Acute lymphocytic leukemia (ALL) in children less than one year of age is characterized by more organomegaly, higher WBCs, an increased incidence of CNS leukemia, a high frequency of chromosome abnormalities involving 11q23, and a poor (30%) event-free survival. Recent molecular studies have suggested mechanisms by which the 11q23 region is targeted. Moreover, gene expression studies suggest that infant ALL is not a homogeneous disease : there may be multiple causes even in infants with similar chromosome abnormalities. Therapeutically, two large multicenter trials (Interfant and Children's Oncology Group) suggest that a cure rate of 50-65% can be achieved using chemotherapy regimens that contain high dose methotrexate, etoposide and cyclophosphamide. In addition, new stem cell transplantation regimens have also showed improved outcomes for infants with ALL. Recommendations for treatment are presented based upon these new biologic and clinical findings.

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Gilbert syndrome is a mild type of hereditary unconjugated hyperbilirubinemias, caused by the mutations of the bilirubin UDP-glucuronosyltransferase gene (UGT1A1). Incidence of a severe and moderate form of hereditary unconjugated hyperbilirubinemias, Crigler-Najjar syndrome, is rare. However, Gilbert syndrome is the most frequent metabolic disease and incidence of Gilbert syndrome is 3-7% of the population. Thus 3-7% of patients with blood disease have Gilbert syndrome simultaneously. In the Japanese population, there are two major polymorphisms of UGTIAI which cause Gilbert syndrome : G71R in exon 1 and A (TA) 7TAA of TATA box in the promoter region. Allele frequencies of those polymorphisms are 0.16 and 0.15, respectively. Not only patients with Gilbert syndrome caused by homozygous or compound heterozygous mutations [G71R and A (TA) 7TAA], but also heterozygous carriers of the mutations contribute to modification of the situation of blood diseases. Recently reports were published on the association of Gilbert syndrome and blood disease. Co-occurrence of hereditary spherocytosis and Gilbert syndrome increases the risk of gallstones. When leukemia patients with UGT1A1 mutations receive chemotherapy, transiently unconjugated hyperbilirubinemia develops. This review provides an overview of bilirubin UDP-glucuronosyltransferase and the association between Gilbert syndrome and blood diseases.

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We have performed an allogeneic umbilical cord blood stem cell transplant to an 12-year-old male patient with T-cell Non-Hodgkin's lymphoma (NHL). The NHL was refractory to conventional intensive chemotherapy. High-dose chemotherapy and radiation therapy was given, but the patient relapsed 3 times. He was then treated with allogeneic umbilical cord blood stem cell transplantation (CBSCT) using a fludarabine, and melphalan-based nonmyeloablative conditioning regimen. GVHD prophylaxis was composed of tacrolimus alone. The patient had neutrophil engraftment within 28 days. Investigation by short tandem repeat (STR) showed that 100% of his bone marrow nucleated cells were reconstituted by donor origin since day +28. He is now alive and well and remains disease-free 7 months after transplant. Although the symptom of GVHD is faint, we evaluated that the graft-versus-lymphoma effect is one of the factors contributing to his remission.

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We report a 3-year-old boy without any underlying diseases who was diagnosed as having acquired hypoprothrombinemia lupus anticoagulant (LA) syndrome. He was admitted for evaluating a sudden onset of severe epistaxis and subcutaneous bleeding on buttocks and lower extremities 10 days after gastroenteritis. Platelet counts were normal, but PT and APTT were markedly prolonged (39.0 and 127.3 sec, respectively). PIVKA II was negative. Mixing test normalized PT, but not APTT. Coagulation studies revealed a low Factor II activity (10%) and a high titer of LA (2.19 of dRVVT/RVVT). Bleeding tendency and abnormal coagulation time became normal without any specific medications.

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We present a case of successful bone marrow transplantation (BMT) in a 15-year-old girl with T-cell acute lymphocytic leukemia (T-ALL) after treatment for hemophagocytic syndrome (HPS). The patient presented with progressive fever, pancytopenia and erythema after reinduction therapy. A bone marrow biopsy specimen demonstrated intense hemophagocytosis and no lymphoblasts. Dexamethasone and VP-16 were initiated, and symptoms of HPS resolved. Two weeks later, however, fever and pancytopenia relapsed, followed by disseminated vesicles of the skin. A biopsy specimen taken from the skin and vesicles showed varicella-zoster virus (VZV) infection, even though the patient had a past history of chickenpox at four years of age. HPS improved after antiviral therapy with acyclovir, but recurred with neurological manifestations after cessation of antiviral therapy. Computed tomography and magnetic resonance imaging of the brain indicated multiple lesions in the central nervous system. HPS was well-controlled after initiation of anti-HPS therapy with aciclovir and anti-VZV high titer immunoglobulin. Allogeneic BMT was performed with pretreatment without irradiation to the brain after improvement of neurological symptoms. The patient remains in complete remission 84 months following BMT. Hemophagocytic syndrome is an uncommon condition during chemotherapy and the underlying cause is sometimes difficult to determine. The possibility of VZV infection should be considered in immunosuppressed HPS patients, even in those lacking characteristic skin lesions. This condition is often misdiagnosed because clinicians are unfamiliar with it. Magnetic resonance imaging of the brain appears helpful in diagnosing central nervous involvement with HPS.

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