The plasma and urinary levels of thrombomodulin (TM), interleukin-1β (IL-1β) and tumor necrosis factor-α (TNF-α) were measured in 24 patients with Henoch-Schönlein purpura (H-SP); 11 patients without nephritis and 13 with nephritis. The plasma and urinary levels of TM increased during the acute phase of H-SP (≤2 weeks after disease onset). In patients without nephritis in the late phase (>2 weeks), the levels decreased significantly as compared to the levels in the acute phase. In patients with nephritis, whose hematuria measured 10/HPF or more, the plasma and urinary TM levels remained significantly elevated in the late phase. The plasma and urinary IL-1β levels did not show significant changes. The plasma TNF-a levels showed changes similar to those of TM, suggesting a specific relation between TM and TNF-cr. From these results, it is suggested that systemic or renal vascular endothelial cell damage continues for more than two weeks after disease onset in patients with nephritis.

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In all neutropenic patients with hematologic disease, selective decontamination of the digestive tract was instituted by oral administration of a nonabsorbable antimicrobial drug, polymyxin B (PLB), to decrease the gram-negative rods. During a 12 year period, from 1983 to 1994, the Department of Pediatrics, Chiba University School of Medicine diagnosed 32 episodes (23%) of a-streptococcal (α-Str) sepsis out of 142 positive blood cultures in children with hematologic disease, indicating that α-Str was one of the most frequent pathogens of sepsis in that period. All 32 episodes occurred at neutrophil counts of less than 100/μ1. Streptococcus mitis was the predominant type isolated from the blood cultures. Three patients died of intracranial hemorrhaging and one of septic shock. The other 28 cases, including six patients who developed interstitial pneumonia, were cured. The careful selection of antimicrobial drugs is important since some of the isolated strains showed a high minimal inhibitory concentration (MIC) against penicillins and first-generation cephems. Empiric antimicrobial therapy for febrile and neutropenic patients with hematological diseases should include antibiotics effective against α-Str.

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Philadelphia chromosome-positive acute lymphoblastic leukemia (Ph1-ALL) is recognized as a chemotherapy-resistant hematological malignancy in children. We report eight cases of patients with Phi-ALL who underwent bone marrow transplantation (BMT) in a single institution. The patients included four boys and four girls ranging in age 3-15 years. Transplants were conducted from July 1986 to May 1994. Transplant types included one case of syngeneic transplant, two cases of autologous transplant and five cases of allogeneic transplant. The donors included HLA identical siblings in three cases, an HLA-B locus mismatched mother and a matched unrelated donor. The transplants were conducted at first remission (four cases), second remission (three cases) and relapse (one case). Engraftment was obtained in seven cases, and second transplants were done from the same donor for the single relapse patient and another patient in which rejection was observed. In the four cases of allogeneic transplants with engraftment, two cases of grade II-III acute graft versus host disease (GVHD) were observed. Three of the patients were alive and in remission 35-79 months after transplantation and five patients died of either relapse (two cases) or veno-occlusive disease (VOD) (three cases) 0-19 months after transplantation.

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A 10-year-old girl was referred to our hospital because of persistent cough, dysphagia of one to two weeks duration and a large anterior mediastinal mass which was detected by a chest roentgenogram. Within six hours after admission, her respiratory condition deteriorated rapidly and she was diagnosed as having superior mediastinal syndrome (SMS), which necessitated immediate tracheal intubation. Then, a needle biopsy of the mediastinal mass was done under mechanical ventilation. She was treated with prednisolone (PSL) and radiation therapy on the same day of the biopsy. After obtaining the diagnosis of non-Hodgkin's lymphoma (NHL), induction chemotherapy using vincristine, doxorubicin, cyclophosphamide, and PSL was performed, and she was successfully induced to complete remission. Mediastinal NHL during childhood, as in this case, may be presented as SMS with rapidly aggravating dyspnea. One should be fully aware of this mediastinal NHL complication and treat it as an oncologic emergency.

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A 5-year-old boy suffering from lymphoma leukemia syndrome complained of remittent fever for eightdays during first complete remission. Laboratory tests were conducted and the data revealed pancytopenia, abnormal liver function and an elevated level of ferritin. His bone marrow was hypoplastic and hemophagocytosis was remarkable. Cytomegalovirus (CMV) DNA was positive with the PCR method, and the IgM and IgG of CMV turned positive in his blood sample. He was diagnosed as having virus associated hemophagocytic syndrome (VAHS) caused by the initial infection of CMV, and successfully treated with VP-16. After an interval of 10 months, he complained of remittent fever and pancytopenia again. Laboratory test data and a bone marrow examination suggested that the recurrence of VAHS was caused by the reactivation of CMV. An initial treatment with VP-16 was ineffective, however, a combination therapy with G-CSF, γ-globulin and ganciclovir was effective. This study reports a rare case of recurrent VAHS caused by CMV.

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We report a case of acute myelomonocytic leukemia (AMMoL) with a cerebral infarction. A 3-year-old girl was admitted to our hospital because of prolonged fever and gait disturbance. An examination of her peripheral blood showed remarkable leukocytosis, anemia, thrombocytopenia and disseminated intravascular coagulation (DIC). A bone marrow specimen revealed infiltration with myelomonoblasts, and a computed tomography of her brain showed low density in the right frontal lobe. These results led to a diagnosis of AMMoL (M4) with a cerebral infarction. The patient was initially treated with several exchange transfusions and anticoagulant therapy, followed by antileukemic drugs. After improving the leukocytotic and DIC conditions, she was started on treatment according to the Children's Cancer and Leukemia Study Group (CCLSG) ANLL9411 pilot protocol. Remission was induced and she could walk five months after diagnosis. After four courses of chemotherapy, she had a bone marrow relapse and subsequently died due to bacterial meningoencephalitis.

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We report on a 5-year-old girl with Fanconi anemia associated with myelodysplastic syndrome (MDS) who received a bone marrow transplantation from an HLA identical sibling. The preconditioning regimen consisted of low-dose cyclophosphamide (CY) and TBI (500 cGy). She received cyclosporin A (CsA), short term MTX and prednisolone for graft versus host disease (GVHD) prophylaxis. Successful engraftment was confirmed on day 13. The patient had grade III-IV GVHD and severe pancreatitis on day 16. Although FK506 and steroid pulse therapy improved the clinical signs of GVHD to some degree, the pancreatitis was refractory to chemotherapy. She died on day 98 after BMT due to multiple organ failure. The autopsy showed acute pancreatitis with fibrosis and infiltration of the lymphocytes and neutrophils. Thus, the pancreatitis in this patient might have resulted from immune reactions such as acute GVHD.

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A 1-year-8-month-old boy with Down's syndrome developed acute megakaryoblastic leukemia. Chemotherapy consisted of eight courses of cytarabine (Ara-C), daunorubicin hydrochloride (DNR) and etoposide (VP-16). The total dosages of Ara-C, DNR and VP-16 were 5, 600 mg/m², 480 mg/m² and 3, 600 mg/m², respectively. Complete remission was easily obtained. The ejection fraction (EF) remained at the normal level until 15 months after the cessation of chemotherapy. After he suffered from acute tonsillitis, his cardiac functions deteriorated. In spite of treatment with diuretics, an angiotensin-converting enzyme inhibitor, catecholamines and others, he died of cardiac failure, probably due to DNR-induced cardiomyopathy. In order to avoid DNR-induced cardiomyopathy, the total dosage of DNR should possibly be restricted to 350 mg/m² or less.

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A study of an 11-year-old boy with polycythemia is reported. His laboratory tests showed erythrocytosis (RBC>8 × 10⁶/, μl) without any of splenomegaly, leukocytosis or thrombocytosis. The serum vitamin B12 levels were within the normal range. An erythroid colony assay of his bone marrow cells showed normal levels. Serum erythropoietin levels were elevated (33.2-80.6 mU/ml), but the regulation was thought to be normal. These findings did not fulfill the criteria of polycythemia vera by the American Society of Polycythemia Study Group. The patient was diagnosed to have secondary polycythemia of an unknown origin after excluding heart disease or other causes of erythrocytosis. He was treated with six phlebotomies over a period of six months without worsening of the erythrocytosis.

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