Document Type

Article

Publication Date

3-28-2018

Comments

This article has been peer reviewed. It is the authors' final version prior to publication in Clinical Hemorheology and Microcirculation, Volume 68, Issue 2-3, March 2018, Pages 105-128.

The published version is available at https://doi.org/10.3233/CH-189002. Copyright © IOS Press

Abstract

Sickle cell disease (SCD) is an inherited disorder of hemoglobin structure that has no established cure in adult patients. Cure has been achieved in selected children with sickle cell anemia (SCA) using allogeneic bone marrow transplantation or cord blood transplantation. SCD is essentially a triumvirate of (1) pain syndromes, (2) anemia and its sequelae and (3) organ failure, including infection. Pain, however, is the hallmark of SCD and dominates its clinical picture throughout the life of the patients. The prevalence of these complications varies with age from infancy through adult life. However, pain, infections and anemia requiring blood transfusion occur throughout the life span of affected patients. The overall medical care of patients with SCD in developed countries has improved such that their life expectancy has almost doubled since 1951. Currently, there are at least five major approaches for the general management of SCD and its complications. These include (i) symptomatic management, (ii) supportive management, (iii) preventive management, (iv) abortive management, and (v) curative therapy.

Language

English

Included in

Hematology Commons

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