Rare presentation of sickle cell anaemia with marrow necrosis precipitated by Pandorea sp., infection

Abstract

Bone marrow necrosis (BMN) is infrequently encountered in clinical practice and rarely reported in association with sickle cell disease (SCD). The occlusion of the bone marrow (BM) microcirculation with subsequent hypoxia and resulting cell injury has been thought to be the common underlying mechanism. Malignancy is the predominant cause in many studies. We present an unusual case of late-onset SCD whose initial presentation was BMN. The patient presented with fever with chills and bilateral thigh pain. Blood cultures grown Pandorea sp. Even after antibiotics according to sensitivity, fever continued. BM examination was done to send BM cultures. Cultures were sterile. Biopsy showed BMN which was an unexpected finding. As the patient was from tribal area, haemoglobinopathy was suspected and haemoglobin (Hb) electrophoresis was sent which suggested SCD. Thus, a high index of suspicion must be borne in mind, particularly in high SCD-prevalent areas, to identify and prevent this rare complication.

Keywords

• bone marrow necrosis
• myelonecrosis
• pandorea infection
• sickle cell anaemia