Cyclosporine A alleviates severe anaemia associated with refractory large granular lymphocytic leukaemia and chronic natural killer cell lymphocytosis

Large granular lymphocytic (LGL) leukaemia and chronic natural killer cell lymphocytosis (CNKL) are chronic indolent disorders often associated with neutropenia and constitutional symptoms. Severe anaemia occurs in about 20% of patients and is currently treated with corticosteroids followed by oral cyclophosphamide in non-responders. 30% of patients fail initial measures, and salvage therapy is inadequate. We describe three transfusion-dependent patients (two with T-LGL leukaemia, one with CNKL) refractory to corticosteroids, cyclophosphamide, and in one case fludarabine. Cyclosporine A (CSA) initiation resulted in prompt transfusion-independence and was well tolerated in all patients, making it an attractive alternative therapy for this disorder.     

Immunocytochemical characterization of lymphocytes in benign and malignant lymphocyte - rich serous effusions

Summary The cytological diagnosis of malignant Lymphoma in serous effusions can be difficult because reactive lymphocytes may be morphologically indistinguishable from malignant cells in lymphocytic and other low grade Non-Hodgkin's lymphomas. As a result of the present study, diagnostic accuracy can be improved by means of B- and T-cell enumeration using an immunoalkaline-phosphatase method (IAP). 30 cytological specimens, including 28 pleural, 1 pericardial and 1 ascitic fluids, were studied with a panel of monoclonal anti B- and anti T-cell antibodies (PAN B, kappa, lambda, T1, T2, OKT4, T8). Reactive lymphocytic effusions were characterized by a predominance of T cells constituting 80% of all lymphocytes with an excess of helper/inducer cells (mean helper to suppressor ratio 3.0) and by a surface kappa to surface lambda ratio of 1.6 on B-cells. Tuberculous effusions showed a similar distribution of lymphocyte-subpopulations whilst most of the carcinomatous fluids showed a lower percentage of T cells (lowest value 67%) and lower Th:Ts ratio (mean 2.0). Lymphoid cells in samples of five B-cell lymphomas were characterized by T-cell depression ( 70%). B-cells in three cases expressed clear cut light chain monoclonality which was at least suggested in the other two cases.Lymphoid cells from two cases of Hodgkin's disease expressed an indistinct immunological pattern. Labelling of cytoplasmic immunoglobulins (heavy and light chains) using the peroxidase antiperoxidase method (PAP) may be important to characterize neoplasms of the plasma cell series.It is concluded that the chosen panel of antibodies in combination with IAP labelling method may be of great value in identifying B-cell lymphomas. The technique can be used in the routine laboratory and storage of unlabelled and labelled slides over long periods is possible.Dedicated to Professor K. Lennert, Kiel, on the occasion of his 65th birthdayThis study was supported by the Krebsliga St. Gallen/Appenzell     

Lymphocytosis of large granular lymphocytes associated with anemia and neutropenia: Proof of monoclonality of the LGL-population,but benign clinical course

Summary A 37-year-old man with severe transfusion-requiring anemia and neutropenia associated with lymphocytosis of large granular lymphocytes (LGLs) has been followed over a period of 3.5 years. Detailed phenotyping of the LGL has been performed, revealing a phenotype of CD3⁺, CD8⁺, CD16⁺, HLA-DR⁺, and Leu-7⁺. Southern-blot analysis of the T-cell receptor beta-chain locus detected a gene rearrangement, thus providing proof of monoclonality of the peripheral blood LGLs.Abbreviations CD Cluster of differentiation - LGLs Large granular lymphocytes - PBMCs Peripheral blood mononuclear cells     

Characterization of lymphoid cell populations in bovine lymphosarcomas (“leukosis”) by flow-cytofluorometry

Acridine orange stained cell suspensions from ten cases of bovine lymphosarcoma were analyzed by flow-cytofluorometry. The green DNA-AO fluorescence showed in all cases, but one, two distinct separate lymphoid cell subpopulations. One corresponded to (normal) diploid lymphoid cells, the other had variable aneuploid values. The histograms of the latter cell populations also indicated cells in S- and G₂-phase. Low-angle light scatter histograms did not show a distinct separation between the different cell types, although two maxima could be distinguished. The relative proportions between the two cell populations varied between 20 and 80% with domination of one or the other cell type.There was some conformity to the histological and cytological picture in the ordinary light microscope, although neither a clear distinction in separable cell types nor differential counts was possible.The diploid lymphocyte subpopulation in the tumor might represent an immunoreactive process against the BLV-induced lymphosarcoma.     

短暂头痛、神经功能缺损伴脑脊液淋巴细胞增多综合征病例综述

短暂头痛、神经功能缺损伴脑脊液淋巴细胞增多综合征是一种表现为发作性头痛、短暂神经功能缺损、脑脊液淋巴细胞增多且反复发作的神经综合征.该综合征临床缺乏特异性,易与短暂性脑缺血发作、颅内肿瘤、病毒性脑炎、偏头痛等疾病混淆,所以其初次诊断的准确率低,容易漏诊,迄今国内仅见1例报道.为提高对此综合征的认识,本文对其病因、临床特征、诊治进展进行综述.     

Persistent polyclonal B-cell lymphocytosis is an expansion of functional IgD(+)CD27(+) memory B cells

Persistent polyclonal B-cell lymphocytosis (PPBL) is a rare disorder of unknown aetiology affecting predominantly young to middle-aged women. It is characterized by a polyclonal expansion of B cells, including typical binucleated lymphocytes, and is associated with the presence of the translocation t(14;18), involving the bcl-2 oncogene. The stage of differentiation of the B cells expanded in PPBL is not known. We analysed the immunophenotype of the expanded B-cell subset in five new patients with PPBL and found a large uniform expansion of a recently defined human memory B-cell population, IgD(+)CD27(+) memory B cells. After in vitro stimulation with interleukin 2 (IL-2) and Staphylococcus aureus Cowan strain I, B cells from PPBL patients produced high levels of IgM immunoglobulins, which is a characteristic feature of IgD(+)CD27(+) memory B cells. Using a quantitative real-time polymerase chain reaction method, we found a high frequency of the translocation t(14;18) in the range of 1000-3000 per 106 B cells in PPBL patients. In contrast, a much smaller number of cells with a t(14;18) was found in B cells from healthy individuals. Our finding that PPBL is an accumulation of memory B cells further suggests that chronic antigeneic stimulation plays an important part in the pathogenesis of this disorder. This IgD(+)CD27(+) memory B-cell population might harbour a certain number of 'physiological' t(14;18) translocations that increases as this population expands in PPBL patients and constitutes the majority of peripheral blood lymphocytes.