Bone mass density in adults with sickle cell disease

Abstract

Summary Sickle cell disease (SCD) leads to many complications including osteoporosis and osteopenia. We studied the prevalence and predisposing factors of low bone mass density (BMD) in adults with SCD. In this retrospective study, dual X‐ray absorptiometry bone scans were used to determine BMD in the lumbar spine, femoral neck and ultra distal radius of 103 patients (73 females, 30 males, aged 15–80 years). Chart reviews and a patient questionnaire were used to collect patient characteristics, disease course and severity, and low BMD risk factors. The 79·6% of patients (mean age 36·5 ± 12·5 years) had an abnormal BMD, with a predilection for the lumbar spine (P = 0·001). Analysis by 3 (low BMD versus very low BMD versus normal) or by 2 groups (abnormal versus normal) showed that abnormal BMD was associated with lower body mass index (BMI) (P = 0·003), lower Hb level (P = 0·001) and higher ferritin (P = 0·003). Low BMD patients were more likely to be SS, SC or Sβ0thal than Sβ+thal (P = 0·022). Abnormal BMD was not related to age, sex, menarche, SCD complications, number of crises, iron overload, treatment with hydroxycarbamide or desferal, renal disease, smoking or alcohol. Patients treated with hydroxycarbamide for at least 6 months were more likely to have an abnormal BMD. In this SCD population, abnormal BMD seemed to be independent of sex, age and menopause, whereas BMI, ferritin level, Hb type and level appeared to play a major role.

Low Bone Mass Density is Associated with Hemolysis in Brazilian Patients with Sickle Cell Disease

“…6 Some studies, however, found no correlation between Hb levels and BMD values. 7,10 Corroborating our findings, Sarrai et al 3 also described an association between abnormal BMD and reduced Hb levels. These conflicting data could be a result of subject selection.…”

“…3,6,10 Bones may be affected by both hemolytic and vaso-occlusive processes in SCD. 2 In our study, we found a correlation between low BMD and increased erythropoietic activity, which was assessed by the strong correlation between LDH, reticulocytes and Hb levels.…”

“…Several factors, including reduced hemoglobin levels, abnormally low body mass index (BMI), increased ferritin levels, vitamin D deficiencies and low plasma zinc or sex steroid levels, have been previously correlated with reduced BMD in adult patients with SCD. 3,[10][11][12][13] There are a limited number of studies and conclusions regarding the risk factors for osteoporosis in adult SCD patients. The present study aimed to establish the prevalence of reduced BMD in adult patients with SCD and examine whether any correlation exists between reduced BMD and clinical characteristics.…”

“…Osteopenia and osteoporosis are often asymptomatic; however, pain, fractures, deformities, and vertebral collapse may occur and require chronic analgesia, mechanical support, and surgical interventions. [2][3][4][5][6] Several studies have shown an overall reduction of BMD in children with SCD. [7][8][9] The causes of BMD abnormalities in patients with SCD are probably Copyright ß 2011 CLINICS -This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http:// creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted noncommercial use, distribution, and reproduction in any medium, provided the original work is properly cited.…”

Bone Disease in Haemoglobin Disorders

“…Bone involvement is the commonest clinical manifestation of sickle cell disease.The bone in SCD is affected by microinfarction, osteopenia, osteoporosis, osteomyelitis, and osteonecrosis (9)(10)(11). The infarction of vertebral bone marrow and the presence of osteoporosis may produce collapse of the vertebrae with the typical fish mouth appearance.…”