A 7-year-old Korean boy was referred for a nontender mass in the left arm. He had a 5-year history of a poorly defined painless mass on the medial side of the left elbow. The ultrasonogram of the left arm indicated an ill-defined, lobulating solid mass involving the deep soft tissue layer. The fat-suppressed T2-weighted magnetic resonance image showed a poorly marginated, homogeneous high-signal intensity mass. On further follow-up of 1 year and 6 months, the mass showed no change in size and nature. At surgery, a poorly demarcated inflammatory mass was dissected from a branch of the superficial radial nerve and excised completely. There was no recurrence and any dysfunction, except temporary localized hypoesthesia of the medial aspect of the left arm at the 3.6-year follow-up.

No abstract

Patients with hemolytic-uremic syndrome (HUS) can rapidly develop profound anemia as the disease progresses, as a consequence of red blood cell (RBC) hemolysis and inadequate erythropoietin synthesis. Therefore, RBC transfusion should be considered in HUS patients with severe anemia to avoid cardiac or pulmonary complications. Most patients who are Jehovah's Witnesses refuse blood transfusion, even in the face of life-threatening medical conditions due to their religious convictions. These patients require management alternatives to blood transfusions. Erythropoietin is a glycopeptide that enhances endogenous erythropoiesis in the bone marrow. With the availability of recombinant human erythropoietin (rHuEPO), several authors have reported its successful use in patients refusing blood transfusion. However, the optimal dose and duration of treatment with rHuEPO are not established. We report a case of a 2-year-old boy with diarrhea-associated HUS whose family members are Jehovah's Witnesses. He had severe anemia with acute kidney injury. His lowest hemoglobin level was 3.6 g/dL, but his parents refused treatment with packed RBC transfusion due to their religious beliefs. Therefore, we treated him with high-dose rHuEPO (300 IU/kg/day) as well as folic acid, vitamin B12, and intravenous iron. The hemoglobin level increased steadily to 7.4 g/dL after 10 days of treatment and his renal function improved without any complications. To our knowledge, this is the first case of successful rHuEPO treatment in a Jehovah's Witness child with severe anemia due to HUS.