A questionnaire concerning patients with acute leukemia (under 15 years of age) complicated with encephalitis-encephalopathy, was sent to 68 pediatric facilities throughout the country; 54 cases were reported by the first survey. In 54 cases, we made the second detailed survey, and 24 cases (ALL) were confirmed in February 1985. Twenty of 24 patients displayed the syndrome of necrotizing leukoencephalopathy associated with cranial irradiation and the administration of methotrexate.The leukoencephalopathy in these cases developed after the therapy for the meningeal relapse of ALL.The remaining 4 cases were suspected of having encephalitis associated with viral infections. This survey suggested that the intrathecal methotrexate administration including cranial irradiation appeared to be the major factor for the development of leukoencephalopathy. As to neurologic sequelae of the therapy for meningeal leukemia, we should take into consideration possible viral infections of the central nervous system.

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Peripheral blood lymphocytes from 7 cases of histiocytosis X were examined with an electron microscope. Frequent and extensive nuclear pocket formation was observed in 3 cases of disseminated type. Average frequency of this structure was 5.69%while it was 0.55% for 4 cases of localized type. This finding may suggest involvement of the immune system in the disseminated type of histiocytosis X.

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The Bone Marrow Transplantation Committee of the society has conducted an annual registry of bone marrow transplantation in children in Japan since 1983. As of June 30, 1986, 233 patients had been transplanted and registered from 39 institutes. Bone marrow transplantation was performed in 65 cases of acute lymphocytic leukemia (25 alive), 41 cases of acute nonlymphocytic leukemia (24 alive), 9 cases of chronic myelocytic leukemia (8 alive), 16 cases of non-Hodgkin's lymphoma (9 alive), 42 cases of malignant solid tumors (18 alive), 35 cases of aplastic anemia (30 alive), 18 cases of severe combined immune deficiency (7 alive), and 7 other cases (4 alive). The details are reported in this paper.

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The relationship between the rate of platelet MDA production in the early phase of MCLS and the incidence of coronary vascular complications of MCLS was investigated retrospectively. Most MCLS patients who showed increased or decreased values of platelet MDA production stimulated with arachidonic acid before aspirin therapy were later found to have cardiac complications as late sequelae of this disease, but no patients with normal production of platelet MDA suffered from such complications. This fact suggests that the measurement of platelet MDA production in the early phase before administration of aspirin could be one marker to predict the occurrence of coronary complications. The ability of the patients' plasma to regenerate PGI₂ like activity in exhausted umbilical arteries fell within the normal range. This ability was, however, apparently reduced after the start of aspirin therapy. Electronmicroscopic studies on the circulating platelet cohort revealed that the platelets of patients with MCLS in the early phase demonstrated such abnormal changes as degranulation and vacuolization of platelets, supporting the biochemical changes of platelet function.

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Results of the Tohoku Children's Leukemia Study Group Protocol ALL 83 for treatment of 111 children with acute lymphoblastic leukemia according to risk group were reported. Patients at high risk (54 cases) had one or more of the following risk factors : age below two or above nine years, a initial white-cell count of 25, 000/μl or more, and the presence of T- or B-cell immunologic cell surface markers. All the other patients (57 cases) were in the standard-risk group. Patients in both the standard-risk and high-risk groups were treated for three years, receiving intensive multi-drug remission induction therapy, central nervous system prophylaxis, multidrug maintenance, and intensification therapy. At a median follow-up of 19 months, the mean (±SE) continuous complete remission rates at three years among the patients treated with Protocol 83-A, 83-B, and 83-C were 94±3%, 80±12%, and 69±9%, respectively. As compared with the previous Protocol ALL 78, this initial intensive multidrug regimen has resulted in improved disease-free survival in children with acute lymphoblastic leukemia.

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Thirty children between the ages of 2 and 16 years of age with leukemia (six with acute nonlymphocytic leukemia, eight with acute lymphocytic leukemia, and four with chronic myelocytic leukemia), malignant lymphoma (two), neuroblastoma (one), severe aplastic anemia (seven), Wiskott-Aldrich syndrome (one), or Morquio's disease (one) were treated with allogeneic or syngeneic bone marrow transplantation between March 1982 and November 1986. Twenty patients are currently surviving and disease free after one to 57 months, with six surviving beyond three years. Causes of death in 10 patients included graft failure (one), severe acute graft-versus-host disease (two), cytomegalovirus pneumonia (one), pseudomonas septicemia (one), congestive heart failure (two), and relapse of leukemia (three). The quality of life in our long-term survivors remains good except in one patient complicated with leukoencephalopathy, and no serious growth impairment has been noted. Slight or subclinical abnormalities in endocrine and/or respiratory systems have been found in some cases.

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Forty-four children with acute leukemia during induction therapy in private-room isolation were prospectively randomized to receive either total antimicrobial modulation (TAM) regimen (Polymyxin B, Kanamycin and Amphotericin B) or selective antimicrobial modulation (SAM) regimen based on the concept of colonization resistance (Trimethoprim-Sulfamethoxazole and Amphotericin B) for prevention of infection, and compared to historical controls in private-room isolation only. Both TAM group and SAM group had significantly decreased incidence of infection per hospitalization and percentage of days febrile to total days (7.1 % in the TAM group versus 4.0% in the SAM group versus 25.0% in the control group, p<0.005). Pneumonia, upper respiratory tract infection, urinary tract infection, colitis, and otitis media occurred less frequently among recipients of TAM and SAM. No Pseudomonas aeruginosa, Escherichia coli, Klebsiella and Proteus species were found in TAM group and SAM group. Infectious deaths were reduced in TAM group and SAM group. The TAM group and SAM group also had higher complete remission rates. However, there were no significant differences between the two treatment groups in the effect of gut decontamination on prevention of infection. SAM group regimen is cheaper and easier to take than TAM group regimen, and it is therefore preferable to TAM group regimen for the prophylaxis of infection.

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To investigate the clinical significance of serum ferritin levels in acute leukemia children, serial measurements of serum ferritin in each case and ferritin in tissue-cultured leukemic cell lines were done. The results are summarized as follows. 1) Significant difference was found in serum ferritin levels between leukemia children in remission and in relapse. But both those levels were still higher than normal control. 2) Abnormal transaminase (GOT) and serum iron strongly influenced serum ferritin levels, so only groups with normal GOT and serum iron showed significance between relapse and remission compared with normal control. 3) Serum ferritins of three Actute Lymphocytic Leukemia (ALL) patients were measured serially in the whole clinical course. One case who always retained normal level had never relapsed and is now off therapy. On the other hand, the other two cases who showed fluctuating and higher serum ferritin levels had a relapse and died. 4) Ferritins of tissue-cultured Acute Myelocytic Leukemia (AML), Acute Monocytic Leukemia (AMoL) erythroleukemic cells showed much a higher level than normal lymphocytes on the other hand, those in ALL cells showed lower levels. From these results, we concluded that measurement of serum ferritin in ALL children is not significant for either early diagnosis or accurately determining the short-term condition of this disease, but might be effective for predicting long-term prognosis of ALL.

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The proliferation of bone marrow fibroblast colony forming cells (CUF-F) was studied in liquid culture in 3 controls, in 17 patients with acute lymphocytic leukemia (ALL), in 6 patients with aplastic anemia, and in 5 patients with idiopathic thrombocytopenic purpura (ITP). The average number of CFU-F in controls was 94 per 2x10⁶ bone marrow cells. Serial studies were done in ALL. At the diagnosis of ALL, colony formation was significantly suppressed (16). The number of CFU-F increased after chemotherapy (125) and at the stage of complete remission (122), and decreased again at relapse (32). There was a correlation between the number of CFU-F and the percentage of bone marrow myeloid cells. An inverse correlation between the number of CFU-F and the percentage of blasts in the bone marrow was also found. In aplastic anemia, colonies varied widely in numbers, ranging from 37 to 235 (mean 141). The growth rate of CFU-F in ITP was normal or greater than in controls (90-266; mean 135). Bone marrow fibroblasts may be important in the pathogenesis and clinical expression of blood diseases in children.

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A study was carried out during the period from 1984 to 1986 in order to clarify the effects of gabexate mesilate (FOY) on DIC of infancy and childhood. Twenty-seven patients were treated either with FOY (18 cases including 7 newborn infants), heparin (5 cases, all newborn infants) or FOY plus heparin (4 cases including 3 newborn infants) followed by replacement therapy. The others (4 cases) were treated with replacement therapy alone. The clinical findings of DIC, particularly bleeding signs, were improved in 17 patients treated with FOY, out of whom 5 cases eventually died. On the other hand, the bleeding sign was deteriorated in 4 out of 9 patients given heparin with or without FOY, and all 4 patients died of bleeding. No difference was seen in changes of laboratory findings on hemostasis and coagulation between the patient group treated with FOY alone and the other treatment groups. It is suggested that the administration of FOY was more effective than heparin therapy for the DIC of infancy and childhood, especially of neonates, because heparin is less effective due to the far lower level of antithrombin-III and more drastic consumption of coagulation factors and platelets in DIC of infancy, in comparison with DIC in children and adults. We recommend that FOY is to be given by continuous infusion at 1.2-2.0 mg/kg/hr, followed by early replacement therapy for the treatment of DIC of infancy and childhood.

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We tested erythrocyte protoporphyrin (FEP) and its fractions (ZnP and FPP) in the various kinds of anemia in children and discussed the relations between the change of FEP and other hematological parameters. In normal adolescents, the ranges of FEP were from 1.26 to 3.13 and from 1.29 to 3.55 μg/g in boys and girls, respectively. The normal range of FPP% was from 2.12 to 15.28% in both boys and girls. In patients with both severe and moderate adolescent anemia and juvenile rheumatoid arthritis, high values of FEP without any change in the ratio of its fractions were observed, suggesting disturbed iron metabolism. In the cases of hemolytic anemia, acute lymphocytic leukemia in which remission induction therapy was successful, and aplastic anemia in which the hemoglobin concentration was distributed from 8.0 to 11.0 g/dl, the high FEP value with FPP% elevation observed was considered to reflect increased erythropoietic activity. Thus, measuring of FEP and its fractions might be a useful parameter indicating erythropoietic activity.

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Seven patients with acute nonlymphocytic leukemia (ANLL) including two in bone marrow relapse and five in remission, and one patient with chronic myelocytic leukemia in megakaryoblastic crisis were treated with a high dose of cytosine arabinoside (HD Ara-C) or an intermediate dose of AraC (ID Ara-C). The HD Ara-C therapy consisted of 3-hr intravenous infusion of Ara-C in a dose of 3g/m² or 1.5g/m² every 12 hours for 6 days. The ID Ara-C therapy consisted of 2-h intravenous infusion of Ara-C in a dose of 1g/m² or 1-hr in a dose of 0.5 g/m². Two patients in bone marrow relapse treated with HD Ara-C achieved complete remission (CR). In both cases, the duration of CR was 4 months. Five patients in remission treated with HD Ara-C or ID Ara-C have remained in CR with a survival duration of 16 to 50 months. Toxicities of HD Ara-C included vomiting, alopecia, conjunctivitis, and marrow suppression. One patient treated with 3g/m² developed hepatic and splenic microabscesses. Toxicities of ID Ara-C were comparable to conventional treatment. These results indicate that HD Ara-C and ID Ara-C therapy are very useful for children with ANLL.

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We examined serum anti-human immunodeficiency virus (HIV) antibody in 87 hemophiliacs, 86 patients with various diseases who had recieved high-dose immunoglobulin therapy or multiple blood transfusions, and health-care workers. In hemophiliacs, 31.0% of patients are HIV antibody positive by both enzyme-linked immunosorbent assay (ELISA) and Western blot analysis. In others, HIV antibody was positive by ELISA in two infants but negative by subsequent Western blot analysis. All other patients and health-care workers were found to be HIV antibody negative. Our data suggest that HIV infection through transfusion of blood products except for the coagulation factor products is rare in Japan. However, we consider that possibility of transfusion-associated HIV infection still remains, and we must be alert to this unfavorable complication of blood transfusion.

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We have studied the in vitro properties of 5 commercially available preparations of heat-treated F. VIII concentrates : Conco-eight, Koate, Hemofil, Haemate and Confact and compared them with those of corresponding 5 and another (Kryobulin) non-heat-treated concentrates with special reference to factor VIII and von Willebrand factor (F. VIII/vWF) activities. The heat-treated F. VIII concentrates were found to contain about 25 U/ml of F. VIII : C, as indicated on the label, 59.5-103.3 U/ml of F. VIII : Ag, 20.0-74.0 U/ml of RCof and 67.3-96.0 U/ml of vWF : Ag. The F. VIII/ vWF activities in the heat-treated concentrates were almost the same as those in the non-heat-treated concentrates. AHF-cryoprecipitate and a new intermediate F. VIII concentrate (RCG-5) contained 2.3 U/ml and 19.5 U/ml of F. VIII : C, respectively, which was roughly equivalent to the amount of F. VIII : Ag, and 7.3 U/ml and 29.0U/ml of RCof, respectively, which was equivalent to the amount of vWF : Ag. The ratio of F. VIII : Ag to F. VIII : C in the F. VIII concentrates ranged from 2.5 to 4.4. The ratio of vWF : Ag to RCof in the concentrates ranged from 1.1 to 4.8. There were, however, no significant differences in F. VIII/vWF activities between the heat-treated and the non-heat-treated F. VIII concentrates. These findings suggest that the inactivation of biological activities of F.VIII : C and RCof, and/or the denaturation of antigenicity of F. VIII protein and vWF protein occurred during the production procedure, while the heat-treatment did not lead to further changes of F. VIII/ vWF protein. In Haemate and Confact the content of RCof was characteristically high and the ratio of vWF : Ag/RCof was low. In addition, the F. VIII/vWF in the two concentrates consisted not only of small but also of intermediate and some of large multimers, whereas in the other products of F. VIII concentrates only small multimers of F. VIII/vWF could be demonstrated.

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The authors developed a new combination chemotherapy with epipodophyllotoxin, VM-26 or VP-16-213, and cytosine arabinoside (Ara-C) for childhood acute nonlymphocytic leukemia (ANLL). Ara-C was administered twice a day for 6 days at a dosage of 500 mg/m² and epipodophyllotoxin was infused on days 1 and 4 at a dosage of 150 mg/m². Four patients in relapse and 1 patient with initial induction failure were treated with this regimen. Three of 4 patients in relapse had been refractory to conventional therapy. Complete remission was achieved in 4 of 5 patients, all of whom were in relapse. The durations of remission were from 1 to over 17 months. Side effects included nausea and vomiting, fever, exanthema, and bome marrow suppression. However, none of these interrupted the continuation of therapy. This regimen is expected to be useful in the therapeutic strategy for refractory ANLL. We are now planning to make use of this regimen as initial induction therapy.

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Twenty-six children with malignant diseases (acute leukemia, non-Hodgkin's lymphoma, neuroblastoma, hepatoblastoma, yolk sac carcinoma), aplastic anemia, and severe combined immunodeficiency received allogeneic or autologous bone marrow transplantation in Kanagawa Children's Medical Center. Of the patients, 2 children with advanced non-Hodgkin's lymphoma (NHL) were reported in detail. Case 1 was a 4-year-old boy with T cell NHL (Stage IV). The bone marrow (BM) cells were harvested at the time of complete remission, and T cells in the BM were purged by pan T monoclonal antibody (CT-2) plus complement, and then cryopreserved in liquid nitrogen. High-dose chemotherapy and total body irradiation (TBI) 12 Gy were administered, followed by auto BMT. The BM engraftment was achieved on day 46. There were three episodes of viral infections, i.e., interstitial pneumonitis due to cytomegalovirus, chicken pox, and herpes zoster after BMT. He has remained in remission more than 14 months. Case 2 was a boy with NHL of the bones (Stage IV). The unpurged BM cells were cryopreserved because the BM involvement of lymphoma cells was not detected. Myeloablative therapy and TBI were done, followed by auto BMT. The BM engraftment was achieved on day 18, and serious complication did not develop. He is still in unmaintained complete remission after more than 15 months post transplant. This study suggests that auto BMT using ex vivo BM treated. with CT-2 antibody plus complement is safe, and intensive therapy and auto BMT may produce prolonged remission in children with a bad prognosis for NHL.

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High-dose multiagent chemotherapy followed by autologous bone marrow rescue was used in the treatment of 10 patients (6 neuroblastomas, 1 Ewing's sarcoma, 1 Wilms' tumor, 1 rhabdomyosarcoma, 1 malignant sacrococcygeal teratoma) with Stage III or IV childhood tumors. Except for one early death case and a teratoma that obtained complete remission due to other chemotherapy, all of the other cases have relapsed, and the response remained transient. The dose of nucleated marrow cells collected by aspiration varied from 2.4 to 6.8 × 10⁸ /kg, and infused mononuclear cells ranged from 0.5 to 12 × 10⁷ /kg. The patients were kept in a laminar flow unit, and gut decontamination was carried out using Vancomycin, Aminoglycoside, Sulfamethoxazole-Trimethoprim, and Amphotericin B. Marrow reconstitution was obtained in all the patients except a case of neuroblastoma who died on the day of autograft, with recovery of leucocyte counts to > 1.0 × 10⁹ /l between 8 and 36 days and granulocyte recovery > 0.5 × 10⁹ /l between 13 and 52 days. Melphalan showed a tendency for delayed bone-marrow reconstitution. Neither graft-versus-host disease nor interstitial pneumonitis occurred. High-dose chemotherapy combined with autologous marrow grafting is a tolerable treatment method for advanced tumors in children, but further study is required to determine whether the procedure is of sufficient value.

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Recently, nine of 23 cases of peroxidase-negative acute leukemia, of which the cellular phenotype was considered to be Ll or L2 on the basis of FAB criteria, failed to achieve remission or had relapsed by 20 months from diagnosis. Four of 9 patients were diagnosed as unclassified ALL (Ia (+), CALLA (-)), one was common ALL, two were monocytic leukemia, and two were myelocytic leukemia as based on immunological analysis. One of the unclassified ALL and 13 of the other patients have survived in continuous first remission. Two of 4 patients with unclassified ALL were identified as the standard risk group according to age at diagnosis and initial WBC, which are generally accepted as having prognostic importance. The absence of CALLA on leukemic blasts might be an additional prognostic factor to be considered in designing therapy.

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A morphological study was performed on sixty-one cases of acute nonlymphocytic leukemia (ANLL) in childhood by means of FAB classification. Thirty cases (49%) were classified as M2 by six pediatric hematologists. In order to confirm the validity of this classification and the heterogeneity of the M2 group, all differential counts of bone marrow smears were quantitatively analyzed according to the original FAB proposals. The result of the analysis revealed that M2 included a wide variety of cases and there was a high probability of contamination between Ml, M2, M3, and RAEB. A quantitative standard with maturation indices is proposed for more objective interpretation of the FAB classification of these groups.

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Quantitative chemiluminescence (CL) was analyzed with polymorphonuclear leukocytes (PMN) obtained from 24 children with acute febrile diseases such as various kinds of bacterial infection, Kawasaki disease, Henoch-Schönlein purpura and so on. No definite tendency was observed between the degree of CL production and the various disease groups. The degree of CL production of PMN from children with bacterial infection, however, was different according to the maturity of PMN : the more immature PMN, the smaller the observed CL production rate (p <0.01). On the contrary, the more hypersegmented the PMN, the greater the observed CL production rate (p <0.05). Polymorphonuclear leukocytes from children with noninfectious disease, on the other hand, did not show any correlation between CL production rate and maturity of PMN. It is necessary to analyze the maturity of PMN when the PMN CL rate from patients with bacterial infection is assessed.

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A case of common acute lymphocytic leukemia, hardly differentiated from M5 in FAB classification is reported. A 3-year-old girl was referred to our hospital because of fever, anemia, and heart murmur at the end of December, 1985. Hepatosplenomegaly, lymphadenopathy, and pancytopenia (WBC 2, 600/mm³, RBC 1.96 × 10⁶/mm³, P1 54 × 10³/mm³) were observed. May-Giemsa-stained bone marrow smear demonstrated a predominance of large-sized leukemic cells (93.5%) with fine granules in relatively abundant cytoplasm and with coarse nuclear chromatin. Those granules showed positive a.-naphthyl butyrate esterase (ANBE) stain, and this reactivity was not inhibited by NaF. Surface marker studies revealed that 97% of blasts were positive for J5 (CALLA) and 48% for Ia (HLA-DR). The diagnosis was made as common acute lymphocytic leukemia (cALL), L2-type in FAB classification. She received chemotherapy according to the ALL standard risk group regimen of the 11 th protocol of Tokyo Children's Leukemia Study Group (TCLSG) and has achieved complete remission sustained for more than one year.

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We treated an 11-year-old boy with hemophilia B who had recurrent pneumonia due to Haemophilus influenzae and who showed profound immunological abnormalities compatible with AIDS-related complex (ARC). He had been frequently treated with prothrombin complex concentrates since early childhood. At 11 years of age, he started to have recurrent pneumonia due to Haemophilus influenzae and abnormalities of lymphocyte functions. He was admitted to hospital six times over one year due3 to pneumonia with high fever and cough. At the time of these admissions, H. influenzae was always isolated from his sputum. Abnormal immunological findings were as follows : marked decrease of the number of OKT 4-positive cells, OKT 4/8 ratio, Tac-positive cells, and interleukin-2 production, absence of delayed-type hypersensitivity and diminished lymphocyte blastogenesis and immunoglobulin production as stimulated by pokeweed mitogen. Furthermore, a decrease of Leu 11-positive cells and depletion of natural killer cell activity were found. Serum human immunodeficiency virus antibody was positive by ELISA and Western Blot. A careful evaluation of clinical symptoms and immunological studies are necessary in hemophiliacs who receive long-term treatment with commercial coagulation factor concentrates.

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We report here a case of the acute blastic phase, the so-called mixed type, of the chronic myeloid leukemia, in which two types of blast cells-i.e. myeloblast and lymphoblast cells-are morphologically noticed. The patient was a 12-year-old girl, who complained of headache and pyrexia. Morphologically, two types of blast cells-myeloblast and lymphoblast cells-were found in this case, whereas, by the analysis of surface markers, 3-population blast cells were noted which had the markers of myeloblast, lymphoblast, and both of these two at the same time (dual marker). On the other hand, by examining this case on the gene level, the rearrangement of Ig gene was observed in Ig gene analysis using JH probe, while the germ line was missing, with the result that it was the single clone of B-cell type. This is a very important case of CML to understand blastic crisis, and may be another evidence supporting the idea that CML is a complex disease of a pluripotent stem cell.

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A ten-year-old boy with myelodysplastic syndrome was presented. Pancytopenia associated with erythroid and myeloid dysplasia such as a Pelger-Hüet-like anomaly was observed. Giant platelets were also noted. Chromosome analysis revealed an abnormal 45XY, -7 (monosomy 7) pattern. Throughout the clinical course, a remarkable proliferation of monocytoid cells were exhibited. Flow cytometric analysis revealed that these cells were identified in the same cytographic region as that of normal monocytes. However, these proliferative monocytoid cells were negative for naphthyl butylate esterase stain, and were positive for peroxidase and naphthol AS-D chloroacetate esterase stain. In addition, immunologic surface marker analysis using monoclonal antibodies revealed that the monocytoid cells had an OKM1 antigen, but did not have OKM5 or Leu M2 antigens at all which were considered to be the monocyte-specific antigens. In spite of aggressive chemotherapies and supportive care, 32 months after diagnosis the patient died of interstitial pneumonia through a partial transformation to chronic myelomonocytic leukemia. This is the first report in which the OKM1 +/OKM5-monocytoid cells are identified although the precise role of these cells in the childhood preleukemic stage remains to be elucidated.

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A 3-year-old girl complaining of epistaxis, in whom thrombocytopenia cyclically occurred at an interval of 20 to 25 days was reported. No antiplatelet antibody could be detected and the platelet aggregation study was normal. When platelet counts decreased, the percentage of macrothrombocytes increased and the life span of platelets was shortened. Treatment with corticosteroid and γ-globulin had no effect upon the cycle of thrombocytopenia. The cycle of thrombocytopenia disappeared after an episode of upper respiratory infection. Only two cases of cyclic thrombocytopenia in childhood have been reported in the literature that we could refer to.

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A case of acute megakaryoblastic leukemia was reported. The patient, a 2-year-old boy, was admitted to our hospital because of petechiae and mild anemia. The WBC count in peripheral blood increased to 29, 300/μl with 48% blasts. Bone marrow revealed 74.2% blasts. In cytochemical studies, a large number of blasts were peroxydase negative, naphthol AS-D chloroacetate esterase negative, α-naphthyl butyrate esterase weakly positive, and acid phosphatase positive. The monoclonal antiplatelet glycoprotein II b/III a complex and factor VIII were present on a number of the cells. In ultrastructural cytochemical studies, PPO positivity in the nuclear envelope and endoplastic reticulum were seen in more than 70% of fresh blast cells, while we could not detect it in cultured cells after 5 days incubation. A definite diagnosis of acute megakaryoblastic leukemia was made on the basis cytochemical and EM investigations. For establishment of the disease entity, further studies on many cases by cytochemical, immunological, and ultrastructural examinations are necessary. Chromosomal analysis revealed a karyotype of 48, XY, +19, +20. The patient was treated with a combination of vincristine, Adriamycin, prednisolone, and cytosine arabinoside, and a remission was obtained.

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A 10-year-old boy with acute lymphoblastic leukemia developed bilateral visual loss and bitemporal hemianopsia, probably due to optic nerve and left maxillary sinus infiltration by leukemic cells 2 months following the cessation of chemotherapy during complete remission. Fundscopy revealed bilateral disc edema. The swelling of the optic nerve and tumor of the left maxillary sinus were proven by CT-scanning. Pleocytosis was present in the cerebrospinal fluid. He was treated with intrathecal injection of methotrexate, radiation to both orbita, and systemic chemotherapy. His vision improved and the appearance of both fundi returned quickly to normal. He had received late intensification chemotherapy 30 months after the cranio-spinal irradiation. He continues in complete remission 55 months after the relapse without noted late side effects.

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A case of an eight-year-old girl with thrombotic thrombocytopenic purpura (TTP) complicated by systemic lupus erythematosus (SLE) was reported. Definite diagnosis of SLE was made by discoid rash, pericarditis, positive LE cell preparation, and elevated titer of antinuclear and anti-DNA antibody. She was also diagnosed as secondary TTP because of fever, unconsciousness, microangiopathic hemolytic anemia, thrombocytopenia, and renal dysfunction. The elevation of circulating immune complexes (CIC) was shown in this case, and the result suggested that CIC play some role in the pathogenesis of the TTP. Though therapy with antiplatelet agents (aspirin and dipyridamole) proved unsuccessful, corticosteroids and urokinase dramatically reversed the TTP syndrome. The possible relationship between SLE and TTP was discussed.

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