1/33. 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) |
2/33. The significance of lymphocytosis in congenital hypoplastic anemia.Two infants with congenital hypoplastic anemia had an unusual number of lymphocytes in their peripheral blood and in the bone marrow. This caused an erroneous diagnosis of acute lymphocytic leukemia to be made in the first case and inappropriate therapy to be administered for three months. The second of these cases provided an unusual opportunity to study human erythrocyte precursor dynamics. Serial bone marrow aspirates, obtained after institution of treatment with a corticosteroid, revealed an initial increase in labeled lymphocytes, a concomitant decrease in their number, and the subsequent appearance of erythroid elements. These findings suggest that cells classified morphologically as lymphocytes may serve as erythroid precursors in human beings.- - - - - - - - - - ranking = 0.5keywords = leukemia (Clic here for more details about this article) |
3/33. Long-term remission in an elderly patient with mantle cell leukemia treated with low-dose cyclophosphamide.We present an elderly patient with mantle cell leukemia who was successfully treated with low-dose cyclophosphamide (CY). A 76-year-old female was diagnosed as mantle cell leukemia based on abnormal lymphocytosis and splenomegaly without lymphadenopathy. She was orally treated with 50 mg of CY daily and had continuous remission over 4 years. Rearrangements of BCL1 and immunoglobulin heavy chain genes in the peripheral blood lymphocytes were detected at diagnosis, but not 1 or 4 years later. Further studies are required to confirm the role of low-dose CY therapy for patients with mantle cell leukemia and lymphoma.- - - - - - - - - - ranking = 3.5keywords = leukemia (Clic here for more details about this article) |
4/33. Persistent polyclonal B-cell lymphocytosis--an important differential diagnosis of B-cell chronic lymphocytic leukemia.Over the last 17 years, 83 cases of polyclonal B-cell lymphocytosis (PPBL) have been published. This rare hematological disorder of unknown etiology is characterized by morphologically atypical lymphocytes, polyclonal immunoglobulin M production in association with smoking, female gender, and HLA-DR7 phenotype. We studied another male patient with PPBL. In contrast to normal B-cells, PPBL cells showed no response to interleukin-4 with regard to CD23 and human leukocyte antigen-DR expression. F2mu antibodies failed to co-stimulate interleukin-4-mediated CD23 expression. Crosslinking membrane immunoglobulin m receptors by F2mu resulted in elevated human leukocyte antigen-DR expression but did not induce in vitro proliferation of PPBL cells. This indicates a different activation and differentiation status than normal B-cells.- - - - - - - - - - ranking = 2keywords = leukemia (Clic here for more details about this article) |
5/33. T-large granular lymphocyte leukemia accompanied by an increase of natural killer cells (CD3-) and associated with ulcerative colitis and autoimmune hepatitis.Clonal expansion of large granular lymphocyte(LGL) have been classified into T-LGL and NK-LGL leukemia. T-LGL leukemia cells have a CD3 phenotype and show clonal T-cell receptor(TCR) gene rearrangement. NK-LGL leukemia cells have a CD3- phenotype and no TCR gene rearrangement. We report a case of T-LGL leukemia accompanied by NK LGL expansions in a 65-year-old man who was observed 3 years earlier to have a LGL lymphocytosis in association with ulcerative colitis(UC) and autoimmune hepatitis (AIH). Phenotypic analysis of peripheral blood by flow cytometry disclosed an increase of both T-LGL(CD3 ,CD56-,CD57 ,and TCRalphabeta ) and NK-LGL (CD3-,CD16 ,CD56 , and CD57 ). Clonal rearrangement of the TCR beta gene was detected. A diagnosis of UC and AIH was made on the basis of the X-ray and mucosal biopsy findings of the large intestine, and on the scoring system for diagnosis of AIH, respectively. The disease was nonprogressive, and mesalazine and prednisolone were successful for treatment of UC and AIH. Previously reported cases of T-LGL, NK-LGL leukemia, or NK cell lymphocytosis had no association with UC or AIH, and there have been no reports having both T-LGL leukemia with T-cell receptor gene rearrangement and chronic NK cell lymphocytosis co-existing in a single patient.- - - - - - - - - - ranking = 5keywords = leukemia (Clic here for more details about this article) |
6/33. Large granular lymphocytes (LGL) following non-myeloablative allogeneic bone marrow transplantation: a case report.We report here the first case of large granular lymphocytes (LGL) expansion following non-myeloablative allo-BMT for chronic myeloid leukemia. We characterized the morphologic, phenotypic and functional features of the LGL subset amplified in vivo 14 months after allo-BMT. Our results indicate that LGL can mediate in vitro a cytolytic activity on tumor cells. In vivo, the timing of the LGL expansion was associated with a sustained complete molecular remission. These observations suggest that LGL are a subset with the properties of effector lymphocytes which may contribute to the graft-versus-tumor effect.- - - - - - - - - - ranking = 0.5keywords = leukemia (Clic here for more details about this article) |
7/33. CD5-negative phenotype of monoclonal B-lymphocytosis of undetermined significance (MLUS).Monoclonal B lymphocytosis of undetermined significance (MLUS) is a benign condition of clonal lymphocytosis. Because of its clonal small lymphocytes and indolent clinical course, MLUS resembles and may be indistinguishable from an early stage of chronic lymphocytic leukemia (CLL). In this study we characterized the immunophenotype of MLUS. The clonal B lymphocytes from three patients of typical MLUS were shown to be CD5(-) and CD23(-) phenotype. Their immunophenotypic features remained unchanged during the clinical course. The results indicate a distinct immunophenotype of MLUS that can be useful for its diagnosis and differentiation from CLL.- - - - - - - - - - ranking = 0.5keywords = leukemia (Clic here for more details about this article) |
8/33. Chronic lymphocytic leukemia presenting with symptomatic central nervous system involvement.leukemic infiltration of the central nervous system (CNS) resulting in neurological manifestations is a rare complication of chronic lymphocytic leukemia (CLL). Furthermore, symptomatic CNS involvement as the initial presentation of previously undiagnosed CLL is extremely rare. In the present report, the authors describe a case of an 89-year-old female previously diagnosed with Alzheimer's disease who suddenly developed rapidly worsening mental changes. Cytological and immunocytological examinations of the lymphoid cells present on the cerebrospinal fluid (CSF) revealed CNS involvement by a clonal B-cell lymphoproliferative disorder, most consistent with de novo B-CLL expressing kappa light chain restriction. Subsequently, flow cytometric analysis done on the peripheral blood lymphocytes confirmed the diagnosis of B-CLL in this patient. Thus, this study shows the potential usefulness of immunocytological evaluation in detecting monoclonal lymphoid populations on CSF samples in adult patients presenting with altered mental status and CSF pleocytosis of lymphocytes.- - - - - - - - - - ranking = 2.5keywords = leukemia (Clic here for more details about this article) |
9/33. cytomegalovirus infection associated with clonal proliferation of T-cell large granular lymphocytes: causal or casual?Clonal proliferation of T-cell large granular lymphocytes (LGL) is an indolent disorder characterized by splenomegaly, lymphocytosis and frequent manifestations of immune disturbances. The LGL are CD3( ) CD4(-) CD8( ) CD56(-). The clonality of the tumor cell population is often only demonstrable by T-cell receptor (TCR) gene rearrangement study because chromosomal abnormality is distinctly rare. We describe a case of T-cell LGL leukemia that presented initially as cytomegalovirus infection. The leukemic LGL are shown to be clonal by both TCR gene rearrangement and chromosomal studies. They persist after subsidence of the cytomegalovirus infection.- - - - - - - - - - ranking = 0.5keywords = leukemia (Clic here for more details about this article) |
10/33. Demonstration of clonality, by X-linked dna analysis, in chronic natural killer cell lymphocytosis and successful therapy with oral cyclophosphamide.The expanded lymphocyte population in large granular lymphocyte (LGL)-leukemia carries the phenotypic characteristics of either cytotoxic T lymphocytes (CD3 ,CD8 ) or natural killer (NK) cells (CD3-,CD15 ). In the former subset, clonality has been demonstrated by T-cell receptor gene rearrangement studies. Since NK cells do not rearrange T-cell receptor genes, the neoplastic nature of chronic NK cell lymphocytosis has not been well defined. We used X-linked dna analysis to study the clonal nature of an expanded NK cell population in a patient with a 3-year history of relative lymphocytosis associated with anemia and neutropenia. Southern blot analysis showed no clonal T-cell receptor gene rearrangement. The majority of the circulating lymphocytes had a NK cell phenotype and demonstrated both direct NK cell-mediated cytotoxicity and antibody-dependent cellular cytotoxicity. However, the in vitro growth characteristics of these cells did not suggest that they were polyclonal expansions of normal NK cells. To determine directly the clonal origin of these cells, we performed X-linked dna analysis. Density gradient centrifugation methods were used to isolate mononuclear cells, and NK cells were positively selected by CD16-immunoconjugated magnetic beads. The dna of these cells was analyzed by restriction fragment length polymorphism-methylation strategy and showed a monoclonal pattern of X-chromosome inactivation while a polyclonal pattern was obtained in corresponding skin tissue. Treatment of the patient with oral cyclophosphamide resulted in complete hematologic remission. We conclude that chronic NK lymphocytosis may be clonal and responsive to immunosuppressive therapy.- - - - - - - - - - ranking = 0.5keywords = leukemia (Clic here for more details about this article) |
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