Multiple autoimmune haemopoietic disorders and insidious clonal proliferation of large granular lymphocytes

Koichi Akashi, Tsunefumi Shibuya, Shuichi Taniguchi, Shin Hayashi, Hiromi Iwasaki, Takanori Teshima, Yasushi Takamatsu, Hisashi Gondo, Takashi Okamura, Mine Harada, Yoshiyuki Niho

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Abstract

We report a patient with clonal proliferation of CD3+8+TCRαβ+ large granular lymphocytes (LGL) presenting multiple episodes of autoimmune cytopenia, including autoimmune neutropenia, idiopathic thrombocytopenic purpura, autoimmune haemolytic anaemia, and pure red cell aplasia. Each disorder appeared separately or as a combination during an 11-year clinical course. The increase of blood CD3+8+TCRαβ+ LGL was detected 6 years after the initial diagnosis of cytopenia, but the absolute number of LGL cells was always <1.0 x 109/1. LGL cells were of monoclonal origin and had a chromosomal abnormality. LGL cells transiently responded to cyclosporine A therapy, which was also effective on all of these autoimmune cytopenias. Accordingly, an undetectable level of proliferation of a clonal LGL population could cause various autoimmune haemopoietic disorders.

Original languageEnglish
Pages (from-to)670-673
Number of pages4
JournalBritish Journal of Haematology
Volume107
Issue number3
DOIs
Publication statusPublished - 1999

All Science Journal Classification (ASJC) codes

  • Hematology

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    Akashi, K., Shibuya, T., Taniguchi, S., Hayashi, S., Iwasaki, H., Teshima, T., Takamatsu, Y., Gondo, H., Okamura, T., Harada, M., & Niho, Y. (1999). Multiple autoimmune haemopoietic disorders and insidious clonal proliferation of large granular lymphocytes. British Journal of Haematology, 107(3), 670-673. https://doi.org/10.1046/j.1365-2141.1999.01734.x