The clinical course of the patients with “dysmyelopoietic syndrome (DMPS)”, according to the definition and the classification proposed by Sultan, Pierre, Hast and Imbert, is presented.
In a 63-year-old male patient (case I), “refractory anemia with proliferative dysplasia” which had been observed during about three years developed into fatal “refractory anemia with excess of blasts”. A 70-year-old female patient (case II) died of acute myelomonocytic leukemia preceded by DMPS. The clinical courses of the 70-year-old and 64-year-old female patients (cases III and IV) diagnosed as DMPS and of a 47-year-old male patient (case V) with PNH are described as well. The successful androgenotherapy of anemia occurring in cases I, III, IV and V indicated a positive effect of this drug upon “ineffective erythropoiesis (dyserythropoiesis)” characteristic of preleukemia.

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Lithium carbonate has been shown to cause neutrophilia in the patients with manicdepressive disorder and recently has been promoted in the treatment of congenital neutropenia and neutropenias of other causes. The effect of lithium carbonate on the neutrophil function is known little and we aware the adverse effect of lithium carbonate on the neutrophil function since lithium carbonate inhibits the activity of adenyl cyclase and reduces the level of c-AMP in the cells. In this study, we investigated the effect of lithium carbonate on neutrophil function in two methods. First is the effect on the carbohydrate metabolism, and the other is the effect on NBT reduction which reflects the activated oxygens. And we also investigated the effect of calcium, magnesium, zinc and copper on the neutrophil function. Although calcium was activated and zinc and copper were supressive, there was no significant change of effect by two methods when lithium was tested. Another kind of investigations on the influence of lithium carbonate on the neutrophil function will be necessary, because lithium carbonate must be used during long period to maintain the blood neutrophil count.

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A comparative study between the results with manual differential and with Hemalog D was carried out.
The types of acute lymphoblastic leukemia were classified by means of the Hemalog D display of peroxydase negative cell population with the index of LUC (large unstained cell).
M1-M3 leukemias were classified on the basis of the display of peroxydase channel with the index of peroxydase activity and with pseudoeosinophil count. Monocytic leukemias were assured on the display of esterase channel (α-naphthyl butyrate as a substrate) with fluoride inhibition.
The results suggested that Hemalog D was useful for the FAB classification of acute leukemias.

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Fifty-one male children with acute lymphocytic leukemia (ALL) were treated at the Department of Pediatrics, the University of Tokyo through January 1965 to December 1976. Forty-three children obtained complete remission and seventeen (39.5%) of them developed testicular leukemia 5 months to 9 years 6 months later as the first relapse. 29.4% and 76.5% of children who developed testicular leukemia did so within 1 year and 3 years of remission, respectively.
Patients were divided into two major groups according to the difference of initial induction therapy. One group was treated with chemotherapy regimens including Prednisolone 300mg/m² for 2 weeks and another group was treated without this high dose of glucocorticoid. Incidence of testicular leukemia was not different between these two groups, but the mean durations of complete remission until interruption by testicular relapse were 53.1 months for the former group and 19.1 months for the latter group and the difference was statistically significant (P=0.01).
Chemotherapy alone was not adequate for treatment of testicular leukemia. Irradiation of more than 2,000 rad to both testes was adequate for complete local control, but many cases repeated relapse at other sites and eventually died.
It was thought that histological detection of occult leukemic infiltration of biopsied materials and early local treatment with reinduction therapy before cessation of intensive chemotherapy was the best and most practical method to prevent testicular leukemia as the first relapse.
Children with ALL and cryptorchidism could develop testicular leukemia and surgical correction during early remission was recommended.

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We reported previously a competitive solid-phase enzyme immunoassay to measure platelet-associated IgG (PAIgG). The results indicated that 93% of patients with idiopathic thrombocytopenic purpura (ITP) had elevated PAIgG values and there was a good inverse correlation between PAIgG values and platelet counts. In this report, using our assay, platelet-binding IgG (PBIgG) in ITP serum was measured. Ten of 20 patients had elevated PBIgG values. There was no correlation between PBIgG values and platelet counts nor correlation between PAIgG values and PBIgG values. It was indicated that PBIgG had differences in the affinity for normal platelet. It is considered that PBIgG is not so important as PAIgG in the destructive mechanism of platelet.

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The pathogenesis of myeloproliferative disorders (chronic myelocytic leukemia, polycythemia vera, essential thrombocythemia, etc.) is thought to be impairment of the pluripotent hemopoietic progenitors. The colonies that both erythropoietic and granulopoietic cells seemed to be the pluripotent hemopoietic progenitors. Peripheral blood mononuclear cells of myeloproliferative disorders were cultured using the methylcellulose method, stimulated by sera of the patients of aplastic anemia. The mixed colonies (CFU-mix) were found in peripheral blood in 5 of 24 cases with myeloproliferative disorders. Such mixed colonies can be easily identified by the difference of color and size, directly observed with inverted microscope. Aspirated colonies were stained with peroxidase and Giemsa stain for identification.
Mixed colonies were not formed in culture of the peripheral blood from myeloproliferative disorders without stimulation of serum of aplastic anemia, and from the normal individuals even in addition with serum of aplastic anemia.
These findings suggest that the serum of aplastic anemia possesses mixed colony forming activity (MCFA) and the myeloproliferative disorders have plenty of pluripotent hemopoietic progenitors in peripheral blood more than the normal individuals.

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The immunopotential activities of leukemic sera were investigated among 35 children with ALL under chemoimmunotherapy.
Immunotherapy was applied monthly with irradiated leukemic cells combined with immunopotentiators such as BCG, Levamisole, OK-432, etc. The suppressor activity was found in the sera of 32 children on admission, when the sera were added to the lymphocyte blastogenic response induced by PHA. But this suppressive activity had disappeared by the time of remission induction. Despite the presense of the same sera, PWM-induced lymphocyte blastogenic response was not suppressed. It was presumed that this serum immunosuppressive factors might act rather specifically on the T-cell moiety by binding competitively with mitogens at the membrane receptors.
On the contrary to the leukemic sera on admission, PHA-induced lymphocyte blastogenic response was enhanced by the sera obtained from leukemic children who had been treated over 6 months.
When the leukemic cells, pre-treated with these sera, were cultured with peripheral mononuclear cells, the cytotoxic reaction was elicited with the sera and the enhancing activity of the sera correlated with the serum level of antibodies against leukemic cells. It was suggested that the sera of the patients under specific immunotherapy contained antibody which took a part in antibody dependent cell mediated immunity.

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Combination chemotherapy with bleomycin, vincristine, adriamycin and prednisolone (BOAP) was performed in 23 patients with advanced malignant lymphoma. In patients without previous chemotherapy complete remission rate was 75% in non-Hodgkin's lymphoma. That was 83% in B cell type and 60% in T cell type. Median duration of their complete remission was longer than 18 months in both B and T cell type. The median survival of these patients was longer than 24 months for complete responders and 2 months for partial and nonresponders. Myelosuppressive toxity was mild. Diffuse pneumonitis was observed in 30%, but they were recovered by the treatment with large doses of adrenal steroids, antibiotics and immunoglobulin. BOAP therapy was considered to be effective in non-Hodgkin's lymphoma of both B and T cell type.

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A case of IgG-K multiple myeloma with hyperviscosity syndrome after operation for prostatic hypertrophy is reported.
A 75-year-old man showed disturbance of consciousness about 3 weeks after operation (TUR of the prostate). Later, bleeding in the oral cavity occurred and he was transferred to our clinic. Data revealed ESR, 102mm/h; RBC, 3,130,000; thrombocytes, 112,000; WBC, 13,800, with 5% of myeloma cells. Serum total protein was 11.4 g/dl, 60.1% of which is M component. Serum creatinine was 4.5 mg/dl, BUN 37 mg/dl, Na 134 mEq/l and K 5.9 mEq/l. Serum viscosity was 5.5. A diagnosis of hyperviscosity syndrome was made and acute renal failure was effectively treated with plasmapheresis.
Cyclophosphamide was used for multiple myeloma, but therapy was stopped because of leukopenia, thereafter left exophthalmus and loss of left visual acuity occurred. Although Bence Jones protein was negative, immature plasma cells were found in urinary sediments. His general condition got worse and he died.
Autopsy revealed bilateral pleural effusion with slightly yellowish color, in which numerous myeloma cells and M protein were observed. Infiltration of tumor cells was found in liver, spleen, pancreas, kidney, lymphnode, bone marrow and others.

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A case with recurrent fever, markedly disseminated skin infiltration of plasma cell and systemic lymphadenopathy with polyclonal hypergammaglobulinemia was reported. The clinical feature of this case was similar to that of immunoblastic lymphadenopathy (IBL).
However, the histological findings of the lymphonode in this case did not show any destruction of the structure but the proliferation of mature plasma cells without atypism in perivascular area and medulla of lymphonode were seen. These findings could not be compatible with the criteria of IBL. Abundant globulin-like products in plasma cell was shown in electron microscopic findings.
Anti-κ and λ positive cells were clearly stained by immunoperoxidase method. These findings suggested that these plasma cells produced polyclonal γ-globulin. Prednisolone, VCR and cyclophosphamide therapy was carried out, but no responses were observed. It can be suspected that this case is so-called “disseminated polyclonal plasmacytosis” and progression into plasmacytoid lymphocytic lymphoma or multiple myeloma was suspected.

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A 50-year-old woman was admitted to our hospital on Dec. 5, 1974, because of pancytopenia and marked splenomegaly. Blood counts revealed RBC 386x10⁴/μl, hemoglobin 10.2g/dl, WBC 1,500/μl and platelets 34,740/μl. A bone marrow aspirate showed erythroid hyperplasia without atypical cells. Neither hepatomegaly nor lymphadenopathy was observed. However, markedly enlarged spleen displaced the stomach to right upward. The celiac and superior mesenteric arteriography indicated marked splenomegaly with moderate sized (9 cm in diameter) hypovascular area, and many aneurysmal dilatation and winding of splenic artery. The echoslenogram and scintigram of liver and spleen suggested huge spleen with a tumor mass. The portography during operation indicated a remarkable dilatation of splenic and portal vein, and the portal pressure was 250 mm H₂O. The spleen weighed 1,120 g, in which a solitary true cyst was discovered. After splenectomy, all of her blood counts returned to normal within 2 weeks, and the patient is very healthy now, 7 years after splenectomy. The case of splenic cyst accompanied with hyperspenism is very rare, and this case is the first case discovered in Japan.

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Three cases of chronic lymphatic leukemia were studied by observing their surface markers on the leukemic cells. 2 cases were classified as a classic type and the other was a bloody change type, according to Scott's classification. All those cases were diagnosed by the bone marrow specimens obtained by needle biopsy, because of dry taps. The specimens showed the following finding; two cases were hyperplastic with typical infiltration of leukemic cells and partial infiltration in the other.
The surface markers in the former two cases were identified as a surface Ig negative non T-CLL and the other one, IgD + IgG type B-CLL by the E-rosset formation test and the fluorescence method using FITC labeled anti-IgF (ab₂) fragment.
The cells of non T-CLL cases possessed C3b and C3d receptors, but C3b positive cells were dominant. However, the cells collected from the patients' lymph nodes showed that C3d receptor were more dominant than C3b receptor. The cells of B-CLL possessed no complement receptors. We concluded the importance of simultaneous observations of surface markers on both peripheral leukemic cells and lymph node cells.

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A case of a 56-year-old man with chronic idiopathic neutropenia accompanied by few febrile episodes and his benigh clinical course was reported. The mechanism of mature neutrophil depletion in this patient was investigated. Patient's bone marrow showed slight hypercellularity and remarkable depletion of mature neutrophils. The in vitro soft agar culture of bone marrow cells showed merely slightly decreased myeloid colony forming cells (CFU-C), which did not mean primary disturbance of stem cells. The liquid culture of bone marrow cells revealed normal maturation of neutrophils and chemotaxis of leukocytes might be lowered. Epinephrine stimulation test showed only slight response. No antineutrophil antibodies were detected in either agglutination test, cytotoxity test or quantitation of neutrophil surface IgG by solid phase enzyme immunoassay. The measurement of circulating immune complexes in this patient by solid phase C₁q enzyme immunoassay revealed a high value. Patient's serum lysozyme and lysozyme index showed high levels. These findings suggest that neutropenia in this patient may be based upon increased destruction of mature neutrophils and that circulating immune complexes might be concerned with increased destruction.

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Case 1.- A 44-year-old woman was admitted on February, 1980. She complained nasal bleeding, purpura and lower abdominal pain. Purpura appeared occasionally since several years ago. Her menses have been regular.
She had thrombocytopenia, normocytic normochronic anemia of unknown etiology. Bone marrow aspiration revealed hypoplasia with scattered megakaryocytes releasing no platelets. On admission, she had collapsed. A Douglas puncture revealed the presence of fresh blood supporting the diagnosis of ovarian bleeding. Daily administration of prednisolone (40 mg) rapidly induced an improvement of her clinical conditions and an increase in platelet count up to 185,000.

Case 2.- A 29-year-old woman experienced massive genital bleeding when her pregnancy was interrupted in 1976. On the following year, purpura, hematuria and genital bleeding developed. She was diagnosed as idiopathic thrombocytopenic purpura based on her laboratory findings. After prednisolone therapy, her platelet counts ranged from 12,000 to 20,000. She was admitted to our hospital on March, 1980. On admission, she complained severe abdominal pain and nausea, then she fell into collapse. Operation revealed the presence of right ovarian bleeding.
Although purpura, genital, gingival and nasal bleeding are known as the common complications of thrombocytopenic purpura, ovarian bleeding is extraordinally rare complication in patients with thrombocytopenic purpura.
We report two unusual cases of thrombocytopenic purpura with a complication of ovarian bleeding.

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