DIRECT EVIDENCE FOR DISSOCIATED MEGAKARYOCYTIC CHIMERISM IN A WISKOTT-ALDRICH PATIENT SUCCESSFULLY ALLOGRAFTED

被引：7

作者：

BRUNEL, V

MOZZICONACCI, MJ

SAINTY, D

FAUCHER, C

ARNOULET, C

SIMONETTI, J

MARANINCHI, D

BLAISE, D

LAFAGEPOCHITALOFF, M

机构：

[1] INST J PAOLI I CALMETTES, HEMATOL & CYTOGENET LAB, F-13009 MARSEILLE, FRANCE

[2] INST J PAOLI I CALMETTES, UNITE TRANSPLANTAT MEDULLAIRE, F-13009 MARSEILLE, FRANCE

[3] INSERM, U119, MARSEILLE, FRANCE

来源：

BRITISH JOURNAL OF HAEMATOLOGY | 1995年 / 90卷 / 02期

关键词：

WISKOTT-ALDRICH SYNDROME; BONE MARROW TRANSPLANTATION; CORRELATIVE INTERPHASE CYTOGENETICS; MIXED CHIMERA; MEGAKARYOCYTES;

D O I：

10.1111/j.1365-2141.1995.tb05155.x

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

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 thoraco-abdominal 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 X 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.

引用

页码：336 / 340

页数：5

共 18 条

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