Fourteen patients with polycythemia vera were treated with oral dibromomannitol (DBM), 250 mg or 100 mg daily, for up to 4 years and 1 month. Two of them had been treated with pipobroman before the treatment with DBM.
Complete remission was obtained in all of the 14 cases. The median duration of remission was 28 weeks. DBM proved effective in cases which had been refractory to pipobroman.
Severe but not fatal bone marrow supression was noted in 3 cases and moderate inpairment of liver function in 2.
A dose of 100 mg/day was considered appropirate for treatment of polycythemia vera. For discontinuation of the drug, normalization of leukocyte and platelet counts proved to be good indication. In addition, it is necessary to pay attention to the rise in serum iron levels.
These data suggest that DBM is effective for the treatment of polycythemia vera.

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Colony forming cells (CFCs) in children with acute leukemia were assayed using methylcellulose clonal cell culture. Marrow from children with acute leukemia formed no colonies or very small numbers of colonies in onset or relapse, small numbers of colonies in partial remission and normal numbers in complete remission.
Blast cell colonies were formed by blood containing 81 percent leukemic cells in a child with AML. Blast cell colonies were observed not only by using PHA stimulated normal or leukemic leukocyte conditioned medium but also by using human placental conditioned medium.
Erythroid colonies in children with acute leukemia were also assayed using methylcellulose clonal cell culture. In culture of marrow, the sum total of erythroid colonies scored at day 7∼8 were none or markedly decreased in onset or relapse, decreased in partial remission, and within normal range in complete remission.

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Chronic granulocytic leukemia (CGL) is cytogenetically an interesting disease because of the presence of Philadelphia (Ph¹) chromosome and the appearance of new chromosomal changes throughout their course in about 75% of the cases. As the majority of new chromosomal changes occur shortly before or during the acute phase, they are as one of the predictive or diagnostic indicators of the acute crisis. However, some cases keep the chronic phase in spite of the presence of new chromosomal changes. If the state of new chromosomal changes in the chronic phase can be differentiated from that in the acute phase, chromosomal examination will be more useful for the understanding of the progress of the disease.
The analysis by banding method of our 21 cases and 120 cases in the literature with one or more additional chromosomes in addition to Ph¹ revealed the followings.
1. Such chromosomal aberrations as the extra 8, i (17 q), the extra Ph¹, missing Y and balanced rearrangement could appear not only in the acute phase but also in the chronic phase. Consequently when these chromosomal aberrations are found, we should not make the diagnosis of acute crisis but carefully follow up their clinical course.
2. Those chromosomal aberrations such as the number of chromosomes exceeding 48, i (17 q) with additional chromosomal aberration, autosomal elimination, unbalanced rearrangement and the abnormality of X chromosome appeared in the acute phase. These chromosomal aberrations might be useful diagnostic indicators of the acute crisis of CGL.

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Increase of monocytes in the peripheral blood of the patients with congenital neutropenia might be a compensatory phenomenon for the lack of neutrophils in defence mechanism.
We attempted to clarify bactericidal and cryptocidal capacity of monocytes, and compared with those of neutrophils. Monocytes were obtained from a patient with cyclic neutropenia, and a patient with severe combined immunodeficiency when neutrophils were absent or greatly reduced with increase in monocytes. Neutrophils were obtained from healthy adults.
Monocyte killing of both Staphy. aureus and Strept. faecalis, and cryptocidal capacity of monocytes were similar to those of neutrophils. The NBT dye reduction capacity of monocytes was not less than of neutrophils.
Myeloperoxidase mediated antimicrobial system provides an especially potent bactericidal activity. Since monocytes do not contain as much peroxidase as neutrophils, peroxidase independent bactericidal system will operate in monocytes more actively than neutrophils.

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A 28-year-old female was admitted to our hospital because of bleeding tendency on December 1, 1978. On admission, nasal and oral bleeding, petechiae and purpurae over the body and moderately enlarged goiter were noted, but exophthalmus or hepato-splenomegaly were not detected. Thrombocytopenia of 8,000/cmm and marked increase of immature megakaryocytes in the bone marrow were consistent with idiopathic thrombocytopenic purpura, though antiplatelet antibody was not detected. Laboratory findings such as T₃ uptake, 54.7%; T₄ level 21.4 μg/dl and ¹³¹I uptake 71.8% confirmed the association of hyperthyroidism. Prednisolone and azathioprine did not provide lasting improvement of the thrombocytopenia, while thyrotoxicosis was controlled by methimazole. On March 28, 1979, splenectomy was performed, when she was in euthyroid state. Soon after the operation, marked thrombocytosis occurred and maintained over 200,000/cmm without additional use of prednisolone or azathioprine.
It was suggested from the experience of this case that splenectomy should be recommendable for idiopathic thrombocytopenic purpura in spite of the assocation of hyperthyroidism.

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Thirtyfive-year-old female was admitted complaining of palpitation, myalgia, arthralgia and left hypochondralgia. The diagnosis was considered to be chronic myelocytic leukaemia (CML) presenting in blastic transformation (BT) from the findings of hepatosplenomegaly, anaemia, leukocytosis, myelogram and positive Ph¹ chromosome. The patient was treated with V-P therapy and partial remission was achieved. After 40 days in partial remission, blasts in the peripheral blood and bone marrow increased rapidly. We continued V-P therapy but no effect was achieved. Then we tested a combination of V-P therapy, ACNU, and cytosine arabinoside. But this combination was not effective. The patient died 10 days after administration of ACNU due to progressive respiratory insufficiency. At autopsy, pathologic diagnosis was done as AML with secondary pulmonary fibrosis.

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A case of Listeria monocytogenes septicemia complicating acute lymphoblastic leukemia was reported. The patient, a 52 years old male, was diagnosed to have acute lymphoblastic leukemia in March, 1977. A complete remission was attained with the chemotherapy consisting of daunorubicin, vincristine and prednisolone. Relapse occurred in September of the year, and he was again given various combination chemotherapies without much success. In February, 1978 when the peripheral leukocyte count was as low as 100 per cmm, a sudden rise in body temperature was observed and blood cultures revealed the growth of Listeria monocytogenes. Gentamicin and cephalothin was given immediately following blood samplings for bacteriological studies, and were proved effective to lead to a complete cure of the sepsis. It was shown later that the isolated strain of Listeria monocytogenes was highly susceptible to both of the antibiotics. The patient died in September, 1978 after repeating probable infections without attaining the second remission.

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Since Baughan et al described the first case of nonspherocytic hemolytic anemia with glucose phosphate isomerase deficiency, more than 25 new cases and more than 17 cases with new variants of glucose phosphate isomerase deficiency have been reported. The purpose of this paper is to describe two additional cases with a new variant of glucose phosphate isomerase deficiency, which is characterized by a profoundly decreased activity in the red cells, normal optimal Michaelis constant, thermal instability, and abnormal electrophoretic phenotypes in the cases and the mother.
Propositus was a 3 year-old-girl. She was born uneventfull, and did not present neonatal severe jaundice. She had had blood transfusion twice since she was 2 years and 4 months old. Her anemia became worse, when she had high fever due to upper respiratory infection without any medication. Her developmental mile stones were normal. There was no consanguinity in her family, and no family members, who needed blood transfusions except her sister.
Physical examination on admission showed mild icterus on conjunctiva, pale skin, no lymphadenopathy, clear heart sounds, liver edge palpable 1 cm below right costal margin, and spleen palpable 2 cm below left costal margin. Lab. data of propositus; Hb 11.1 g/dl, Rbc 353×10⁴, Hct 34.1%, MCV 97 fl, MCHC 32.6%, reticulo 128‰, Coombs test negative, total bilirubin 3.4 mg/dl, direct bilirubin 1.7 mg/dl, Fe 38 μg/dl, U.I.B.C. 219 μg/dl, HbF 8.2%, G.O.T. 13, G.P.T. 14, LDH 202, normal osmotic fragility test, normal Ham's Test, hyperplasia of erythroid cells in bone marrow aspiration, 3.8 days of red blood cell's half life, glucose phosphate isomerase activity 7.5 μmol/min/g Hb (normals: 27.9∼44.9).
Her sister, 1 year and 10 months old, had similar history of blood transfusions. She also had pale skin, and mild icterus on her conjunctiva, but didn't have splenomegaly. Lab. data of the sister; Rbc 313×10⁴, Hb 10.2, Hct 31.8%, MCV 101 fl, MCHC 31.9%, reticulo. 140‰, t. bili 1.9 mg/dl, direct bili 0.8 mg/dl, Fe 115 μg/dl, GOT 17, GPT 4, glucose phosphate isomerase activity 6.4 μmol/min/g Hb.
The glucose phosphate isomerase activity in the mother was also low (8.4 μmol/min/g Hb). The activity in the father was intermediate (20 μmol/min/g Hb). pH curves showed that the optimal pH was 8.5 like normal enzyne. Thermal stability was much decreased in the propositus, the sister, and the mother. It was normal in the father. Michaelis constants of G.P.I. were all within normal limits. On starch gel electrophoresis in the mother, the main band moved less from the origin than two other minor bands towards the cathode. The electrophoretic patterns of the propositus and the sister showed that three bands moved slightly less than that of normals.
We concluded the G.P.I. of the propositus, the sister, and the mother are new variant which have never reported in the world literature.

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A 73-year-old female was admitted on December 12, 1978, because of dizziness, tinnitus and general malaise. Physical examination revealed pale conjunctiva, hepatosplenomegaly and generalized lymphadenopathy. The hemoglobin was 6.1 Gm/100 ml., red cell count 3,000,000 per cu. mm., hematocrit 18 per cent and reticulocytes 3.4 per cent. The white cell count was 590,000 per cu. mm. with 1.0 per cent myeloblasts and the maturation of the myeloid series was normal. The erythroblasts were seen 17 per 200 white cells. The platelet count was 115,000 per cu. mm.
A sternal marrow aspirate revealed hypercellular marrow with normal megakaryocyte count and marked myeloid hyperplasia. Axillary lymph node biopsy material revealed a severe infiltration by the mature granulocytes, small grouped erythroblasts and atypical megakaryocytes.
The Philadelphia chromosome was negative. The neutrophil alkaline phosphatase score was within normal limits. The hemoglobin F was 0.6 per cent.
She suffered from acute renal failure before the initiation of the antileukemic therapy and died two weeks after admission.
On autopsy infiltration of the leukemic cells was noted in almost all organs with the granulocytes of every maturation stage, erythroblasts and atypical megakaryocytes. No other malignant changes were observed on autopsy.

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Autoimmune hemolytic anemia may be accompanied in a wide variety of diseases, but it is far more rarely encountered in the myelogenous leukemia.
A 77-year-old male with acute myelogenous leukemia, when he was in the partial remission, was admitted because of exacerbation of the anemia.
On admission, his hemoglobin level was 5.1 gm/100 ml, reticulocytes 42‰, the platelet count 54,000/mm³ and leukocyte count 2450/mm³ with 1% myeloblasts. Bone marrow smear revealed 15% myeloblasts and hyperplasia of erythroid elements. The erythrocyte life span (T 1/2) as measured by ⁵¹Cr was reduced. (8 days). The serum indirect bilirubin was 0.8 mg/100 ml, Type I of LDH isozyme increased and haptoglobin undetectable. The direct Coombs test was strongly positive and the autoantibodies to the erythrocytes were found to be IgG (κ).
After initiation of treatment with prednisolone, the direct Coombs test became negative and anemia gradually alleviated.
These findings strongly suggested that autoimmune hemolytic anemia had occurred in the course of acute myelogenous leukemia.

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HBsAg has been found in 7.5 to 20% of the cases with leukemia. Though asymptomatic increases in serum transaminase levels are common, overt hepatitis has been regarded rather rare. Among three patients with ALL treated in our hospital, two cases of fulminant hepatitis resulted in death and one case recovered from acute hepatitis.
In case I, 13-year-old boy, fulminant hepatitis developed after the completion of remission induction therapy with vincristin and prednisolone and intrathecal injection of methotrexate as CNS prophylaxis. In case 2, 13-year-old boy, fulminant hepatitis occurred after two courses of DCMP therapy for the remission induction. In case 3, 10-year-old girl, acute hepatitis with ascites occurred 2 months later from the day of complete hematological remission.
In these three cases, hepatitis B was evident when the patients were lymphopenic. Explanation by the development of hepatitis B by the immunological hypothesis, which has been previously presented, could not be applied to the aboved-mentioned cases.

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Megaloblastic bone marrow was observed in a 26 years old women suffering from idiopathic thrombocytopenic purpura after 10 days administration of azathioprine (100mg per day). Serum and red cell folate levels were within normal limits. Administration of 10 mg of folic acid did not improve the megaloblastosis of the marrow. Merely discontinuation of azathioprine brought disappearance of the megaloblastosis dramatically. This is the first case of megaloblastic anemia induced by azathioprine in Japan.

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