide, doxorubicin, and prednisone (CHP). Approval as frontline therapy was based on the ECHELON-2 phase 3 clinical trial (n = 452). Brentuximab plus CHP was superior to CHOP for progression-free survival (P = .011). Overall survival was also improved compared with CHOP (P = .024).^[53]

Two thirds of patients treated with low doses of recombinant interferon alfa-2a (used as a single agent) achieved an objective remission, while, in the remaining one third of patients, no change or progressive disease was observed. The median remission duration was 3.5 months. Thus, interferon seems a promising agent in the treatment of AITL, but its role must be further defined.

Other therapeutic approaches that have been tried include the following:

Radiation therapy
Cyclosporine, 2-chlorodeoxyadenosine, and cyclophosphamide
Cyclophosphamide, Oncovin (vincristine), prednisone, bleomycin, Adriamycin (doxorubicin), and Matulane (procarbazine) (COP-BLAM)
Ifosfamide, mesna uroprotection, methotrexate, and etoposide (IMVP-16)
Rituximab ^[54]

Methotrexate has not been found to be effective.

High-dose chemotherapy followed by autologous bone marrow transplantation represents a promising new treatment modality for patients with advanced lymphoma and may conceivably be useful in AITL. Rodríguez et al described prolonged survival for patients with AITL after high-dose chemotherapy and autologous stem cell transplantation.^[55]Shinohara et al reported durable remission after the administration of rituximab for Epstein-Barr virus (EBV)–negative, diffuse large B-cell lymphoma that developed after autologous peripheral blood stem cell transplantation for AITL.^[56]

Dunleavy et al have suggested that novel therapeutic strategies that include immunomodulation with agents such as cyclosporine and angiogenesis inhibition with drugs such as bevacizumab may prove helpful.^[5]There are reports of sustained remission from AITL induced by alemtuzumab.^{[57, 58]}

Bendamustine has shown promise as a treatment agent for patients with angioimmunoblastic lymphadenopathy in a phase 2 trial, in particular in this study of 60 patients, 27 (45%) of whom were refractory to previous treatments.^[59]

Korean researchers reported on a case of cervical lymphadenopathy mimicking AITL that was actually dapsone-induced hypersensitivity syndrome. It promptly responded to steroid therapy.^[37]

A report in 2013 from the Netherlands noted a response to lenalidomide durable out to 2 years in a patient refractory to 2 other lines of chemotherapy treatment.^[60]

Surgical Care

In a few cases, the removal of the spleen has improved the symptoms of angioimmunoblastic T-cell lymphoma (AITL) or induced remission. A 5-month-old girl with AITL was treated with interferon-alfa, cyclosporin A, deoxyspagarine, and azathioprine, alternating regimens of low-dose 6-mercaptopurine, cyclophosphamide, and methotrexate, and results were inconsistent. At age 58 months, a splenectomy was performed, which induced a prolonged complete remission of the AITL, without any medication.

Nakashima et al reported successful coil embolization of a ruptured hepatic aneurysm in a patient with polyarteritis nodosa accompanied by AITL.^[61]

Consultations

Management of angioimmunoblastic T-cell lymphoma (AITL) requires consultations with all specialists who can deal with its myriad specific manifestations. An oncologist/hematologist should supervise the care of these patients. Because most patients die of infectious complications, an infectious disease specialist should also be involved.

Long-Term Monitoring

Patients must be monitored for infections and must be educated about the importance of seeking medical care if they develop a fever or other constitutional symptoms. In most patients, angioimmunoblastic T-cell lymphoma (AITL) eventually progresses to immunocompromise, and the assessment and treatment of infection becomes a critical aspect of care.

Medication

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