Sustained engraftment post bone marrow transplant despite anti-platelet antibodies in Glanzmann thrombasthenia

Veronica H. Flood, F. Leonard Johnson, Lynn K. Boshkov, Gregory A. Thomas, Diane J. Nugent, Antony C. Bakke, H. Stacy Nicholson, David Tilford, Mary P. Brown, Kamar T. Godder

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24 Scopus citations

Abstract

Background. Patients with Glanzmann thrombasthenia (CT) have normal platelet counts but abnormal platelet aggregation and carry the risk of life-threatening bleeding. We report three patients who received bone marrow transplantation (BMT) for type I CT and discuss the risk and management of anti-platelet antibodies. Patients and Results. Diagnosis of CT was made through abnormal platelet aggregation studies or the absence of GPIIb/IIIa by flow cytometry. All patients had severe bleeding requiring multiple red blood cell transfusions. One patient received an unrelated donor transplant and two received matched sibling donor transplants following conditioning therapy with busuifan, cyclophosphamide, and fludarabine. Two patients developed an anti-platelet antibody, treated in one with intravenous immune globulin (IVIC). Engraftment of white blood cells and platelets was achieved on day +13 to +14 and +17 to +25, respectively. Complete donor chimerism and CPIIb/IIIa+ platelets are sustained at +22 to +30 months post transplant. Conclusions. In summary, patients with CT and history of severe hemorrhage can be cured with BMT, but the presence of anti-platelet antibodies should be sought and platelet transfusions minimized prior to transplant. IVIG may be helpful in cases of refractory immune thrombocytopenia related to anti-platelet antibodies. Improvement in transplant-related complications with current transplant regimens allows consideration of BMT for life-threatening non-malignant disorders such as CT.

Original languageEnglish (US)
Pages (from-to)971-975
Number of pages5
JournalPediatric Blood and Cancer
Volume45
Issue number7
DOIs
StatePublished - Dec 1 2005

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Keywords

  • Anti-platelet antibodies
  • Bone marrow transplant
  • Glanzmann thrombasthenia
  • Matched unrelated donor

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Oncology

Cite this

Flood, V. H., Johnson, F. L., Boshkov, L. K., Thomas, G. A., Nugent, D. J., Bakke, A. C., Nicholson, H. S., Tilford, D., Brown, M. P., & Godder, K. T. (2005). Sustained engraftment post bone marrow transplant despite anti-platelet antibodies in Glanzmann thrombasthenia. Pediatric Blood and Cancer, 45(7), 971-975. https://doi.org/10.1002/pbc.20365