| Literature DB >> 28643468 |
Maria Gabelli1, Antonio Marzollo1, Lucia Dora Notarangelo2, Giuseppe Basso1, Maria Caterina Putti1.
Abstract
Wiskott-Aldrich syndrome (WAS) is an inherited X-linked disorder characterized by microthrombocytopenia, immunodeficiency, and eczema. Hematopoietic stem cell transplantation (HSCT) is the treatment of choice. Eltrombopag, a thrombopoietin receptor agonist, may be useful to prevent bleeding while awaiting HSCT. We present a case of a male with WAS, profound thrombocytopenia, and bleeding diathesis successfully managed with eltrombopag before HSCT. Eltrombopag was given for 32 weeks obtaining a stable platelet count without any platelet transfusion. The patient did not experience any bleeding symptom. Eltrombopag may be a suitable therapeutic option for patients with WAS and severe thrombocytopenia as "bridge" to definitive cure.Entities:
Keywords: Wiskott-Aldrich syndromezzm321990; eltrombopag; hematopoietic stem cell transplantation; thrombocytopenia
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Year: 2017 PMID: 28643468 DOI: 10.1002/pbc.26692
Source DB: PubMed Journal: Pediatr Blood Cancer ISSN: 1545-5009 Impact factor: 3.167