Magnetic resonance imaging of bone marrow in sickle cell disease: clinical, hematologic, and pathologic correlations

Abstract

A longitudinal, prospective, controlled evaluation of magnetic resonance images (MRI) of long bones in sickle cell patients was undertaken simultaneously with assessment of clinical status and hematologic parameters, including dense erythrocytes. MRI of bone marrow in sickle cell patients during steady states appeared patchy and were markedly different from those in matched controls (P approximately 0). Patients with severe patchiness were older than those with mild or moderate patchiness (P less than .03). Sixty-nine MRI were performed during 28 painful episodes occurring in 14 subjects with sickle cell disease (SCD). Increased signals on intermediate and T2-weighted images were detected in 35.7% of painful episodes. These abnormalities were distinct and not observed to occur spontaneously during the steady- state examinations (P approximately 0). Bone marrow infarcts were confirmed by biopsy in two instances and autopsy in one instance. Dense red cells decreased by 40.81% of baseline during pain crises (P = .00005), more remarkably in those who had pain in the lower extremities (P = .0145). Patients with change in MRI during pain crises had a greater percentage change in the dense cells than those without the change in MRI (69.7% v 31.3%, P = .0120).

Bone marrow characterization in sickle cell disease: inflammation and stress erythropoiesis lead to suboptimal CD34 recovery

“…The difficulty in obtaining high cell doses and a robust engraftment of transduced HSPCs in SCD patients suggests, however, that the unique inflammatory BM environment associated with SCD may have significant impacts on HSPCs (Kanter et al, 2016Cavazzana et al, 2017;Kwiatkowski et al, 2017). A patient with SCD is said to be in steady state when there is absence of infection, acute clinical symptoms or overt vaso-occlusive crisis for at least three months (Bookchin & Lew, 1996), yet imaging studies of BM suggests that even during steady state, the BM of patients with SCD appears patchy, probably due to hyperplasia of the haematopoietic marrow in addition to repeated episodes of infarction and fibrosis, and is markedly different from those in matched healthy controls and those with nonsickle haemoglobinopathies (Rao et al, 1989;Mankad et al, 1990;Aguilar et al, 2005). This patchiness worsens during vaso-occlusive crisis and with age, and suggests steady state BM from patients with SCD is uniquely different.…”

Outcome in hemoglobin SC disease: A four‐decade observational study of clinical, hematologic, and genetic factors

“…Our observed higher frequency of osteonecrosis and retinopathy over the recent 2 decades is likely to be related to improved diagnostic imaging capability documenting abnormalities earlier during the course of the disease. Modern techniques of magnetic resonance imaging (MRI) confirmed sickle-related bone disease during the initial symptomatic presentation [45,[48][49][50]. During the decades prior to the availability of MRI, osteonecrosis presented as a localized avascular necrosis of the femoral or humoral heads or the vertebral bodies was confirmed by plain roentgenographic procedures.…”

Bone involvement in sickle cell disease

“…Magnetic resonance imaging (MRI) is also a very sensitive imaging technique for detecting bone and bone marrow infarction (Mankad et al, 1988;van Zanten et al, 1989;Mankad et al, 1990;Bonnerot et al, 1994;Deely & Schweitzer, 1997;Frush et al, 1999). Abnormal periosteal signal intensity and soft tissue changes are frequently seen in the first few days of a vasoocclusive crisis; however, as with scintigraphy, these changes are often difficult to distinguish from those seen in osteomyelitis (Bonnerot et al, 1994;Frush et al, 1999).…”