Shwartzman reaction in pregnant animals was invited by single injection of endotoxin without any preparatory procedure. This specific phenomena had been explained by hypercoagulability of blood during pregnancy, which produced intravascular fibrin deposition. In this paper, it was dealt with the possible role of plasmin system in this specific type of Shwartzman reaction during pregnancy.
In human subject, total plasmin (caseinolytic and fibrinolytic activities of SK-euglobulin) and spontaneous proteolytic activities of euglobulin fracti onwere elevated as the stages of pregnancy advanced. The whole blood or plasma, however, showed suppression of fibrinolytic which might be caused by predominat antiplasmin level during pregnancy. A marked elevation of fibrinolytic was found in blood samples from vasa efferentia of the non-pregnant uterus, while no fibrinolytic activity was proved in blood from the pregnant uterus. Plasminogen activator extracted from kidneys of pregnant rat, guinea pig and rabbit had significantly lower activity than the non-pregnant controls.
The results described above seemed to suggest that in pregnant state, Shwartzman reaction was easily induced by not only hypercoagulability but also lowered fibrinolytic activity, depositing fibrin and remaining it in the vessels.

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Correlation between the plasma fibrinolytic activity, measured by SK-and α-CT-activation methods and plasma total protein, A/G ratio, and the influence of inhalant anesthetics were studied.
1. Samples from 80 cases were measured with SK-and α-CT method; positive results were obtained only in 2.5 per cent with the former and 8.8 per cent with the latter.
2. A relationship between SK-and α-CT activation method was ascertained, but there was no relationship between the fibrinolytic activity and plasma total protein and A/G ratio.
3. Hypoproteinemia was found in only 6.3% of the total cases.
4. Different fibrinolytic activity in vitro was shown in different kinds of inhalant anesthetics.
The antifibrinolytic effect of trans-4-Aminomethylcyclohexane-1-Carboxylic Acid (trans-AMCHA or Transamin), was as follows:
1) Its antifibrinolytic activity was demonstrated at the SK activation method, but hardly been at the α-CT method.
2) It was maintained in stored blood all through the storage.

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The fibrinolytic activity, the antifibrinolytic effect of trans-4-Aminomethylcyclohexane -1- Carboxylic acid (trans-AMCHA or Transamin), known as antiplasmin were evaluated from surgical view-point. Results obtained were as follows:
1) Higher level was observed in uterine cancer and gastric cancer at preoperative period, compared, with that of tympanoplasty.
2) Pre-medication which was consisted of pentobarbital, phenothiazine, atropine and meperidine, which caused the decrease of fibrinolytic activity.
3) Compared with that of pre-and post-operative periods, the fibrino-lytic activity during surgery, measured by SK-activation method was slight, but those measured by α-CT-activation method resulted in a marked increase.
4) Administering trans-AMCHA during surgery the fibrinolytic activity were suppressed remarkably than that of pre-operative level by SK-activation method, but they were scarcely suppressed, while that of pre-operative level by α-CT-activation method did remarkably.
5) An increase of fibrinolytic activity following blood transfusion is explained by physical and chemical reactions which will result from the stimulation of transfusion and by the surgical stress itself.
6) There was no untoward-side effect caused by trans-AMCHA during general anesthesia.

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A 15 year-old boy with congenital afibrinogenenmia was reported, and a review of 14 cases in Japan was made. Episodes of bleeding of this case began with umbilical bleeding and complicated with multiple bruises, nose bleeding and massive bleeding after teeth extractions, but no hematuria, melena, hematemesis, or spontaneous hemoarthralgia was seen.
General laboratory examinations revealed on specific abnormalities except for delayed sedimentation rate. Fibrinogen in the blood was not proved by the immunological method. Examination on clotting factors showed a complete absence of clotting due to absence on fibrinogen in the blood as well as remarkable decrease of fibrinolytic activity. The half of fibrinogen due to transfused Cohn Fraction I was 3.2 days. This seems to be the first case, in which the decreased fibrinolytic activity was found.

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Fourteen cases of chronic mylogencus leukemia were treated with 2 mg daily dosis of myleran (Group A), and their clinical and hematological states were compared with those of 13 cases who were treated with 4-8 mg daily dosis (Group B).
In the Group A, reduction of the peripheral leucocyte count and spleen size occured more slowly than in the Group B. Thrombocytopenia was observed not infrequently in the Group B, but was rare in the Group A.
Although the 2 mg therapy could not prevent the occurrence of the acute exacerbation, its incidence within 20 months after the start of the treatment was significantly reduced by the therapy, and 50 percent survival was prolonged from the 30 months of the Group B to the 60 months in the Group A.
From the above observations, it was considered that the therapy with 2 mg daily dosis was superior to that with 4-8 mg daily dosis in treating chronic myelogenous leukemia.

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Massive and continuous administration of Vitamin B₁₂ was given for seven and a half years long on a 53-year-old female suffering from pernicious anemia with advanced spinal cord lesions.
The treatment has had a marvelous effect on her neurological lesions. However, sensory and motor disturbances and ataxia are even now markedly present. These neurological involvements still show gradual improvement.

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A case of β-thalassemia minor with thalassemia gene on the mother's side was reported.
The propositus, a four-month-old boy, with hepatosplenomegaly had a hypochromic microcytic anemia and in the periperal blood smear anisocytosis, poikilocytosis and basophilic stippling were observed.
Osmotic fragility was markedly decreased. Target cells were seen in mother's periperal blood smear.
On starch block electrophoresis the amount of Hb A₂ comprised 3.1 per cent of the total hemoglobin at the age of eight months.
His mother, maternal grandfather and aunt had A₂ hemoglobin fractions of 4.3-5.4 per cent.

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Among the various forms of inherited diseases of porphyrin metabolism, congenital erythropoietic porphyria stands out as a well-defined and very rare disorder. So far, a total of 13 proved cases have been reported in Japan. The metabolic defect is found in the maturing erythroid cells of the bone marrow and leads to overproduction of porphyrins of type I isomer. We recently had an opportunity to observe and investigate a patient with clinical manifestations of this type.
Report of a Case
A 5-year-old Japanese girl was first seen at our clinic on May 21, 1966, with cutaneous lesions of the face and back of the hands, and an excretion of dark reddish brown urine of four years duration. Family history elicited nothing of significance.
Fine dark hair resembling lanugo covered markedly the face and extremities. Deciduous teeth showed a brownish discoloration (erythrodontia), and under ultraviolet light exhibited red fluorescence.
Splenomegaly was absent. Hypertension and abdominal or neurologic symptoms were not found.
Laboratory studies; liver function tests were all within normal limits, and liver biopsy showed no particular change.
Hematologic examination; hemoglobin concentration 12.4 gm%; red blood cell count 3.74 million; white blood cell count 6,300; reticulocytes 8‰. A decreased erythrocyte life span was demonstrated by the Cr⁵¹ method. Bone marrow showed erythroid hyperplasia and fluorescence microscopic studies of unstained bone marrow preparations revealed intense porphyrin fluorescence in nucleated red cells, particularly in nuclei. Many fluorescing normoblasts exhibited morphologic abnormalities in nuclear structure, consisting of PAS (-) inclusion. Protoporphyrin concentration of circulation red blood cell was 22.0 μg/100 ml.
Chemical examination: porphyrin determinations in urine are given in Table 1. The amount of uroporphyrin excreted were always increased. In addition, the urine contained large amount of coproporphyrin, but the concentration was less than that of uroporphyrin. Smaller amounts of porphyrins with six and five carboxyl groups were also demonstrated. These urinary porphyrins were identified as the type I isomer. Porphobilinogen and protoporphyrin were consistently absent from the urine.

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A 73 years-old-male patient had been fairly well until six months prior to the admission, when he complained of anorexia and palpitation. He had had rib caries, hepatitis, gallstone over 10 years ago.
Physical examination on admission revealed sligfit generalized pittig edema, anemia and jaundice. His tongue was atrophic. The heart was enlarged towards the both sides.
A systolic murmur was audible at the apex. EKG showed low voltage, prolonged QRS and depressed T wave. The liver and spleen were not palpable.
Hematological examination disclosed hyperchromic anemia and 13 erythroblast per 200 leucocytes. Over half of nucleous cells in bone marrow smears were typical megaloblast. Schiling test was 1.04%.
After admission he was treated with high dosage of V. B12 (1000 mcg/day), cardiac stimulants and antibiotics. On the 5 th day of admission reticulocytes increased to 68‰ and on the 6 th day, however, he suddenly developed dyspnoea and died.
The autopsy revealed fatty degeneration of heart, central necrosis of hepatic lobule, fatty degeneration of tubular epithel of kidney, atrophy of gastric mucosa and marked proliferation of erythroblast in bone marrow.

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A 44 years old house-wife was admitted to the hospital in September, 1964, with complaints of general malaise, tachypnea. fever and hemorrhagic diatheses. Physical examinination revealed severe anemia, but neither hepatolienomegaly nor lymphadenopathy. Blood examination showed hemoglobin, 3.8 g/dl, leukocytes, 16,600 with the following differentials: blast cells 74% promyelocytes 1%, metamyelocytes 3%, neutrophils 12%, monocytes 3%, and lymphocytes 17%, and platelets, 76,000. Sternal marrow study showed hypercellularity (261,000) with a good number of myeloblasts (32%).
She was treated with blood transfusios of 3,560 ml for 54 days, dexamethasone of 135 mg for 54 days and 6-mercaptopurin 2,050 mg for 35 days, in total, respectively, with an uneventfull effect, and discharged in November, 1964. Thirty days after the discharge, however, leukocyte counts in the blood raised to 29,400 with dominant myeloblasts (85%). She was readmitted in December, 1964. Blood transfusions, dexamethasone and 6-mercaptopurin were administered with no remarkable effect this time.
In March, 1965, she complained of back pain for 7 days, followed by untolerable leg pain in about 7 days and then a complete transverse myelitis at the 3rd tholacic segment suddenly developed which comprised paraplegia of the lower extremities, anesthesia in both the abdomen and lower extremities, and retention of urin. She died on 24 th April 1965, about 30 days after the developement of such neurological complications.
Post mortem examination disclosed marked infiltration of myeloblasts throughout the tissue, particularly in the bone marrow, spleen, liver and kidneys. Besides, there was found marked myeloblastic infiltration in the dura mater over the whole length of the spinal cord, resulting in liquefaction necrosis of the spinal cord from the 3rd tholacic segments to the cauda equina.

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A fifty-year-old housewife was first admitted on October 22, 1964, with complaints due to anemia. There was no splenomegaly. Hemoglobin 4.2 g/100 cc, RBC 1,620,000, WBC 3,850 with leukoerythroblastosis and platelets 94,000. Repeated bone marrow aspirations were unsuccessful. Needle biopsy of the bone marrow: myelofibrosis. Ferrokinetics: PID T 1/2 was 150 minutes and RCIU 50%. Body surface counting: ⁵⁹Fe activity was high over the liver and low over the spleen and sacral region. PMN alkaline phosphatase was normal on three occasions.
She was re-admitted in February 1966 because of marked albuminuria. Hemoglobin 4.9 g/100 cc and WBC 3,800 with 2% of myeloblasts. On March 17 WBC was 4,000 with 42% of myeloblasts. Improved after the treatment with prednisolone and 6-MP. The second needle biopsy of the bone marrow on April 22: Myelofibrosis accompanied by the proliferation of myeloblasts. She was discharged on June 18, when splenomegaly was not present.
She was admitted on October 11, 1966, for the third time with enlarged liver by four fingerbreadths and spleen palpable on the level of the navel. Hemoglobin 3.7 g/100 cc, RBC 1,400,000, WBC 28,000 with 14.5% of myeloblasts and thrombocytes 45,000. She died on October 14, 1966.
Autopsy findings: Myelofibrosis terminating in the acute myelogenous leukemia. Extensive reticulo-collagenous fibrosis and marked monotonous myeloblastic cell infiltration in the entire bone marrow examined. Splenomegaly (535 g) and hepatomegaly (1880 g) with leukemic cell infiltration and minimal extramedullary hematopoietic foci. Leukemic infiltration to the renal cortices, lymph nodes, myocardium, lungs and hypophysis.

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A 38 year-old housewife was admitted to the Gunma University Hospital complaining of hemorrhagic diathesis. The past and family history were noncontributory. In December 1963, she was admitted to another hospital with the chief complaints of generalized purpura, abnormal genital bleeding and epistaxis. Prednisolone therapy was started under the diagnosis of ITP. As hemorrhagic tendency continued in spite of the treatment, she was transfered to our hospital for further evaluation on June 15. 1964.
Physical examination on admission revealed the generalized purpura, mild hepatosplenomegaly. Laboratory findings; elevated ESR (103 mm/h). Decreased serum albumin and increased serum gamma-globulin concentrations. Hypochromic anemia of Hb. 8.6 g/dl and highly decreased platelets with normal WBC counts. A few juvenile granulocytic leukocytes were always observed on the peripheral blood smears. Prolonged bleeding time, positive tourniquet test and impaired prothrombin consumtion. Decreased serum iron and increased serum copper concentrations. Apparent half survival time of RBC (51 Cr-method) was 25 days. Ferrokinetic study; PID T/2 28 min., %RCU 100% (5 days), PIT 0.84 mg/kg/day and RCIT 0.84 mg/kg/day. No abnormality of bone marrns picture was found in both myeloid and erythroid series. The number of megakaryocytes was within normal limits, although young form was observed.
After admission prednisolone and fresh blood transfusion were performed without remarkable effect. Splenectomy was performed on November 25, 1964. The histology of the spleen revealed marked periarterial fibrosis (onion-skin lesion). After the operation platelet count transiently increased. but soon became as low as previous count. Hemorrhagic diathesis was decreased under the therapy of prednisolone, oestriol succinate and Norluten R.
After discharge (Feb. 1965) her purpura gradually disappeared and platelet count gradually increased in spite of the discontinuation of adrenal cortical hormone therapy Platelet count was 100,000/cm in June 1967. No fever, joint involvement, cutaneous lesions and neuropsychotic symptoms have been observed so far, although slight proteinuria was detected.

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Numerous elliptic erythrocytes were found in peripheral blood smears of a 31-year-old male who had a severe pancytopenia and hypoplastic marrow on admission. According to Günther's method, 72.5 per cent of his erythrocytes belonged to classes III and IV. Since the family survey revealed a mild degree of elliptocytosis in one of the patient's children and one of his sisters, hereditary elliptocytosis complicated with aplastic anemia was suspected. The number of elliptic cells, however, gradually decreased with improvement of anemia on steroid therapy, and elliptocytes virtually disappeared after the anemia was cured. In such an anemic case with elliptocytosis prudent consideration should be paid to establish the diagnosis of hereditary elliptocytosis.

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