CASE REPORTS
Adult T-cell leukemia/lymphoma (ATL) in Bahia, Brazil
Achiléa L. Bittencourt
Adult T-cell leukemia/lymphoma (ATL), an aggressive type of leukemia/lymphoma was first described by Uchiyama et al (1977) in Japan. Subsequent studies demonstrated its association with the human T-cell lymphotropic virus (HTLV-I). One-third of all cases of T-cell lymphoma in Salvador, Bahia, Brazil are found to be associated with this virus, which is endemic in the region.
In the 74 cases of ATL observed in Bahia, the mean age of patients was 48.2 years, lower than that found in patients in Japan, and there were two cases in adolescents of 15 and 17 years of age and one case beginning in childhood. Forty-four per cent of the cases had history of severe eczema occurring early in childhood, probably corresponding to the infective dermatitis associated to the HTLV-I. Myelopathy associated with HTLV-I (HAM) was present in 13.5% of cases. According to the classification of Shimoyama et al (1991) 22 cases were classified as lymphoma (29.7%), 21 as smoldering (28.4%), 19 as acute (25.7%), and eight as chronic (10.8%). Four cases (5.4%) were classified as a new subtype, which has been previously proposed by us: the primary cutaneous tumoral form that is characterized by the absence of lymphadenomegaly, lymphocytosis, leukemia, hypercalcemia and involvement of internal organs in the presence of normal or slightly elevated LDH levels. I suggest that this new subtype should be included in Shimoyama's classification, since although the cases have only skin lesions they are tumoral and has a worse prognosis than the smoldering type (Table 1). So we would have five subtypes: 1. smoldering with cutaneous non-tumoral lesions; 2. primary cutaneous tumoral; 3. chronic; 4. acute; 5. lymphoma (tumors in lymph nodes and/or in internal organs). In forty-one cases (55.4%) the lesions began in the skin and were considered as a primary cutaneous form. In table 1, are referred the duration of disease, survival and frequency of deaths in each clinical type. Seventy seven per cent of the patients are dead and one has been lost-to-follow-up.
Histologically, a diagnosis of non-specific peripheral T-cell lymphoma was made in 52 cases (70.2%), while 16 cases (21.6%) were diagnosed as mycosis fungoides and 6 cases (8.2%) as anaplastic large cell lymphoma. In 18 of the 74 cases studied clonal integration by southern blot or inverse PCR was evaluated with positive results. Molecular studies are in course in the other cases.
Some aspects observed in these ATL cases were not frequently reported in the literature: 1) earlier appearance, including the occurrence in two adolescents and one child; 2) occurrence of a new clinical subtype, the primary cutaneous tumoral form; 3) presence of other morphological types of lymphoma besides the nonspecific peripheral T-cell lymphoma (mycosis fungoides and anaplastic large cell lymphoma), 4) higher association with HAM (13.5%); and 5) frequent history of severe eczema in childhood.
Publication Dates
-
Publication in this collection
06 Jan 2006 -
Date of issue
Oct 2005
