Hemophagocytic Lymphohistiocytosis in Infants: A Single Center Experience from India. (1st April 2014)

• Record Type: Journal Article
• Authors: Ramzan, Mohammed; Yadav, Satya Prakash; Kharya, Gaurav; Chinnabhandar, Vasant; Enteserian, Miriam; Henter, J Inge; Sachdeva, Anupam
• Is Part Of: Pediatric hematology and oncology. Volume 31:Number 3(2014:Apr.)
• Page Start: 285
• Page End: 292
• Publication Date: 2014-04-01
• Subjects: Pediatric hematology -- Periodicals; Tumors in children -- Periodicals; Blood -- Diseases -- Periodicals; Hematologic Diseases -- Child; Hematologic Diseases -- Infant; Neoplasms -- Child; 618.9215
• DOI: 10.3109/08880018.2013.862754
• Languages: English
• ISSNs: 0888-0018

Abstract

<abstract> <title> <x xml:space="preserve">Abstract</x> </title> <p>There is paucity of outcome data for hemophagocytic lymphohistiocytosis (HLH) in infants from India, especially post stem cell transplant (SCT). We report outcome data of eight infants diagnosed with HLH. Mean age was 7.1 months (range 2–11). Mutation analysis was possible in seven patients. One patient had Griscelli syndrome. In three patients, no known mutation could be identified, while in remaining three homozygous mutations in Perforin, Munc and STX11 gene were identified. All were treated as per HLH 2004 protocol. Four died during induction phase. One patient abandoned therapy. Two underwent SCT, while one is awaiting SCT. First patient is alive and disease-free at 22 months postmatched sibling donor SCT. Second underwent unrelated double cord blood transplant, but died 5 months posttransplant due to renal failure. It is feasible to offer SCT for infants with familial HLH in the developing world although barriers like sepsis and disease refractoriness remain.</p> </abstract>