11/60. HTLV-I-associated T-cell leukemia in hawaii. adult T-cell leukemia (ATL) is a disease found endemic in Southern japan and the Caribbean basin. The human T-cell leukemia virus I (HTLV-I) has been implicated epidemiologically as the causative agent in this disease. This paper describes the identification of this disease in hawaii in a second generation immigrant from Southern japan. The disease was initially indolent, then clinically explosive, characterized by cutaneous lesions, leukemic lymphocytes with convoluted nuclei of T-cell phenotype, hypercalcemia, and a terminal infection. ( info) |
12/60. Spinal myoclonus associated with HTLV III/LAV infection. We describe spinal myoclonus in a 35-year-old homosexual man with concurrent human T-cell lymphotropic virus type III/lymphadenopathy--associated virus (HTLV III/LAV) infection of the central nervous system as indicated by intra-blood-brain barrier synthesis of HTLV III/LAV-specific IgG. The spinal myoclonus was characterized by asymmetric, rhythmic contractions of the abdomen with a frequency ranging between 40 and 70 per minute. The myoclonus was self-limited, resolving over the course of two months. Human T-cell lymphotropic virus type III/lymphadenopathy--associated virus should be considered among the viral causes of spinal myoclonus. ( info) |
13/60. Hypercalcaemia in T cell acute lymphoblastic leukaemia: report of two cases. Two young adults presenting with acute lymphoblastic leukaemia (ALL) associated with hypercalcaemia and osteolytic lesions were both found to have T cell ALL. Hypercalcaemia is a rare feature of ALL and has not previously been related to T cell disease. Both cases, in some respects, resembled (age between 10 and 20 years and low white cell count) the few other previously reported cases. In one of our cases increased concentrations of vitamin D3 seemed to have a role in the pathogenesis of the hypercalcaemia. ( info) |
| chromosome aberrations and clinical features of three patients with adult T cell leukemia-lymphoma (ATL) not associated with human T cell leukemia virus type I (HTLV-I) are described. From their clinical features, two patients were diagnosed as acute type and one patient was diagnosed as chronic type, which later converted to acute crisis. Clonal and many chromosomal abnormalities were observed before therapy in the two acute type cases and at relapse in the chronic type case. karyotype aberrations, including trisomy 3, trisomy 7, trisomy 21, del(6)(q21), del(10)(p13), 14q11 translocation, and loss of x chromosome, all of which are frequently found in HTLV-I associated ATL, were also seen in these cases of HTLV-I-negative ATL. ( info) |
15/60. Chromosomal abnormalities in adult T-cell leukemia/lymphoma (ATL). A report of six cases with review of the literature. Chromosomal studies were performed on six patients with adult T-cell leukemia (ATL). Structural abnormalities of chromosome 3 were the most common. In one case a complete loss of the short arm of chromosome 10 (10 p-) was noted while in another case a balanced translocation involving chromosome 10p and 4q was found. These abnormalities have not been previously reported. After reviewing the literature, it was concluded that chromosomes 3, 6, 10, 13, 14, and X were most frequently involved in abnormalities. Specific and consistent chromosomal abnormalities were noted in each study. Therefore, it is hypothesised that the mutation rate for this virus may be higher than first expected. Furthermore, the relative consistency of heterogeneous findings in different localities may reflect a geographic clustering of specific chromosomal abnormalities which may in turn be related to specific and geographically associated viral mutations. To support these suggestions not only are more cytogenetic data required but a molecular evaluation of these patients must be carried out to establish a relationship, if any, between genetic abnormalities and the epidemiology of ATL. ( info) |
16/60. T-helper phenotype chronic lymphocytic leukemia (Thp-CLL): characterization of an Italian case with particular biological findings. A patient with Chronic Lymphocytic leukemia (CLL) characterized by an expansion of helper phenotype mature T lymphocytes is here described. The phenotype of these cells was OKT3 , OKT4 , Leu 9 , 5/9 , OKT8-, Tac- and functional studies showed a strong helper activity on B cell differentiation; an "in vivo" presence of an IgG-lambda paraproteinaemia has been demonstrated. Cytogenetic studies showed multiple clonal, numerical and structural rearrangements which included a tandem t(14;14) (q11;32) translocation. Hybridization showed HTLV I related specific bands indicating the presence of exogenous sequences related to prototype virus but derived from a different Retrovirus (HTLV 1c). The clinical course was aggressive and unsuccessful treatments with various polichemotherapeutic protocols, associated with multiple leukaphereses, were performed. The authors underline that despite the morphological, immunological, biological and virological heterogeneity, the common feature of T-helper CLL is the inexorable clinical course which needs a new therapeutic approach. ( info) |
17/60. Hypercalcaemia-induced acute renal failure as a presenting feature of T-cell leukaemia. A 14-year-old patient presented with hypercalcaemia-induced acute renal failure. Investigation yielded a diagnosis of T-cell leukaemia. Chemotherapy resulted in complete remission, a return of serum calcium levels to normal and consequent improvement of renal function. ( info) |
18/60. Acute myelopathy associated with primary infection with human immunodeficiency virus. A 29 year old white homosexual man presented with a two and a half week history of severe sore throat, fever, and extreme fatigue. His symptoms did not respond to antibiotics. He had mild bilateral conjunctivitis, a rash over his chest and back, and enlarged lymph nodes, but examination of the nervous system yielded normal results. He had low total white cell and platelet counts. The results of enzyme linked immunosorbent assay for human immunodeficiency virus (hiv) were equivocal when hiv IgM was detected in serum. Despite treatment with ampicillin his temperature remained high and he developed abnormal neurological signs, including a paraparesis and hyperreflexia of the arms. hiv was isolated from lymphocytes from blood and cerebrospinal fluid. Over the next six weeks the patient improved and was discharged. Two months later abnormal neurological signs persisted in his legs. Although various neurological syndromes associated with seroconversion to hiv have been described, this is probably the first report of a patient with myelopathy at the time of seroconversion. ( info) |
19/60. A case of CD4 /CD8- adult T-cell leukemia with good response to interferon-beta terminating as a CD4 /CD8 adult T-cell lymphoma. The leukemic cells of adult T-cell leukemia (ATL) usually express the helper/inducer associated antigen reactive with anti-CD4 antibodies but not with anti-CD8. We present a 63-yr-old woman with ATL characterized by circulating leukemic cells with CD4 /CD8- phenotype, hepatosplenomegaly with no lymphadenopathy, and the presence of proviral dna of human T-cell leukemia virus I in the leukemic cells. She was successfully treated with interferon beta and the remission lasted for 12 months. She then relapsed in the lymph nodes with minimal peripheral blood involvement. The neoplastic cells of the lymph node now co-expressed CD4 and CD8 antigens indicating that the change in clinical manifestation was accompanied by a phenotypic change of the leukemic cells. ( info) |
20/60. Massive acroosteolysis in adult T-cell leukemia/lymphoma. adult T-cell leukemia/lymphoma is a relatively uncommon disease, most often found in japan, the Caribbean, the southeastern united states, and south america. To date there have been few reports of its skeletal manifestations. A case is reported in a 44-year-old man in which a short history of swelling of the hands and feet and painful motion in the fingers was followed by the rapid development of severe acroosteolysis. ( info) |