Allogeneic hematopoietic stem cell transplantation in Glanzmann thrombasthenia complicated by platelet alloimmunization.

Abstract:

BACKGROUND:For Thrombasthenia Glanzmann (GT) patients presenting with a severe clinical phenotype due to complete lack of thrombocyte function or increased titres of anti-platelet antibodies hematopoietic stem cell transplantation (SCT) is the only curative therapy. CASE REPORT:We report the case of a 13-month-old boy, presenting with a severe course of GT, who was successfully treated with an HLA-identical sibling bone marrow transplant. SCT was complicated by anti-platelet alloimmunization after platelet transfusion successfully treated with high dosage immunoglobulins (2 g/kg) and partial plasma exchange. CONCLUSION:SCT may be a viable option for selected patients with GT. However, SCT in GT carries its own significant risks, resulting from the development of anti-platelet antibodies. A critical risk-benefit analysis is mandatory prior to SCT.

journal_name

Klin Padiatr

journal_title

Klinische Padiatrie

authors

Wiegering V,Winkler B,Langhammer F,Wölfl M,Wirbelauer J,Sauer K,Kobsar A,Meyer T,Strauss A,Bakchoul T,Eyrich M,Schlegel PG

doi

10.1055/s-0031-1273726

subject

Has Abstract

pub_date

2011-05-01 00:00:00

pages

173-5

issue

3

eissn

0300-8630

issn

1439-3824

journal_volume

223

pub_type

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