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The chest radiographs of 33 adult hemophiliacs were reviewed for possible pulmonary features of this disease. Only 7 patients showed no abnormalities; 12 patients exhibited scarring, fibrosis, and pleural thickening of the lung, interpreted as the sequelae of intrapulmonary hemorrhage or hemothorax. The remaining 14 patients demonstrated abnormalities of the pulmonary vessels, and 4 of them had evidence of hyperinflation. Possible etiologic factors are discussed.

Hemophilia A and B are commonly considered male diseases because of their X-linked recessive inheritance, but clinical hemophilia is occasionally seen in females. This may represent an extreme example of the Lyon hypothesis in which a heterozygous female has only the one X chromosome bearing the aberrant gene in the active state.

Angiography in the hemophilic patient is a safe and reliable procedure for recognition of surgically treatable vascular abnormalities. When untreated, these abnormalities can cause repeated bleeding and progressive joint destruction.

Three patients with cutaneous and/or synovial hemangiomas demonstrated roentgenographic alterations in the knees, simulating hemophilic arthropathy. In 2 cases, unilateral synovial hemangiomas appeared to be present; in the third patient, who had bilateral abnormalities, the Kasabach-Merritt syndrome was evident, resulting in a consumption coagulopathy and hemorrhagic tendency. The pathogenesis of this unusual arthropathy is discussed.

The ratio of patellar length to width was analyzed in comparison with subjective examination using radiographs of the affected knees in patients with hemophilia, juvenile rheumatoid arthritis (JRA), and controls to determine the usefulness of this measurement in differentiating the two diseases. A statistically significant difference was found between the proportion of JRA patients, controls, and patients with hemophiliac arthritis involving the knee. It was difficult to distinguish hemophilia from JRA by subjective analysis of the appearance of the knee using standard criteria. The patellar ratio is a more objective criterion which should be helpful in the radiological differentiation of JRA from hemophilia affecting the knee.