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Direct evidence for dissociated megakaryocytic chimaerism in a Wiskott‐Aldrich patient successfully allografted
Author(s) -
BRUNEL VERONIQUE,
MOZZICONACCI MARIE JOELLE,
SAINTY DANIELLE,
FAUCHER CATHERINE,
ARNOULET CHRISTINE,
SIMONETTI JACQUELINE,
MARANINCHI DOMINIQUE,
BLAISE DIDIER,
LAFAGEPOCHITALOFF MARINA
Publication year - 1995
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 - 10.1111/j.1365-2141.1995.tb05155.x
Subject(s) - wiskott–aldrich syndrome , bone marrow , megakaryocyte , cyclophosphamide , immunology , transplantation , conditioning regimen , haematopoiesis , biology , stem cell , cancer research , medicine , chemotherapy , genetics , gene
Summary. We report a Wiskott‐Aldrich patient who underwent allogeneic bone marrow transplantation from his HLA‐identical sister at the age of 25. Conditioning regimen consisted of cyclophosphamide (180mg/kg) and thoracoabdominal irradiation (6 Grays). Cytogenetic follow‐up revealed rapid and complete lymphoid chimaerism, but prolonged mixed bone marrow chimaerism. Correlative interphase cytogenetics performed on bone marrow smears using dual‐colour fluorescence in situ hybridization with × and Y specific probes showed that the proportion of donor cells was significantly higher within megakaryocytes than in other lineages. This patient therefore presented with dissociated lineage engraftment, which is not exceptional in congenital diseases and aplastic anaemia, but has not previously been described in Wiskott‐Aldrich syndrome. Bone marrow transplantation was successful despite this delayed engraftment which ensured adequate production in the involved cell lines.