1/190. 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. ( info) |
2/190. 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. ( info) |
3/190. 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. ( info) |
| 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. ( info) |
| 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. ( info) |
6/190. Aggressive natural killer-like T-cell malignancy with leukemic presentation following solid organ transplantation. NK-like T-cell malignancies are part of a spectrum of lymphoproliferative diseases that complicate immunosuppression associated with solid organ transplantation. We describe 2 patients with long-standing immunosuppression following solid organ transplantation. Both patients had systemic symptoms that included fever, myalgia, and weight loss. Organ involvement and lymphadenopathy were not initially observed. Unique to these 2 cases are the initial leukemic symptoms, which led to further characterization and identification of NK-like T-cell malignancies. Both patients exhibited an anomalous T/NK phenotype, CD56 positivity, and atypical blastic architecture of the large granular lymphocytes. Clonal rearrangement of T-cell receptor genes was detected in both patients. In 1 patient, a cytogenetic abnormality involving 8q24 was demonstrated. The disease course in both patients was aggressive, with involvement of multiple sites and rapid demise. This study emphasizes the importance of including NK-like T-cell malignancies in the differential diagnosis of lymphoproliferative disorders associated with immunosuppression and recognizing that an aggressive clinical course may follow leukemic presentation of disease. ( info) |
7/190. 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. ( info) |
| 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. ( info) |
9/190. 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. ( info) |
10/190. Natural killer (NK) cell leukaemia in a patient with a B cell non-Hodgkin's lymphoma. We describe a 74-year-old woman with the diagnosis of natural killer (NK)-cell leukaemia and autoimmune pathology. Four years previously, a diffuse large B cell non-Hodgkin's lymphoma had been diagnosed and treated effectively. Although NK-cell leukaemia has been thought to be a distinct highly aggressive clinicopathological entity, our case shows no further evolution at the present time. As far as we know, this association has not been previously described in the literature. ( info) |