By use of prophylactic central nervous system (CNS) therapy in acute childhood leukemia, the incidence of CNS relapse was dramatically reduced and the survival rate was significanly increased. But 10∼20% of patients still show CNS relapse and one-third of children with ALL still have hematological relapse.
While major advances have been made in the treatment of childhood leukemia, therapy related complications such as immunosuppression, neurotoxicity, endocrine dysfunction and secondary cancer are significantly. One of the most worrisome complications is the neurotoxicity which is related to both CNS prophylaxis and systemic chemotherapy.
The clinical manifestations of neurotoxicity may be abnormal IQ EEG and CT fiudings especially below the age of 5 years. Another important complication is the pituitary dysfunction. About 90% of the patients show low level of urinally growth hormone secretion despite of normal body height development.
New approaches to CNS therapy in children with ALL may be necessarry for decrease the serious complications.

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The unusual manifestations of central nervous system involvement in leukemia are remarkable obesity and diabetes insipidus. 26 cases of hypothalamic syndrome or Cushing syndrome and 4 cases of diabetes insipidus from 26 hospitals were analysed. As for hypothalamic syndrome, the degrees of obesity ranged from +30 to +82%. Leukemic cells in cerebrospinal fluid were demonstrated in 88%. Hormonal study did not show any specific findings in hypothalamic syndrome. The frequency of this syndrome in central nervous system leukemia was estimated between 10 to 20%.
One of 4 cases with diabetes insipidus revealed leukemic cells in cerebrospinal fluid. Autopsy findings indicated that the diabetes insipidus was related to lesions in the supraoptico-hypophyseal system. The frequency of diabetes insipidus in leukemia seems to be around 0.05%.

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Complete remission was achieved and clinical course could be followed in seventy-three adult cases with overt acute leukemia during January 1972 through December 1986.
Incidence of the central nervous system leukemia (CNS-L) was 15.3% in acute nonlymphocytic leukemia (ANLL, 9/59) and 28.6% in acute lymphoblastic leukemia (ALL, 4/14).
Before 1979 no CNS prophylaxis was done and CNS-L occurred in 6 out of 26 cases of ANLL and 2 out of 2 cases of ALL; after intermittent intrathecal prophylaxis with MTX CNS-L occurred in only 1 out of 31 cases of ANLL and 2 out of 12 cases of ALL.
Among the cases with ANLL in whom no prophylaxis was done many high-risk ones, i. e., marked leukocytosis above 50,000/μl, were involved.
The treatment of CNS-L was begun with intrathecal MTX and, if its effects were insufficient, cranial irradiation was added. Complete recovery was seen in 3 out of 11 cases, and in two cases residual nervous symptoms were observed without any relapse; in remaining 6 patients symptoms relieved only partially or relapse occurred.
In the cases with remarkable leukocytosis above 50,000/μl at the first visit, more frequent injections of MTX with other antileukemic drugs would be necessary to avoid CNS-L.

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We analysed 234 adult patients with acute leukemia regarding CNS-leukemia. Thirteen (7.2%) of 180 patients with ANLL and twenty-one (38.9%) of 54 patients with ALL developed CNS-leukemia during the course of their illness. CNS-leukemia in ANLL occured mostly at diagnosis of leukemia or early in remission. On the other hand, CNS-leukemia in ALL was more likely to occur during the first complete remission. In 43 patients with ALL who achieved complete remission, one prophylactic intrathecal injection of MTX and/or Ara-C decreased the rate of CNS-relapse. Two or more prophylactic administrations resulted not only in the decrease of CNS-relapse rate, but also in the decrease of bone marrow-relapse rate. The probability of survival was not different between the two groups, patients who developed CNS-leukemia and patients who did not. No one in the group of patients who developed CNS-leukemia survived more than five years. Leucoencephalopathy also occured in two of these patients. Data from International Bone Marrow Transplant Registry show that CNS-involvement is not a risk factor associated with the five-year actuarial probability of disease-free survival or relapse following HLA-identical bone marrow transplants. The incidence of CNS-leukemia occured in an early period of ANLL was slightly higher (6.3%) in patients treated with BHAC-DMP than in patients treated with DCMP (2.9%), but not statistically significant.

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As a method of preventive CNS therapy, simple whole skull irradiation including retro-orbital spaces but not the first two cervical vertebrae, or Pinkel's radiation method has been given to child-patients with acute lymphoblastic leukemia (ALL) after remission by chemotherapy in Japan. The ratio of initial CNS-relapse was 13.4% at 1985 in 1,236 of ALL who had been treated from 1966 to 1979. However, those of ALL in our hospital was 39% and 86% of these relapses occured within 2 years.
As we retrospectively examined the radiation ports of these patients, we found that many small parts of the brain were out side of radiation port for prevention of CNS-leukemia. These results forced us to study and to change the cranial radiation port, and we found that Pinkel's method does not include the anterior part of cribriform plate in which cerebrospinal fluid circulate. So we thought that the actual relapse ratio by his method was reported about 10% in child patients.
We tried to modify Pinkel's radiation port of cranial irradiation to include the anterior part of lamina cribrosa, all child-patients with ALL have been given CNS therapy with our new modified cranial irradiation since March 1982 and all of 48 patients, 37 of them were followed for more than 2 years, were followed with none having CNS-relapse to date.
We widened the radiation port untill the upper edge of lens at anterior part of orbit to include the anterior part of cribriform plate and we thought the radiation exposure doses might increase even if we used X-ray of Linear Accelerator without penumbra that γ-ray of Co-60 is accompanied by. In measuring the exposures of radiation in lens by using TLD-tips, those of lens were not more than those of Pinkel's radiation port with γ-ray of Co-60. Therefore we thought that it is possible to widen the radiation port without an increase of lens exposures with high energy X-ray of Linear Accelerator as a preventive therapy of CNS-leukemia.
Based on these results, we discussed whether cranial radiation doses may be reduced without an occurrence of CNS-relapses. And we thought that the radiation doses to cranial region could be reduced to 1200 cGy or at least to 1400∼1600 cGy. We think it is very important to try to reduce the radiation dosage to brain to avoid an occurrence of several side effects hampering development of brain growth in child patients receiving preventive CNS-therapy with radiation, since a long survival period can be expected with our new modified radiation port along with the development of chemotherapy.

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Risk factors for developing central nervous system leukemia (CNSL) were investigated in 21 patients who developed CNSL during complete remission. 20 out of 21 patients developed CNSL within 18 months after complete remission was achieved. The frequency of CNSL was predominant in males. The incidence of CNSL was significantly higher in acute lymphoblastic leukemia (ALL) (34.4%, 12/34) than those in acute myelogenous leukemia (AML) (10.5%, 6/57) and acute promyelocytic leukemia (APL) (10.0%, 1/10). The most frequent complaints or signs at the onset were fever and lymphadenopathy in ALL and headache in AML respectively. Visual disturbance, complained of at onset, was cured during the induction therapy but recurred again during hematological complete remission. Two or more the following four criteria were significantly higher in patients with CNSL: 1) peripheral leukocyte counts at onset. 2) nucleated cell counts in the bone marrow at onset. 3) percentage of leukemia cells in the bone marrow at onset. 4) pressure of cerebrospinal fluid performed when complete remission was achieved. Out of 20 patients who had the prophylaxis with intermittent intrathecal methotrexate and prednisolone, only one patient developed in CNSL. It suggests that adequate continuously prophylaxis is effective in preventing CNSL in adults with acute leukemia.

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Fourteen children with idiopathic thrombocytopenic purpura (ITP), including eight acute, four chronic, and two recurrent cases, were treated with pulse methylprednisolone therapy (30mg/kg/day×3 days). Pulse therapy was needed 1∼4 courses case by case.
Positive responses were observed in all patients. Five (63%) of 8 cases with acute ITP, one (25%) of 4 cases with chronic ITP, and all (100%) of 2 cases with recurrent ITP showed excellent effect to pulse therapy. Other 6 cases showed good effect, and none of patient showed no effect. Moreover, severe side effects with pulse therapy were not observed.
2 cases, who had been treated with high dose gammaglobulin previously and had responded good effect, responded excelent to pulse therapy. We suspected the difference of mechanism between high dose gammaglobulin therapy and pulse therapy.
As to the mechanism of intravenous administration of methylprednisolone on immune process, lymphocyte functions such as T lymphocyte function, immunoglobulin synthesis and natural killer cell activity were inhibited.
Pulse therapy is worth considering treatment to ITP children, who are unresponsible to the standard prednisolone therapy or high dose gammaglobulin therapy, or who need rapid increase of platelets for the risk of severe hemorrhage or as a preoperative precedure.

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Of 137 patients with symptomatic myeloma who received various regimens of chemotherapy for at least 3 months, twenty-four (17.5%) survived over five years after treatment, and three (2.2%) over ten years. No patients with Bence-Jones or IgD myeloma survived longer than five years. Twenty-four among 93 patients with IgG or IgA myeloma, whose treatment were started between Jan. 1968 and Jun. 1981, had significantly longer survival (more than five years) and belonged to stage II, were of the diffuse-proliferation (osteoporosis) type and belonged to a “good control” group defined according to the new criteria of M-protein and albumin levels. Moreover, the pretreatment values of albumin (≥3.5g/dl), hemoglobin (≥10g/dl) and bone marrow plasma cell (<20%) were significantly related to long survival. Conversely, patients surviving for shorter periods significantly belonged to stage III or were of a mixed type of diffuse-proliferation and multiple focus. The five-year survival rate of 114 patients with IgG or IgA myeloma who were registered between Jan. 1968 and Jun. 1983 was 26.6%. Among prognostic factors associated with the markedly high rate of five-year survival, it was demonstrated that the stage II, diffuse-proliferation and “good control” designations were significantly important.

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We studied for an inhibitory effect of deferoxamine (DFX) on inhibition of cell proliferation and DNA synthesis in 5 lymphoid cell lines (3 cell lines derived from acute lymphoblastic leukemia, 2 cell lines derived from adult T-cell leukemia) in our institute.
DFX had inhibitory effect on cell proliferation in all 5 cell lines and on DNA synthesis in one cell line studied. Culturing these cell lines after addition of FeCl₃ and washing out by RPMI-1640 led reversible recovery of inhibited cell proliferation and DNA synthesis. Inhibitory effect on cell proliferation by DFX was different and varied between these cell lines.
DFX was expected to be useful for an anticancer therapy and for study on malignant cells.

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We analysed 293 febrile episodes which were observed in 105 patients with acute leukemia.
Sixty-three percent of febrile episodes was suspected due to septicemia, but no pathogen was detectable. 106 documented infections comprised 26 of septicemia, 47 of pneumonia, 23 of urinary tract infection and 10 of phlegmone.
Pathogens were identified in 53% of the documented infections in life. They were mostly gramnegative bacilli (GNB), mainly E. cloacae and E. coli. The infectious pathogens were mostly fungi (Candida spp. and Aspergillus spp.) in postmortem examination.
Most febrile episodes were observed during severely neutropenic state (<100/mm³).
Oral administration of kanamycin was ineffective on prophylaxis against infection. We consider that the intestinal bacilli are resistant for kanamycin.

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Sequential sera from nine hemophiliacs infected with human immunodeficiency virus (HIV), including three AIDS related complex (ARC), were examined by Western blot analysis and confirmatory EIA for the development and change in anti-HIV antibody during over three years. Reactivity to core protein p18 appeared earliest in one patient. After seroconversion, fluctuations of antibodies to various HIV protein was revealed in five patient. No relationship was found between spectrum of antibodies in infected subjects and disease stage, total lymphocyte counts, CD4 positive cell number, CD8 positive cell number, and immunoglobulin value. However, two of three ARC patients lost antibodies to gag protein p18 or p24.

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Morphology, tartarate-resistant acid phosphatase (TRACP) activity and immuno-phenotypes of 6 cases of B-chronic lymphocytic leukemia (CLL) were studied before and after treatment with phorbol ester (TPA) or B cell growth factor (BCGF). Many of the 6 cases expressed some of the immunophenotypes of TRACP⁺, sIg⁺, B2^- and Leu M5⁺ which are characteristic for hairy cell leukemia (HCL).
After TPA-treatment, leukemic cells had a characteristic morphology of fibroblasts with high TRACP activity as reported in HCL. Each of sIg⁺, Leu 1⁺, Leu 4⁺, Leu M5⁺ or IL-2R⁺ cells increased after TPA-treatment. After BCGF-treatment, leukemic cells transformed to large cells with basophilic cytoplasms. Also the increment of each of sIg⁺, Leu 4⁺ or My7⁺ cells was observed in 3 cases.
These findings may draw these four conclusions; 1) Japanese CLL has the phenotypes resembled HCL with TRACP⁺, sIg⁺, B2^- and Leu M5⁺, 2) this resemblance to HCL becomes more obvious by TPA-treatment, 3) the expression of Leu 1 by TPA-treatment is thought to be a useful tool for the definit diagnosis of CLL, and 4) a functional BCGF receptor is expressed on leukemic cells of CLL.

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Fourty-two pretreated and untreated adult patients with acute leukemia underwent the combined remission induction chemotherapy (B-triple V) consisted of behenoyl-ara C (BH-AC), etoposide (VP-16), vincristine and vinblastine.
Of 34 patients with AML, an overall response rate of 73.5% was achieved with 67.6% complete remission (CR) and 5.9% partial remission (PR). CR was obtained in 9 out of 9 previously untreated patients and in 14 out of 25 pretreated patients.
A high CR rate was seen in M2 (81.8%) and M5 (80.0%) according to the FAB classification.
While there were 2 PR achieved among 5 patients with ALL, no response was noted in 2 patients with CML in blastic crisis and one with myelofibrosis in leukemic transformation.
Frequent side effects included gastrointestinal disturbance, alopecia and liver dysfunction although they were well tolerated.
In conclusion, B-triple V therapy should be considered one of the valuable regimens for remission induction or intensification treatment for AML.

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A marked decrease in plasma fibrinogen content, an increase in FDP in serum, and prolongation of prothrombin time, frequently seen in patients with acute promyelocytic leukemia (APL), have been attributed to disseminated intravascular coagulation (DIC). However, we have observed that the levels of antithromlin III and protein C in plasma are not decreased in patients with APL on diagnosis. On the other hand, marked decrease in the levels of plasminogen and α₂-plasmin inhibitor (α₂-PI) is seen in these cases, which might suggest the possibility of enhanced fibrinolysis rather than DIC.
To elucidate relevance to fibrinolysis in APL, levels of α₂-PI-plasmin complex and α₂PI were immunologically determined in 10 cases of APL and in cases of DIC caused by other diseases. In the both groups, the levels of α₂PI-plasmin complex increased at the time when levels of fibrinogen decreased and concentrations of FDP increased. The levels of α₂PI-plasmin complex were significantly higher in the former group than in the latter. Concentrations of fibrinogen and α₂PI were inversely proportional to those of α₂PI-plasmin complex. There is no evidence to support that fibrinogen in plasma was destroyed by plasmin, because it was not known whether increased FDP in serum was produced by proteolysis of fibrinogen or fibrin. However, it is evident that the fibrinolytic system is activated in patients with APL.
Further sutdy is required to clarify the pathogenesis of coagulopathy in APL.

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We have evaluated the clinical usefulness of discontinous Percoll gradients to isolate stem cells from leukapheresis-derived mononuclear cells in children with malignant disorders. The cells recovered in the interphase of 40 and 60% gradients were depleted of platelets and erythrocytes and contained all of granulocyte-macrophage colony-forming units (CFU-GM) and granulocyte, erythroid, macrophage, megakaryocyte colony-forming units (CFU-GEMM) in less than 20% of the original volume of cell suspension. The enrichment factor was 2.4 for CFU-GM and 3.3 for CFU-GEMM and this procedure allows the reduction of DMSO volume to be infused to patients. This stem cell-enriched fraction was subsequently frozen and stored in liquid nitrogen, without loosing their proliferative ability, for the stem cell-rescue operation following high-dose chemotherapy. We found that this procedure is a valuable measure for the enrichment of stem cell-fraction from leukapheresis-derived cells and can be used on a regular basis.

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Case I was a 72-year-old man who had no symptom. The WBC count was 11,000/mm³ (56% eosinophils). Bone marrow smears showed eosinophilic hyperplasia (39% eosinophils) and increase of plasma cells (4.5%). Ph¹ chromosome was not detected. Serum immunoglobulin levels were: IgG 2, 336mg/dl, IgA 144mg/dl, IgM 101mg/dl and IgE 105U/ml, and immunoelectrophoresis revealed IgG λ monoclonal protein. Vitamin B₁₂ level was in the normal range. Urinalysis revealed the presence of λ light chains in low quantities. The parasite was not detected in the stool.
Case 2 was a 77-year-old female who presented with generalized itching and exanthema. The patient had no past history of allergy or evidence of parasitic disease. Her blood count showed: Hb 12.2g/dl and WBC 6,930/mm³ (21% eosinophils). A bone marrow aspirate was normocellular with 21% eosinophils and 2.2% mature plasma cells. Ph¹ chromosome was not detected. Serum electrophoresis revealed a monoclonal peak, defined as IgG κ type by immunoelectrophoresis. Urinalysis revealed mild proteinuria (κ chains).
Three possible explanations of plasma cell dyscrasia with hypereosinophilia are suggested: 1) hypereosinophilia caused by plasma cell dyscrasia, 2) disturbance of pluripotent stem cell, and 3) a foutuitous association.

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An 84-year-old male with benign monoclonal gammopathy (BMG) of 11 years showed a gradual increase of serum M protein as well as bone marrow plasma cells to the level satisfying the diagnostic criteria for multiple myeloma. Neither bone pain nor lytic bone lesion was noted throughout the course. Previous case reports describing the patients with BMG developing multiple myeloma were remarkable for such an asymptomatic clinical course and minimal or no lytic bone lesion. Patients with BMG seem to evolve into overt myeloma via asymptomatic indolent form of myeloma after a prolonged observation period.

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An 82-year-old man had been given bood transfusions and cimetidine (200mg 4 times daily, intravenously) for his bleeding from gastric ulcer since April 18, 1984. On May 8, pruritic eruptions appeared on his face and neck. All medications were withdrawn and replaced with prednisolone and anti-allergic drugs. While the skin rashes subsided, leukopenia (neutropenia) developed and progressed rapidly. On May 21, his leukocyte count was 100/μl without neutrophil, platelet count 30,000/μl and reticulocyte count 0%. An aspiration biopsy of the bone marrow on May 22 revealed markedly depressed hematopoiesis. He died of suspected GI bleeding on the next day.
Detailed scrutiny of his clinical course pointed strongly to cimetidine as a possible cause of severe aplastic anemia in this patient.

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A 37-year-old female with stomatocytic hereditary elliptocytosis (HE) accompanied by chronic hemolysis who developed severe hemolytic crisis triggered by medicines for a cold is reported. Membrane lipids were normal but a complate absence of membrane protein band 4.2 was revealed. Concerning membrane transport functions, red cell Na⁺ concentration was normal, though Na⁺ influx was found to be about two times normal. By ectacytometory, deformability in intact cells decreased, but that in membrane ghost was normal. In family studies, her father showed mild degree of stomatocytosis and her mother showed ovalocytosis, but both with normal results on membrane transport function, lipid and protein composition, and deformability. No abnormality was detected in her yonger brother and nephew. The abnormalities of red cell shape were probably transmitted from her parents, but a complete absence of membrane protein band 4.2 appeared sporadic. This is the first case of HE with complete deficiency of membrane protein band 4.2.

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A 59-year-old female of adult-onset cyclic neutropenia with 15 day cycle is reported. The peripheral blood picture revealed that the neutropenia recurred every 15 days. The other formed elements of the blood such as monocytes, platelets and reticulocytes also showed periodic fluctuations corresponding to cyclic hematopoiesis. As with the cycle in peripheral blood, myeloid series in the marrow also cycled successively from myeloblasts to segment forms. The concentration of CFU-C decreased prior to the neutropenic episode. The surface marker analysis of the lymphocytes revealed the increase in OKT₈ population at the neutropenic phase and the chemiluminescence of neutrophils and monocytes were accerelated at febrile state.

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A 9-year-old boy was admitted to our hospital because of fever and painful lower expremities, and the diagnosis of deep venous thrombosis was made. In the course, he was found to have a low level of t-PA in the blood. t-PA inhibitor was in normal range. Blood level of t-PA was normal in his family members. He was treated with an intravenous infusion of t-PA, but could not obtain any good results.
We thus supposed that he had a defective capacity to release t-PA from the blood vessel walls, and there was still a problem concerning the infusion methods of t-PA.

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Two cases of acute leukemia with both myeloid and lymphoid phenotypes (AMixL) are reported. Case 1 and 2 were classified into ALL (L1) and ALL (L2) by FAB criteria. However, myeloperoxidase (MPO) was demonstrated by electronmicroscope in 66% of blasts in Case 1 and in 20% of blasts in Case 2, whereas CALLA and TdT were also demonstrated 12.5% and 40.6% of blasts in Case 1, and 51.4% and 89.4% of blasts in Case 2. Case 1 failed to attained a remission following 3 courses of induction therapy for AML, however he achieved complete remission (CR) following 1 course of induction therapy for ALL. CR was also attained in case 2 by 2 caurses of the therapy for ALL. The results of remission induction therapy for AMixL have been reviewed in the literature. Thirty-three of 43 cases were firstly treated with protocol for ALL and 23 cases were in CR. Three of 10 cases treated firstly with protocol for AML were in CR. Seven cases failed to attained a remission were subsequently treated with protocol for ALL and 5 cases achieved CR. Above findings suggest that the protocols for ALL are superior for initial treatment for AMixL.

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We report here a case of malignant lymphoma occurred subsequent to Evans' syndrome. A 42-year-old woman was admitted december 1986 because of right cervical swelling. She had been diagnosed to be suffered from autoimmune hemolytic anemia in 1978 and also developed to Evans syndrome in 1984, and has been treated with dexamethasone plus azathioprin.
The pathological finding of biopsied lymph node indicated that she was suffered from Non-Hodgkin lymphoma classified into small lymphocytic lymphoma with plasmacytoid differentiation according to Working Formulation classification. The immunosteining showed that the proliferating cells in the biopsied lymph node were morphologically postive for cytoplasmic IgG and light-chain kappa. Southern blot analysis showed that they contained the rearranged J_H genes, thus indicating monoclonal B-cell proliferation of them. She was treated with semi-mantle type irradiation and discharged in April 1987.
Furthermore, we reviewed and discussed here on reported cases of malignant lymphoma occurred subsequent to autoimmune disease.

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We serially mesured serum ferritin levels in two patients with malignant histiocytosis (MH) and a patient with virus assosiated hemophagocytic syndrome (VAHS). In all three patients, serum ferritin levels elevated above 10,000ng/ml at the deteriorated phase of the disease just before the treatment. (a range from 19,700 to 39,300ng/ml)
The MH patients received a commbination chemotherapy consisting of adriamycin, vincristine, cyclophosphamide and prednisone (ACOP), and the VAHS patient was given prednisone alone. After the initiation of chemothrapy, serum ferrtin levels gradually returned down to the normal range in one of the two MH and the VAHS patients, who are now alive in remission. The remaining MH patient continuously showed hyperferritinemia ranging 2,000∼3,000ng/ml, in spite of clinical improvement, and relapsed 3 months later, resulting in an unfavorable outcome.
Although our experience is limited to a small number of caces, our results suggest that markedly elevated levels of serum ferritin in MH/VAHS patients may reflect proliferation and activation of monocyte-histiocyte lineage, and that serum ferritin is a useful clinical marker for activity and prognosis of these diseases.

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A 38-year-old woman was admitted to our hospital with purpura, profuse menstruation, and general malaise. The diagnosis of thrombotic thrombocytopenic purpura (TTP) was made because of the presence of microangiopathic hemolytic anemia, thrombocytopenia, neurologic signs, renal dysfunction, fever, and platelet aggregating substance (PAS). Furthermore, an autopsy revealed diffuse hyaline-like thrombi of the kidney.
She was treated with corticosteroid, anti-platelet drugs, plasma exchange (PE), and high-dose gamma globulin (γ-gl) without remarkable clinical improvement. The high level of platelet associated IgG (PAIgG) in this patient let us add vincristine (VCR) to the treatment of PE, by which she achieved remission. PAS disappeared under the infusion of high-dose γ-gl, while still markedly elevated PAIgG declined to the normal range after the treatment with VCR. These results indicate that not only PAS but the process of peripheral platelet consumption, probably inhibited by VCR, might be involved in the pathogenesis of TTP in this case.
Only 18 cases of TTP treated by VCR were reported, but the response rate was 80%, so we suggest that the combination therapy of VCR and PE is effective in patients with refractory TTP.

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