Severe Mediterranean Anemia

A HYPOCHROMIC microcytic anemia in children was first described by Cooley and Lee’ in 1915. Later, milder forms of this condition were recorded. 2, 3 The highest incidence of the disease has been shown to occur in persons of Mediterranean stock (Italians, Greeks, Syrians, and Armenians). However, cases have been reported in individuals of English, German, Spanish, Egyptian, Chinese,4 and Negro5 descent. Foster6 was apparently the first to describe a typical case of Cooley’s anemia in a Chinese child.* Of the 2. cases reported by Gardner,8 the mother of Case i did not have the stigmata of the minor form of the disease in her peripheral blood, which is contrary to all other reports. It is interesting that the father of this same patient, and two siblings, all showed microcytosis and hypochromia, without definite anemia. An error is suggested in Gardner’s Case 2., in that the red blood cell count and hemoglobin indicate a normocytic anemia, though the smear reveals microcytosis. The serum bilirubin value and the x-ray films were consistent with a hemolytic process. Scott9 records the fourth case, in which the clinical and laboratory data are typical of the disease. This paper records the findings in what we believe to be the fifth reported case of Cooley’s anemia in a Chinese child. It is important to recognize the disease clinically for several reasons: (a) It presents many of the findings of other types of hemolytic anemia, but splenectomy is usually of little or no value; (i) both forms of the disease resemble closely irondeficiency anemia, but do not respond to iron therapy; (3) it is not a rare disease, as was first thought, since Neal and Valentine#{176} in a study of persons of Italian descent found one severe case in every 1,368 live births, and one case with the trait in every 2.5 persons studied (Smith” found an even higher incidence in a study of sixteen Italian families); and (4) early recognition may prevent unnecessary treatment and permit a more accurate prognosis.