Adult T-cell leukemia/lymphoma treatment in Bahia, Brazil

Abstract

BackgroundAdult T-cell leukemia/lymphoma is a peripheral disease associated with human T-cell lymphotropic virus type 1. Treatment is carried out according to clinical type with watchful waiting being recommended for less aggressive types. Aggressive adult T-cell leukemia/lymphoma is generally treated with chemotherapy and/or antivirals. The objective of this study was to correlate the survival of patients diagnosed in Bahia, Brazil, with the therapeutic approaches employed and to evaluate what issues existed in their treatment processes.MethodsEighty-three adult T-cell leukemia/lymphoma patients (26 smoldering, 23 chronic, 16 acute, 13 lymphoma and five primary cutaneous tumoral) with available data were included in this study.ResultsComplete response was achieved in seven smoldering patients with symptomatic treatment, in two with chronic disease using antivirals/chemotherapy, in one with acute disease using antivirals and in one lymphoma using the LSG15 regimen [vincristine, cyclophosphamide, doxorubicin, and prednisolone (VCAP); doxorubicin, ranimustine, and prednisolone (AMP); and vindesine, etoposide, carboplatin, and prednisolone (VECP)]. Smoldering patients who received symptomatic treatment presented longer survival. Favorable chronic patients treated with antivirals presented longer survival compared to the unfavorable subtype. However, for the acute form, first-line chemotherapy was better, albeit without significance, than antivirals. Only one of the patients with lymphoma and primary cutaneous tumors responded.ConclusionsWatchful waiting associated with phototherapy represents the best option for smoldering adult T-cell leukemia/lymphoma with survival in Bahia being superior to that described in Japan. There was a trend of better results with zidovudine/interferon-alpha in favorable chronic disease. Excellent results were achieved in the lymphoma type treated with the LSG15 protocol. Patients are diagnosed late probably due to lack of knowledge of adult T-cell leukemia/lymphoma by primary healthcare doctors and a Brazilian treatment protocol needs to be established.

Human T‐Cell Lymphotropic Virus Type 1 and associated diseases in Latin America

“…Most studies have been performed in blood donors, pregnant women and hospitalised patients. Also, ATLL cases have been reported (Table ), .…”

Lymphotropic Viruses EBV, KSHV and HTLV in Latin America: Epidemiology and Associated Malignancies. A Literature-Based Study by the RIAL-CYTED

“…Perhaps the most distinctive feature is an early presentation. Iwanaga et al reported that in Brazil the median age of presentation is 44 yrs, while in Japan it is 68 yrs [510], and, in spite of being named as an adult disease, there are pediatric cases of ATLL in Brazil [323,325,328,340,471]. The Chilean ATLL cohort also had a younger disease presentation than Japan, with a median age of 50 yrs [266].…”

“…For instance, ATLL cases have been reported in the urban areas of North East Brazil, by and large in the Bahía state that presents the largest prevalence of HTLV-1 infection [326,327,333,335,336,[469][470][471]. Characteristics of these ATLL are that they displayed cutaneous involvement [323,324], or were associated with infective dermatitis (IDH) [336], hyalohyphomycosis [333], Strongyloides stercoralis [335] or Hodgkin-like features [332]. Remarkably, ATLL has also been described in 31 pediatric patients [472].…”

HTLV-1-Associated Lymphoma Presented as Massive Lymphadenopathy

“…According to the Shimoyama classification, both cases were most likely diagnosed with ATL lymphoma type. 5 , 15 Case 1 presented with elevated LDH, hypercalcemia, and massive cervical lymphadenopathy causing compression of the airway, and esophagus, as well as the absence of leukocytosis and abnormal peripheral lymphocyte. Case 2 had a much longer disease process that lasts for 3 years.…”