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Takeshi NOMA, Kuniteru KOU, Izumi YOSHIZAWA, Yutaka KAWANO, Masahiko I ...
1992 Volume 33 Issue 12 Pages
1809-1817
Published: 1992
Released on J-STAGE: April 24, 2009
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Virus-associated hemophagocytic syndrome (VAHS) is a nonneoplastic, generalized histiocytic proliferation disorder with marked hemophagocytosis associated with a systemic viral infection. We descrive a female child with EBV-related VAHS, in whom Southern blot analysis showed monoclonal proliferation of bone marrow cells having EBV genome as detected with Xho-1 fragment of latent infection membrane protein (LMP) genome. The EBV serology showed anti-EBNA, anti-VCA-IgG, anti-VCA-IgA elevation and positive EBNA of SRBC-rosette forming bone marrow cells in late period of her clinical course, which indicated primary infection or secondary alteration of EBV immunity. The DNA analysis of the bone marrow cells also demonstrated monoclonal rearrangement of T cell receptor-β and -γ chain genes. Thus our study suggests that EBV might infect T cells and the T cells might proliferate monoclonally. Repeated administration of VP16 was capable of inducing remission of the disease, although adrenocortical steroid, vincristine and cyclophosphamide were administrated unsuccessfully. This is the first case of VAHS in which the monoclonal proliferation of EBV-infected T cells was demonstrated.
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Eishi ASHIHARA, Nariaki OKU, Akira NISHIO, Katuhiro TUJI, Mituo NAKAMU ...
1992 Volume 33 Issue 12 Pages
1818-1823
Published: 1992
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A 64-year-old female was admitted for treatment of refractory myeloma (IgG-λ). Because of severe liver cirrhosis, the patient was treated with interferon (IFN) alone (natural IFN 6×10
6 IR/day i.m. for 28 days). Pneumonia developed during IFN therapy. The IFN therapy was completed, restoring suppressed IgM and IgA to their normal ranges, while pneumonia was cured by antibiotics. Because the M-component remained, an additional IFN therapy was resumed and M-protein disappeared. As the period within which the M-component disappeared in this case was shorter than that reported previously, we supposed that the pneumonia might have enhance the effects of IFN. To verify this, we administered OK-432 for 4 weeks as a model of immunoactivation by pneumonia between two successive courses of IFN therapy when M-protein reappeared. To monitor the immune state, natural killer (NK) and lymphokine activated killer (LAK) activities were measured: NK activity suppressed by IFN was restored by OK-432 and was suppressed less by subsequent IFN administration. LAK activity was increased by IFN and OK-432. These observations suggested synnergic effects of OK-432 on IFN-activated immunity. This case suggests that IFN combined with immunotherapy may be effective in some cases of myeloma.
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Toshiya OKUBO, Norio ASOU, Hitoshi SUZUSHIMA, Shintaro MATSUMI, Mitsuh ...
1992 Volume 33 Issue 12 Pages
1824-1828
Published: 1992
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Six of 14 patients with acute myeloblastic leukemia (AML) complicated reactive histiocytosis during initial remission induction therapy. All six patients had a high fever without signs of infection during initial chemotherapy, and periods of myelosuppression were prolonged. Histiocytes with a mature appearance, some of which phagocyted erythrocytes, thrombocytes or neutrophils, increased in the bone marrow. All of 3 patients tested showed high serum levels of ferritin. Two of 3 patients treated with 125 mg/day methylprednisolone achieved complete remission. In the remaining 3 patients, one patient achieved complete remission, but the others died of fungal pneumonia or sepsis. Thus, reactive histiocytosis is one of the severe complications in patients with AML undergoing chemotherapy.
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Harue YOKOO, Yutaka OKADA, Kazunori TOMINAGA, Morifumi TSUJI, Toshiyuk ...
1992 Volume 33 Issue 12 Pages
1829-1833
Published: 1992
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The chromosome der(1;7)(q10;p10) is a derivative chromosome consisting of the short arm of chromosome 7 and the long arm of chromosome 1. We observed this abnormality in three patients with acute myeloblastic leukemia (AML), myelodysplastic syndrome (MDS), or myeloproliferative disorder (MPD). Case 1 was a 76-yr-old male with a history of IgG myeloma treated with melphalan, cyclophosphamide, vincristine, and prednisolone (MEVP). AML-M1 developed one and half years after discontinuation of the MEVP therapy. Case 2 was a 39-yr-old male with MDS. Case 3 was a 56-yr-old male with refractory anemia with excess of blasts in transformation that evolved from primary myelofibrosis. Chromosome analyses revealed der(1;7)(q10;p10) in bone marrow cells of the three patients. All patients failed to respond to chemotherapy, and died within four months after the diagnosis. Thus, der(1;7)(q10;p10) may indicate a very poor prognostic outcome in patients with malignant hematologic disorders.
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Takashi MURATA, Hiroshi HARANO, Yoshimi HASHIMOTO, Michio MATUZAKI, Hi ...
1992 Volume 33 Issue 12 Pages
1834-1838
Published: 1992
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A 73-year-old man was admitted with severe abdominal fullness. Physical examination suggested much ascites. Laboratory data revealed that BUN was 39 mg/d
l, Cr was 2.2 mg/d
l, UA was 19.7 mg/d
l, and LDH was 1,569 U/
l. In ascites, there were many cells with large nuclei and many vaculoes. Immunocytological characterization indicated that most of these cells were B lineage. And diagnosis of malignant lymphoma, the patient was treated with VEPA therapy. However, he soon died with acute renal failure. We examined the possibility of gene rearrangements between the
c-myc and immunoglobulin genes and found that
c-myc gene was rearranged at the first intron, and joined with immunogulobulin J
H locus as head to head. These results suggested that the patient carried Burkitt's lymphoma of the American type.
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Takashi MIZUGUCHI, Hisayuki KOHNO, Makoto TAKISHITA, Masaaki KOSAKA
1992 Volume 33 Issue 12 Pages
1839-1844
Published: 1992
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A case of malignant histiocytosis with rearrangements of both T-cell receptor and immunoglobulin genes. The patient was a 69 year-old woman suffering from high fever, which was unresponsive to the administration of various antibiotics and steroids for more than two weeks. Laboratory findings on admission revealed disseminated intravascular coagulopathy and liver dysfunction. The bone marrow examination showed an increased number of giant cells. Some of the giant cells had phagocytosis of various blood cells and were cytochemically stained with non-specific esterase, but not with myeloperoxidase and PAS. Immunohistochemical study revealed that α
1-antitrypsin α
1-antichymotrypsin, lysozyme and CD15 were all detected in the cytoplasm of some giant cells while CD30 was not detected. Of interest was the rearrangements of the T-cell receptor, Ig heavy chain and κ chain genes on bone marrow mononuclear cells demonstrated by Southern blot analysis.
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Akitoshi HANAMURA, Hideaki MAEDA, Wakako KUWAYAMA, Yasuo TAKANO
1992 Volume 33 Issue 12 Pages
1845-1850
Published: 1992
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An 80-year-old male was admitted to our hospital because of multiple tumors in October 1989. A pathological diagnosis of non-Hodgkin's lymphoma (diffuse, medium-sized cell type) was made with the histological examination of his biopsied tumors. His clinical stage was stage IV A. Chemotherapy (CHOP) brought him to complete remission. In May 1990, relapse of non-Hodgkin's lymphoma was observed in the neck and both eyes. The diagnosis of uveitis due to involvement of lymphoma cells was confirmed by an aspiration biopsy of left aqueous humor. Complete recovery of his visual aquity was possible with additional chemotherapy, and lymphoma cells in his right aqueous humor were expelled completely, while his cervical tumor remained 1/3 of the original size. He eventually died of pneumonia on December 9th, 1990. Intraocular involvement of malignant lymphoma is rare in comparison with extraocullar involvement. Especially, the patient who has only turbid aqueous humor as an ophthalmic sign is very rare. In this paper, we reported a case of lymphoma with intraocular involvement and discussed the mechanisms of infiltration of lymphoma cells into the aqueous humor and the therapy.
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Haruki KONDO, Toyotaro TAKASO
1992 Volume 33 Issue 12 Pages
1851-1856
Published: 1992
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A 56-year-old man had a leiomyosarcoma of the small intestine in 1987. After surgery, he received cyclophosphamide for 2years. In December, 1990, he exhibited severe pancytopenia. His hematological data were as follows: Hb 7.4 g/d
l, ret. 0.8%, WBC 1,700/μ
l with leukoerythroblastosis and 2.8×10
4/μ
l platelets. A bone marrow aspiration was a dry tap. A bone marrow biopsy specimen showed a hypercellular marrow with myelofibrosis, leukemic infiltration (10.2%) and slight dyserythropoiesis. Both PPO and GPIIb/IIIa reaction were positive for blast cells and atypical megakaryoblasts. A diagnosis of MDS with an abnormality in megakaryocytic lineage was made. The patient was treated with 1,25-dihydroxy-vitamin D
3, however this therapy was temporary and he developed into acute megakaryoblastic leukemia (M
7). This report suggested that some cases of therapy-related leukemia (TRL) mainly involve megakaryocytic lineage and are diagnosed as MDS with myelofibrosis which transform to M
7. The fact that PAS stain of erythroblasts in the patient reported here was positive may suggest involvement of a stem cell capable of aberrant differentiation along each of these pathyways. In the near future, the development of more precise immunological markers of differentiation and EM study will permit better diagnosis of TRL and may therefore facilitate new therapeutic approaches.
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Tadatsugu SATO, Yoshihisa WAKABAYASHI, Toshimi SATO, Tetsuo NISHIKAWA, ...
1992 Volume 33 Issue 12 Pages
1857-1862
Published: 1992
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In myeloproliferative disorders, aggravation of splenomegaly was reproted as an adverse effect of erythropoietin (EPO). Recently, we experienced the adverse effect of EPO in myelodysplastic syndrome (MDS). A 65-year-old male was admitted to our hospital for scrutiny of pancytopenia in July 8, 1991. He was diagnosed as having MDS (refractory anemia: RA). After discharge, daily subcutaneous administration of EPO (3,000U) was started on August 1 because his Hb concentration had decreased to 9.2 g/d
l. After the daily dose of EPO was increased up to 6,000U in August 15, left hypochondralgia gradually developed. EPO administration was haltedon August 22. His splenomegaly was aggravated from 2 finger breadths below the left costal margin before EPO administration to 4.5 finger breadths. Bone marrow examination revealed a change to extremely hypercellular marrow from slightly hypocellular marrow before EPO administration. The peripheral blood cell count was not altered. We concluded that he was a rare case of MDS in which aggravation of splenomegaly was observed, probably as a result of extramedullary hematopoiesis induced by administration of EPO.
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Shin FUJISAWA, Michio MATUZAKI, Hiroshi HARANO, Shigeki MOTOMURA, Taka ...
1992 Volume 33 Issue 12 Pages
1863-1868
Published: 1992
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A 57-year-old man, diagnosed as Polycythemia vera (PV), had been treated with administrations of Busulfan since 1984. Three years later, the number of neutrophils in peripheral blood increased to 50,000/μ
l with progression of splenomegaly, and the case was diagnosed as Chronic neutrophilic leukemia (CNL) based on the criteria by Miura et al, in November, 1989. In spite of 6MP and Busulfan therapy, marked neutrophilia and splenomegaly progressed, and the patient died due to liver dysfunction in June of 1991. To clarify the pathophysiolosy of PV and CNL, we studied the
in vitro growth kinetics of hematopoietic progenitor cells in bone marrow of this unique case and made a comparison with those of 4 cases of PV and 4 normal volunteers employing methylcellulose culture. As in other cases of PV, erythroid colonies were formed in culture of bone marrow from this patient without addition of erythropoietin. Furthermore, spontaneous colonies derived from CFU-GM and CFU-Mix increased remarkably in this case only. The results suggest that the hematopoietic abnormalities in this case involve the multipotent stem cells as well as erythroid and granuloid-macrophage progenitors.
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Fumiaki NAKAJIMA, Kazushi TAKAYA, Shigeyoshi HIBI, Shinjiro TODO, Shin ...
1992 Volume 33 Issue 12 Pages
1869-1874
Published: 1992
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A 9-year-old boy with chronic granulomatous disease was hospitalized on May, 1991, because of continued fever and pain in the right elbow. Increased bone intensity at the distal end of right humerus on x-ray and a 33×36mm space-occupying lesion in the spleen on abdominal CT scan were recognized. Under a diagnosis of periosteitis and spleen abscess, intravenous infusion of rhG-CSF at a dose of 200 μg/m
2/day was started in combination with antimicrobial therapy. Fever, tenderness, swelling in the right elbow and inflammatory indices improved three weeks after the institution of therapy. Hydrogen peroxide (H
2O
2) formation by neutrophil increased significantly, although intermittently, during the therapy. The spleen abscess had completely vanished on CT scan on February, 1992. The therapy was well tolerated and no significant side effects were observed. The use of rhG-CSF in combination with potent antibiotics is recommended for patients with serious infections in chronic granulomatous disease to avoid a fatal course.
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Junji SUZUMIYA, Eiji MORIOKA, Nobuhiro KIMURA, Morioki ISHIBASHI, Tosh ...
1992 Volume 33 Issue 12 Pages
1875-1879
Published: 1992
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We report a case of an acute promyelocytic leukemia patient who showed remarkable thrombopoiesis prior to granulopoiesis by human macrophage colony-stimulating factor (hM-CSF) treatment after chemotherapy. A 50 year-old man was diagnosed as acute promyelocytic leukemia (FAB classification: M3) with t(15;17). He received two courses of induction therapy with daunorubicin and cytarabine followed by consolidation therapy with cytarabine and mitoxantrone. After each course of chemotherapy, hM-CSF (8×10
6 units, daily) was given for 14 days. After each treatment with hM-CSF, the increase in number of platelets preceded increase of granulocytes and reticulocytes. When serum levels of granulocyte-macrophage-colony stimulating factor (GM-CSF), granulocyte-colony simulating factor (G-CSF), interleukins-3 (IL-3) and -6 (IL-6) were measured, only that of G-CSF was increased after hM-CSF treatment.
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Tetsuya TSUKADA, Nadim MAHMUD, Masanori TANIGUCHI, Takahiro TAKAHASHI, ...
1992 Volume 33 Issue 12 Pages
1880-1883
Published: 1992
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A 41-year-old man with untreated acute promyelocytic leukemia (APL) was treated with all-trans retinoic acid (ATRA) 80 mg/body/day per os. Complete remission was reached in 16 day without bone marrow hypoplasia and aggravated disseminated intravascular coagulation. The chromosomal abnormality, t(15;17), which presented before therapy has not been found since the 29th day of therapy. During the course of induction therapy with ATRA, there was no complication worth of mentioning. Induction therapy with ATRA is thought to be more effective and safer than conventional chemotherapy to attain complete remission in APL. The complete remission has been maintained for 11 months with conventional postremission chemotherapy.
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Eiji SADA, Kosuke YANAGISAWA, Hitoshi HASEGAWA, Shigeru FUJITA, Yuzuru ...
1992 Volume 33 Issue 12 Pages
1884-1889
Published: 1992
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A 36-year-old male was admitted to the Ehime University Hospital with anemia, eosinophilia and hepatosplenomegaly. Peripheral blood examination demonstrated severe anemia (Hb 7.1 g/d
l), thrombocytopenia (Plt 6.8×10
4/μ
l) and increase of peripheral leukocytecounts (53,000/μ
l) with 32.0% of eosinophils which had lobulated nuclei, abnormal distribution of eosinophilic granules and a few vacuoles. The level of serum IgE was low (<5IU/m
l), while that of serum vitamin B
12 was elevated. A diagnosis of eosinophilic leukemia was made. He was noted to have spontaneous fluctuations in his eosinophil and total leukocyte counts. To analyze the mechanism of cyclic eosinophilic leukocytosis, we examined eosinophil colony stimulating activity of the serum and plasma of the patient. These examination showed that eosinophil colony-stimulating activity was not found in his serum and plasma, and cyclic eosinophilic leukocytosis was due to the hemopoietic stem cell disorder.
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Sachiko ANDO, Seisho ANDO, Norito OTANI, Tadashi KANBE
1992 Volume 33 Issue 12 Pages
1890-1894
Published: 1992
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58-year-old man was admitted with cervical tumor and leukocytosis. Physical examination indicated splenomegaly and cervical abscess. Laboratory data showed WBC 55,000/μ
l, Hb 7.9 g/d
l, and PLT 4.5×10
4/μ
l. After cure of the abscess, WBC counts were still high with 1,500-2,000/μ
l monocytes, and anemia and thrombocytopenia persisted. Bone marrow aspiration showed myeloid hyperplasia and trilineage myelodysplasia. The Ph
1 chromosome could not be detected. The case was diagnosed as chronic myelomonocytic leukemia and treated with oral etoposide (25 mg/day). After 2 weeks, the dose was increased to 50 mg, and then modified according to the blood counts. WBC counts are presently being maintained between 7,000 and 12,000/μ
l, and RBC and PLT counts have gradually become normal. Splenomegaly almost disappeared and dysplastic change in bone marrow improved somewhat. At nine months following the start of chemotherapy with etoposide, remission is maintained by treatment with 25 mg of etoposide on alternate days. This case suggests that low-dose etoposide is useful for treating CMMoL.
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