Allogeneic Hematopoietic Stem Cell Transplantation in Glanzmann Thrombasthenia Complicated by Platelet Alloimmunization

被引：6

作者：

Wiegering, V. ^{[1
]}

Winkler, B. ^{[1
]}

Langhammer, F. ^{[1
]}

Woelfl, M. ^{[1
]}

Wirbelauer, J. ^{[3
]}

Sauer, K. ^{[2
]}

Kobsar, A. ^{[2
]}

Meyer, T. ^{[4
]}

Strauss, A. ^{[4
]}

Bakchoul, T. ^{[5
]}

Eyrich, M. ^{[1
]}

Schlegel, P. G. ^{[1
]}

机构：

[1] Univ Childrens Hosp Wurzburg, Pediat Stem Cell Transplantat Program, Dept Pediat Hematol Oncol & Neurooncol, Wurzburg, Germany

[2] Univ Wurzburg, Inst Clin Biochem & Pathobiochem, Cent Lab, D-97080 Wurzburg, Germany

[3] Univ Childrens Hosp Wurzburg, Dept Pediat Intens Care & Neonatol, Wurzburg, Germany

[4] Univ Wurzburg, Pediat Surg Unit, Dept Gen Visceral Vasc & Pediat Surg, D-97080 Wurzburg, Germany

[5] Univ Giessen & Marburg, Ctr Transfus Med & Hemotherapy, Marburg, Germany

来源：

KLINISCHE PADIATRIE | 2011年 / 223卷 / 03期

关键词：

Glanzmann thrombasthenia; platelet alloimmunization; hematopoietic stem cell transplantation; platelet function; refractory to transfusion; IMMUNE THROMBOCYTOPENIC PURPURA; CHILDREN;

D O I：

10.1055/s-0031-1273726

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

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.

引用

页码：173 / 175

页数：3

共 11 条

[1] Molecular basis of Glanzmann's Thrombasthenia and current strategies in treatment [J].