Large granular lymphocyte/natural killer cell proliferative disease: Clinical and laboratory heterogeneity

Bassan, Renato; Introna, Martino; Viero, Piera; Chisesi, Teodoro; Mantovani, Alberto; Barbui, Tiziano

doi:10.1111/j.1600-0609.1986.tb01779.x

Cited by 17 publications

(1 citation statement)

References 20 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…However, these features may vary, even among cells from the same patient ( Loughran, 1993 ). The granulation can range from fine to coarse, and some cells may have otherwise characteristic features but lack granules (sometimes called large agranular lymphocytes) ( Bassan et al , 1986 ). Occasionally, clonally expanded lymphocytes with a characteristic CD3 + , CD57 + phenotype may not have LGL morphology on a peripheral smear ( Ahern et al , 1990 ) but may represent in vivo antigen-activated cytotoxic effector T cells.…”

Section: Diagnosis and Initial Evaluationmentioning

confidence: 99%

Acquired pure red cell aplasia: updated review of treatment

2008

View full text Add to dashboard Cite

Pure red cell aplasia (PRCA) is a syndrome characterized by a severe normocytic anaemia, reticulocytopenia, and absence of erythroblasts from an otherwise normal bone marrow. Primary PRCA, or secondary PRCA which has not responded to treatment of the underlying disease, is treated as an immunologically-mediated disease. Although vigorous immunosuppressive treatments induce and maintain remissions in a majority of patients, they carry an increased risk of serious complications. Corticosteroids were used in the treatment of PRCA and this has been considered the treatment of first choice although relapse is not uncommon. Cyclosporine A (CsA) has become established as one of the leading drugs for treatment of PRCA. However, common concerns have been the number of patients treated with CsA who achieve sustained remissions and the number that relapse. This article reviews the current status of CsA therapy and compares it to other treatments for diverse PRCAs.

show abstract

Section: Diagnosis and Initial Evaluationmentioning

confidence: 99%

Acquired pure red cell aplasia: updated review of treatment

2008

View full text Add to dashboard Cite

show abstract

Fulminant clonal expansion of large granular lymphocytes: Characterization of their morphology, phenotype, genotype, and function

Ohno¹,

Kanoh²,

Arita³

et al. 1988

Cancer

View full text Add to dashboard Cite

A 39-year-old woman exhibited abrupt malignant transformation of the large granular lymphocytes (LGL) after a chronic course of T,-lymphoproliferative disease (T,-LPD). The T,-lymphocytes were CD2+, CD3-, CD8-, CD16+, Leu7-, and Leul9+ with morphologic characteristics of LGL. Newly appearing LGL were much larger and had more prominent azurophilic granules. Although fundamentally they had the same phenotype as the LGL in chronic stage, they showed increased Ia-like antigen and decreased CD16 antigen expressions. Immunoglobulin (Ig) G-kappa type monoclonal component was detected in the patient's serum. The LGL showed a germ-line configuration for T-cell receptor (TCR) beta and gamma chain genes, whereas the clonal chromosomal abnormalities indicated the neoplastic nature of the LGL. The LGL exhibited competent natural killer (NK), interleukin 2 (IL2) activated killer (AK), and antibody-dependent cell-mediated cytotoxicity (ADCC) activities. The LGL may have derived from NK cells at their mature stage with prethymic phenotype and may have influenced the homeostasis of the patient's humoral immune response.

show abstract

Autoimmunity and b-cell dysfunction in chronic proliferative disorders of large granular lymphocytes/natural killer cells

Bassan

Pronesti

Buzzetti

et al. 1989

Cancer

View full text Add to dashboard Cite

Clinical and laboratory findings of B-cell immune dysfunction were evaluated in ten patients with a large granular lymphocyte/natural killer cell proliferative disease (LGL/NK-PD). Increased immunoglobulin synthesis with autoantibody formation was documented: polyclonal hypergammaglobulinaemia (six patients), neutrophil autoantibody (one patient), antinuclear antibody (one patient), and rheumatoid factor (five patients). In addition, serum beta 2-microglobulin level was raised in seven patients, a benign monoclonal gammopathy was detected in one, and concurrent B-type hairy cell leukemia in another. Most patients experienced the complications of hemocytopenia, polyarthritis or rheumatoid arthritis, and immediate allergic reactions to drugs or environmental substances, rather than from symptoms of progressive LGL/NK-PD. A review of the literature confirms that an increased immunoglobulin production is common in LGL/NK-PD, and that patients are likely to develop multiple autoantibodies. The incapacity of proliferating, abnormal LGL/NK cells to suppress B-cell activation and immunoglobulin synthesis, documented by several in vitro studies, offers a pathogenetic explanation for these phenomena. This study shows that an anomalous B-cell function contributes greatly to the morbidity of disease in LGL/NK-PD, and points out the utility of investigating the LGL/NK cell system in patients with autoimmune pathologic characteristics of unclear origin.

show abstract

Large granular lymphocyte/natural killer cell proliferative disease: Clinical and laboratory heterogeneity

Cited by 17 publications

References 20 publications

Acquired pure red cell aplasia: updated review of treatment

Acquired pure red cell aplasia: updated review of treatment

Fulminant clonal expansion of large granular lymphocytes: Characterization of their morphology, phenotype, genotype, and function

Autoimmunity and b-cell dysfunction in chronic proliferative disorders of large granular lymphocytes/natural killer cells

Contact Info

Product

Resources

About