Clinical Utilities of Serum Ferritin as a Marker of Disease Activity and Prognostic Factor in Childhood Tumors / 소아과

PURPOSE: In order to assess the usefulness of serum ferritin as a marker of disease activity and prognostic factor in pediatric malignancy, serum ferritin levels were measured. METHODS: Peripheral blood samplings for ferritin level were made at presentation, in remission following therapy, and in relapse in 95 children with malignancy admitted to the Department of Pediatrics, Kosin University Gospel Hospital between January, 1986 and August, 1995. The patients were comprised of 35 acute lymphoblastic leukemia(ALL), 17 acute myelogenous leukemia(AML), 20 non- Hodgkin's lymphoma(NHL) and 23 neuroblastoma(NB). RESULTS: The mean values of serum ferritin at presentation were 465.3+/-53.9 ng/mL in ALL, 468.9+/-69.4 ng/mL in AML, 274.1+/-69.2 ng/mL in NHL and 337.3+/-64.4 ng/mL in NB. Those values were increased significantly compared to the mean of 20 control children(69.5+/-12.9 ng/mL). The mean values of serum ferritin concentration in remission stage(first, second, and third remission) tend to be lower compared to those in the active stage(at presentation, first relapse and second relapse). But these differences reached a statistical significance only in patients with ALL when the mean values of the active stage were compared to those checked in the remission stage over 12 months(P= 0.0002). Comparison of overall survival according to initial serum ferritin levels(below and above 200 ng/mL) did not show any significant difference in ALL, AML and NHL. However, there was a borderline relationship in NB(relative risk 3.12, P=0.06). CONCLUSION: The study showed that normalization of serum ferritin levels were found in ALL who had continuous, complete remission for more than 12 months. And patients with lower serum ferritin levels were not associated with better survival except in patients with NB in which the lower ferritin group showed borderline significance.

A Case of Rothmund-Thomson Syndrome with Pure Red Cell Aplasia, Autoimmune Hemolytic Anemia and Chronic Respiratory Infection / 소아과

Rothmund-Thomson syndrome(RTS), or poikiloderma congenita, is a rare, multisystem disorder. It is inherited genetically as an autosomal recessive trait, occurring predominantly in females(1.4 : 1). The RTS is comprised of poikiloderma, short stature, sparse hair, juvenile cataracts, skeletal defects, dystrophic teeth and nails, photosensitivity, and hypogonadism. We report a case of RTS who died of bleeding from esophageal varices, pulmonary hemorrhage and septic shock at 25 years of age and had suffered from various diseases such as transient pure red cell aplasia, autoimmune hemolytic anemia, chronic maxillary sinusitis, bronchiectasis, secondary hemochromatosis, and liver cirrhosis in addition to poikiloderma, alopecia, and sexual infantalism which are typical of RTS.