Adult T-cell leukemia/lymphoma: report of two cases

Abstract

Adult T-cell leukemia/lymphoma is a lymphoproliferative disorder of mature T lymphocytes associated with infection with human T-cell lymphotrophic virus type I (HTLV-I). Adult T-cell leukemia/lymphoma is characterized by clinical and laboratory polymorphism that allows it to be classified into four distinct subgroups: smoldering, chronic, acute and lymphomatous types. We present here two cases of adult T-cell leukemia/lymphoma, respectively in the acute and lymphomatous forms of the disease. Case 1 was a 35-year-old woman who presented abdominal distension accompanied by hepatosplenomegaly, adenomegaly, skin lesions, positivity for anti-HTLV-I antibodies and leukocytosis with the presence of flower cells. Case 2 was a 38-year-old man who was admitted with generalized lymphadenomegaly, positivity for anti-HTLV-I antibodies, hypercalcemia and osteolytic lesions. In this paper, we correlate the clinical-laboratory findings of these two cases with data in the literature. Key-words:Human T-cell lymphotrophic virus. Adult T-cell leukemia/lymphoma. Flower cell. RESUMO A leucemia/linfoma de células T do adulto é um distúrbio linfoproliferativo de linfócitos T maduros associado à infecção pelo vírus linfotrópico de células T humanas tipo I (HTLV-I).A leucemia/linfoma de células T do adulto tem polimorfismo clínico e laboratorial, que a classifica em quatro subgrupos distintos entre si: smoldering, crônica, aguda e linfomatosa. Apresentamos neste artigo, dois casos de leucemia/linfoma de células T do adulto, respectivamente, nas formas aguda e linfomatosa da doença. O caso 1: uma paciente de 35 anos apresentava distensão abdominal com hepatoesplenomegalia, adenomegalia, lesões cutâneas, anticorpos anti-HTLV-I positivo e leucocitose com presença de flower cell. O caso 2: homem de 38 anos, internado com linfadenomegalia generalizada, anticorpos anti-HTLV-I positivo, hipercalcemia e lesões osteolíticas. Neste artigo correlacionamos os achados clínicos e laboratoriais destes dois casos com dados da literatura. Palavras-chaves:Vírus linfotrópico de células T humanas. Leucemia/linfoma células T do Adulto. Flower cell.

Adult T-cell leukemia/lymphoma in South and Central America and the Caribbean: systematic search and review

“…Finally, 90 articles were chosen including 940 ATL reported cases; 24 papers were found in the Caribbean, 15,24,25,28–48 two in Central America (Table 2) 49,50 and 64 in South America (Table 3). 9,16,51…”

“…128 The association with IDH is rare, and except for one all cases were diagnosed in Brazil. 39,55,69,70,79 The clinical classification of ATL was not reported in some papers, in spite of the fact that it is of prime importance in order to determine the prognosis and the adequate therapeutic approach. 12 Acute ATL was much more frequent than other types, possibly because it is generally easier to diagnose due to marked lymphocytosis, hypercalcemia and presence of many 'flower' cells in peripheral blood.…”

“…128 The association with IDH is rare, and except for one all cases were diagnosed in Brazil. 39,55,69,70,79…”

Adult T‐cell leukemia/lymphoma in a Peruvian hospital in human T‐lymphotropic virus type 1 (HTLV‐1) positive patients

“…In Latin America, treatment available consists of chemotherapy (CHOP) and AZT/IFN. Several reports indicate that each one alone or combined leads to mixed results …”

Mechanisms of Humoral Hypercalcemia of Malignancy in Leukemia/Lymphoma

“…50-90% of ATLL patients develop HHM and osteolytic lesions in the long bones and calvaria (Olivo et al, 2008). Bone resorption may act as a 'vicious cycle' for ATLL growth in bone, since factors released by resorbing bone increased the growth of ATLL and HTLV-1-infected T-cells in vitro (Shu et al, 2010).…”