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Aplastic anaemia in a case of hereditary neutrophil Fcγ receptor IIIb deficiency
Author(s) -
Tournilhac Olivier,
Kiladjian JeanJacques,
Cayuela JeanMichel,
Noguera MariaEléna,
Zini JeanMarc,
Daniel MarieThérèse,
Maarek Odile,
Gluckman ÉLiane,
Socié Gérard,
Sigaux François
Publication year - 1997
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1046/j.1365-2141.1997.3813195.x
Subject(s) - cd16 , immunology , medicine , exon , human leukocyte antigen , peripheral blood mononuclear cell , monoclonal antibody , bone marrow , antibody , antigen , biology , gene , cd3 , cd8 , genetics , in vitro
CD16 antibodies recognize Fcγ receptors III of a and b types. In a patient with severe idiopathic aplastic anaemia (AA), polymorphonuclear cells, which in normal subjects express FcγRIIIb, were found to be CD16 negative. The FcγRIIIb gene configuration was analysed by PCR on peripheral blood mononuclear cells. Bi‐allelic deletion encompassing at least part of the coding exon 5 was found in the patient and his brother, suggesting a hereditary defect. The patient underwent successful bone marrow transplantation from his HLA‐matched brother despite a similar phenotype and genotype. This observation suggests that FcγRIIIb hereditary deficiency in donor and/or recipient does not impair engraftment and justifies the use of other monoclonal antibodies in addition to CD16 in the study of GPI‐anchored antigen expression.