LRBA defects should always be kept in mind as a differential diagnosis for patients with autoimmune disease affecting multiple organs, chronic diarrhea, and organomegalies. In our experience, early HSCT is a life-saving therapeutic strategy.

A high serum sCD44 level and/or tumor tissue expression at diagnosis is associated with poor prognostic criteria and/or unfavorable outcome in childhood leukemias and lymphomas.

Genistein sensitizes cancer cells to chemotherapy and radiation by modulating cell survival pathways. At the same time, genistein's antioxidant and anti-inflammatory effects may protect normal tissues from adverse effects of chemotherapy and radiation, which are largely due to oxygen-free radicals and inflammation. We conducted a small pilot study with a soy isoflavone mixture containing 8 mg of genistein in children receiving chemotherapy and/or radiation to investigate genistein's potential toxicity preventive effect. We monitored clinical and laboratory parameters in children with cancer who received their first cycle of chemotherapy without genistein and the subsequent cycles with genistein. Patients served as their own controls, and the clinical-laboratory data from the first cycle were compared to the data from subsequent cycles. Nine cycles of chemotherapy were administered without genistein and 57 cycles with genistein. Patients experienced less myelosuppression, mucositis, and infection when they received genistein with chemotherapy. During supplementation, serum genistein levels were 2 to 6 times higher compared to presupplementation levels. Patients who received abdominal radiation reported less pain and diarrhea when they took the genistein supplement. Further clinical investigation of soy isoflavones in pediatric cancer patients receiving chemotherapy and/or radiation should be conducted.

Advanced intraocular tumors and metastatic disease in retinoblastoma patients still occur frequently in developing countries. The aim of this retrospective study was to describe the clinical and epidemiological characteristics of patients with retinoblastoma and the effects of these features on disease prognosis in the authors' pediatric oncology unit as a developing country profile to define the problem. A retrospective chart review of 91 patients who presented to the unit between May 1996 and December 2003 was conducted in this study. Patients with unilateral disease presented at a median age of 24 months and those with bilateral disease at a median age of 9.5 months (p < .01). Most of the eyes with retinoblastoma (68.6%) had Reese-Ellsworth stage V disease. Metastatic disease was diagnosed in 19 (20.9%) patients. Cases with metastatic disease presented at a median age of 24 months and those without metastatic disease at a median age of 12.5 months (p < .05). In 31 patients (34.1%) there was a delay in diagnosis. The enucleation ratio in eyes with advanced intraocular stage was significantly higher than in eyes with early intraocular stage (57.9 vs. 3.8%) (p < .001). In patients with metastatic disease, tumor recurrence was more frequent than in the nonmetastatic patients (36.8 vs. 4.2%) (p < .01). Seven children (7.7%) died due to central nervous system (CNS) metastasis (p < .01). Advanced intraocular disease and distant metastases occur more frequently in Turkish children with retinoblastoma than in children in developed countries, causing a higher rate of enucleation and mortality. Late referral might account for the delayed diagnosis.

h A very unusual clinical presentation of Hodgkin disease with immune thrombocytopeni a and autoimmune hemolytic anemia is reported. A 6.5-year-old boy presented with thrombocytopenia, Coombs' positive hemolytic anemia, and multiple small posterior cervical lymph nodes. After a course of high-dose methylprednisolon e therapy with a diagnosis of Evans syndrome, complete response for thrombocytopenia and partial response for anemia was achieved. Six weeks later there was a sudden increase in the size of left posterior cervical lymph nodes and a biopsy was compatible with Hodgkin disease, mixed cellularity type. The child was successfully treated with chemotherapy and radiation therapy. He has been off therapy for 28 months and has no clinical or laborator y evidence of autoimmune cytopenia. A combination of immune thrombocytopenia and autoimmune hemolytic anemia may be associated with Hodgkin disease. The recognition of this clinical picture as a complication of Hodgkin disease has important implications. This complication appeares to be managed best by the de® nitive treatment of Hodgkin disease.