Megakaryocytes and platelets in primary thrombocythemia were studied in 3 patients. Each patient presented with marked thrombocytosis with platelet count over 1.5×10⁶/cmm. There were mild to moderate leucocytosis with neutrophilia in 2 of them and elevated neutrophil alkaline phosphatase activity in all patients. Bone marrow examinations revealed preponderant megakaryocytes of matured type with large platelet aggregates on smear. Megakaryocytes were significantly greater in size than those of the normal control. Similar megakaryocyte metric survey in other myeloproliferative syndromes indicated that megakaryocytes in 19 cases of chronic granulocytic leukemia were smaller, whereas those in 6 cases of polycythemia vera were significantly larger. Platelet factor 3 activity as assessed by the thromboplastin generation test was abnormally low in all patients, but the activity was rectified by increasing the concentration of platelet suspensions. Platelet aggregation to epinephrine was absent in 2 patients in whom thrombo-hemorrhagic symptoms were more severe than the other patient in whom platelets aggregated normally. Platelet size in 2 patients were larger than the normal control and that of the other within the normal range. Platelet life span was studied using autologous labeled platelets in 2 patients; one with splenic infarction was within the normal range (8.0 days) and the other with splenomegaly was slightly shortened (6.1 days). The foregoing findings provide evidence for an enhanced thrombopoiesis associated with normal to shortened platelet life span in primary thrombocythemia.

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Studies on platelet functions of 21 patients with renal insufficiency have been preformed. Platelet counts were significantly decreased in uremic patients, although severe thrombocytopenia less than 100×10³ platelets/mm³ was detected only in two patients. Platelet adhesiveness to glass was remarkably decreased in uremic patients. Platelet factor-3 availability was impaired in some of these patients, while platelet factor-3 activity was not decreased. Clot retraction was slightly defective in few patients. The significant correlation was not found between platelet adhesiveness and BUN, creatinine, uric acid or inorganic phosphate level in the serum of these patients. The abnormalities of platelet functions were partially corrected after dialysis.
In vitro, urea at high concentration showed an inhibitory effect on platelet aggregation. Guanidinosuccinic acid had less inhibitory effect on platelet aggregation, and creatinine had no inhibitory effect.

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The important functions of the neutrophils are phagocytic and bacteriocidal activities, and these were studied on the relationship with neutrophil alkaline phosphatase (NAP) activity in various diseases, especially in CML.
Ink-phagocytosis was regarded to represent phagocytic and nitroBT (NBT) dye test, bacteriocidal activity.
Azo dye method was applied in NAP, test tube method phagocytosis and Park's modified method in NBT dye test, and those positive rate and score were counted.
These results were as follows.
Generally, in infectious diseases high values of these test were gained, but CML gave low values. (One case of atypical CML with high NAP activity gave low phagocytic and bacteriocidal activities, too.)
The results of our procedures suggested that though morphologically neutrophils of CML looked like matured cells, these functions were decreased.

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The conventional methods for the examination of the osmotic fragility of erythrocytes have inherent disadvantages of poor reproducibility, requirement of a large amount of blood sample and time-cosuming procedure.
Coil planet centrifuge method presented here gave a high reproducibility with a minute amount (1∼3 μl) of blood and required a relatively simple technique.
According to this method, erythrocytes were forced to travel through a long linear density gradient in a fine vinyl tube from physiological saline solution to hypotonic solution at a constant speed. So the erythrocytes were exposed to a gradually decreasing osmotic pressure down to the point where hemolysis occurred, thus the hemoglobin distribution pattern, in other words, the erythrocyte osmotic resistance curve, was observed.
The erythrocytes osmotic resistance curve obtained by our method showed some clinical informations which could not be obtained by the conventional methods.

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In a previous paper, (I reported on) the establishment and characteristics of lymphoblastoid cell lines (LCL) from patients with Down's syndrome (DS) and normal persons.
This paper concerns long-term cytogenetic studies performed at regular intervals on these cell lines (2 DS patients and 2 normal individuals). It was revealed that the chromosome constitutions of LCL from DS patients as well as from normal persons maintained the donor's karyotypes from over 12 months, particularly G 21-trisomy being quite stable.
By the banding technique using trypsin treatment and Giemsa staining, it was shown that an extra G group chromosome of LCL from DS patients belonged to chromosome 21.
Although a low percentage of EB virus-positive cells were detected in all these 4 cell lines, there was no apparent chromosomal change associated with this virus.

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This experiment was conducted to know the effect of thrombin, trypsin or plasmin upon the platelets in the purified system by observing a platelet aggregation after using ADP or collagen.
(1) It was clearly shown in these experiments that platelet aggregation was inhibited by means of ADP or collagen from the interaction between washed platelets and thrombin, trypsin or plasmin.
Thrombin, trypsin or plasmin in the vessels would appear in the case of DIC. From the results of these experiments, it was suggested that platelet aggregation might be inhibited and related to the bleeding tendency in vivo.
(2) It was considered that thrombin, trypsin and plasmin are serine proteases, so they show the same influence of platelets, and that they hydrolyse the protein which is the constituents of the platelet membrane.

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It has been reported that hemopexin (Hx) is a β₁-glycoprotein with high affinity for heme and its serum level is reduced in certain patients such as hereditary spherocytosis, sickle cell anemia, hemorrhagic pancreatitis and thalassemia major, correlated with the degree of intravascular hemolysis or more specifically the plasma heme pigment level.
Recently, we had two clinical cases of the transient hypohemopexinemia after surgical procedures. Case one was 67 year old man with aneurysm of left common iliac artery who undertook the reconstructive operation under blood transfusion of 5250 ml. Case two was 35 year old woman with primary aldosteronism who was treated by the left adrenectomy.
From the various investigations, these results were obtained. 1) The transient hypohemopexinemia may follow the surgical procedures. 2) This may appear in the patient with profound ahaptnglobinemia. 3) This may relate to the excessive hemolysis. 4) This may occur due to the consumption of Hx.

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A 7 year old boy was admitted to the university hospital with hemoglobinuria. Two months prior to admission, bilateral ureteral retransplantation had been carried out for vesico-ureteral reflux, and one month later, he had varicella for a week. hemoglobinuria occurred after an exposure to a cold weather and was associated with spiking fever and shaking chills.
On serologic tests, direct and indirect Coombs' tests and Donath-Landsteiner's test were positive, but cold agglutinin and heterophile hemagglutinin titers were respectively 1:64, and 1:224. Serologic test for syphilis was negative. Hemoglobinuria was not provocated by Rosenbach's test, but patient developed high fever.
The clinical course was transient and Donath-Landsteiner's antibody turned negative two months after initial hemoglobinuria.

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A case of bleeding tendency attributed to unusual platelet dysfunetion was described. The retention of platelets in glass bead column was markedly decreased. The in vivo adhesiveness of platelets and platelet factor-3 availability were also decreased. Collagen—induced platelet aggregation and epinephrine-induced aggregation were markedly impaired, while ADP-induced aggregation was less defective.
The amount of platelet ADP was remarkably diminished, accompanied by slightly decreased ATP level. The release of ADP from platelets after collagen-induced aggregation was not observed. The labeling pattern of platelets by incubating them with adenine-8-¹⁴C was almost normal. However, a retarded metabolism of labeled ATP during collagen-induced aggregation was found. From these findings, a new platelet abnormality attributed to the defective adenine nucleotide metabolism, that is, “storage pool disease” was highly suspected.

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Spontaneous splenic rupture in a case with chronic myelogenous leukemia which appeared causatively related to the anomalous lobation of the spleen was described.
A 40-year-old male patient had been maintained in complete remission for six years with initial induction accompanied by occasional short courses of busulfan, until he was hospitalized in May, 1973 because of fever, jaundice, nausea and fatigue of three days duration. The early stage of acute blastic crisis was suspected on the basis of a mild increase in the percentage of myeloblast and promyelocyte in the peripheral blood. On the day of admission he vomited, then complained of an extremely severe upper abdominal pain of abrupt onset, which was then immediately followed by a progressive vascular collapse, resulting in death 12 hours later.
Autopsy revealed a fair amount of blood with some coagula in the peritoneal cavity. Fresh rupture was found at the base of an unusual lobe formation of the spleen. Neither splenic infarction nor localized leukemic cell growth was observed as a possible cause of rupture.

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The patient, a 24-year-old male, with acute lymphatic leukemia was reported in this paper. The initial symptomes were general lymphadenopathy, hemorrhagic tendency due to thrombocytopenia and hepatosplenomegaly.
The peripheral blood finding revealed leucocytosis (WBC 179,250/mm³, blast 81.5%) and remarkable thrombocytopenia (7,500/mm³).
After about 5 months, he first complained headache, right orbitalgia and the painfull swelling of bilateral testis. The spinal fluid contained many blasts which were similar to those in the peripheral blood.
In spite of the intrathecal methotrexate treatment, facial palsy, visual disturbances, ptosis, swallowing difficulties and sensory disturbances and motoric paresis of both extremities appeared one after another.
The chief autoptsy findings were as follows:
1) General lymphadenopthy and the swelling of thyroid, liver and spleen due to leukemic infiltration.
2) Leukemic infiltration of pia mater, bilateral optic nerve, oculomotoric nerve, facial nerve, radix anterior and posterior of spinal cord, and femoral nerve.

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A case of reticulosis with persistent macroglobulinemia and transient macro-pyroglobulinemia during the course is presented.
The patient, 68 year-old female, was admitted to the Chukyo Hospital because of nasal bleeding in November, 1972. She noticed a subcutaneous induration without pain in the right thigh in 1970. Tumor-like indurations with size of up to hen's egg appeared on the chest, trunk and thigh, and bleeding tendency such as purpura and gum bleeding developed in September, 1972. She responded to steroid therapy with decrease of the size of tumors and bleeding manifestations.
Biopsy of tumors having character of lymphatic tissue disclosed proliferation of reticulum cells, moderate depletion of lymphocytes and slight increase of plasma cells and macrophages. Although cell infiltration into outside the capsule and destruction of nodal architecture were noted, cataplastic reticulosis was diagnosed histologically because of the presence of variety of lymphoreticular cells.
Serum analysis revealed the polyclonal increase of IgG and A, and monoclonal elevation of IgM which exhibited the nature of pyroglobulin transiently during the course of the diseaee.
In view af the lymphoreticular proliferation and persistent presence of monoclonal IgM, the reticulosis with macroglobulinemia was considered as pertinent to the patient's state, though she should be carefully followed up for the transformation to the typical Waldenströn's macroglobulinemia.

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A forty-eight-year-old male was first seen on November 15, 1972, with a chief complaint of anemia unresponsive to the iron treatment. He had been diagnosed to have hypertension, diabetes mellitus, gastric ulcer (partial gastrectomy) and liver cirrhosis. He had been a heavy drinker, taking more than 500 ml of sake a day for twenty-five years.
On physical examination he was anemic and the liver swollen. Laboratory examinations revealed hyperchromic, macrocytic anemia, leukopenia, thrombocytopenia, elevated LDH and serum iron levels and lowered UIBC; bone marrow aspirates revealed hypercellular material with hyperplasia of erythroblats and occasional megaloblasts. A Schilling test was normal. While the vitamin B₁₂ level was normal, folic acid was remarkably low in his serum.
A diagnosis of megaloblastic anemia due to folic acid deficiency was made, and a slight improvement of anemia and decrease in marrow meagloblasts were noted on a normal hospital diet and abstinence after admission. After the oral therapy of folic acid with daily dose of 50 mgm. a reticulocyte crisis was noted and a simultaneous increase of hemoglobin, RBC, WBC and platelets followed.
The main cause of the folic acid deficiency in this case was regarded to be the inadequate intake of folic acid and liver dysfunction.

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