We have recently reported a new type of lymphocytic leukemia with the following characteristics: (1) onset in adulthood, (2) leukemic cells with T-cell properties, (3) leukemic cells with morphologically characteristic features, (4) frequent skin affection, (5) lymphadenopathy and hepatosplenomegaly, (6) no mediastinal masses, (7) subacute or chronic course, and (8) predominance among natives of Kyushu. This disease presents many intriguing aspects for research. The current problems in the investigation are as follows.
1. Expeditions are necessary for the comparative study of the T cell malignancies reported from the different places of the world.
2. More precise analysis of the past history of each patient is also needed.
3. HLA study is under way.
4. Our chromosome study suggests that 14q translocation may be significant.
5. RNA dependent DNA transcriptase is absent in the cultured leukemic cells.
6. TdT activity is present in the leukemic cells from three patients studied.
7. Fc receptor is absent in the leukemic cells in most cases.
8. The leukemic cells can be distinguished from T-ALL cells by the use of appropriate antisera.
9. Functional aspects of the leukemic cells are also of grave concern. MIF activity and suppressive effect on PWM-induced normal B-cell differentiation have been demonstrated in some cases.
10. Further work is needed for the establishment of cell lines and hybridomas.

抄録全体を表示

The clinical and hematological features of 28 patients with adult T cell leukemia (ATL) are described. ATL has been becoming a disease attracting special attention because of its peculiar geographic distribution of the patients' birthplace, converging on the southern part of Kyushu, and its fulminant clinical course and inefficient treatment.
The disease affects equally both sexes in 40 years and over. Presenting symptoms predominantly include abdominal distention, skin lesion and lassitude. Moderate to massive enlargement of the liver and/or spleen and skin lesion, different from those in Sézary syndrome and mycosis fungoides, are frequent findings, while peripheral lymphadenopathy is inconspicuous or absent. No patient has a mediastinal tumor.
Leukemic cells with pleomorphic nuclear irregularity and T cell property are found at high rate in peripheral blood (83.1 precent of 87,100/cmm WBC) and bone marrow. Dysfunction of the liver are detected on several occasion by blood-chemical examinations.
The remission is hardly obtained with usual anti-leukemic chemotherapy, being accompanied with lethal complications, such as generalized mycosis, pneumonia and interstitial pneumonitis, suggesting the deficiency of cellular immunity. Two months is the median survival time from diagnosis.

抄録全体を表示

The neoplastic cells of patients with non-Hodgkin's lymphoma (NHL) and chronic lymphocytic leukemia (CLL) have been studied for the presence of specific membrane receptors, and the annual incidence rates of malignant lymphoma in Nagasaki district in 1973 and 1974 have been examined. Considering the national and ethnical backgrounds, the clinical and geographic difference between our cases and the cases reported in the other parts of Japan and Western countries is discussed in this synposium.
Unlike the majority of CLL cells studied in Western countries, which have been of B-cell origin with surface-IgM, CLL cells in present series had Ig-determinants for IgG, and some CLL with s-IgG positive cells were associated with serum IgG M-components.
Then, according to a study of surface characteristics on tumor cells from 41 NHL patients, 46% were classified as T-cell origin, 27% as B-cell origin and 27% as non-T, non-B cell type. The majority of T cell lymphoma, which were compatible with adult T-cell leukemia reported by Uchiyama et al, had the specific clinical, histological and geographic characteristics. Therefore, the authors confirmed that there is distinctive T cell lymphoma localized predominantly in Kyushu Island.

抄録全体を表示

The T-cell type lymphoid malignancies were heterogenous disease. A classification of T-cell type lymphoid malignancies was proposed as based on clinical, cytological and immunological analysis of the disease.
1. Thymus (+), lymphoblastic type
2. Thymus (-), lymphoblastic and/or lymphocytic type
3. Adult, pleomorphic type
4. IBL-like or Hodgkinoid type
The characteristics of each disease is summarrized as follow; 1) Thymus (+), lymphoblastic type—The disease usually developed predominantly in young male (less than 30 years old) with thymus enlargement and was progressive with high incidence of CNS involvement and leukemic manifestation. Tumor cells had high TdT activity, T-cell specific antigen, En-rosette forming capacity (En-RFC) and freguently C₃-d receptor. 2) Thymus (-), lymphoblastic and/or lymphocytic type—The disease developed predominantly in male without specific age distribution. Thymus enlargement was not detected throughout the course. Tumor cells had low TdT activity, En-RFC, T-cell specific antigen occasionally with Fcγ-receptor. About 80% of the patients, who had lymphoblastic or mixed cell morphology, had poor prognosis, while 20% of the patients, who all showed lymphocytic or CLL morphology, survived longer. 3) Adult, pleomorphic type—The disease usually developed in older people (more than 20 years old) with slight predominance in male. More than half of patients came from Kyushu districts. About 20∼30% of patients was accompanied with hypercalcemia, eosinophilia, interstitial pneumonitis and Herpes Zoster. High incidence of skin involvement and leukemic manifestation without thymus involvement was characteristics of the disease. Tumor cells showed bizarre and pleomorphic appearances and had low TdT activity, T-cell specific antigen and En-RFC. C₃ and Fcγ-receptors were not detected. About 60% of patients died early, but 30∼40% of patients were alive for longer period. 4) IBL-like or Hodgkinoid type—This group of patients was diagnosed histopathologically as likely immunoblastic lymphadenopathy or Hodgkinoid. However, cytology of aspirates from lymphnodes revealed many tumor cells with atypical morphology, which always carried T-cell marker. We tentatively classified these cases as “IBL-like or Hodgkinoid” type.
The approprieteness of the new classification of T-cell type lymphoid malignancies according to phenotype of immunological markers, morphological characteristics and clinical features and their presumed origin is discussed.

抄録全体を表示

Malignant lymphomas (ML) in Kagoshima Prefecture are very characteristic, not only in their high incidence among prefectures in Japan, but also in a variety of clinical and morphological appearances. Our recent study based on the 1007 biopsy materials indicated that 1) the incidence rate per 100,000 population of ML has been markedly increasing, revealing 3.93 in 1970, 5.1 in 1975, 6.26 in 1976 and 9.16 in 1977, 2) the diffuse growth pattern predominated histologically, resulting in poor prognosis, 3) high incidence in the higher age group of the 60s and 70s with a male predominance, and 4) prevalence of cutaneous lymphomas.
By the analysis of surface marker of tumor cells, we studied 57 patients with T cell type malignancies and 4 patients with B cell type and 12 patients with null cell lymphoma. The primary involvement of the T cell lymphoma was seen in 31 cases in lymph nodes, 20 in the skin, 4 in the tonsils and 2 in the bone marrow. Histologically, all of the cases showed polymorphous tumor cells with diffuse growth pattern. Cytologically the T cell lymphomas were classified as follows: 11 cases of lymphocytic histiocytic mixed type, 10 cases of immunoblastic type, 6 cases of pleomorphic type with Sternberglike giant cells, 6 cases of so-called reticulum cell type, 6 cases of convoluted lymphoblastic type and 2 cases of mycosis fungoides.
Twenty autopsies out of 57 T cell lymphomas revealed that a preferential tumor cell involvement was seen in the spleen, bone marrow, aderenal, lungs and liver, and they had severe herpescomplication in form of interstitial pneumonitis due to pneumocystis carinii, cytomegalo and virus and fungus infections. In addition, metastatic calcification in the lung and kidney resulted from hypercalcemia was observed in 30%.

抄録全体を表示

According to modified Rappaport classification, 31 cases of proved T-cell lymphomas were divided into 3 lymphoblastic, 6 lymphocytic, 7 mixed, 11 histiocytic, and 3 pleomorphic histiocytic types. Three lymphoblastic cases were all males of young age (average 19.0 years) and two of them were of E and EAC double marker. Histiocytic type composed of 11 cases were the largest single histologic type in the present series. Pleomorphic histiocytic type, although small in number, was a distinct histological class with worst prognosis.
When compared as a group with B-cell lymphomas, T-cell lymphomas showed more pleomorphism and variations in the size of neoplastic cells even in a given histological group. Often histological appearances differed at portions of the nodes. While B-cell lymphomas showed nodular and expansive growth, T-cell tumors showed infiltrative pattern with frequent spearing of the peripheral sinuses, and in early stages, of the cortical structures of the lymph nodes.
Enzyme histochemically, dot-like localized activity of lysosomal enzymes characterized T-cell lymphomas, white ATPase positive lymphomas were invariably of B-cell origin.
Findings reported herein may be useful to judge the subclass of the lymphoma on the histological ground.

抄録全体を表示

A total of 25 children with previously untreated acute myelogenous leukemia (peroxidase positive) in sequence between 1972 and 1977 were treated with a five-drug protocol (MINI-COAP regimen) using vincristine (VCR), prednisolone (Pred), cytosine arabinoside (Ara-C), cyclophosphamide (CPM) and 6-mercaptopurine (6 MP).
The rate of complete remission was 76%. Of 17 children who achieved complete remissions including one partial remission, eight, 1972∼1974, were maintained with daily 6MP plus periodic reinforcement by MINI-COAP induction every 4 weeks, and nine patients, 1975∼1977, were randomized to maintenance regimen A and regimen B. The maintence chemotherapy program of both A and B was the same as that for the patients of 1972∼1974 including reinforcement of MINI-COAP induction every 3 weeks instead of 4 weeks. The patients in Regimen A received additional immunotherapy with BCG-CWS.
The overall median duration of the initial complete remissions was 6 months, but three patients (one in Regimen A, two in Regimen B) are still remaining in initial complete remission for 13∼22 months. There is no difference in the duration of complete remission or survival between regimen A and B.
Although the results do not compare well to those of the lymphoblastic leukemia, long-term disease free survival can be achieved with a multiple-drug intensive treatment in acute mylogenous leukemia in childhood.

抄録全体を表示

A combination of Sulfamethoxazole and Trimethoprim (SMX-TMP) was given orally to 19 acute leukemia patients with infections. All patients had failed to respond to previous antibiotics consisting of Penicillins, Aminoglycosides or Cephalosporins. SMX-TMP was given either as single agent or as supplement to previous antibiotic regimen.
Eleven patients responded and overall response rate was 57.9%. Three out of 7 patients who were treated by SMX-TMP alone responded. All cases (3 out of 3) with Klebsiella pneumoniae infection and 3 out of 5 with E. coli infection responded.
Only 2 patients showed mild nausea and no other side effects were observed. There was no deterioration of the hematological state.
SMX-TMP seems to be useful agent in the treatment of serious infections in acute leukemia.

抄録全体を表示

A 63-year-old man was referred to our hospital for investigation of severe anemia recurring for past 14 years. He had had rheumatoid arthritis since 31 years of age. On admission, he showed deformity and stiffness of bilateral elbow and ankle joints. Hematological data on admission were as follows: red cell count 130×10⁴-cu mm, hemoglobin 5.0g/dl, hematocrit 15%, reticulocyte 1.9%, white cell count 3,600/cu mm and platelet count 12.2×10⁴/cu mm. The diagnosis of pernicious anemia was made because of megaloblasts in the bone marrow, low serum vitamin B₁₂ and positive Schilling's test. Daily intramuscular injections of cyanocobalamin were effective to improve anemia. Serologically autoantibodies to the parietal cell, intrinsic factor and microsome fraction of the thyroid gland in addition to rheumatoid factor were detected. It was suggested that the long-standing systemic rheumatic changes might have accelerated the development of pernicious anemia in this patient.

抄録全体を表示

A case of 39-year-old-male of mastocytoma was reported. He was admitted to our clinic because of cough, chest pain and fever on September 8, 1977. On physical and laboratory examination enlargement of cervical lymphnode, hepatosplenomegaly, left pleural effusion and remarkable eosinophilia were recognized. The lymphnode biopsy, pleurocentesis and bone marrow aspiration revealed that unclassifiable cell with basophilic granules as well as eosinophils were infiltrated to these organs. The cytochemical and electron microscopic examinations showed that the unclassifiable cell had a characteristics specific to mastocytoma. The patient had been put on combination therapy in VENP but was not induced to the remission. Thereafter the lymphocyte-like immature cell with no granule became more dominant in the peripheral blood and bone marrow. The immature cell gave negative reaction to metachromasia staining; peroxidase and non specific esterase. The surface marker of the cell was null cell type and its TdT activity was not increased.
The leukemic manifestation of immature cell gave arise rapid increase of peripheral WBC up to 113,400 cmm. The patient was treated with VAMP therapy inducing partial remission but died of acute pneumonia and pericarditis. Autopsy showed marked infiltration of the mastocytoma cells in the lymphnode, liver, spleen, lung, heart and kidney. The case reported here is the interesting case of mastocytoma with leukemic proliferation of its immature cells in the hematopoietic organs.

抄録全体を表示

The patient was a 11 1/12-year-old girl who was admitted to our hospital because of anemia and bleeding tendency. On admission, promyelocytes were present as many as 16% of leukocytes in peripheral blood and 90% of nucleated cells in bone marrow. She developed fibrinogenopenic bleeding and laboratory evidences for disseminated intravascular coagulation.
The electron micoscopy revealed peculiar configurations of rough-surfaced endoplasmic reticulum (rough ER) and honeycomb-like inclusions in the organelle. The peculiar configurations of rough ER consisted of central locus with regular arrangement of round cisternae and multiple cisternae of rough ER radiating from the locus. The honeycomblike inclusions in rough ER were observed in about 16% of the leukemic cells.

抄録全体を表示

A 35-year-old man with Ph¹-positive chronic myeloid leukemia in acute phase responded to vincristine and prednisolone therapy. The blasts were lymphoid in appearance and had a high level of terminal deoxynucleotidyl transferase activity, 27.8U/10⁹ cells. Surface marker analysis showed that these blasts did not have surface immunoglobulins and receptors for sheep erythrocytes and Fc portion of either IgG or IgM. Vincristine and prednisolone therapy resulted in a normal leukocyte number and disappearance of the blasts in the peripheral blood, which might indicate return to chronic phase of CML, but the lymphoid cells remained about 70% in the bone marrow. Twenty weeks after the diagnosis of blastic transformation, typical myelobasts, but not lymphoid cells, increased markedly in the peripheral blood, and the patient died from infection and cerebral bleeding. The significance of TdT activity in the treatment of CML blast crisis was discussed.

抄録全体を表示

Eight patients with hyperviscosity syndrome and two patients with autoimmune diseaes underwent a course of therapeutic pheresis on an intermittent flow cell separator.
A patient with dermatomyositis had not responded to prednisolone, and spontaneous pneumothorax had developed following massive cough caused by interstitial pneumonitis.
Three plasma exchanges combined with azathioprine therapy produced reduction of circulating immune complex and clinical improvement.
A patient with myasthenia gravis whose symptoms were not controlled with anticholinesterases had suffered from respiratory failure before thymectomy. Plasma exchange was performed five times over fourteen days period, with removal of total of 9,700ml of plasma.
The levels of C3c and C4 in patient's serum and have subsequently been normal, and a dramatic improvement in all affected muscle groups was observed.
The vital capacity rose from 1,940ml to 2,940ml, therefore thymectomy was performed without a difficulty of anesthetic management. Thereafter, she has been maintained on anticholinesterases alone.
In two patients with hyperviscosity syndrome who were treated with plasma exchange for one case and cytapheresis for the other, an improvement in the value of A-aDO₂ was observed following the procedure.
It is cocluded that therapeutic pheresis using a cell separator may be valuable as an adjuvant to the treatment of hematologic and autoimmune diseases and it is safe for the patient.

抄録全体を表示

Plasma exchange was performed in each case of idiopathic thrombocytopenic purpura, myasthenia gravis and multiple myeloma and favourable effect was observed in every case.
The effect appeared in a short time after the plasma exchange in all cases, but did not last long in a patient with multiple myeloma.
This therapeutic procedure can be carried out easily by using Haemonetics Model 30. The indication for this operation should be strict, because only limited amount of fresh frozen plasma is available in our country at the present time.

抄録全体を表示

In this report, our recent experience with the use of Haemonetics Model 30 for surgical patients is discussed. Main indications of platelet transfusion by this equipment are: 1. Emergency operation with massive blood replacememt. 2. Elective surgery for patients with thrombocytopenia.
In the former, massive blood loss and replacement with stored blood may lead to dilutional thrombocytopenia. Quick preparation and infusion of platelets by this method can prevent life threatening bleeding.
In the latter, patients with severe thrombocytopenia may have received multiple transfusions, and may be refractory to platelets from random doners. In such cases platelet transfusion with HLA identical single doner is important.
We reported a case of successful total hip replacement surgery for a patient with aplastic anemia using HLA identical platelet transfusion.

抄録全体を表示

Granulocyte collection using Hemonetics Model 30 was compared with that using Leucolumn from an healthy donar, and also from a case with chronic myelogenous leukemia.
1. By granulocyte collection with Leucolumn, the WBC count and neutrophil percentage were higher and RBC count was lower than those by Hemonetics.
2. The morphology and motile function of granulocytes were maintained better with Hemonetics than with Leucolumn.
3. More drugs are needed to be administered to the donar in Hemonetics. Further long-term study might be necessary, if the granulocyte collection be frequently repeated from the same donar.
4. From a CML case mature neutrophil or basophil leukocytes were more easily collected than immature myelocytes by Leucolumn. By Hemonetics the differential count of collected leukocytes was very simmilar to that of the peripheral blood of the patient.

抄録全体を表示

The effect of granulocyte transfusions on septicemia in pasients with acute leukemia was evaluated by comparing 23 patients receiving only antibiotics therapy with 7 patients receiving both antibiotics therapy and granulocyte transfusions. Only 26% of patients treated with antibiotics therapy (3 drug combination; cephalosporin+broadacting penicillin+aminoglucoside) survived from septicemia, while 85.7% of patients treated with both antibiotics therapy and granulocyte transfusions survived from septicemia. This indicates that granulocyte transfusions enhance survival in patients with septicemia occured in granulocytopenic periods of acute leukemia.

抄録全体を表示

There are several different blood comp nents; red blood cell, granulocyte, lymphocyte, platelet and plasma etc. It is considered that there are several alloantigens on the red blood cell, granulocyte, lymphocyte, platelet and plasma in man. Therefore, the between HLA antigen and blood comp nents must be tested and investigated. The relation relation between HLA antibody production and HLA incompatibility n father and mother, the split of HLA antigens and soluble HLA antigens in the serum have been pres nted and discussed.

抄録全体を表示