Rapid response to therapy of neurocutaneous melanosis with leptomeningeal melanoma |
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Authors: | Vivek Subbiah MD Johannes E.A. Wolff MD PhD |
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Affiliation: | Department of Pediatrics, University of Texas MD Anderson Cancer Center, Houston, Texas |
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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. |
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Keywords: | bleeding disorders especially newborn KINDLIN leukocyte adhesion deficiency neutrophil function non‐malignant condition BMT platelet aggregation |
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