Severe Sickle Cell Disease-Pathophysiology and Therapy

George Buchanan, Elliott Vichinsky, Lakshmanan Krishnamurti, Shalini Shenoy

Research output: Contribution to journalArticle

25 Scopus citations

Abstract

Over 70,000 people live with sickle cell disease (SCD) in the United States and multitudes worldwide. About 2000 afflicted babies are born in this country each year. In African countries such as Nigeria, over 100,000 babies are born with the disease each year. Great strides have been made in the conservative management of SCD. However, the medical and psychosocial cost of supporting patients with this chronic illness is enormous and spans a lifetime. Hematopoietic stem cell transplantation (HSCT) can abrogate SCD manifestations, and is the best option for cure today. Yet, this treatment modality is underutilized as less than 500 transplants are reported in the Center for International Blood and Marrow Transplant Research (CIBMTR) database because of its significant risk of morbidity and mortality. There is growing understanding of the pathophysiology of the disease, and this, coupled with advances in transplantation and new approaches to therapy, continue to improve care of patients with SCD both in children and during adulthood. Continuing investigation seeks to predict the course of the disease and to determine timing and modality of therapy in order to optimize outcomes.

Original languageEnglish (US)
Pages (from-to)S64-S67
JournalBiology of Blood and Marrow Transplantation
Volume16
Issue number1 SUPPL.
DOIs
StatePublished - Jan 1 2010

Keywords

  • Bone marrow transplant
  • Chronic transfusion therapy
  • Sickle cell disease

ASJC Scopus subject areas

  • Hematology
  • Transplantation

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