Bone infarcts in sickle-cell anemia.

Abstract

Bone infarcts in children with sickle-cell anemia may produce a striking radiographic picture of extreme bone destruction and rapid rebuilding. We have recently seen a case which we believe to be an excellent example of this process; in a second case, in which an initial diagnosis of osteomyelitis was entertained, the subsequent course was more suggestive of bone infarct. The phenomenon of infarction in sickle-cell anemia has long been recognized, and aseptic necrosis resembling infarction pathologically has been described in virtually every organ and tissue of the body (6). Bone infarcts have received less attention in the literature than other features of this disease, and it is not at all certain that there is uniformity in the interpretation of certain of the bone changes, especially with regard to infarcts and osteomyelitis. Four cases, for example, similar to Case I of the present study have been reported as bone infarcts (1, 4, 9, 11), while comparable cases have been described merely as “unusual bone changes” (2, 3, 7). One of the first accounts of bone infarcts in sickle-cell anemia was published in 1937, by Diggs, Pulliam, and King (4), who stated that patients with this disease were subject to attacks of severe bone pain with localized tenderness, fever, and leukocytosis, leading to a diagnosis of osteomyelitis and subsequent surgery; on opening the bone, however, no exudate was encountered and cultures were sterile. The authors concluded that the lesions represented bone infarcts. One patient in their series actually had a proved staphylococcal osteomyelitis in addition to sickle-cell anemia. In 1948, a series of 48 cases of sickle-cell anemia was published by Macht and Roman (9). They found bone destruction, which they called infarction, in 5 cases, involving the long bones and the metatarsals. In 1954, Tori (11) reported 102 cases of sickle-cell anemia; in 1 of these, areas of destruction in the metacarpals were believed to be infarcts. It is our contention that many cases of bone infarction in individuals with sickle-cell anemia go unrecognized either because no films are made, or because of an insufficient interval between the onset of symptoms and roentgen examination. Cases in which bone lesions are discovered, on the other hand, may be mistakenly diagnosed as osteomyelitis when the lesions are in reality uncomplicated bone infarcts. Case Reports Case I: J. R. F., a 22-month-old Negro boy, was admitted to the hospital on Nov. 22, 1955, because of a “swollen arm.” At eight months of age, he had been seen by a physician because of irritability and at that time a diagnosis of anemia was made. Swelling of the hands and feet subsequently developed, and since September 1955 the child had refused to walk. The swelling was associated with pain and tenderness and often accompanied by fever.