血液循環により生命活動を行なっている生体にとって，止血機構は重要な生命保全の手段であるが，この止血は血液の凝固作用と血液細胞および血管内皮細胞の機能との相互作用によって協調的に行なわれる。例えばいま組織の一部が損傷を受けると，そこからトロボプラスチンが血液中に放出されて凝固がおこり，できたフィブリンがその部位に定着する。茲に周囲の組織から線維芽細胞と毛細血管内皮細胞が伸びてきて，血管の豊富な肉芽組織となる。そして最終的にはこの肉芽組織は上皮や粘膜上皮細胞におき代って瘢痕となり，創傷は治癒することとなる。この際もし炎症反応が加わると，白血球や各種蛋白分解酵素がこの機序に参加してフィブリンの除去が促進されるが，この炎症反応がない場合には，ここで活性化された血液中および組織内の線溶系が，このフィブリンの溶解除去，瘢痕形成に主役を演ずることとなる。
もとより血液凝固には血中（および組織内）のフィブリノゲン，トロンビン，トロンボプラスチンが主導的な意味をもつが，その出血ないし組織損傷部位の凝固過程はまた阻害物質によっても大きく影響をうける。そしてそれは線溶についても同様で，これら凝固・線溶およびそれらに対する阻害物質の間には，止血機構という意味からも一定の協調・制御が働いている。
出血とは閉鎖血行路から血液が逸脱することであるが，これには大きく分けて3つの型がある。すなわち1) 血友病など凝固因子の欠如による出血，2) 肺における血栓・塞栓症における組織トロンボプラスチン放出による凝固と出血，3) 妊娠および出産時におけるトロンボプラスチン放出の出血性合併症である。
凝固あるいは線溶系が活性化されて出血がおこる際，その程度が一定の限度内で制御が効く範囲であれば，やがて止血されるが，この程度がその限度をこえる時は，出血は続き，創傷の治癒は後れる。この最も代表的な例はいわゆるDIC（汎発性血管内凝固）である。この際は凝固がおこってフィブリンが析出すると，線溶活性化物質がその表面に吸着され，プラスミノゲンは活性化され易いリジン型になって容易にプラスミンとなり，これによりフィブリンはとける。しかしこのプラスミンの作用を抑えるα₂アンチプラスミンがあれば，プラスミンの作用は一層つよくなり，出血は促進される。DICはまた脱フィブリン症候群(defibrination syndrome)ともよばれるが，凝固が一次的に先行し，そのため線溶が二次的におこるという機序は必ずしも全てのDIC例で確められるとは限らず，ことに不顕性(covert) DICの場合は血小板やフィブリノゲンの減少，FDPの増加，プロトロビン時間の延長などが検査で認められなくても，局所的に臓器内血栓ができ，血栓性発作などの臨床症状がおこることがあり，あるいは終始この症状の出ないこともあって，その診断は困難である。治療としては凝固を阻害するためにヘパリンを投与することがいわれているが実際に大量出血中の患者にヘパリンを直ちに注射するのには勇気を要する。この際比較的過量のカルシウムを投与すると凝固が却て遅延することがあるので，試みる価値があろう。
循環血中で凝固反応さらに血栓症がおこる際凝固促進物質の証明されることがある。血友病患者で外傷や出血がおこる時にはしばしばこの物質が認められるが，これは内因性機序の一部の活性化によるものであろう。
止血機序によっておこるフィブリン沈着などの局所の変化は創傷治癒に重要な影響を与えるが，これは臓器別に，また動物種によって差異がある。臓器や動物種によって凝固・線溶活性物質の量や活性化の機序に著しい相異があるからである。今後この方面の研究にはこれらの事項を十分に脳裏に収めておく必要がある。

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Platelet-bound surface IgG (PL-IgG) was quantitatively measured in 16 patients with I. T. P., 15 normal subjects and six patients with hypersplenism using complement lysis inhibition assay. Also the correlation between PL-IgG and in vivo ⁵¹Cr-platelet survival was studied in 16 I. T. P..
The mean values of PL-IgG in I. T. P., normal subjects and hypersplenic patients were 241.2±151.8, 35.1±16.9, and 44.1±13.6 ng/10⁷ platelets respectively. However, the values of PL-IgG in I. T. P. were widely distributed from 50 to 525 ng/10⁷ platelets. Two cases in 16 patients showed near normal level of PL-IgG.
The negative correlations between PL-IgG and ⁵¹Cr platelet survival (γ=-0.686, P<0.05), and platelet count (γ=-0.499, P<0.05) before treatment in 16 I. T. P. cases were observed. However, two cases out of 16 patients showed low platelet count and low level of PL-IgG. Also two cases in 8 patients whose ⁵¹Cr-platelet survival time showed within 24 hours revealed near normal PL-IgG.
The changes of PL-IgG were followed during treatment in three cases with I. T. P.. Case 14 responded well to steroid therapy. The initially high PL-IgG promptly decreased as circulating platelets increased. Case 15 had transient response to steroid therapy. High PL-IgG did not decrease, although circulating platelets increased. Patient was placed on cyclophosphomide and then PL-IgG decreased to normal range. Case 16 had no initial response to steroid therapy. PL-IgG did not decrease in spite of 2 months of high doses of steroid. Patient had splenectomy and then PL-IgG rapidly decreased and platelet count increased to normal range.
In conclusion, PL-IgG could be measured by complement lysis inhib tion assay. The reduction of PL-IgG became good indication to evaluate an effectiveness of treatment and to predict a prognosis of I. T. P.

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FDP (fibrin/fibrinogen degradation products) in serum from patients with DIC (disseminated intravascular coagulation) have been characterized using a method of immunoabsorption followed by SDS-polyacrylamide gel electrophoresis. In all five patients with DIC, fragment D-dimer, derived from cross-linked fibrin, as well as fibrinogen degradative fragments (X, Y, D) were detected. It appears that in DIC primary and secondary fibrinolysis may take place, and both anticoagulants and anti-fibrinolytic agents should be given.

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Infections are the most dangerous complications in malignancies. We examined PMN leukocyte functions in ALL and other malignancies of children with regard to chemotaxis, phagocytosis, NBT test, deformability, adherence and intracellular killing activity. Chemotaxis, phagocytosis and adherence activity were decreased to about 50% of the control values. NBT test was within normal limit in all cases, deformability was suppressed moderately only in ALL and intracellular killing activity was also depressed.
Based on these findings, it was suspected strongly that impaired PMN leukocyte function was one of the most important causes of the undue susceptibility to infections of children in ALL and other malignancies.

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Clinical observations were performed in 11 cases of testicular involvement in childhood acute leukemia.
1) The incidence of this involvement was 18.6% of male children with acute leukemia. When studied in relation to the type of leukemia, the incidence was higher in acute monocytic leukemia (AMoL) and acute lymphoblastic leukemia (ALL) than in acute myeloblastic leukemia (AML): the value was the highest in T cell ALL (T-ALL). The incidence increased as the survival time was prolonged.
2) The median interval from the diagnosis of leukemia to onset of testicular involvement was 30.9 months.
3) Testicular involvement was frequent among children diagnosed at from 2 to 8 years of age and with marked organomegaly.
4) Of 9 cases with isolated testicular leukemia 7 had subsequent relapse with the median interval of 8.4 months. Of 11 cases with testicular involvement 8 died with the median interval of 14.4 months.
5) The testicular involvement in long-term survivors did not necessarily mean a poor prognosis.

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An 80-year-old man was admittde to Tokyo Metropolitan Geriatric Hospital on Feb. 13, 1978 because of the swelling and pain in the left submaxillar region. Histological study on the specimen biopsied near the gingiva of the left lower molar revealed that the tumor occupying the left portion of mandibula was plasmacytoma. Hb was 10.8 g/dl, RBC 342×10⁶/μl, Ht 31.5%, WBC 3,300/μl. Bone marrow smear showed 19.8% of atypical plasma cells. The patient was diagnosed as multiple myeloma (IgA, λ+BJP, κ) from the immunoelectrophoretic analyses. Electrocardiogram showed AV block, and CTR was 67% on an X-ray film of the chest. There were abundant atypical plasma cells in the aspirated pericardial effusion, indicating an infiltration of myeloma cells into pericardial cavity. Local therapy with ⁶⁰Co-irradiation to tumor decreased the size of tumor. In addition, a chemotherapy was carried out, and CTR decreased from 67 to 60%.
A case of multiple myeloma with two different light chains and pericardial infiltration is presented.

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A case of pyruvate kinase variant and its family study are reported. The patient, 26 year-old female, was admitted to our hospital in January 1980 because of anemia and jaundice. On physical examination, anemia and jaundice in the conjunctiva and hepatomegaly were detected. During her hospitalization, there were no symptoms except general fatigue and slight dizziness. Laboratory data revealed macrocytic anemia, slight hyperbilirubinemia with predominant elevation of indirect bilirubin, elevated serum LDH level, reticulocytosis and positive hemosiderin in urine. The enzymatic analysis of red blood cell showed low PK activity (14.9%). In the family study of PK activities in her father, mother, elder brother and younger brother were 47.6%, 102.3%, 64.1%, and 21.3%, respectively. From these data it was suggested that her father and elder brother were heterozygotes, and her mother was a heterozygote of the PK variant which was probably different in character from that of her fater. The patient and her younger brother were homozygotes. Although the site of hemolysis in PK variant has been proposed to be mainly in the spleen, the possibility of intravascular hemolysis could not boe denied.

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A 26 year-old female was diagnosedas acute myelomonocytic leukemia in May, 1977. A complete remission was attained with the chemotherapy consisting of daunorubicin, cytosine arabinoside, 6-MP, and prednisolone. During remission, breast cancer (medullary adenocarcinoma) was complicated in June, 1978. Relapse was observed in September of the year after mastectomy. She was again given various combined chemotherapies with little success.
In July 1979, a rise in body temperature was observed and periproctal abscess by E. coli was diagnosed. Antibiotics were effective for the abscess, but the body temperature rose again. In August, she died because of complete atrioventricular block.
At autopsy, fungeal infection with leukemic infiltration was observed in the conduction system of the heart.

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A case of common variable immunodeficiency (CVID) associated with drug-induced marked granulocytopenia is described.
A 17-year-old boy referred to our hospital for further examination on agammaglobulinemia. He had a history of recurrent infections since 9 years of age. Family history was non-contributory. Laboratory studies on admission disclosed marked hypogammaglobulinemia of all classes and depressed antibody titers against bacteria, virus and blood groups, but normal responses in vivo skin test and in vitro lymphocyte stimulation with mitogens, normal numbers of peripheral T and B-cells, normal morphology and in vitro function of neutrophils, normal complement activities, and intrinsic defect of B-cells, which were demonstrated by using coculture study of T and B-cells for secreting immunoglobulins in vitro. A diagnosis of CVID was made. Gammaglobulin was given intravenously and two days after antibiotics treatment with sulbenicillin sodium (SB-PC) and gentamicin (GM) a marked leukopenia with 7% of immature neutrophils was found. For 6 days thereafter neutrophils completely disappeared from peripheral blood, and drug-induced granulocytopenia was suspected. Bone marrow showed maturation arrest of granulopoiesis. SB-PC and GM were stopped. Over the next 3 days leukocytecounts gradually returned to normal with transient leukemoid reaction and without any bacterial infections. Antiteukocyte agglutinating antibodies could not be demonstrated.

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Two cases of Pre-B-cell leukemia in childhood were reported. Immunologic studies demostrated no rosette formation with SRBC at 37°C lack of surface immunoglobulins, and presence of cytoplasmic-IgM in the lymphoblast.
On electron microscopy, the lymphoblast had round nuclei, abundant free ribosomes, several strands of rough endoplasmic reticulum in the cytoplasm, and no microvilli on the surface.
Clinically, the patients easily entered to complete remission. However, the duration of first remission was somewhat short, being about one year. There was a tendency to repeat relapses.

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A case of Ph¹-positive ALL in a t (13q 14q) carrier is reported. A 34-year-old woman was admitted to the Nihon University Hospital complaining of high fever, palpitation, chest pain and generalized lymphadenopathy on August 17, 1979. Hematological examination on admission revealed that the WBC count was 211,000/μl with 97.5% blast cells, and the nucleated cell count in the bone marrow was 880,000/μl with 97.8% blast cells. A diagnosis of ALL (L2 by the FAB classification) was made. The blast cells had high TdT activity but had neither T nor B markers. At the time of diagnosis, she had no hepatosplenomegaly. Blood examination showed normal NAP score and no increase in basophils. The level of vitamin B₁₂ in serum was more than 3,200pg/ml. However, a complete remission could not be achieved with either BH-AC·DMP or LVP; the latter combination therapy induced partial remission. In spite of vigorous chemotherapy and supporting therapy, she died of pulmonary bleeding in January 1980.
On admission, cytogenetic analysis of the bone marrow cells revealed a karyotype of 44, XX, -8, -13, -14, +t (13q 14q), t (9q+; 22q-) in 100% (25/25) of mitoses. Peripheral lymphocytes and skin fibroblasts of the patient showed a karyotype of 45, XX, -13, -14, +t (13q 14q). When the patient was in partial remission in October 1979, Ph¹ chromosome could not be found out in any of 25 mitoses of her bone marrow cells, but t (13q 14q) still existed in them. From these clinical and laboratory findings, this case was diagnosed as Ph¹-positive ALL in a t (13q 14q) carrier.
The coincidence of Ph¹ chromosome and t (13q 14q) is discussed in this paper.

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A case of immunoblastic lymphadenopathy which underwent transformation into immunoblastic sarcoma associated with polyclonal hypergammaglobulinemia at later date is reported.
A 52-year-old man hospitalized on March 11, 1980, with anemia and generalized lymphadenopathy. Laboratory findings showed marked anemia, reticulocytosis and positive Coombs' test. Serum gammablobulin level remained slight increase on admission. The lymph node of the neck was examined by biopsy and initially interpreted as showing immunoblastic lymphadenopathy. Therapy with prednisolone was started. However, there was no objective effect on the patient's condition, and the lymph nodes remained large. In addition to prednisolone, cyclophosphamide was administered, but anemia and hepatosplenomegaly aggravated. Two months after admission, histological findings of rebiopsied lymph node of the neck demonstrated monotonous proliferation of immunoblasts and monoclonal staining pattern of heavy chain class Ig G and lambda light chain type in the cytoplasm of these cells using immunoperoxidase method (PAP technique). A diagnosis of immunoblastic sarcoma was made. Serum gammaglobulin level rapidly increased and of interest was the finding that there was diffuse, polyclonal hepergammaglobulinemia on immunoelectrophoresis. The level of serum gammaglobulin reached 4.9 g/dl. Hepatosplenomegaly increased and high fever continued, and thus, combination chemotherapy with VEMP was tried. However, his condition continued to deteriorate with progressive pancytopenia and high fever. He died of sepsis four months after admission.

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The 6th case of IgE myeloma in Japan (15th case in the world) was reported in the present communication.
71 year-old female was admitted to Orthopedic clinic of Sakura National Hospital on November 12, 1979 due to right shoulder pain which appeared on June 1979.
Osteolytic and osteoporotic foci were found in the skull, right scapula and right ilium bone without any physical and neurological signs. Complete blood countings were as follows: RBC 248×10⁴/mm³, Hemoglobin 8.3 g/dl, WBC 3,100/mm³, platelet 15.4×10⁴/mm³. Blood smear showed red cell rouleaux formation without any abnormal lymphoid or plasmacytoid cells.
Total serum protein level was 7.6 g/dl. Monoclonal protein was observed at fast γ-region by cellulose-acetate electrophoresis. On immunoelectrophoresis, this monoclonal protein made specific M-bow against anti-IgE and anti-kappa antisera. By single radial immunodiffusion, amount of IgE was calculated as 1.5 g/dl. 53.6% of bone marrow cell was myeloma cells which were stained with anti-IgE and anti-kappa antisera by indirect immunofluorescent technique.
Electronmicroscopic observation of myeloma cells revealed that those cells had plasmacytoid characteristics. Bence Jones protein was negative during clinical course and no renal dysfunction was observed.
Those findings supported the diagnosis of IgE myeloma with kappa light chain.
She has been treated with melphalan, cyclophosphamide, vincristine, ACNU and prednisone.

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