X-linked severe combined immunodeficiency (XSCID) is characterized by the faulty development of T and NK cells and nonfunctional B cells. XSCID is a disease of pediatric emergency, and rapid diagnosis and treatment are required. Hematopoietic stem cell transplantation is the choice of treatment. When an HLA identical donor is not found, HLA haplotype identical bone marrow transplantation from the parents is widely recommended after T cell depletion by soy bean lectin and E-rosettes. However, immunological reconstitution by HLA haplotype identical bone marrow transplantation is not perfect and often requires immunoglobulin supplement therapy. GVHD, severe viral infection, and autoimmune hemolytic anemia are also reported as frequent complications. Recently Fischer et al reported successful gene therapy for XSCID by using autologous bone marrow CD34 positive cells. γc chain gene transduced T cells were proliferated under strong selective pressure. Polyclonal CD45RA positive T cells were restored with normal blastogenesis by PHA and anti-CD3. Specific antibody responses and the appearance of NK cells were also confirmed. These results are quite promising as a treatment of XSCID. The efficacy and safety issues must be solved quickly by the accumulation of more patients who will undergo XSCID gene therapy.

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A female case of Down syndrome with transient abnormal myelopoiesis (TAM) associated with severe liver fibrosis is reported. Because of hyperviscosity syndrome secondary to leukocytosis at birth, she received mechanical ventilation, exchange transfusion, and chemotherapy. Herblast cells disappeared, and her general conditions get better through this management. However, her bilirubin level was maintained to elevate gradually. An abdominal CT scan revealed an atrophy of the right lobe of the liver and splenomegaly at 123 days of age. She died of hepatic failure at 145 days of age. The autopsy showed severe liver fibrosis without infiltration of blast cells. According to literature we investigated, all but one patient with Down syndrome complicated with TAM and liver fibrosis died within 100 days of age. TAM is generally considered to be a self-limiting disease. The prognosis, however, could be much worse if the syndrome is complicated with liver fibrosis. A further accumulation of data is needed to establish an effective therapy for a patient with TAM complicated with liver fibrosis.

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An 8-year-old boy who had a subaponeurotic hematoma with a significantly low level of factor XIII (F XIII) was experienced. There was no abnormality in platelet counts and in the solidification function screening test. But the F XIII level decreased to 13% on the 6th day after onset (the lowest level was 6%). The hematoma was gradually improved by the frequent intravenous administration of F XIII concentrate. The activity of F XIII stayed below 70% for the first 4 months, but the patient recovered well without hemorrhage after that, and the F XIII level has been normal. We suspected that a significant decrease of the F XIII level is caused by a massive hematoma and a transient appearance of antibody against the F XIII subunits. In cases of prolonged bleeding with normal platelet counts and a normal test result of solidification function screening, a decrease of F XIII activity should be suspected.

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We report here two cases of childhood Sjögren's syndrome (SjS), who were complicated with aseptic meningitis and hemophagocytic syndrome. Sialography and auto-antibody studies (positive SS-A and SS-B) had indicated SjS. An 11-year-old boy was admitted to our hospital for headache and fever. Laboratory findings revealed leukopenia, thrombocytopenia, increased serum concentrations of ferritin and TNF-α, and elevated leukocyte count in cerebrospinal fluid (CSF). Two years after diagnosis he was diagnosed as having aseptic meningitis again. An 8-year-old girl was admitted to our hospital for headache and fever. Laboratory findings revealed leukopenia, thrombocytopenia, increased serum concentrations of ferritin and transaminase, and elevated leukocyte (containing of macrophage) count in CSF. A half year after diagnosis she was suffering from headaches again. We report these cases because aseptic meningitis and hemophagocytic syndrome are uncommon complications in childhood SjS.

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We report a 9-year-old-boy with Burkitt's lymphoma suffering from disseminated varicella in the remission induction phase. He complained of severe back pain in the fourth week of the remission induction phase. X-ray and CT showed no abnormality. Vesicular skin rash followed a few hours later, leading to a diagnosis of varicella, and acyclovir treatment was started. Morphine chloride was administered to relieve the severe back pain. The liver dysfunction was rapidly progressive, and the exanthema became hemorrhagic in some areas and spread throughout the body. On the 10th day, the symptoms improved. Varicella-zoster virus DNA was detected in the vesicle specimen. The varicella-zoster virus antibody titer rose soon afterward. Disseminated varicella should be considered when a patient on chemotherapy complains of severe abdominal or back pain of unknown origin, even if a skin rash is absent, and empirical administration of acyclovir is mandatory.

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