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Rapid response to therapy of neurocutaneous melanosis with leptomeningeal melanoma
Authors:Vivek Subbiah MD  Johannes E.A. Wolff MD  PhD
Affiliation:Department of Pediatrics, University of Texas MD Anderson Cancer Center, Houston, Texas
Abstract:
Leukocyte adhesion deficiency‐III (LAD‐III) is a rare disorder characterized by abnormal signaling to β integrins, leading to defective leukocyte adhesion and chemotaxis and platelet aggregation. Here we present the first case of an African‐American female infant with this disorder. She had history of multiple infections, bleeding, and leukocytosis since birth. She was successfully treated with allogeneic bone marrow transplant using a reduced intensity‐conditioning regimen. Mutations in KINDLIN‐3 have been described in LAD‐III but the mutations in KINDLIN‐3 in her case are unique. Pediatr Blood Cancer 2010;55:180–182. © 2010 Wiley‐Liss, Inc.
Keywords:bleeding disorders  especially newborn  KINDLIN  leukocyte adhesion deficiency  neutrophil function  non‐malignant condition BMT  platelet aggregation
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