The haptoglobin (Hp) is a group of mucoproteins belonging to the alpha-2 globulin which is characterized by a pronounced affinity for hemoglobin (Hb) and its level in serum is abnormal in diseases accompanied by inflammatory, neoplastic, hepatocellular or hemolytic changes.
The purpose of this study is to investigate the serum Hp level before and after splenectomy in hereditary spherocytosis and idiopathic thrombocytopenic purpura and to clarify the clinical significance of serum haptoglobin, which was measured by the single radial immunodiffusion method.
The results were as follows.
1. In 19 patients with various blood diseases, one hereditary spherocytosis, one paroxysmal noctural hemoglobinuria and 4 idiopathic thrombocytopenic purpura were ahaptoglobinemic. And one hemophilia, one allergic purpura and one aplastic anemia were hypohaptoglobinemic.
2. In one hereditary spherocytosis with ahaptoglobinemia, the serum Hp level was raised within 24 hours after splenectomy. This evidence may indicate that the main field of hemolysis in this disease is the spleen and the absence of Hp is a result of the consumption of Hp and not of the failure of synthesis.
3. In 4 idiopathic thrombocytopenic purpura with ahaptoglobinemia, the serum Hp returned to the normal level within 3∼7 days after splenectomy, but in 3 of 4 cases platelet counts did not increase. The splenectomy in these cases should be carefully considered.
4. There may be a significant correlation between serum Hp and histological findings of spleen.

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Platelet count, fibrinogen content, thrombelastograph, prothrombin time, thrombin time, euglobulin lysis time and fibrin degradation products were examined in cases of disseminated intravascular coagulation (DIC).
All patients had elevated fibrin degradation products (FDP). Thrombocytopenia, prolonged prothrombin time and prolonged euglobulin lysis time were found in 94%, 89% and 82% of the cases respectively, and these three tests were most helpfull in diagnosing DIC in addition to detecting FDP. In contrast to these tests fibrinogen content, r value of thrombelastograph and thrombin time were found abnormal in 24%, 51% and 73% respectively, and were found less diagnostic.
The return of platelet count and euglobulin lysis time to normal under heparin therapy would confirm the diagnosis. No correlation was found between the amount of FDP and each of these parameters.

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In the present experiment it was attempted to clarify the correlation of erythrocyte shape and packed red cell volume (Ht). For the determination of Ht, conventional centrifugation method and Coulter Counter Model S (a full automatic electronic particle counter) were adopted. In microcytosis, Ht measured by centrifugation was found to be much higher than that measured by Coulter Counter. This finding indicates that the intercellular free space is larger in microcytosis than in normocytosis. Furthermore in hereditary spherocytosis or spherocytosis caused by Isoton treatment, the conventional Ht was observed to be relatively low. This phenomenon indicates that abnormal high value of the mean corpuscular hemoglobin concentration (MCHC) in hereditary spherocytosis is mainly due to relatively low Ht caused by spherical shape of erythocytes.

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Platalet aggregation by ADP was measured by screen filtration pressure (SFP) method, which was described by Swank (1961). The SFP apparatus measures the resistance to flow of a fluid in mmHg as the blood is forced at a steady rate through a screen with multiple micro-pores 20×20 micra square and approximately 20 micra deep. Whole blood was used in SFP method, so destruction or injury of plateles which may occur during centrifugation to collect platelet rich plasma are able to avoid using this method. Nineteen human subjects, aging from 24 to 60 years, 11 males and 8 females, were used. Variations of SFP of blood added with 1/10 volume of 3×10^-5 molar of ADP were less than 20 percent of the average value in all cases. So the repeated variability of SFP method was not so high and SFP method may be valuable for clinical use. In pores of used screen large number of aggregated platelets masses and few red blood cells were detected. The finding suggests that SFP is mainly due to grade of aggregated platelet mass in blood. Other factors which may influence of SFP were discussed.

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Studies were performed to examine the erythropoietin activity during pregnancy and at the time of delivery with one hundred and thirty one specimens of different lunar month and thirteen paired sera from women at the time of delivery and from umbilical cord of neonates.
Bioassay methods utilizing starved rats and polycythemic mice were employed for this study.
No increased erythropoietin activity was found during the first half period of pregnancy. However, elevated erythropoietin were observed after fifth lunar month which increased remarkably at seventh month lasting until full gestation.
The distribution rate of cases with elevated erythropoietin was much higher in the group assayed with polycythemic mice as compared with that of starved rats.
Sera obtained from mothers during delivery revealed higher erythropoietin activity than those from cord blood of neonates in most cases.
No significant relationship between hemoglobin concentration of peripheral blood and erythropoietin level was observed.

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The anemia associated with chronic renal failure does not respond to usual therapeutic measures and is a very serious problem.
We postulated that decreased production of erythropoietin (ESF), presence of erythropoiesis inhibiting factor (EIF), decreased responsiveness of the bone marrow to ESF, increased red cell destruction and impaired immunological reactivity take part in the pathophysiology of this type of anemia.
Numerous reports have suggested that androgen may enhance the endogenous production of ESF. On the other hand, in some patients well-maintained on a regimen of long-term hemodialysis, ESF activity in plasma was detectable.
The present study was undertaken to investigate the mechanism underlyning the influence of large dose of androgen on erythropoiesis in patients undergoing chronic hemodialysis programs.
The clinical material comprises 14 male and 3 female patients who have been maintained on long-term hemodialysis. Intramuscular injections of androgen (Methenolone enanthate) 400 mg weekly were given to them, usually just after hemodialysis, for 7 to 15 weeks. Our patient's general condition was improved. And androgen produced a significant rise in hemoglobin values, hematocrit and especially in reticulocyte counts. There was no significant difference in the serum iron level, but the unsaturated ironbinding capacity returned to normal. In ferrokinetic studies, most of patients showed marrow improvement to respond to androgen. We have observed the increase in ESF activity on response to androgen therapy in six patients.
Kidney on long-term hemodialysis showed fat deposit and plasma exsudate in the glomeruli in addition to obsolescence of the nephron. It is suggested that “this kidney” without functioning renal tissue cannot excrete the sufficient amount of ESF.
On the basis of these observations, it appears to be question that an extrarenal ESF site(s) may respond to the stimulation with exogenous androgenic hormone.

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Fifty-five children with various types of acute leukemia who had received no prior treatment were treated with 6-mercaptopurine riboside. The patients were induced to a complete remission by combination chemotherapy including 6-mercaptopurine riboside with a dose of 120 mg/m². The remissions were maintained by 6-mercaptopurine riboside alone, 80 mg/m² daily. The rate of remission was 78%.
Toxic hepatitis or liver function impairment and bone marrow depression were major side effects.
Central nervous system involvement by leukemic infiltration was observed in 14 cases (27%) of the treated cases during complete remission.

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The staining of neutrophil alkaline phosphatase (NAP). periodic acid Schiff (PAS) and lactic dehydrogenase (LDH) on neutrophils, lymphocytes and leukemic cells were carried out and searched for the relation of their scoring and serum LDH activity.
1) In AML there were two groups of NAP score, namely low and high score groups. The former showed in general fairly clinical course but the latter did not so good as the former except a few case dropped down to normal score range, and also, so called atypical aplastic anemia showed high score dropped down to normal range.
2) In spite of high or low serum LDH activity, blood cell LDH score showed broad distribution. In polycythemia vera N-, L-PAS score and N-, L-LDH score were all high but were low in CML. In AML there was reverse change of N-PAS score and N-LDH score, even in remission low N-PAS score and high N-LDH score in many cases were observed. High N-, L-LDH score were seen in aplastic anemia.
3) There was reverse correlation in LC-PAS score and LC-LDH score, but that did not be observed in N-PAS score and N-LDH score and also in L-PAS score and L-LDH score. This seemed to be cellular specific characteristics which the glycolysis, respiration and LDH isozyme pattern were different in each type of cells.

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A 25-year-old male was admitted because of dark-red urine, jaundice and purpura. 5 days prior to admission, the patient noticed that the urine color became dark red, and purpuric rash and jaundice were noted by his wife.
Examination revealed scleral icterus, pallor, numerous petechiae and an ecchymosis. The sensorium was clear. The neurologic examination was normal.
The patient had hemolytic anemia, thrombocytopenic purpura, fever and proteinuria. Shortly after admission, fluctuating neurologic symptoms developed, and the patient was diagnosed as thrombotic thrombocytopenic purpura.
He was treated with prednisolone and heparin without benefits, and expired on the 14 th hospital day.
Hyaline thromboses of the vessels of liver and kidney were demonstrated by the examination of the specimens obtained by post mortem needle puncture.
Coagulation studies disclosed that partial thromboplastin time and prothrombin time were slightly prolonged, however, factor V activity and fibrinogen titre were not low. Fibrinogen degradation products determined by tanned red cell hemagglutination inhibition immunoassay were markedly increased, although euglobulin lysis time was not shortened.
Immunological analysis of serum proteins showed the increase of acute phase reactants, the decrease of β₁-AC and haptoglobin, and the appearance of fibrinogen degradation products.
These results were discussed in relation to the pathogenesis of the disease.

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A 26-year-old man was introduced in our clinic as suspected ALL. Hematological examination in this patient revealed prominent lymphoblastic cells in the peripheral blood and bone marrow. Under the diagnosis of ALL, inguinal lymph node specimen was examined. On electron microscopic investigation, many nuclear pockets were found in the blastoid cells. Nuclear pockets are formed by a finger of cytoplasm indenting the nucleus. They contain the same material of the surrounding cytoplasm and have four membranes which are made up of two outer nuclear membranes and two inner nuclear membranes.
Nuclear pockets have been described in leukemic cells and Burkitt lymphoma cells. But they are reported in neutrophils, lymphocytes and also in monocytes of healthy man. Nuclear pockets can therefore be considered not to be specific for leukemic cells and other malignant cells. It seems that it is related to the functional development and differentiation of the cells.

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Chlorambucil was administered by mouth in doses ranging from 6 to 10 mg daily for 2 to 4 weeks to 4 patients with chronic lymphocytic leukemia (one, with leukosarcomatosis). Two patients who were treated by chlorambucil from the onset responded to the drug with disappearance of lymphadenopathy, hepatosplenomegaly and decrease of peripheral lymphocyte count as well as elevation of hemoglobin. They were given chlorambucil intermittently with good results, one for 2 years and the other for one and a half years.
The other 2 patients had been successfully treated with large dose of steroid, cyclophosphamide and vinca alkaloid at first, and responded to these drugs in several relapses. They became resistant to these drugs later, when chlorambucil was administered to them with remarkable effect on lymphadenopathy and splenomegaly. Remission has persisted for 10 months in one patient so far.
These 4 patients displayed no apparent immunologic aberration except for transient monoclonal macroglobulinemia in one patient, and depressed cellular immunity with negative Manteux reaction in spite of active lung tuberculosis in another patient.

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