[T-cell lymphoma/leukemia secondary to HTLV-1 in adults. Report of a case].

Revista medica de Panama Pub Date : 1998-01-01
A León, R Días, F Gracia
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Abstract

The Human T-Cell lymphotropic virus type I (HTLV-I) is endemic in the Caribbean basin, Japan, Central Africa and South Pacific. It as been associated to Lymphoma-Leukemia of Adult T-Cells (ATLL) and a progressive spastic paraparesis (TSP/HAM). The nationwide seroprevalence of Panama is of 1-2%. We report a case of a 73 year old male, albino, single, patient, Panamanian descendent from Jamaican immigrants who presented a skin disorder which started 1 year ago, characterized by the appearance of infiltrative, intensely pruritic papules, nodules and a non exfoliative erythroderna involving face, neck, trunk and extremities. He also had painless enlarged cervical lymph nodes, non tender hepatomegaly. Laboratory studies revealed a keukocyte count of 128,000/ml wit 67% atypical lymphocytes, serum calcium was 12.5 mg/dl, DL in 583 UNI, "flower cells" and atypical lymphocytes with hyperlobulated nuclear contour was observed in the peripheral blood smear, seropositivity to TLV-I detected by enzyme-linked immunosorbent assay (Elisa) and confirmed by Western blot assay. The skin biopsy shows a bandlike dermal infiltrates of atypical lymphoid cells with epidermotropism and Pautrier's microabscesses. Once the treatment was initiated with prednisone, cyclophosphamide, and systemic antibiotics for a bronchopneumonic process most of the cutaneous lesions cleared up although the clinical condition of our patient became progressively worse and died after a acute renal failure and a lower gastrointestinal bleeding. In 1986, the tree first cases of ATLL were identified in Panama, there has not been apparently new cases reported until now.

成人HTLV-1继发t细胞淋巴瘤/白血病。病例报告]。
人类t细胞嗜淋巴病毒I型(HTLV-I)在加勒比盆地、日本、中非和南太平洋流行。它与成人t细胞淋巴瘤白血病(ATLL)和进行性痉挛性截瘫(TSP/HAM)有关。巴拿马的全国血清患病率为1-2%。我们报告一例73岁男性,白化,单身,病人,牙买加移民的巴拿马后裔,表现为1年前开始的皮肤疾病,其特征是浸润性,强烈瘙痒丘疹,结节和非剥脱性红皮病,涉及面部,颈部,躯干和四肢。颈部淋巴结无痛肿大,肝无压痛性肿大。实验室检查显示:keukocyte计数12.8万/ml,不典型淋巴细胞67%,血钙12.5 mg/dl, 583例UNI,外周血涂片见“花细胞”和非典型淋巴细胞,核轮廓呈高分叶状,酶联免疫吸附试验(Elisa)检测TLV-I血清阳性,Western blot证实。皮肤活组织检查显示非典型淋巴样细胞带状真皮浸润,伴表皮性和鲍特里氏微脓肿。一旦开始使用强的松、环磷酰胺和全身抗生素治疗支气管肺炎过程,大多数皮肤病变就消失了,尽管我们的患者的临床状况逐渐恶化,并在急性肾功能衰竭和下消化道出血后死亡。1986年,巴拿马发现了第3例ATLL病例,直到现在才有明显的新病例报告。
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