Hemorrhagic bursitis and bone crises in chronic adult gaucher's disease: a case report

✍ Scribed by Gilbert Gelfand; Harry Bienenstock

• Publisher: John Wiley and Sons
• Year: 1982
• Tongue: English
• Weight: 472 KB
• Volume: 25
• Category: Article
• ISSN: 0004-3591
• DOI: 10.1002/art.1780251115

✦ Synopsis

Adult Gaucher's disease (type I) is characterized by a deficiency of the catabolic lysosomal enzyme glucocerebrosidase that results in the accumulation of abnormal cells that contain glucocerebrosides in skeletal and reticuloendothelial tissues. Splenomegaly, hepatomegaly, anemia, thrombocytopenia, and changes in the bone, conjunctiva, and skin are features of the disease. This report is the first description of a patient with Gaucher's disease who, during an episode of bone crisis, developed a persistent suprapatellar hemorrhagic bursitis and an extensive "cold" lesion of the entire left tibia1 plateau, visible on bone imaging.

CASE HISTORY

A 21-year-old white man was admitted to Coney Island Hospital in 1980 with severe left knee pain of several days' duration. When he was 5 , Gaucher's disease had been diagnosed, and since then he has been admitted to several hospitals for episodes of fever and bone pain. In 1974 his spleen was removed, and in 1976 he suffered a pathologic fracture of the right femur. The patient stated that he had been admitted to the hospital on several occasions for osteomyelitis and aseptic necrosis of the left femoral head.

On the day of admission, he was febrile (102°F) and unable to walk because of severe pain and swelling of his left knee. His hemoglobin level was 11.1 gm/dl;