Bone marrow biopsy was carried out 68 times in 54 cases with various hematological disorders by Jamshidi needle. All specimens were obtained successfully except one in a case with malignant reticulosis.
Clinically important evidences were revealed 38 times and definitive diagnostic evidences which were not obtained by aspiration methods were found 16 times. The important role of the bone marrow biopsy was discussed in detail in 4 cases with lymphoproliferative disorders, 10 cases with nonhemic malignancy, 7 cases with myeloproliferative disorders, 5 cases with acute myelocytic leukemia and 7 cases with aplastic anemia.
The device enabled author to get successful biopsy material even from spinal porcess, ribs and sternum when carried out cauciously.
Bone marrow biopsy should be done routinely to elucidate precise architecture of the marrow in various hematological disorders. This would be rewarded with further progress of the hematology in diagnosis and treatment by new evidences of the pathophysiological feature in the bone marrow.

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A total of 42 children—17 with acute myeloblastic leukemia (AML), 6 with poorly differentiated myeloblastic leukemia (AML (P)), 11 with acute monocytic leukemia (AMOL), 7 with frequently relapsed lymphoblastic leukemia (ALL), and one chronic myelogenous leukemia have been treated with combination of multiple drugs. This combination was as follows: cytosine arabinoside 100 mg/m² for the first 5 continuous days, daunomycin 30 mg/m² at the 2 and 5th days, and vincristin 1.5 mg/m² at the 7th day. Prednisolone 40 mg/m² was also given orally for the patients who had already been administered before this regimen. This schedule was repeated with seven to ten day intervals until remmision was obtained.
Complete (partial) remmision was achieved in 6 (4) of 17 AML, 5 (2) of 11 AMOL, 4 (1) of 6 AML (P) and 5 of 7 ALL. By including this regimen, median survival was 9 months in AML, 29 months in AML (P) and 11 months in AMOL.
This intensive combination chemotherapy proved to induce satisfactory remission in refractory childhood leukemia, although good supportive therapy was required, because of susceptibility to infections due to bone marrow suppression.

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Plasma platelet factor 4 (Pf 4) assay using the modified Fuster's method was carried out in 15 patients with hypoplastic anemia, 7 patients with idiopathic thrombocytopenic purpura and 6 patients with disseminated intravascular coagulation.
(1) According to this assay method, the mean value of plasma Pf 4 level in 25 healthy subjects was 14.64+5.09% (M±SD).
(2) In cases of hypoplastic anemia with severe haemorrhage Pf 4 level was elevated from 20 to 38%.
(3) In cases of ITP with haemorrhage, Pf 4 level was decreased from zero to less than 10%.
(4) In cases of DIC with haemorrhage, Pf 4 level was markedly increased form 25 to 40%.
(5) As the platelet counts increased and bleeding symptoms relieved, Pf 4 levels returned to normal in the cases of hypoplastic anemia, ITP and DIC.
It was concluded that the plasma Pf 4 level was a useful parameter to estimate the platelet function in thrombocytopenic disorders.

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Fibrin agar plate for fibrinolysis estimation was manufactured with agar and bovine fibrinogen which was purified by Blombäck and Blombäck's method and lysine-Sepharose affinity chromatography procedure, and found employable for quantitative estimation, applying the fact that the plasmin activity had a linear relationship in semi-logarithmic plotting with the diameter of lysis windows. The estimated values were highly reproducible.
The similar data were also obtained with human fibrin agar plate and no significant difference was proved between these bovine and human fibrin plates. These plates were able to be stored at least for a year in refrigerators, avoiding bacterial contamination without any definite change in estimated values.
Using these plates, estimation procedures were contrived for plasminogen amount, activator and inhibitor as well as plasmin activity in test samples including plasma. Particularly plasmin inhibitor activity was measured directly from the highest plasmin value which was neutralized and stopped to lyse the fibrin plate with a certain amount of samples.

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Congenital factor V deficiency is a relatively rare disease and up to now, only 28 cases (20 families) have been reported in Japan.
A report was made of 6-year-old Japanese boy with a bleeding tendency charactarized by excessive bleeding from the operation wound, after tooth extraction, and following injury. Familial history revealed that father, paternal uncle, and grandfather suffered from hemorrhagic symptoms. The hemostatic findings showed a prolongation of whole blood clotting time, one stage prothrombin time, and partial thromboplastin time. On thromboplastin generation test using human brain extract, the patient's absorbed plasma showed a delay with a normal terminal yield. Circulating anticoagulant was not present. Fibrinolysis was normal. Factor V activity was remarkably reduced. (3% of the normal value) But Factor I, II, VII-complex, VIII, IX, X, and XI-XII were within normal range. A diagnosis of congenital facton V deficiency was made.
The properties of the deficient factor in the patient were pursued. A review of the previously reported 28 cases of congenital factor V deficiency was made.

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Two cases of IgD (K-type) myeloma were reported.
The first patient, 48-year-old male, complained of severe lumbago, shoulder pain and generalized weakness. X-ray films of the skeleton disclosed punched out lesions in the skull and pelvis. A bone marrow aspirate showed 88% replacement of normal marrow elements by atypical myeloma cells resembling reticulum cells. A marked Bence Jones proteinuria was detected. Diagnosis was made as IgD (K-type) myeloma following immunoelectrophoretic analysis by specific antisera.
The second patient, 65-year-old male, with pains of back and lower extremities was diagnosed as multiple myeloma because of multiple bony lesions, marked plasma cell proliferation in the marrow and Bence Jones proteinuria. The latter case was identified as IgD (K-type) myeloma by immunochemical analyses.
Both of the present cases, in addition, were found to have IgD catabolic fragment proteins in urine.
High susceptibility of IgD myeloma protein to papain digestion was proved in the present cases.
The accelerated degradation of IgD myeloma proteins in vivo might cause the excessive excretion of fragment proteins.

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A 48-year-old woman who had primary macroglobulinemia with acute clinical course was reported. She was admitted because of drug-induced hepatitis in Oct. 1971, with chief complaints of jaundice and general rash developed during treatment for common cold, and the recovery was satisfactory. During the four-months' hospital days, none of laboratory findings was suggestive of macroglobulinemia. Six months after discharge she again entered our hospital due to high fever, hematuria and palpitation. Serological findings revealed monoclonal gammapathy with an increase of IgM (3880 mg/dl). Hematological data showed pancytopenia and appearance of lymphoblastoid cells in peripheral blood and sternal bone marrow. She died on the eighth hospital day.
This case with acute clinical course seems to be unusual because most cases of primary macroglobulinemia are chronic.

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Purpura hyperglobulinemica is a syndrome of which three cases featured with hyperglobulinemia and purpura were described by Waldenström for the first time in 1943, and in view of the subsequently reported cases, it is considered to comprise the primary and the secondary cases. In recent years, such secondary cases which accompany auto-immune diseases are often taken up.
Two cases of secondary purpura hyperglobulinemica have been described. Case 1 appeared secondary to myxedema and Sjögren syndrome. The antinuclear antibodies were positive. It was inferred that not only the elevation of γ-globulin level but also the decrease in platelet count, a reduction in platelet adhesiveness and aggregation, the presence of circulating anticoagulant, a tendency for euglobulin clot lysis time to be reduced, and abnormality in the correction of thrombin time were influential over the development of purpura in this case. The administration of thyroid hormone and corticosteroid resulted in the improvement in these symptoms. Case 2 appeared secondary to Sjögren syndrome and renal tubular acidosis. Not only γ-globulin level was elevated but also euglobulin clot lysis time tended to be reduced.
Because there are the “primary” and the “secondary” cases of purpura hyperglobulinemica, it has been proposed to indicate the identity as a remark to the diagnosis of this disease.

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A 2-month-old boy was admitted on August 3, 1973, because of high fever, hepatosplenomegaly and leukocytosis. After admission, he continued to develop high fever, heomrrhagic diathesis including melena and petechial hemorrhages scattered over the entire body and eczematous skin lesions with the concomitant development of suppuration. Cultures of blood, urine and secretions from suppurative skin lesions grew Enterococcus, Klebsiella and Staphylococcus aureus, respectively, at the different stages of the whole course of the disease.
Among other laboratory findings, anemia, leukocytosis with striking leukoerythroblastosis, thrombocytopenia, hyperimmunoglobulinemia G, A, M and E and significant reduction of isohemagglutinin titer were noteworthy. The vigorous antimicrobial therapy was instituted immediately after admission and was continued throughout the hospital course, but gave no remarkable improvement. Consequently his general condition was deteriorated gradually and he died on October 29, 1973.
An autopsy performed about 3 hours after the death revealed severe bronchopneumonia, a number of cytomegalic inclusion bodies in submaxillary glands and kidneys, and markedly atrophic thymus gland weighed 0.5gm.
Moreover, the histology of the thymus gland showed indistinct “corticomedullary” boundary attributable to lymphocyte depletion in the thymic cortex. Several lymph nodes revealed profound lymphocyte depletion especially in thymus-dependent paracortical area with no definite development of reticular hyperplasia and plasmacytosis.
Beside these findings, on family survey, 3 other boys in maternal side were found to have developed symptoms and signs similar to those in this case and have succumbed to overwhelming infections within a year after birth.
Based on the clinical, laboratory and histological findings above described, we discussed hyperimmunoglobulinemia E and cytomegalic inclusion disease in this case chiefly in relation to the striking atrophy of the thymus gland.
It seems likely that marked elevation of serum IgE and other classes of Ig in this case was ascribed to T cell deficiency secondary to thymic atrophy, and cytomegalovius might be transferred into the patient via multiple fresh blood transfusions.

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