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Four patients with purpura who manifested an unusual response to bruising were studied. This response was characterized by the development of an area of painful ecchymosis at the site of trauma followed by progressive erythema and edema. This unusual tissue response was seen only in women. The various features of the cases suggested an autosensitization by the patients to their own blood. Special studies utilizing skin testing procedures indicated an abnormal tissue response of sensitivity to red blood cells. The factor responsible was present in the red cell stroma and was not associated with the hemoglobin. The clinical manifestations and possible therapy are discussed. This syndrome may represent another example of autosensitization such as has been speculated for lupus erythematosus, some forms of acquired hemolytic anemia and of thrombocytopenic purpura, and for an increasing number of disease states.

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Abnormalities of adenosine deaminase, a critical enzyme of the purine salvage pathway, have been reported in association with immune dysfunction, acute leukemia, and hereditary hemolytic anemia. We report data showing that erythrocyte adenosine deaminase activity is also abnormal in congenital hypoplastic anemia (the Diamond-Blackfan syndrome). Adenosine deaminase activity in erythrocytes from 12 patients (mean +/- S.D., 2.20 +/- 0.77 IU per gram of hemoglobin) was substantially greater than that observed in 50 controls (0.62 +/- 0.13 IU per gram). Enzyme activity in affected patients was also greater than that seen in cord blood or in erythrocytes from patients with hemolytic anemia, acquired aplastic anemia, Fanconi's hypoplastic anemia, acquired pure red-cell aplasia, or transient erythroblastopenia of childhood. These observations indicate that erythrocyte adenosine deaminase activity may be a unique marker for identifying congenital hypoplastic anemia.

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