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Mayumi YAMAMURA, Takashi YOSHIDA, Shinobu NAKAMURA, Ken-ichi HATTORI, ...
1980 Volume 21 Issue 12 Pages
1869-1876
Published: 1980
Released on J-STAGE: January 26, 2009
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A 56-year-old female was admitted on May 2, 1979 for a persistent high fever, eczema and left exophthalmus of one month duration. On physical examination, left exophthalmus with chemosis and erythematous nodules of about 5 cm in diameter scattered through the trunk were noted, but neither superficial lymphadenopathy nor hepatosplenomegaly was seen. Blood counting showed mild pancytopenia with a few histiocyte-like cells with erythrophagocytosis. These atypical cells, also were found in a small number on the bone marrow smears. Skin biopsy revealed proliferation of atypical cells within capillaries and blood vessels of the skin. Morphological study showed that such atypical cells in the blood, bone marrow and the skin belonged to histiocytes, some of which were immature in dominancy of the nucleus with prominent nucleoli, but many of them were mature in erythrophagocytosis in their huge cytoplasm.
From the above-mentioned clinical manifestations and laboratory data, a diagnosis of malignant histiocytosis (Rappaport) was made. The patient was treated with combination therapy of vincristine and corticosteroids with a very transient effect, deteriorated rapidly, and died on June 11, 1979, two months after onset. Permission of autopsy was not obtained.
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Hideki MUKAIYAMA, Ichiro TSUKIMOTO
1980 Volume 21 Issue 12 Pages
1877-1884
Published: 1980
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Four cases with congenital factor XI (PTA) deficiency from a Korean family were reported. An 11 year old girl with nasal bleeding was found to have prolonged whole blood clotting time and plasma recalcification time, abnormal prothrombin consumption test and abnormal thromboplastin generation test.
The factor XI activity examined using PTA deficient plasma (DADE) as a substrate was around 20%. Other plasmatic clotting factors were within normal range. The patient's father, mother and younger sister (7 year old) showed 36, 44 and 32% of factor XI activity, respectively. All of them have had mild hemorrhagic tendencies such as nasal and gingival bleeding.
With these laboratory findings, four patients were diagnosed to be factor XI deficiency, and were classified as a minor deficiency type under Rapaport's criteria.
Following a single fresh plasma infusion of 5 U/kg of body weight, the factor XI activity was elevated from 23% to 44% and returned to the preinfusion level within 24 hours. But plasma recalcification time and activated partial thromboplastin time were kept normal for 48 to 72 hours after infusion.
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—A Variant of PiM (PiMnumazu)—
Hideki OHTANI, Tadao FUNATO, Masayuki SAITO, Tomoyuki TOMITA, Yutaka T ...
1980 Volume 21 Issue 12 Pages
1885-1891
Published: 1980
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A 56-year-old male with severe α
1-antitrypsin deficiency and four cases in his family are reported. The patient (propositus) was admitted because of dyspnea. X-ray films of the chest disclosed marked emphysema. Respiratory examinations revealed increase of residual volume/total lung capacity ratio and forced expiratory volume, and decrease of CO diffusion.
Cellulose acetate electrophoresis revealed decrease of α
1-fraction. Serum α
1-antitrypsin was 10 mg/d
l by single radial immunodiffusion. Trypsin inhibitory capacity also markedly decreased. Immunoelectrophoresis showed no precipitin line of α
1-antitrypsin developed with anti-α
1-antitrypsin.
The α
1-antitrypsin phenotype of the patient was designated as a variant of PiM, PiMnumazu, by cellulose acetate isoelectric focusing followed by crossed immunoelectrophoresis, which was more sensitive than acid starch gel electrophoresis and useful for distinguishing the genetic variability of α
1-antitrypsin deficiency.
Family study revealed that the parents, the youngest brother, one of his uncles and one of his aunts were heterozygous for α
1-anititrypsin gene. They had no pulmonary emphysema except for the propositus.
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Chihiro SHIMAZAKI, Tetsuo NAKATA, Akira NISHIO, Harue HARUYAMA, Akito ...
1980 Volume 21 Issue 12 Pages
1892-1897
Published: 1980
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This paper presents a case of T-cell lymphoma with a long survival achieved by radiation and immunochemotherapy.
A 46 year old male, a non-native of Kyushu district, was admitted to our service for the further evaluation and treatment of subcutaneous tumor of r. shoulder and l. arm on January 1979. Two years prior to admission the patient was diagnosed as malignant lymphoma from the histological findings of dissected nasal septal tumor, which was treated by irradiation therapy alone. A tumor of the r. inguinal region was extripated for further evaluation and was diagnosed as poorly differentiated malignant lymphoma. These tumor cells formed rosette with sheep erythrocytes. Bone marrow aspiration revealed hypocellularity without tumor cells and he was treated by prednisolone alone with poor response and additional local administration of OK-432 was started into the tumor which became soften in consistency and decreased in size. Positive direct Coomb's test and antiplatelet antibody were detected in short time of thrombocytopenia, and the increased administration of prednisolone improved the platelet counts. In spite of immunochemotherapy including prednisolone, Bleomycin, oil-Bleo and OK-432, the tumor size was increased and symptom was aggravated and expired on March 1979 due to shock induced by oil-Bleo injection.
Through the clinical evaluation of this patient, it is emphasized that 28 months long survival was achieved responding to the treatment with irradiation and local OK-432 administration. This case of T-cell lymphoma suggested the effectiveness of the combined therapy with irradiation and immunopotenciator.
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—Evaluation of Hemopoietic Disorder by in vitro Culture Technique—
Koichiro IKUTA, Hideki SASAKI, Yoshitaka KOISO, Toshitsugu KAWASHIMA, ...
1980 Volume 21 Issue 12 Pages
1898-1903
Published: 1980
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A ten-month-old boy with Shwachman's syndrome (chronic neutropenia and exocrine pancreatic insufficiency) was evaluated.
Hemopoietic disorder in this case was studied by in vitro culture technique. An assay of bone marrow granulocyte-macrophage precursor (CFU-C) in a double agar culture system demonstrated its reduced number. Production of colony stimulating factor (CSF) from patient's peripheral blood leukocytes appeared normal when tested on normal marrow. No serum inhibitor against CFU-C could be demonstrated.
The reduced number of erythroid progenitor cells (CFU-E) was shown in a plasma clot culture system.
These findings suggested that the bone marrow dysfunction in this case might be resulted from a stem cell disorder.
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Yasuharu MITOMO, Masao HIGAMI, Masakazu NITTA, Kimiaki YOSHIKAWA, Mako ...
1980 Volume 21 Issue 12 Pages
1904-1908
Published: 1980
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A 33-year-old man was admitted because of abdominal distension on May 23, 1975. A diagnosis of chronic myelocytic leukemia (CML) was made because of leukocytosis, splenomegaly, Piladelphia chromosome (Ph
1) positive and a low score of neutrophil alkaline phosphatase activity (NAP). The splenectomy was performed on the chronic phase after 2 years 4 months from the diagnosis in order to prevent the devolopment of acute crisis in a spleen. In this case, no agent has been administered after the splenectomy except a small amount of vercyte immediately after the operation. Lymphoadenopathy, disseminated intravascular coagulation (DIC) and leukemic meningitis were developed at the final course. The variation of leukocyte and platelet count were observed, and the leukocyte count increased to 228×10
3/cmm and then showed a plateau before the acute crisis. Cell infiltration was recognized rather the generalized lymphnodes than the liver by the pathological findings, and cell kinetics after a splenectomy of CML was discussed. A new protocol of chemotherapy after the splenectomy of CML to induce a more prolonged survival will be expected, and then an effect of the splenectomy should be discussed.
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Yasuo TAKANO, Toshiaki CHINEN, Makoto OGAWA, Yo KATO, Tomoyuki KITAGAW ...
1980 Volume 21 Issue 12 Pages
1909-1915
Published: 1980
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A 53 years old mastectomized woman for breast cancer treated with radiotherapy (total doses 12,600 rad) and with long term oral administration of cyclophosphamide (CPM) and ftorafur (FT), developed aplastic anemia and thereafter acute myelogenous leukemia. About six months after discontinuation of the above therapies, she developed anemia and leukopenia and was referred to our clinic.
Hematological improvement was obtained by the administration of anabolic hormone, however, two months later she became pancytopenic again. At that time, quite atypical myeloblasts contained peroxidase positive granules, were found 39% in the peripheral blood and 89.4% in the bone marrow, respectively. Leukemic hiatus was present. A bone marrow biopsy revealed coexistence of leukemic cells and breast cancer cells. A diagnosis of breast cancer complicated with acute myelogenous leukemia was made. A combined therapy of adriamycin, CPM and FT was ineffective. OAP regimen of vincristine, cytosine arabinoside and predonisolone revealed transient hematologic improvement. Finally, the patient died of septicemia due to klebsiella. Autopsy revealed wide spread coexistence of leukemia and cancer in the bone marrow, liver, and thyroid.
The authors discuss some possible explanations for development of acute leukemia after radiotherapy and chemotherapy.
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A Special Reference to Antibody-Dependent Cell-Mediated Cytotoxicity by Peripheral Blood Leukocytes as a Mechanism of Hemolysis
Masatoshi TAKAYA, Yukinobu ICHIKAWA, Shigeru ARIMORI
1980 Volume 21 Issue 12 Pages
1916-1922
Published: 1980
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A 21-year-old male patient was admitted because of acute onset of generalized purpura and thrombocytopenia (0.2×10
4/cmm) in Dec., 1977, and treated with prednisolone (PSL) under the diagnosis of idiopathic thrombocytopenic purpura. In Sept., 1979, he noticed jaundice and general malaise. Marked anemia and mild splenomegaly were demonstrated. Examinations of peripheral blood showed normocytic anemia (Hb 5.2 g/d
l), leukocytosis (10,700/cmm), thrombocytopenia (6.1×10
4/cmm), and reticnlocytosis (12.2%). Hemolytic anemia was confirmed by other laboratory data, such as indirect bilirubin, serum LDH, serum iron, and myelogram. Both direct and indirect antiglobulin test were positive, and anti-erythrocyte antibody in his serum was confirmed as IgG. Treatment with large dosage of PSL (100 mg/day) was started. Anemia and then thrombocytopenia were gradually improved.
Antibody-dependent cell-mediated cytotoxicity (ADCC) of human peripheral leukocytes (PBL) was investigated by using
51Cr-labelled human erythrocytes sensitized with the patients serum. It was concluded that ADCC of PBL in this case was not participated in the mechanism of hemolysis, because neither cytotoxic nor phagocytic activity of PBL was demonstrated against target erythrocytes.
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Masafumi TANIWAKI, Taira MAEKAWA, Junichi EDAKAWA, Yoshiaki SONODA, To ...
1980 Volume 21 Issue 12 Pages
1923-1930
Published: 1980
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TdT positive and subsequently occurred TdT negative blast crises were observed in a 49-year-old patient with Ph
1-positive CML. In the first crisis, blast cells of the patient showed lymphoid morphology (L
1) and they had high TdT activity and surface markers of non-T cell, such as negative surface immunoglobulin, positive C
3-receptor, Fc-receptor and Ia-like antigen. In the second crisis, however, the blast cells were considered myeloblasts (M
1) based on morphologic impression and showed no TdT activity. Both crises responded well to VP therapy. This case suggests that loss of TdT activity in blast cells does not always imply the emergence of cells resistant to VP therapy. The relationships between TdT, VP therapy and karyotype and the origin of target cell in the blast crisis were discussed.
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Hidehiko JYOSE, Hideo MUGISHIMA, Junichi MIMAYA
1980 Volume 21 Issue 12 Pages
1931-1937
Published: 1980
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5-year-old-girl with recurrent subcutaneous bleedlings and epistaxis was admitted to Shizuoka Children's hospital for further investigation. She had a history of abnormal post-traumatic bleeding of the tongue at the age of 3 years. Hemostatic screening tests on admission revealed prolonged A-PTT and abnormal TEG patterns. She had low level of VIII: C (7.6%∼16%) but normal level of VIII R: AG (115%) and VIII R: WF (94%) Platelet count, bleeding time, PT, fibrinogen, Platelet retention, ristocetin induced platelet aggregation and factor VIII-crossed immunoelectrophoresis showed normal values. No factor VIII inhibitor was demonstrated. Her Karyotype revealed 46 chromosomes with two X chromosomes. Mother showed no abnormal hemostatic figures, but father was bleeder, althrough he was not precisely tested. From these findings, she was diagnosed to be a female hemophilia A of heterozygous type.
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Kunizo MARUO, Haruya YOSHIKAWA
1980 Volume 21 Issue 12 Pages
1938-1943
Published: 1980
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The 33-year-old-man was admitted because of purpura and pain above the both legs. One month ago, he suffered from lacunar tonsillitis and was treated with antibiotics.
On admission, he appeared actually ill, with petechiae and purpuric spots over the extremities and the face. He complained of severe pain in the both thighs, though neither muscle weakness nor atrophy was prominent. The serum creatinine phophokinase value was 41 units (normal: 0 to 35 U) and 24-hour excreations of creatine in urine was 572 mg. Test for C-reactive protein was positive; antistreptolysis 0 titer (ASLO) was 480 Todd units; antistreptokinase titer (ASK) was 81,920 U; Electromyogram revealed a pattern of low amplitude neuromuscular units.
Clinical signs and symptoms suggested his disease as vascular purpura with polymyositis syndrome. Microscopical findings of the muscle showed to be uneven muscle fibers and wide-opened endomysim. They were compatible with polymyositis of vasculo-interstitial type.
Five months later, he suffered from glomerulonephritis.
Considering that the common pathognomonic factor between the vascular purpura and polymyositis is an angitis, it could be presumed that the infection with hemolytic streptococcus caused the angitis as well as glomerulonephritis.
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Nobuo NARA, Tadaaki MIYAMOTO, Akira KURISU, Hiroshi TSUNEMOTO, Kazuyas ...
1980 Volume 21 Issue 12 Pages
1944-1950
Published: 1980
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Two siblings with Fanconi's anemia developing squamous cell carcinomas were reported.
Case 1, a 23-year-old male, was diagnosed as Fanconi's anemia in November, 1975. He was in a small stature with small head, hyperpigmentation, severe pancytopenia and chromosomal abnormalities (chromatid breaks, exchanges, gaps, etc.). He had been treated with methenolone acetate until June, 1979, when carcinoma of the right lower gum was noticed. With irradiation therapy by electron and fast neutron beams, tumor regressed gradually. He was well under follow-up in April, 1980 at present.
Case 2, a 14-year-old female and an elder sister of the patient in case 1, was diagnosed as aplastic anemia in July, 1969. Treated with prednisolone followed by methenolone acetate, she died of gingival and esophageal carcinomas 7 years later.
Consanguinity of their parents was noticed. The occurrence of Fanconi's anemia associated with squamous cell carcinomas in two siblings suggested genetic factor as an etiology of the disorders. The pathogenesis of congenital aplastic anemia and oncogenesis were discussed.
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Kunio KONDO, Chizuko ISHINO, Shigeki OHTAKE, Shintaro SHIOBARA, Kosei ...
1980 Volume 21 Issue 12 Pages
1951-1958
Published: 1980
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Autologous bone marrow transplantation (Auto-BMT) was carried out a 27 year old man with stage IV malignant lymphoma of 4 months' duration. This procedure was performed under bioclean room therapy, after high dose cyclophosphamide (CY), 120 mg/kg and 1,000 rad total-body irradiation (TBI). The autologous marrow was removed 2 months before BMT, gradually frozen in the anticryopreservation and kept in liquid nitrogen until use. Just before use, the cryopreserved marrow was thawed rapidly, 2 times washed and intravenously infused to the patient. Sequential blood study showed WBC of 0 on day +5, but WBC of over 1,000 on day +21. He has survived from gangrenous stomatitis and acute hepatitis by intensive supportitive therapy such as hyperalimentation, fractionated blood transfusion, intravenous immunoglobulin, etc. He is now healthy with no chemotherapy, approximately 6 months after BMU. This is the first successful cases in Japan of Autp-BMT aimed to cure the patient. In summation, to malignant tumors with no marrow invasion by tumor cells and with hypersensitivity to drugs or TBI, Auto-BMT would be the most potential therapeutic weapon.
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Tatsuro KISU, Tsunefumi SHIBUYA, Shozaburo JIMI, Yoshiyuki NIHO, Keita ...
1980 Volume 21 Issue 12 Pages
1959-1965
Published: 1980
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A case of myeloproliferative syndrome manifested by fever, hepatosplenomegaly and pancytopenia was described. This 21 year-old Japanese female patient had a outcome of fatal course after nine months in spite of various treatments. The diagnosis of this case was very difficult although many investigations were done. We had suspected malignant lymphoma or malignant reticulosis, but could not diagnose definitely before autopsy.
There was marked hepatosplenomegaly, but no evidence of the swelling of lymph nodes were seen. The autopsy showed neither tumor formation nor focus of infection, and bone marrow revealed a compact red marrow. The microscopic examination revealed the marked increase of erythroblastic, granulocytic and megakaryocytic series in the bone marrow, and marked infiltration of mature neutrophils and erythroblasts in the sinusoid of the liver. This was also seen in the spleen and lymph nodes.
These findings were compatible to the myeloproliferative syndrome advocated by Dameshek in 1951. This syndrome includes acute and chronic granulocytic leukemia, polycythemia vera, acute and chronic myelosclerosis with myeloid metaplasia, Di Guglielmo syndrome, thrombocythemia and other disorders of the proliferation of one or more marrow constituents. In this context, this rare case was included into myeloproliferative syndrome but did not belong to any single entity of this syndrome.
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