Abnormal NK Cell Lymphocytosis Detected after Splenectomy: Association with Repeated Infections, Relapsing Neutropenia, and Persistent Polyclonal B-Cell Proliferation

被引：0

作者：

Elisa Granjo

Margarida Lima

Manuela Fraga

Filipe Santos

Conceição Magalhães

Maria Luís Queirós

Ilidia Moreira

Sandra Rocha

Alice Santos Silva

Irene Rebelo

Alexandre Quintanilha

Maria Letícia Ribeiro

Jorge Candeias

Alberto Orfão

机构：

[1] Hospital S. João,Department of Hematology

[2] Hospital S. António,Service of Hematology, Unit of Cytometry

[3] Hospital S. João,Department of Immunotherapy

[4] Hospital S. João,Department of Immunology

[5] Faculdade de Farmácia,Service of Cytometry

[6] Instituto de Ciências Biomédicas Abel Salazar,Serviço de Hematologia Clínica

[7] Instituto de Biologia Celular e Molecular,undefined

[8] Centro Hospitalar de Coimbra,undefined

[9] Hospital Universitario de Salamanca,undefined

[10] Hospital S. João,undefined

来源：

International Journal of Hematology | 2002年 / 75卷

关键词：

NK cells; Neutropenia; B-cells; Spherocytosis; Splenectomy;

D O I：

暂无

中图分类号：

学科分类号：

摘要：

We report the case of a boy with hereditary spherocytosis who presented with mild microcytic hypochromic anemia and recurrent leg ulcers that had been present since childhood. Chronic natural killer (NK) cell and B-cell lymphocytosis was detected 1 year after therapeutic splenectomy during investigation of recurrent episodes of neutropenia and persistent lymphocytosis. NK cells proved to be abnormal at immunophenotyping studies, and B-cells were polyclonal and displayed a normal immunophenotype. Genotypic analysis of T-cell receptor (TCR)-β and TCR-γ genes showed a germ-line pattern. The clinical course of this patient was characterized by multiple pulmonary infections and amygdalitis. We discuss the potential roles of persistent immune stimulation due to chronic hemolysis and severe leg ulcers and of splenectomy in the origin of NK cell lymphocytosis and the relationship between NK cells and recurrent infections, relapsing neutropenia, and polyclonal B-cell response.Int J Hematol. 2002;75:484–488.

引用

页码：484 / 488

页数：4

共 46 条

[1] Hassoun H(1996)Hereditary spherocytosis: a review of the clinical and molecular aspects of the disease Blood Rev. 10 129-147
[2] Palek J.(1999)Lymphocyte populations in hereditary spherocytosis pre and post splenectomy and under oxidative stress Mol Biol Hematopoiesis 6 347-352
[3] Granjo E(1994)Hereditary spherocytosis and haematological malignancy N J Med. 91 95-97
[4] Moreira I(1995)Natural killer cells wear different hats: effector cells of innate resistance and regulatory cells of adaptative immunity and of haematopoiesis Semin Immunol. 7 83-88
[5] Santos Silva A(2001)Immunophenotypic characterization of normal blood CD56+lo versus CD56+hi NK- cell subsets and its impact on the understanding of their tissue distribution and functional properties Blood Cells Mol Dis. 27 731-743
[6] Conti JA(1996)Classification of natural killer (NK) cell and NK-like T cell malignancies Blood 87 1207-1210
[7] Howard LM.(1996)Chronic natural killer cell lymphocytosis Leuk Lymphoma. 20 245-248
[8] Trinchieri G.(1999)A long-term study of patients with chronic natural killer cell lymphocytosis Br J Haematol. 106 960-966
[9] Lima M(2001)Immunophenotypic analysis of the TCR-Vγ repertoire in 98 persistent expansions of CD3+/ TCRαβ+ large granular lymphocytes: utility in assessing clonality and insights into the pathogenesis of the disease Am J Pathol. 159 1861-1868
[10] Teixeira MA(1993)Transient and persistent expansions of large granular lymphocytes (LGL) and NK associated (NKa) cells: the Yorkshire Leukaemia Group Study Br J Haematol. 83 505-517

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