1/149. Poor outcome of autologous stem cell transplantation for adult T cell leukemia/lymphoma: a case report and review of the literature.A limited number of patients with adult T cell leukemia/lymphoma (ATL) who received autologous stem cell transplantation (ASCT) have been reported. We report here a case of fatal systemic candida krusei infection in a female patient with ATL undergoing ASCT. All of the eight patients (including seven patients in the literature) with ATL who received ASCT developed relapse of ATL or death due to ASCT complication, irrespective of subtype or remission state of ATL, source or selection of SCT or conditioning regimen. At present, ASCT appears to provide little benefit for ATL in contrast to that for other types of aggressive non-Hodgkin's lymphoma.- - - - - - - - - - ranking = 1keywords = leukemia (Clic here for more details about this article) |
2/149. 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 = 0.4keywords = leukemia (Clic here for more details about this article) |
3/149. Detection of human papilloma virus type 58 in a case of a perianal bowen's disease coexistent with adult T-cell leukemia.A case of bowen's disease (BD) that appeared in the perianal region of a 65-year-old Japanese woman coexistent with chronic adult T cell leukemia (ATL) is described. Histopathological findings revealed that irregularly arranged tumor cells with atypical nuclei throughout the epidermis, which itself disclosed hyperkeratosis, dyskeratotic cells, and clumping cells. Positive staining for HPV antigens was immunohistochemically seen in several nuclei of the tumor cells. Electron microscopic study of the tumor tissue disclosed virus particles of about 50 nm in diameter form the squamous cells. A positive band at 256 bp was obtained by PCR using HPV-L1 primer. The amplified dna by L1 primer completely corresponded to that of HPV-58.- - - - - - - - - - ranking = 1keywords = leukemia (Clic here for more details about this article) |
4/149. Allogenic bone marrow transplantation as a treatment for adult T-cell leukemia.We performed allogenic bone marrow transplantation (BMT) in two adult T-cell leukemia (ATL) patients with HLA-identical siblings as donors. One patient, with acute ATL, relapsed 3 months after BMT. The other, with chronic ATL, has become free of disease over 18 months after the BMT from his human T-cell lymphotropic virus type 1 (HTLV-1)-negative sibling, and we were unable to detect HTLV-1 in the patient's peripheral blood. Based on our results and those of others, although there have been limited numbers of patients, BMT may represent the only potentially curative treatment for ATL, and the presence of graft-vs.-host disease tends to be related to good results, which suggests the possibility that graft-vs.-leukemia effects may play an important role in allogenic BMT for ATL.- - - - - - - - - - ranking = 1.2keywords = leukemia (Clic here for more details about this article) |
5/149. Concurrent infection with legionella pneumophila and pneumocystis carinii in a patient with adult T cell leukemia.A 48-year-old woman was admitted to our hospital with high fever, chills, cough, and exertional dyspnea. On admission, the chest roentgenogram and computed tomography scan showed bilateral alveolar infiltration in the middle and lower lung fields. Microscopic examination of the bronchial lavage fluid showed flower cells typical for adult T-cell leukemia (ATL) and cysts of pneumocystis carinii, and legionella pneumophila serogroup 1 grew on buffered charcoal yeast extract (BCYE)-alpha agar. The patient was successfully treated with antibiotics including trimethoprim/sulfamethoxazole, erythromycin, and sparfloxacin. Remission of ATL was achieved after three courses of antileukemic chemotherapy. Mixed infection of opportunistic pathogens should be considered in patients with ATL.- - - - - - - - - - ranking = 1keywords = leukemia (Clic here for more details about this article) |
6/149. indium-111-chloride scintigraphy visualized gastric involvement in a case of adult T-cell leukemia.We report a case of adult T-cell leukemia (ATL) with gastric involvement visualized by 111In-chloride scintigraphy. A 55-year-old male patient presented with upper abdominal pain and appetite loss. His barium gastric series showed multiple ulcerating polypoid tumors due to gastric involvement of ATL. These lesions accumulated 111In-chloride and were also avid for 67Ga-citrate. Although the mechanism of 111In-chloride accumulation is unknown, 111In-chloride scintigraphy may be useful in evaluating patients with ATL.- - - - - - - - - - ranking = 1keywords = leukemia (Clic here for more details about this article) |
7/149. Molecular remission in adult T cell leukemia after autologous CD34 peripheral blood stem cell transplantation.This report describes a patient with acute-type adult T cell leukemia/lymphoma (ATLL) successfully treated by autologous CD34 peripheral blood stem cell transplantation after fractionated total body irradiation and high-dose cytarabine and cyclophosphamide. A newly established inverse polymerase chain reaction method was used to demonstrate the disappearance of ATLL clonal cells. The patient achieved a sustained molecular remission after transplantation, but died from opportunistic infection 4 months after transplantation. Thus, autologous CD34 peripheral blood stem cell transplantation is promising for this type of malignancy. However, a prudent clinical attitude toward immunological fragility after transplantation is needed for better outcome.- - - - - - - - - - ranking = 1keywords = leukemia (Clic here for more details about this article) |
8/149. Seropositive polyarthritis and skin manifestations in T-prolymphocytic leukemia/Sezary cell leukemia variant.Sezary cell leukemia (SCL) is a rare T cell neoplasia that has been suggested to be a variant of T-prolymphocytic leukemia (T-PLL). Both disorders have an aggressive clinical course, lymphocytosis with characteristic morphology, lymphadenopathy, hepatomegaly, characteristic cytogenetic abnormalities and mature T cell phenotypes. Skin lesions, however, are mainly found in T-PLL. We describe a patient with T-PLL/SCL, who atypically presented with severe seropositive polyarthritis and skin lesions, responding to treatment with human CD52 antibody, CAMPATH-1H and pentostatin. Meningeal leukemia and an assumed myocardial infiltration subsequently developed. Polyarthritis is common in T large granular lymphocyte leukemia and adult T cell lymphoma-leukemia, but both entities could be ruled out in the present case. In rheumatoid arthritis, an expansion of CD4 and/or CD8 T lymphocytes is well documented and this phenomenon is believed to be of pathogenetic importance. We speculate that the T cell clone in the present case had special homing properties or cytokine effects resulting in synovitis.- - - - - - - - - - ranking = 2.6keywords = leukemia (Clic here for more details about this article) |
9/149. A novel natural killer cell line (KHYG-1) from a patient with aggressive natural killer cell leukemia carrying a p53 point mutation.We present the establishment of a natural killer (NK) leukemia cell line, designated KHYG-1, from the blood of a patient with aggressive NK leukemia, which both possessed the same p53 point mutation. The immunophenotype of the primary leukemia cells was CD2 , surface CD3-, cytoplasmic CD3epsilon , CD7 , CD8alphaalpha , CD16 , CD56 , CD57 and HLA-DR . A new cell line (KHYG-1) was established by culturing peripheral leukemia cells with 100 units of recombinant interleukin (IL)-2. The KHYG-1 cells showed LGL morphology with a large nucleus, coarse chromatin, conspicuous nucleoli, and abundant basophilic cytoplasm with many azurophilic granules. The immunophenotype of KHYG-1 cells was CD1-, CD2 , surface CD3-, cytoplasmic CD3epsilon , CD7 , CD8alphaalpha , CD16-, CD25-, CD33 , CD34-, CD56 , CD57-, CD122 , CD132 , and TdT-. Southern blot analysis of these cells revealed a normal germline configuration for the beta, delta, and gamma chains of the T cell receptor and the immunoglobulin heavy-chain genes. Moreover, the KHYG-1 cells displayed NK cell activity and IL-2-dependent proliferation in vitro, suggesting that they are of NK cell origin. Epstein-Barr virus (EBV) dna was not detected in KHYG-1 cells by Southern blot analysis with a terminal repeat probe from an EBV genome. A point mutation in exon 7 of the p53 gene was detected in the KHYG-1 cells by PCR/SSCP analysis, and direct sequencing revealed the conversion of C to T at nucleotide 877 in codon 248. The primary leukemia cells also carried the same point mutation. Although the precise role of the p53 point mutation in leukemogenesis remains to be clarified, the establishment of an NK leukemia cell line with a p53 point mutation could be valuable in the study of leukemogenesis.- - - - - - - - - - ranking = 2keywords = leukemia (Clic here for more details about this article) |
10/149. adult T cell leukemia/lymphoma with lymphopenia in a Korean.We experienced a case of adult T cell leukemia/lymphoma (ATLL) in a 48-year-old Korean female, who has never been abroad since birth and no history of blood transfusion. The patient had hypercalcemia and multiple lymphadenopathy. Histopathologic study of left cervical lymph node (LN) and bone marrow (BM) revealed that infiltrates of malignant lymphoid cells were composed of small, medium and large cells with pleomorphic nuclei. Smears of peripheral blood (PB) showed lymphopenia (16%) with the appearance of a few atypical lymphoid cells (less than 2%), but not the typical clover leaf cells seen in ATLL. Immunophenotypic study of LN and BM revealed T cell phenotype. PB showed increased CD4 T cell (T(H), CD3/CD4 , 57%) and decreased CD8 T cell counts (T(S), CD3/CD8 , 6.7%). The sera of the patient and her family were reactive for HTLV-I antibody. The specific sequences of pol, env, and tax of HTLV-I dna were detected in the lymphoma cells and peripheral blood mononuclear cells (PBMC) using polymerase chain reaction. Ultrastructural examination of PBMC confirmed numerous type c virus particles in extracellular space. This case was an acute type of ATLL without overt leukemic features in PB. Despite chemotherapy and intensive conservative treatment, she died 3 months after admission.- - - - - - - - - - ranking = 1keywords = leukemia (Clic here for more details about this article) |
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