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Naoya SAKAMOTO, Yoshitaka AOYAGI, Yukio OZAKI, Masaaki KANAYAMA, Yuji ...
1990 Volume 31 Issue 2 Pages
172-176
Published: 1990
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A thirty-eight-year-old man developed gradually progressing right retroorbital pain, diplopia on the left lateral gaze, and left ptosis. On examination paresthesia was present on the first division of right trigeminal nerve. Orbital venography revealed obstruction of right superior orbital vein on the entering portion to the cavernous sinus. A daily administration of 30 mg of prednisolone resulted in a rapid improvement of the symptoms. Diagnosis of Tolosa-Hunt syndrome was made on the basis of neurological symptoms, roentogenographic findings and responsiveness to prednisolone. One year later, weakness of right leg accompanied with left cervical and axillary lymphadenopathy was developed. A biopsy specimen of cervical lymph node was identical with the Hodgkin's disease, lymphocyte predominance type. He was treated with COPP regimen; lymphadenopathy decreased in size. We reported a rare case of Hodgkin's disease preceded by Tolosa-hunt syndrome which might be caused by the extranodal lesions.
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Takasi MURATA, Sigeki MOTOMURA, Hirosi HARANO, Heiwa KANAMORI, Hiroko ...
1990 Volume 31 Issue 2 Pages
177-182
Published: 1990
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We describe a patient with CML who developed hypercalcemia in his course of blast crisis.
A 25-years-old man was diagnosed as CML with priapism in April 1985, and controlled with BHAC-DVP, VMP, busufan therapy. In Decenber 1987, he readmited to our hospital with abdominal pain. Investgations at that time showed: white blood cell count 11600/μ
l (blast cells 9%); hemoglobin 8.4 g/μ
l; platlets 19.0×10
4/μ
l; serum calcium 13.2 mg/d
l; BUN 44 mg/d
l; creatinine 2.7 mg/d
l.
Treatment with predonine, 6-MP and vincristine was begun. But serum calcium level rose gradually up to 16.5 mg/d
l. So we tried middle dose Ara-c therapy, serum calcium decreased to 6.8 mg/d
l.
At once he was in a chronic phase, but he relapsed and died of heart failure.
Necropsy showed extensive leukemic blast-cell infiltration of the bone marrow, liver, spleen, lung, and kidney.
The cause of hypercalcemia in our case was suspected of local osteolitic hypercalcemia, because multiple bone destruction was found.
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—A Case Report and Its Gene Analysis—
Yukio HATTORI, Yasuhiro YAMASHIRO, Kiyomi YAMAMOTO, Machiko MORISHITA, ...
1990 Volume 31 Issue 2 Pages
183-188
Published: 1990
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Hereditary HbH disease was found in a Japanese family. The propositus showed hypochromic microcytic anemia and chronic hemolysis. HbH inclusion bodies were detected in red cells, and an abnormal band corresponding HbH was found in an isoelectric focusing of the hemolysate.
Gene analysis of the propositus revealed double heterozygosity for α
+ and α
0 thalassemias. Four of six his siblings shared the α
0 thalassemia and one the α
+ thalassemia. Another one was normal. The α
+ thalassemia was of 3.7kb-deletion type and α
0 was close to Southeast Asian type.
This is the fourth Japanese family with hereditary HbH disease.
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Hiroshi KOJIMA, Yuichi HASEGAWA, Kazuko SHIBUYA, Masaki NAKAZAWA, Yasu ...
1990 Volume 31 Issue 2 Pages
189-193
Published: 1990
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A 55-year-old man was admitted to our hospital for the evaluation of neutropenia. On physical examination, he had apthae and splenomegaly. CBC showed 1,000/μ
l WBC with 5% neutrophils, and microcytic anemia consistent with iron deficiency. Bone marrow examination revealed a marked decrease of mature neutrophils, but normal percentage of immature myeloid cells. There was no morphological abnormality in the hemopoietic cells. He had no drug or family history responsible for the neutropenia. Anti-neutrophil auto-antibody was negative. Hence, a diagnosis of chronic idiopathic neutropenia (CIN) was made. He developed frequent episodes of infection such as penitis, peri-anal infection, gingivitis, and pharingitis. He was treated with steroid pulse therapy, anabolic hormone, and high dose γ-globulin infusion, but no significant improvement occured. Then, recombinant granulocyte-colony stimulating factor (rG-CSF) was started. The neutrophil count was normalized by the 7th day of 5 μg/kg/day rG-CSF administration. The administration of G-CSF was discontinued after a 14-day course. Thereafter, the neutrophil count remained at near normal level (approximately 1,500/μ
l) and there have been no episodes of infection in the last 5 months. However this cannot be explained simply by the direct effect of rG-CSF on the myeloid precursors; rather, it suggests some unknown effect of G-CSF on the bone marrow microenvironment regulating myeloid hemopoiesis. We consider this to be a rare case of CIN with frequent episodes of infection, which was successfully treated with G-CSF.
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Hiroyuki NOZAKI, Keiko TANAKA, Toshihiko KUBOTA, Mitsumoto KOMATSUDA, ...
1990 Volume 31 Issue 2 Pages
194-197
Published: 1990
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A 41-year-old female, who had been diagnosed as having paroxysmal nocturnal hemoglobinuria (PNH) in 1985, was admitted to our hospital with cough, fever, diarrhea, vomiting and palpitation in April 1988. The chest X-ray showed pneumonia in her right lung. In the peripheral blood, red blood cell count was 1.64×10
6/μ
l, hemoglobin 4.7 g/d
l and reticulocytes 19%. The levels of serum LDH, indirect bilirubin and creatinine were high. Pneumonia improved by the administration of antibiotics, however, anemia and renal failure deteriorated. After washed red blood cell transfusions totalling 2,000 m
l and six times of hemodialysis, renal function returned to normal. This patient with PNH appeared to have developed acute renal failure by dehydration and hemolytic crisis due to pneumonia.
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Mari AKIBA, Yasuyuki KUNIEDA, Shiro MAEDA, Makoto OITA, Mihiro OKABE, ...
1990 Volume 31 Issue 2 Pages
198-203
Published: 1990
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The anti-tumor therapy followed by tumor lysis syndrome may cause the metabolic disorders including hyperkalemia, hyperphosphatemia and hyperuricemia. It should be known that it occurs frequently in lymphoproliferative diseases, especially in Burkitt's lymphoma.
Two cases of T-ALL accompanied by this syndrome, from which the patients were recovered, at the induction therapy of the first complete remission are reported here.
Case 1. A 28-year-old man received VP therapy under the diagnosis of T-ALL with massive hepatosplenomegaly and bilateral enlarged kidneys. During the therapy, metabolic disorders with both renal failure and ventricular tachycardia happened. They were resolved by certain series of treatments. The patient was brought to a complete remission with normal size of liver, spleen and kidneys.
Case 2. A boy aged 15 having received the intrasubarachnoidal infusion of MTX and 1-Ad-VP therapy under the diagnosis of T-ALL accompanied by this syndrome which was improved by an appropriate treatment, and the patient was lead to the remission.
The risk factors of this syndrome. such as 1-high drug sensitivity of the tumor; 2-renal dysfunction; 3-rapid cytokinetics of the tumor cell; 4-bigger size of the tumor, as well as the preventive treatment of this syndrome are reviewed.
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Yuji SATO, Akira SHIBUYA, Hideki KUDO, Yamao ADACHI, Keishi KINUGASA, ...
1990 Volume 31 Issue 2 Pages
204-208
Published: 1990
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A 70-year-old man, who had been diagnosed as primary myelofibrosis in 1987 at Tokyo Womans Medical School, was admitted to our hospital because of left hip-joint pain in May 1988.
Physical examinations revealed marked hepatosplenomegaly and multiple ruddy papules on the skin. The peripheral blood showed marked leukoerythroblastosis and severe anemia with poikilocytosis.
Bone marrow aspirations were dry tap. Needle biopsy of iliac crest showed a diffuse fibrosis. Biopsy of the papules showed an extramedullary haematopoiesis.
He was treated with low dose Ara-C for seven days. Although hepatosplenomegaly and skin papules were reduced, he died of pneumonia a month later.
At autopsy, leukemic cells massively infiltrated into the multiple organs, including bone marrow. The cells were identified with megakaryoblast, as those were positive for factor VIII related antigen.
As far as we know, this is the third case of primary myelofibrosis transformed into acute megakaryoblastic leukemia.
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Hiroko SHIGEMI, Satoshi MURAKAMI, Nariaki OKU, Kunihiko ITOH, Naohisa ...
1990 Volume 31 Issue 2 Pages
209-213
Published: 1990
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A 43-year-old man was admitted to our hospital because of legs edema and periorbital edema in Dec. 1983. Laboratory findings showed massive proteinuria (3.7 g/day), Bence Jones protein (BJP) in urine, and hypoproteinemia. Peripheral blood examinations were normal and a bone marrow aspiration showed hypocellularity with slight increase of monocytes and plasma cells. Serum immunoelectrophoresis showed two M-components (IgGκ, IgAλ). Serum IgG was 1,690 mg/d
l, IgA 379 mg/d
l and IgM 160 mg/d
l. No remarkable findings were obtained in bone survey, Ga-scintigraphy and rectal biopsy, and a diagnosis of diclonal gammopathy with nephrotic syndrome was made.
In Aug. 1986, serum IgA started to increase rapidly with conocomitant decrease of IgG. He died of pneumonia due to pancytopenia in Dec. 1986, when serum IgG was 450 mg/d
l, IgA 1,014 mg/d
l, and IgM <39 mg/d
l. Immunoelectrophoresis showed two M-components (IgGκ, IgAλ) in serum and BEP (κ, λ), IgG (κ) and IgA (λ) in urine. An autopsy showed massive infiltration of myeloma cells which were positive for λ light chain in bone marrow, suggesting a development of myeloma from a diclonal gammopathy in about 3 years.
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Chizuko OHTA, Koushi OHISHI, Hiroshi ANAZAWA, Akinori ISHIHARA, Takao ...
1990 Volume 31 Issue 2 Pages
214-218
Published: 1990
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An 18 years old female was admitted to hospital due to pancytopenia on May 25, 1987 and found to have petechiae, mild hepatomegaly and severe splenomegaly. The bone marrow was found to contain 12% of blast cells. Splenectomy was performed followed by CHOP therapy. In September, 1987 the peripheral blood was found to contain tumor cells, which turned out to be resistant to various combination chemotherapies. The patient died on August 21, 1988.
The phenotype of tumor cells in this case was CD2
+ CD7
+ CD3
+ CD4
- CD8
- WT31
-. Genetic analysis detected rearrangement of the β and γ chain of TcR but not transcription or translation of the β chain of TcR, while the antibodies of δ TCS 1 and TcRδ 1 to the δ chain of TcR were positive. From this fact, the present case was considered to be the malignant counterpart of normal CD3
+ WT31
- double negative T cells. The reactivity of this tumor cells to IL-2 and IL-1β suggested the assosiation of the IL-2Rβ chain.
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Shinji IJICHI, Yuji SANO, Fumiho UNE, Ikuro MARUYAMA, Mitsuhiro OSAME
1990 Volume 31 Issue 2 Pages
219-223
Published: 1990
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Protease inhibiting agents, which have inhibiory effects on complement system, were used to treat a patient with autoimmune hemolytic anemia (AIHA) associated with B cell chronic lymphocytic leukemia (B-CLL). Although this patient had failed to respond thoroughly to prednisolone, the additional use of protease inhibitors induced a disease remission of hemolytic anemia. The result suggests that complement components are of importance in the pathogenesis of hemolysis in AIHA, and supports the accessory use of protease inhibiting agents in autoimmune hematologic diseases associated with the complement activation and hypersplenism.
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Masayuki KAWASAKI, Yujiro YAMANO, Yutaka KIKUCHI, Tatsuo SATA, Mine HA ...
1990 Volume 31 Issue 2 Pages
224-228
Published: 1990
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A 28-year-old man was admitted to our hospital because of cardiac tamponade and pleural effusion. Although his peripheral blood picture was normal, his pericardial fluid contained a lot of myeloblasts with Auer bodies and a diagnosis of acute myeloblastic leukemia (M2) was made from the finding of bone marrow smears. The pericarditis was successfully treated with instillation of mitoxantrone into the pericardial space after drainage. He responded well to the systemic chemotherapy and achieved a complete remission. Although 27 cases of leukemia complicated with cardiac tamponade have so far reported, only three cases gave normal findings of the peripheral blood on admission. Leukemic pericardial effusion with cardiac tamponade in very rare as an initial manifestation of AML. The efficacy and safety of pericardial infusion of mitoxantrone were demonstrated in this patient.
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Mieko MATSUI, Miwako MATSUZAKI, Yoshinori SHIMAMOTO, Mutsumi SHIGYO, M ...
1990 Volume 31 Issue 2 Pages
229-233
Published: 1990
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A case of non-Hodgkin's lymphoma with nephrotic syndrome and acute renal failure is described.
A 60-year-old Japanese male was admitted to our hospital in November, 1988, because of lymphadenopathy, fever, night sweat, weight loss. On physical examination, lymphadenopathy was present in both cervical, submandibular, supraclavicular, axillar and inguinal regions. The leukocyte count was 9,700/μ
l with 85% neutrophils and 2% atypical lymphocytes. Renal function was normal. Lymph node biopsy showed non-Hodgkin's lymphoma of diffuse, large cell type. Immunohistologic examination showed T cell type.
A few days later, he fell into acute renal failure. After systemic chemotherapy, he showed prompt improvement in renal function. Echo and computerized tomography (CT) of abdomen revealed no compression of the ureters and bladder. Renal biopsy findings suggested mesangium proliferlative glomerulonephritis without invasion of tumor cells.
Our case seemed to be rare and was compared with previous reports.
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Kazuya SHIMODA, Yuji YUFU, Hiroyuki TAKAHIRA, Masahiro YAMAMOTO, Hiros ...
1990 Volume 31 Issue 2 Pages
234-238
Published: 1990
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It is quite rare that aplastic anemia except Fanconi's type occurs in members of a single family. Here we reported two patients with aplastic anemia, a 14-year-old female and a 49-year-old her mother. In both cases, hematological findings were compatible to that of a typical aplastic anemia. In the case of daughter, CFU-E formation of control bone marrow cells was significantly inhibited by the addition of the patient's bone marrow cells, suggesting the presence of cellular immune mechanism. In the case of mother, no inhibitory activity on CFU-C formation of control bone marrow cells was detected in the patient's serum, though the ratio of helper/suppressor T lymphocytes in the peripheral blood was increased. We could not sufficiently clarify the common immune mechanism causing aplastic anemia in both cases. It was noticeable, however, that both cases share HLA DR2, which is frequently found in patients with various autoimmune diseases and is belived to relate closely to the pathogenesis of autoimmune diseases. This evidence suggests the possibility that genetically inherited susceptibility may also be the important factor for causing aplastic anemia.
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Hideyo NATORI, Eiji NAKAMURA, Ken TANAKA, Katsunobu NAITO, Koichiro EG ...
1990 Volume 31 Issue 2 Pages
239-244
Published: 1990
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A 22-year-old female was admitted to our hospital because of general fatigue. The lymph nodes, liver and spleen were not palpable. She was without cutaneous lesions.
Haematological examinations revealed leukocytes 3,200/μ
l with 44% blasts of myelomonocytic origin, and platelets 15,000/μ
l. Bone marrow smears were hypercellular marrow with 51% blasts of myelomonocytic origin and focal involvement of mast cells. Serum histamine and vitamine B
12 level was high.
Mast cells were round with rounded or segmented nuclei. The nucleoli were inconspicious and the cytoplasm contained a number of metachromatic granules. Cytochemically, mast cells stained possitive for α-naphthol-AS·D-chloroacetate esterase and acide phosphatase, and negative for peroxidase, Sudan black B and α-naphtyl butylate esterase. In toluidine blue staining, mast cells had stained similarly with pH values from 2.5 to 6.5.
She was diagnosed as acute myelomonocytic leukemia with benign mastocytosis, and treated with BH·AC-DNP. A complete remission was obtained, but mast cells in the marrow did not decrease.
Relationship between leukemia and mastocytosis was not known, but it was suggested that mast cells responded to the proliferation of the leukemic cells.
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Masuhira SAI, Yoshikazu KAWANISHI, Yoshikazu ITOH, Shin AIZAWA, Takesh ...
1990 Volume 31 Issue 2 Pages
245-248
Published: 1990
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We present a patient with refractory anemia (RA) who developed Sweet's syndrome during the treatment of recombinant human granulocyte colony-stimulating factor (rhG-CSF). A 30-year-old man was admitted to the hospital for evaluation of anemia. He was diagnosed as MDS (RA). As a phase II study in MDS, rhG-CSF therapy was begun. Fever associated with cutaneous lesion developed over the left shoulder. Antibiotics showed no effects. skin biopsy revealed Sweet's syndrome. This skin lesion disappeared thoroughly with discontinuance of G-CSF and administration of prednisolone. To examine whether Sweet's syndrome was related to the G-CSF therapy, we analyzed the effect of G-CSF on the function of patient's neutrophils. However, the function of patient's neutrophils was not activated by G-CSF administration.
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Takashi SHIMAMOTO, Junko OHYASHIKI, Kenta UTSUMI, Kazuma OHYASHIKI, Ke ...
1990 Volume 31 Issue 2 Pages
249-253
Published: 1990
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We report here a patient who was diagnosed as having acute lymphoblastic leukemia (ALL-L2) and colon carcinoma simultaneously and a successful operation for the colon carcinoma was performed preceded to the treatment of ALL. The patient was a 43-year-old male who presented with acute abdomial symptoms and was diagnosed as ALL by the hematological examination and as colon carcinoma (well differentiated adenocarcinoma, Borrmann II) by colon fiberscope. The patient was underwent sigmoidcolectomy for colon carcinoma and was received antibiotics as well as recombinant human granulocyte colony-stimulating factor (rhG-CSF), Approximatelly two weeks later, the patient received AdVP (Adriamycin, vincristine, and prednisolone) for ALL and achieved a complete remission. It might be possible for some leukemia patients manifesting acute abdomial symptoms to perform surgical approaches, since the prognosis of leukemia patients improved and supportive therapies, including an application of rh-CSF, have developed.
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