[Effective infliximab treatment for a recurrent type of acute intestinal graft-versus-host disease accompanied by steroid-induced depression].

A 22-year-old man with chronic active Epstein-Barr virus infection underwent allogeneic bone marrow transplantation (allo-BMT) from an HLA two allele-mismatched unrelated donor. Ten months after allo-BMT, he developed protein-losing enteropathy following a respiratory syncytial virus infection. A diagnosis of a recurrent type of acute graft-versus-host disease (GVHD) was made based on the histopathological findings, such as the infiltration of T lymphocytes into the superficial epithelium and crypts, and apoptotic bodies in crypts.

Quantitative RT-PCR analysis of the MOZ-CBP fusion transcript in therapy-related acute myeloid leukemia with t(8;16)(p11;p13).

We developed a real time reverse transcriptase polymerase chain reaction (RT-PCR) assay system for detecting the MOZ-CBP fusion transcript and used it to monitor minimal residual disease (MRD) status in a patient with therapy related acute myeloid leukemia (t-AML) harboring t(8;16)(p11;p13). Expression of the MOZ-CBP fusion transcript was determined by RT-PCR analysis of the patient's bone marrow at the time of diagnosis. Thereafter, real time RT-PCR was used to evaluate MRD levels throughout the entire course of treatment.

Complications of Evans' syndrome in an infant with hereditary spherocytosis: a case report.

Hereditary spherocytosis (HS) is a genetic disorder of the red blood cell membrane clinically characterized by anemia, jaundice and splenomegaly. Evans' syndrome is a clinical syndrome characterized by autoimmune hemolytic anemia (AIHA) accompanied by immune thrombocytopenic purpura (ITP). It results from a malfunction of the immune system that produces multiple autoantibodies targeting at least red blood cells and platelets.

Evaluation of angiopoietins and cell-derived microparticles after stem cell transplantation.

Although stem cell transplantation (SCT) is being used for hematopoietic reconstitution following high-dose chemotherapy for malignancy, it involves certain serious transplant-related complications such as graft-versus-host disease (GVHD). Angiopoietins play important roles in angiogenesis. However, the role of angiopoietins after SCT is poorly understood.

Role of soluble tumor necrosis factor-related apoptosis-inducing ligand concentrations after stem cell transplantation.

Although stem cell transplantation (SCT) is being used for hematopoietic reconstitution following high-dose chemotherapy for malignancy, it involves certain serious transplant-related complications such as graft-versus-host disease (GVHD). Tumor necrosis factor (TNF)-related apoptosis-inducing ligand (TRAIL) plays important roles in regulating cell death, immune response, and inflammation. However, the role of soluble TRAIL (sTRAIL) after SCT is poorly understood.

Outcome of non-T-cell-depleted HLA-haploidentical hematopoietic stem cell transplantation from family donors in children and adolescents.

Non-T-cell-depleted HLA-haploidentical hematopoietic stem cell transplantation (SCT) from family members has been reported, but its effectiveness and safety are not fully known. In this study, we examined the outcomes of 83 children and adolescents with nonmalignant (n = 11) or malignant (n = 72) disorders who underwent SCT mismatched at 2 or 3 HLA loci, either from the mother (n = 56), a noninherited maternal antigen (NIMA)-mismatched sibling (n = 14), or the father/a noninherited paternal antigen (NIPA)-mismatched sibling (n = 13). Engraftment was satisfactory.

A randomized prospective study on the use of 2 g-IVIG or 1 g-IVIG as therapy for Kawasaki disease.

A single, 2 g/kg dose of immune globulin (IG), denoted 2 g-intravenous (IV)IG, has become a standard regimen for treating Kawasaki disease (KD) because of its highly preventive effect on coronary arterial lesions (CAL). However, IG is obtained from blood specimens, a drawback to many patients, and is also very expensive. This randomized prospective study reported here was carried out with the aim of developing a treatment regimen that would reduce the total dose of IG.

Role of platelet-derived chemokines (RANTES and ENA-78) after stem cell transplantation.

Stem cell transplantation (SCT) is being used for hematopoietic reconstitution following high-dose chemotherapy for malignancy. Some patients seem to have an imbalance of the immune response after SCT and cytokines are known to regulate this response. Recently, platelets have been shown to contain members of the chemokine family, suggesting a role of platelets as inflammatory cells.

[A woman complicated with immune thrombocytopenic purpura, subclinical Graves disease and peripheral neuropathy 5 years after allogeneic bone marrow transplantation].

A 21-year-old woman developed immune thrombocytopenia (ITP), subclinical Graves disease and peripheral neuropathy without typical chronic graft-versus-host disease (GVHD) 5 years following an allogeneic bone marrow transplantation from an HLA-identical sibling. She received high-dose intravenous immunoglobulin (IVIG) and prednisolone (PSL), which resulted in transient recovery of platelet numbers and muscle weakness. A combination of cyclosporine and PSL induced a durable response against not only the thrombocytopenia but also her high levels of thyroid stimulating antibody (TSAb), muscle weakness and sensory abnormality.

Successful autologous peripheral blood stem cell transplantation with rituximab administration for pediatric diffuse large B-cell lymphoma.

A 13-year-old boy with chemotherapy-resistant diffuse large B-cell lymphoma (DLBCL) was successfully treated with autologous peripheral blood stem cell transplantation (auto-PBSCT) with administration of rituximab. Previous reports indicate that auto-PBSCT without rituximab for adult chemotherapy-resistant DLBCL is only marginally successful. The addition of rituximab administration might have intensified anti-tumor activity before the transplant procedure and might have enhanced the in vivo purging of the auto-graft, resulting in a successful outcome in this case.

Prognostic value of early response to treatment combined with conventional risk factors in pediatric acute lymphoblastic leukemia.

To determine useful prognostic factors in treating childhood acute lymphoblastic leukemia (ALL), we correlated conventional risk factors and bone marrow response 14 days after induction chemotherapy. Our study included 116 precursor B-cell (n = 104) and T-cell (n = 12) ALL patients treated with our protocol between 1988 and 1999. The patients were classified into 3 initial risk groups on the basis of conventional risk factors (56 in the low-risk, 33 in the high-risk, and 27 in the very high-risk groups).

First description of somatic mosaicism in MYH9 disorders.

MYH9 disorders are characterized by giant platelets, thrombocytopenia, and Dohle body-like cytoplasmic granulocyte inclusion bodies that result from mutations in MYH9, which encodes non-muscle myosin heavy chain-A (NMMHCA). These disorders are known to be transmitted in an autosomal dominant manner, although about 20% of cases are considered to be sporadic. We report here the first case of a MYH9 disorder because of somatic mosaicism.

Invasive adenoviral infection in a recipient of unrelated bone marrow transplantation: Problems with diagnostic PCR.

A hematopoietic stem cell transplantation (SCT) recipient developed severe diarrhea and fever. A rapid test for the presence of adenovirus (AdV)-specific antigen in the patient's stools was positive; however, AdV genome was not detected by conventional or real-time polymerase chain reaction (PCR). AdV was confirmed by specific PCR for AdV serotype 7 and by an AdV hexon/fiber gene DNA sequence homology search of the PCR product.

Granulocytic sarcoma presenting with severe adenopathy (cervical lymph nodes, tonsils, and adenoids) in a child with juvenile myelomonocytic leukemia and successful treatment with allogeneic bone marrow transplantation.

The occurrence of adenopathy in patients with myelodysplastic syndrome-associated extramedullary myeloid cell tumors has rarely been reported. We describe a 7-year-old girl with juvenile myelomonocytic leukemia who showed the novel chromosomal abnormality t(9;12)(p22;q24.1) and who developed severe adenopathy of the cervical lymph nodes, tonsils, and adenoids that was manifested as granulocytic sarcoma.

[Granulocyte transfusions for severe infections prior to allogeneic hematopoietic stem cell transplantation].

The efficacy and safety of granulocyte transfusions were evaluated in two acute lymphoblastic leukemia patients for the control of severe infections (cervical cellulitis, sepsis) prior to hematopoietic stem cell transplantation. One patient received 6 transfusions and the other 2 transfusions. The donors were given subcutaneous granulocyte-colony stimulating factor plus oral dexamethasone/betamethasone 12 hours before the scheduled collection.

Feasibility of HLA-haploidentical hematopoietic stem cell transplantation between noninherited maternal antigen (NIMA)-mismatched family members linked with long-term fetomaternal microchimerism.

Based on the hypothesis that long-term fetomaternal microchimerism is associated with acquired immunologic hyporesponsiveness to noninherited maternal antigens (NIMAs) or inherited paternal antigens (IPAs), several groups have recently reported successful cases of non-T-cell-depleted hematopoietic stem cell transplantation (SCT) from HLA-haploidentical family members mismatched for NIMAs. In this study, we examined the outcomes of 35 patients with advanced hematologic malignancies who underwent HLA-2-antigen- or HLA-3-antigen-incompatible SCT from a microchimeric NIMA-mismatched donor. After standard-intensity or reduced-intensity preparative regimens, all patients had sustained hematopoietic recovery with tacrolimus-based graft-versus-host disease (GVHD) prophylaxis.

Secondary acute promyelocytic leukemia following chemotherapy for non-Hodgkin's lymphoma in a child.

Of the several kinds of therapy-related leukemia, therapy-related acute promyelocytic leukemia (t-APL) is most closely associated with topoisomerase II inhibitor administration for treatment of malignancies in adults. Although rare in children, the majority of therapy-related malignancies have been etoposide-related APL associated with Langerhans cell histiocytosis. The authors describe the development of t-APL after chemotherapy administered for non-Hodgkin's lymphoma (NHL) in an 8-year-old girl.

Early blastic transformation following complete cytogenetic response in a pediatric chronic myeloid leukemia patient treated with imatinib mesylate.

This article reports early blastic transformation of chronic myeloid leukemia (CML) in a child following a complete cytogenetic response induced by imatinib mesylate. A 14-year-old Japanese boy was diagnosed with t(9;22) cryptic CML in the chronic phase and treated with imatinib. His response to treatment was slow, but a major cytogenetic response was obtained at 142 days of therapy.