Nephrotic Syndrome Associated With a Clonal T-Cell Leukemia of Large Granular Lymphocytes With Cytotoxic Function

Orman, S.; Schechter, Geraldine P.; Whang‐Peng, Jacqueline; Guccion, John G.; Chan, Clara S. P.; Schulof, Richard S.; Shalhoub, Robert J.

doi:10.1001/archinte.1986.00360210223034

Cited by 25 publications

References 15 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

Natural killer cell proliferation and renal disease: A functional and phenotypic study

et al. 1996

View full text Add to dashboard Cite

We describe a 57‐year‐old woman who presented with a constitutional syndrome, glomerulonephritis, and lymphocytosis. The phenotypic study, using flow cytometry, showed an expansion of natural killer (NK) cells (CD2+, CD3−, CD16+, CD56+, and CD7+). We performed a functional study of peripheral blood mononuclear cells (PBMCs) and of purified CD16+ cells (NK cells) and CD3+ cells (normal T cells). The expanded NK cell population, CD16+, did not proliferate with phytohemagglutinin (PHA) or anti‐CD3 but showed a dose‐dependent proliferation with recombinant interleukin‐2 (rlL‐2) and also proliferated with phorbol dibutyrate. This population showed very strong NK and lymphokine‐activated killer cell (LAK) activities. The patient's symptoms resolved spontaneously without treatment. Three years later, however, there is still abnormal renal function, and the expansion of NK cells persists, although with no indication of malignancy. We review the features of the different large granular lymphocyte proliferations and their seldom described relationship with renal disease. © 1996 Wiley‐Liss, Inc.

show abstract

Natural killer cell proliferation and renal disease: A functional and phenotypic study

et al. 1996

View full text Add to dashboard Cite

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

Chromosomal abnormalities define clonal proliferation in CD3‐ large granular lymphocyte leukemia

et al. 1990

View full text Add to dashboard Cite

Six patients with lymphoproliferative disorder of large granular lymphocytes (LDGL) were studied for chromosomal abnormalities. When the patients were divided into two groups depending on the expression of a mature antigen of T cell lineage, CD3, two with CD3+ phenotype had a stable disease, whereas three of the four patients with CD3- phenotype had marked hepatosplenomegaly and a highly aggressive disease. Chromosomal abnormalities were detected in four of six patients, indicating a clonal proliferation of large granular lymphocytes in individual patients. In particular, chromosomal abnormalities were found in three of the four patients with CD3- LDGL, all of which had a germ-line configuration of the T cell receptor beta-chain gene. Thus, the chromosomal abnormalities provide definitive evidence for the clonal origin of the expanded cells in the CD3- LDGL.

show abstract

Nephrotic Syndrome Associated With a Clonal T-Cell Leukemia of Large Granular Lymphocytes With Cytotoxic Function

Cited by 25 publications

References 15 publications

Natural killer cell proliferation and renal disease: A functional and phenotypic study

Natural killer cell proliferation and renal disease: A functional and phenotypic study

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

Chromosomal abnormalities define clonal proliferation in CD3‐ large granular lymphocyte leukemia

Contact Info

Product

Resources

About