1/54. Clonal expansion of gammadelta-T lymphocytes in an HTLV-I carrier, associated with chronic neutropenia and rheumatoid arthritis.We report on an HTLV-I carrier showing clonal proliferation of gammadelta-T lymphocytes associated with chronic neutropenia and rheumatoid arthritis (RA). A 75-year-old Japanese woman had a 20-year history of RA and was found to have neutropenia and lymphocytosis by routine examinations. Her white cell count was 5,800/microl with 89% lymphocytes. The proliferating gammadelta-lymphocytes did not show the typical morphology of large granular lymphocytes (LGL) and were positive for CD3, TCRdelta1, and HLA-DR but negative for CD4, CD8, and deltaTCS1. Clonally rearranged TCRgamma-chain (Jgamma) and TCRbeta-chain (Cbeta1) genes were detected by Southern blot analysis. Clonality of these proliferating gammadelta-T cells was confirmed by CDR3 size analysis for the TCRdelta-chain. Anti-HTLV-I antibody was positive and the pX region of HTLV-I proviral dna was detected by PCR analysis, but clonal integration of HTLV-I proviral dna was not detected by Southern blotting analysis. The patient's clinical course has been stable, except for infrequent infectious episodes. The association of HTLV-I/II infection with T-LGL leukemia has been reported by several groups, although most cases exhibit TCRalphabeta type T cells. Analysis of the junctional sequence of TCR on T-LGL leukemia cells may clarify the role of HTLV-I/II infection in clonal T-cell proliferation.- - - - - - - - - - ranking = 1keywords = leukemia (Clic here for more details about this article) |
2/54. Primary gastric T-cell lymphomas: report of two cases and a review of the literature.To understand more fully the clinicopathological features of primary gastric T-cell lymphomas (PGTL), we report two cases of PGTL and review the literature. The present cases were not associated with human T-cell leukemia virus type 1 (HTLV-1) and were at clinical stage IIE. In both cases, T-cell origin of the lymphoma cells was diagnosed immunohistochemically. The clinical courses of these two cases were different: one followed a very aggressive clinical course and the patient died 6 months after the diagnosis, whereas the other patient survived more than 2 years without adjuvant chemotherapy. Clinicopathological features of 23 patients with PGTL are summarized with regard to their differences from primary small intestinal T-cell lymphomas (PSITL) and by association with HTLV-1. The median age at onset of PGTL was 58 years. The gender ratio was male-dominant (M:F = 2.3:1). About two-thirds (10 of 17) of PGTL cases had evidence of HTLV-1 infection. The most common presenting symptom for PGTL was upper abdominal discomfort and/or pain (76%), whereas that in PSITL was weight loss (61%) and diarrhea (42%). Typical lesions for PGTL were large ulcerations at the corpus to antrum. Neoplastic cells had no typical morphological characteristics for PGTL including HTLV-1-associated cases. CD3 4 8- was the most frequently observed surface phenotype of PGTL cells. Laboratory findings at diagnosis were not informative. Most patients were treated by gastrectomy with or without chemotherapy. PGTL, excluding that with HTLV-1, showed better prognosis than PSITL, although PGTL with HTLV-1 had a poorer prognosis.- - - - - - - - - - ranking = 0.5keywords = leukemia (Clic here for more details about this article) |
3/54. High prevalence of HTLV-I infection among the family members of a patient with adult T-cell leukemia/lymphoma from northeastern japan.Human T-cell lymphotropic virus type I (HTLV-I) is transmitted through infected lymphocytes mostly by breast feeding. In the present study, high prevalence of HTLV-I infection was disclosed in the family members of a patient with adult T-cell leukemia/lymphoma (ATL), all of whom were residents of Iwate, northeastern japan. Long-term follow-up is necessary for people with HTLV-I infection because of the risk of developing ATL after a certain period of latency. New inventive treatments for the acute and lymphomatous types of ATL are needed.- - - - - - - - - - ranking = 2.5keywords = leukemia (Clic here for more details about this article) |
4/54. Sensorimotor polyneuropathy associated with chronic lymphocytic leukemia, IgM antigangliosides antibody and human T-cell leukemia virus I infection.A 65-year-old man presented with a sensorimotor polyneuropathy associated with B-cell chronic lymphocytic leukemia (CLL) and immunoglobulin m (IgM) antibody to various gangliosides. Electrophysiological studies denoted significant abnormalities of motor and sensory nerve conduction. Although the pathology of sural nerve biopsy looked minimally affected, immunohistochemical studies showed specific binding of IgM to the human peripheral nerve. Our patient also had high titer of antibody to human T-cell leukemia virus I (HTLV-I) in both serum and cerebrospinal fluid (CSF), which might activate B-cell-mediated immunity and facilitate the production of IgM antibody. The other unique feature is the reactivity of antibody to gangliosides. The patient had IgM antibody reactivities to gangliosides with disialosyl residue such as GT1b, GQ1b and GD3, but not to GD1b. IgM antibody to gangliosides with disialosyl residue has been reported in ataxic symptoms, but our patient failed to demonstrate ataxia. Without reactivity to GD1b, sensory ataxic neuropathy might not develop even in the presence of antibody reactive to other gangliosides with disialosyl residue.- - - - - - - - - - ranking = 5keywords = leukemia (Clic here for more details about this article) |
5/54. Improvement of quality of life after splenectomy in an HTLV-I carrier with T-cell prolymphocytic leukemia.A 34-year-old woman of HTLV-I carrier with T-PLL, whose quality of life improved and survival was prolonged after splenectomy, is described. The patient had marked splenomegaly, generalized lymphadenopathy and marked proliferation of abnormal lymphocytes in the peripheral blood with an irregular nucleus, deeply basophilic cytoplasm and a single prominent nucleolus, which were positive for CD2, CD3, CD5, CD7, CD4 and CD8. Although the patient had serum antibody against HTLV-I, HTLV-I proviral dna integration was not detected. She was diagnosed as an HTLV-I carrier with T-PLL and received combination chemotherapy and 15.1 Gy splenic irradiation. However, the generalized lymphadenopathy and splenomegaly did not improve. The patient underwent splenectomy to palliate abdominal distension and hypersplenism. After the operation, her symptoms improved dramatically and within a week her hemoglobin concentration and platelet count normalized. She was discharged from hospital two weeks after the splenectomy, however 11 months later, she relapsed and despite treatment with chemotherapy and alpha-interferon, she died two months after the second admission. autopsy findings revealed that PLL cells had invaded the bone marrow, lymph nodes, liver, lungs, kidneys, uterus, ovaries and adrenal glands.- - - - - - - - - - ranking = 2keywords = leukemia (Clic here for more details about this article) |
6/54. HTLV-I associated infective dermatitis may be an indolent HTLV-I associated lymphoma.When present for a first time blood donation, a 28-year-old Brazilian white female reported a pruritic eczema of the scalp and retroauricular areas since childhood that had been frequently infected. Her mother had been diagnosed as having HTLV-I-associated myelopathy (HAM), and the patient was found to be a human T-lymphotropic virus type-I (HTLV-I) carrier. The patient had been breast-fed for 6 months. The patient had a complete examination, and a biopsy was taken from eczema in the retroauricular area. The biopsy indicated chronic lymphohistiocytic dermatitis with no abnormal lymphocytes. Eleven months later, the patient had an infiltration in the skin of the retroauricular area and a new biopsy revealed atypical lymphocytes. Nested polymerase chain reaction (PCR) was positive for HTLV-I and immunohistochemistry of the tissue at this time confirmed adult T-cell leukemia/lymphoma (ATLL). Retrospective immunohistochemistry showed that the first fragment submitted from the biopsy 11 months before was also compatible with the diagnosis of ATLL. This case fulfilled all major criteria for diagnosis of HTLV-I-associated infective dermatitis (HTLV-I-ID). We postulate that the patient had indolent ATLL associated with HTLV-I infective dermatitis since childhood. We recommend that tissue immunohistochemistry analysis be done in any patient with HTLV-associated infective dermatitis.- - - - - - - - - - ranking = 0.5keywords = leukemia (Clic here for more details about this article) |
7/54. An unusual case of mediastinal lymphadenopathy caused by amyloidosis.A 71-year-old Japanese female Nagasaki-atomic-bomb survivor was admitted for evaluation of a mediastinal mass. She was infected with human T-cell leukemia virus type I. Histological examination of the biopsy specimen, obtained thoracoscopically from the mass, revealed amyloid lymphadenopathy confirmed by congo-red staining and electron-microscopic examination. Amyloid deposits and the serum monoclonal peak consisted of immunoglobulin lambda light chains. No atypical cells were detected in bone marrow. The case was diagnosed as an unusual amyloidosis affecting the mediastinal lymph node. No other sites were found to be affected by amyloidosis, although systemic involvement could not be conclusively ruled out.- - - - - - - - - - ranking = 0.5keywords = leukemia (Clic here for more details about this article) |
8/54. Successful bone marrow transplantation for adult T-cell leukemia from a donor with oligoclonal proliferation of T-cells infected with human T-cell lymphotropic virus.We describe a patient who underwent successful BMT from her sibling for the treatment of adult T-cell leukemia/lymphoma. Pre-transplant examination of the donor revealed oligoclonal integration of HTLV-I proviruses within the germ line, and our concern was that clinical sequelae of HLTV-I infection might become evident in the setting of post-transplant immunosuppression. However, the patient has been in complete remission for 14 months after transplantation, and no clonality of HTLV-I provirus was detected in the peripheral blood cells using southern blotting analysis. Our experience supports the possibility of transplantation from HTLV-I positive donors.- - - - - - - - - - ranking = 2.5keywords = leukemia (Clic here for more details about this article) |
9/54. Human T-cell leukemia virus type I infection in various recipients of transplants from the same donor.BACKGROUND: The human T-cell lymphotrophic virus (HTLV) causes adult T-cell leukemia-lymphoma, tropical spastic paraparesis-HTLV type I, and associated myelopathy. methods: An analysis was performed of serum samples from a multiorgan donor and the five recipients. Also studied was the donor's family and the partner of one of the renal recipients. Serologic detection of anti-HTLV antibodies was carried out by enzyme immunoassay and Western blot to confirm and discriminate between HTLV types. Analysis of proviral dna was performed by polymerase chain reaction and sequenced in the long terminal repeat region and the env gene. Peripheral blood mononuclear cell samples from all the recipients of the HTLV-I-positive organs and the donor's mother were studied. RESULTS: Two years after transplantation, three organ recipients positive for antibodies to HTLV-I were detected (two kidney transplants and one liver). All the recipients' serum samples were negative at the time of transplantation except those from the multiorgan donor. The donor's mother was born in venezuela and was confirmed positive for antibodies to HTLV-I. The remaining family members were negative. HTLV-I dna sequences were recovered, amplified, and sequenced from all the samples from the HTLV-I-positive recipients and the donor's mother. The homology of HTLV-I sequences was 100% in all cases. CONCLUSIONS: The authors are reporting the first documented case of HTLV-I infection in several transplant recipients sharing the same donor. The donor was infected by vertical transmission. HTLV-I infection has devastating consequences for some immunocompromised organ recipients. This emphasizes the need for a systematic survey of HTLV antibodies in all potential donors.- - - - - - - - - - ranking = 2.5keywords = leukemia (Clic here for more details about this article) |
10/54. Human T-cell leukemia virus-1-positive cell line established from a patient with small cell lung cancer.A stable cell line, KHM-3S, was established from a patient with small cell lung cancer (SCLC), who had a high serum level of soluble interleukin 2 receptors (sIL2-R) and was seropositive for human T cell leukemia virus (HTLV)-1. KHM-3S cells were positive for IL2-R (Tac) and NKH-1, but negative for other lymphocytic markers such as OKT 11, OKT 4, OKT 8, T cell receptor (WT 31), B 1, and B 4. Moreover, the KHM-3S cells were negative for leukocyte common antigen and strongly positive for neuron-specific enolase (NSE). Secretion of sIL2-R and NSE by the KHM-3S line was detected by an enzyme-linked immunosorbent assay. Rearrangement of the T cell receptor gene and monoclonal HTLV-1 integration were found by Southern blot analysis of KHM-3S dna. However, Northern blot analysis showed no T cell receptor mRNA. KHM-3S may be useful for studies on the role of HTLV-1 in carcinogenesis and IL2-R expression in SCLC.- - - - - - - - - - ranking = 2.5keywords = leukemia (Clic here for more details about this article) |
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