Medical and Surgical Treatment Options for Early Osteonecrosis in Sickle Cell Disease

Review Article

Ann Hematol Oncol. 2016; 3(5): 1092.

Medical and Surgical Treatment Options for Early Osteonecrosis in Sickle Cell Disease

Al-Jafar HA¹*, Al-Ali NS² Ali A³ and Alobaid A³

¹Department of Hematology, Amiri Hospital, Kuwait

²Department Endocrinology, Amiri Hospital, Kuwait

³Consultant in Orthopedic Surgeon, Al-Razi Hospital, Kuwait

*Corresponding author: Hassan A. Al-Jafar, Consultant Hematologist, Amiri Hospital, Kuwait,

Received: June 17, 2016; Accepted: July 13, 2016; Published: July 15, 2016

Abstract

Sickle cell disease is one of the most common genetic disorders and the first molecular disease identified. Osteonecrosis de?ned as necrosis of bone and bone marrow. It occurs with sickle cell disease due to the polymerization of the sickle cell hemoglobin HbS, which followed by sickling and dehydration of the red blood cells. That causes slowing down of blood circulation and obstruction of the microcirculation leading to osteonecrosis. Many medical and surgical measures could be applied to reserve the joints with osteonecrosis from collapse and may be to avoid major surgical interventions. Both the hematologist and the orthopedics treating their sickle cell patients need to be aware of these available measures. The future trend also promising with the new hypo methylating drugs, new anticoagulants, autologous stem cell-based therapy, and many other new agents could be used to improve osteonecrosis and other sickle cell disease complications.This article reviews a subject poorly discussed in the literature from medical and surgical prophylactic and treatment points of view for the early detected osteonecrosis.

Keywords: Osteonecrosis; Sickle cell anemia; Sickle cell hemoglobin

Introduction

Sickle cell disease (SCD) is the most common and the first molecular disease identified. It has a high prevalence in Africa, around the Mediterranean, Middle East, United States of America, and West Indies and in Brazil [1]. In Africa, the highest prevalence is found within the sicklemic belt of Lehmann. The prevalence of SCD is variable; it is 5-20% in West Africa and reaches up to 40% in Central Africa [2]. The abnormality is due to the substitution of a glutamic acid by a valine at the sixth position on the chain of β-globin, resulting in pathologic hemoglobincalled hemoglobin S. This abnormality results from an autosomal recessive mutation on chromosome 11 [3]. The classic physiopathology of the SCD is based on the polymerization of hemoglobin S, followed by sickling and dehydration of the red blood cells. The slowing down of blood circulation and obstruction of the microcirculation by the deformed red blood cells is the reason of the manifestations and complications of SCD. Bone microcirculation is a common place for red blood cell sickling, which leads to thrombosis, infarct, and Osteonecrosis (OSN) [4].

This bone lesion is also referred as a vascular necrosis, ischemic necrosis or aseptic necrosis. It is defined as a necrosis of the bone and bone marrow [5]. OSN, when investigated with advanced technology, could found in as many as 76% of the people with SCD [6]. It results from temporary or permanent loss of the blood supply to the bones. Around 10,000 to 20,000 Americans typically aged between 20 and 50 years old develop OSN annually from different etiologies [7]. Vertebral bone compression and collapse induced by OSN in people with SCD have also been reported [8]. OSN in SCD found in different sites which seem it potentially affect any bone in the body as a part of the multi-organ infarcts [9]. The diagnosis and staging of OSN must be precise as the condition is complex and requires specific treatment according to the grade of bone and joint involvement [10]. There are a number of classifications or staging systems used for grading OSN joint involvement such as Steinberg classification [11] (Table 1).

Citation: Al-Jafar HA, Al-Ali NS, Ali A and Alobaid A. Medical and Surgical Treatment Options for Early Osteonecrosis in Sickle Cell Disease. Ann Hematol Oncol. 2016; 3(5): 1092. ISSN : 2375-7965