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Kenji WATANABE, Takuro SHIMBO, Mareo NAITOU, Norio TANAHASHI, Masaru K ...
1989 Volume 30 Issue 12 Pages
2148-2151
Published: 1989
Released on J-STAGE: February 02, 2009
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A 34 year-old female was admitted because of anemia and leukopenia. Her bone marrow contained abundant blastic cells, which were histochemically positive for peroxidase and α-naphthyl butyrate esterase, but negative for ASD chloroacetate esterase. She was diagnosed as acute monocytic leukemia (FAB, M5a). Complete remission was achieved after the administration of BHAC, daunorubicin, 6MP and prednisolone, and she was discharged after consolidation therapies. But shortly later, she noticed hoarseness and erythematous nodules on her breast and abdomen. Though the examinations of peripheral blood and bone marrow did not show any abnormality, hoarseness rapidly worsened and she complained of dyspnea. X-ray and CT scan demonstrated narrowing of the trachea under the cricoid cartilage, and trans-tracheal biopsy revealed leukemic involvement. In addition, erythematous skin lesion showed the infiltration of leukemic cells by biopsy. Although radiation and chemotherapy was initiated, she died of pneumonia. We tried to discuss the laryngo-tracheal and skin involvement of acute monocytic leukemia as early symptoms of relapse.
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Minoru TAKAHASHI, Yoshihiro MOGI, Yoshiro GOTO, Nobuyasu TSUSHIMA, Yas ...
1989 Volume 30 Issue 12 Pages
2152-2156
Published: 1989
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A 63-aged woman with cutaneous T cell lymphoma successfully treated with local administration of tumor necrosis factor (TNF) was reported. She was admitted to our hospital because of tumors and subcutaneous nodules on her bilateral inner thigh. A pathological study of her skin of right inner thigh showed mononuclear atypical cells with hyperlobulated nuclei. In peripheral blood the same lymphoid cells were found. Immunohistochemical staining of these cells was positive for OKT-3 and OKT-4, but negative for OKT-8. No lymph node swelling and no visceral involvement were detected by a CT scan and a Echography of the chest and the abdomen. A diagnosis of cutaneous T cell lymphoma was made (stage IIb TNM classification). Althogh the chemotherapy of VEPA and CHOP was done, about 70% (PR) of the bilateral inner thigh tumors were retracted. Owing to the interstitial pneumonia aroused in the period of bone marrow suppression and cardiomyopathy after chemotherapy, we gave up further systemic chemotherapy. And then the local administration of TNF was done and the disappearance of the bilateral inner thigh tumors was obtained. Our therapy with local administration of TNF for CTCL in the first report.
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Ryosuke ITO, Kyoichi KAWAUCHI, Tsutomu TOKI, Mikio KASAI, Koji ARAI, T ...
1989 Volume 30 Issue 12 Pages
2157-2162
Published: 1989
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A 7-year-old boy was admitted to our department complaining pale face and subcutaneous bleeding in August, 1987. Peripheral blood analysis showed pancytopenia of WBC 2,600/μ
l, RBC 148×10
4/μ
l and platelets 5,000/μ
l. Bone marrow biopsy revealed hypocellularity. Granulocytes 104/μ
l, reticulocytes 4,290/μ
l and platelets 5,000/μ
l were compatible with the diagnosis of severe aplastic anemia based on the criteria of the Ministry of Pubric Welfare in Japan. Prednisolone (PDN) was initially indicated and bolus methylprednisolone, metenolone and ALG therapy followed with no hematological improvement.
Fifteen months after admission, in addition to 0.5∼1 mg/kg/day of metenolone, Cyclosporin A (CyA) was started at a dose of 12 mg/kg/day for a week and 6 mg/kg/day thereafter. After a week from administration of CyA, 1 mg/kg/day of PDN was given because his bleeding tendency became worse. But this combination was complicated with liver damage and hyperglycemia to discontinue both drugs. These adverse effects were subsided within 7 days by cessation of the drugs. CyA was started again at a dose of 6 mg/kg/day without any response for 4 weeks. Then PDN was added together at areduced dose of 0.5∼1 mg/kg/day. Hematological response was obtained promptly. Granulocytes reached 1,500/μ
l, hemoglobin 10.2 g/d
l and platelets 26,000/μ
l after 3 months of therapy. Afterward the patient became transfusion independent.
The most effective method of CyA administration for aplastic anemia is still controvertial. Alternative use of CyA, considering combination of steroids or anabolic steroids, in patients who failed to respond to conventional immunosuppressive treatments should be further investigated.
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Kanji SUGITA, Shinpei NAKAZAWA, Midori SAITO, Takaaki GONDA, Yutaka KU ...
1989 Volume 30 Issue 12 Pages
2163-2168
Published: 1989
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A 7-year-old girl with an acute leukemia was reported whose blasts showed conversion from a T-lymphoid to a myeloid phenotype. At the onset of the disease, the blasts were negative for peroxidase and displayed FAB L1 morphology. Surface marker analysis revealed only CD7 antigen. Although complete remission was achieved, an extramedullary relapse was identified as having a several subcutaneus tumors 15 months later. Tumor cells showed the same marker expression as that of the blasts at the onset. After short term culture without an addition of any differentiation stimulators, the blast cells expressed CD2, CD3, CD4, CD8, and CD25 antigens. The karyotype was 46, XX, t(12;21) (p11;q22). The intensive chemotherapy and radiation therapy were carried out, however, a hematological relapse occurred 12 months later. At this time, the blasts were strongly positive for peroxidase and expressed HLA-DR and CD33 antigens with disappearance of the CD7 antigen. Chromosome analysis revealed the additional abnormalities (del (7) (p15), -17, +der (17) t(17;?) (p13;?)).
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Noriko NAKAMURA, Kazuhiko KUME, Mayumi MORI, Masao NAMIKI
1989 Volume 30 Issue 12 Pages
2169-2172
Published: 1989
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Portal hypertension in chronic lymphocytic leukemia (CLL) is rare. A 64-year-old woman with CLL for 5 years and increasing hepatosplenomegaly developed portal hypertension and bleeding gastric varices. There was no portal vein thrombus by abdominal echography and angiography. Following splenectomy and devascularization of the fornix, the gastric varices disappeared. The liver biopsy showed dense leukemic cell infiltration in portal triads, but no fibrosis. The portal hypertension in this case may be mainly due to increased portal flow from the enlarged spleen and leukemic cell infiltration in the liver. Previously reported cases are summarized.
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Yoshio HATAE, Takeo TAKEDA, Hisaya NAKADATE, Yukiko HATAYAMA, Akira SA ...
1989 Volume 30 Issue 12 Pages
2173-2177
Published: 1989
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We have experienced and treated seven patients of pre-B cell leukemia in childhood. Clinical, cytological and ultrastructual characteristics of them were studied. Most of them had higher counts of white blood cells, hepatosplenomegaly, high value of lactic dehydrogenase and various karyotype abnormalities at onset. The chromosomal translocation t(1;19) that is supposed to be specific to pre-B cell ALL was found in four of seven of our cases.
In the seven patients, survival was studied in comparison to that of 27 common ALL patients at our hospital that are common in childhood acute leukemia. Although no difference in remission duration and survival time between pre-B cell ALL patients and common ALL group, there have been seen the tendency that remission and survival were of shorter duration for patients with pre-B cell ALL.
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Yoko TSUSHIMA, Tomotaka YUKITA, Takafumi MIKAMI, Hideki TAKAMI, Morio ...
1989 Volume 30 Issue 12 Pages
2178-2182
Published: 1989
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A 21-year-old man was admitted to our hospital because of anorexia and general malaise in July, 1988. On admission, the white blood cell count of 18,600/μ
l with 72% leukemic cells. The bone marrow aspirate showed 76.8% immature monocytes, 10% mature and immature eosinophils. Leukemic cells were 66.6% myeloperoxidase positive cells, and 20.6% naphtylbutylate esterase positive cells. The lysozyme activity in urine was high. Cytogenetic analysis revealed the presence of 46 XY inv (16) (p13q22). Under the diagnosis of acute myelomonocytic leukemia with eosinophilia (M4Eo) associated with inv (16) (p13q22), one course of DCMP induction therapy was performed. After complete remission, the bone marrow aspirate showed disappearance of inv (16) (p13q22), and associated with decreased residual leukemic cells
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Yutaka TAKAI, Hiromichi KOYA, Shigeru HIMEI, Hisatomo HAYASHI
1989 Volume 30 Issue 12 Pages
2183-2188
Published: 1989
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Acquired inhibitor of von Willebrand factor-platelet interaction occurring in a 57 year-old female has been partially characterized. She had no personal or familial bleeding tendencies, but presented a subcutaneous hematoma of recent origin. She was diagnosed as having an acquired von Willebrand syndrome because she had low levels of FVIII complex in plasma, with platelet adhesiveness to glassbeads and RIPA decreased.
This inhibitor was classified as an IgA immunoglobulin, and had no activity against any component of FVIII complex. The purified IgA by the chromatographic technology interacted with normal platelets to inhibit RIPA. Following 1-deamino-8-D-arginine vasopressin (DDAVP) infusion, she had higher immediate rise in all components of FVIII complex in plasma, with no rapid decline. Plasma von Willebrand factor (vWF) multimers analyzed by 1.5% SDS-AGE technology revealed to be identical with those of normal plasma.
These studies suggest that the abnormality of ristocetin-induced vWF-platelet interaction by IgA RIPA inhibitor and the reduction of all vWF multimers (like type IA von Willebrand disease) may have a relationship with the pathogenesis of bleeding diathesis in this case.
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—Effect of Anti-platelet and Anti-fibrinolytic Therapy—
Makoto TOMEOKU, Tsutomu NAKASE, Kouichi MORITA, Masami KATOU
1989 Volume 30 Issue 12 Pages
2189-2193
Published: 1989
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Case 1: 75 years old male was admitted to our hospital with anterior chest subcutaneous bleeding. Coagulation study revealed that fibrinogen and α
2-PI decreased, and FDP, FPA, Bβ 15∼42 and D-dimer increased. Case 2: 78 years old male was admitted to Shingu City Hospital with a left hip subcutanous hematoma. Coagulation study revealed that fibrinogen, ATIII and α
2-PI decreased, and FDP increased. US and CT showed abdominal true aneurysm in both cases. Either severe infection or malignancy was not found. Ticropidine and T-AMCHA were medicated for 8 days in case 1, and for 18 monthes in case 2. Symptom and coagulation study improved in these cases. Due to some side effects such as appetite loss and liver dysfunction in case 1, and diarrhea in both cases, we changed the therapy to mini-dose heparin therapy. This therapy also proved effective. It is concluded that antiplatelet and anti-fibrinolytic therapy are effective for chronic DIC with abdominal true aneurysm.
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Tadao ISHIDA, Mitsuru YOSHIMOTO, Akihiro ONO, Niwako SAITOH, Tatsumi N ...
1989 Volume 30 Issue 12 Pages
2194-2198
Published: 1989
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A 38-year-old woman was diagnosed as acute lymphoblastic leukemia (L2) in Oct. 1985. After VP and AdVEMP therapy, complete remission was obtained. In Oct. 1987, she noticed bilateral breast tumors and leukemic cell infiltrations were shown in a biopsy specimen of the breast tumor. Bone marrow was occupied with 94 percent blasts. The second complete remission was achieved by the AdVP therapy.
In Nov. 1988, she developed double vision and photophobia. The examinations of CT and MRI showed cavernous sinus tumor, and 20 percent blasts were recognized in a bone marrow aspirate. The leukemic cells were negative for peroxidase, but were positive for both lymphoid and myeloid cell surface markers (CD2, CD5, CD7, CD33). The two color flowcytometry showed that CD5 and CD33 were simultaneously expressed on the leukemic cells.
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Kazue TAKAI, Masayoshi SANADA
1989 Volume 30 Issue 12 Pages
2199-2204
Published: 1989
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A case of peripheral T-cell lymphoma presenting with secondary myelofibrosis and meningeal involvement is described.
A 65-year-old female was admitted because of remarkable weight loss and pancytopenia. On admission, she was confused and showed tiny cervical lymph nodes but no hepatosplenomegaly. Bone marrow aspiration resulted in dry tap and its biopsy showed remarkable myelofibrosis with marked decrease of hematopoiesis and increase of lymphoid cells. Lymph node biopsy revealed diffuse medium sized cell lymphoma, which was diagnosed as CD3
+4
+8
- peripheral T-cell lymphoma with immunohistochemistry (anti-HTLV-1 antibody negative). The lymphoid cells of bone marrow expressed the markers of T-cell lineage (LCA
+ UCHL1
+ MT1
+ L26
- MB1
-). The cerebrospinal fluid examination revealed many lymphoma cells. She was treated with CHOP regimen and intrathecal injection of MTX. After three months, bone marrow biopsy showed recovery of hematopoiesis and disappearance of lymphoma cells and reticulin fibers.
Immunohistochemical analysis of bone marrow specimen was useful for the diagnosis of atypical myelofibrosis.
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—Involvement of vWf and Endothelial Cell Injury—
Hideo TANAKA, Shizuyo KUSUMI, Motoyoshi TAKEMOTO, Shuji MAEHAMA, Kingo ...
1989 Volume 30 Issue 12 Pages
2205-2209
Published: 1989
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A 49-year-old man with a one-week history of general fatigue and several other symptoms, including hematuria, numbness of the mouth, anemia and thrombocytopenia, was admitted because of an episode of convulsions and unconsciousness after blood transfusion. A diagnosis of thrombotic thrombocytopenic purpura (TTP) was then made, and treatment with steroids, anti-platelet agents, transfusion of fresh frozen plasma was started. However, since no improvement was seen, on the third day of admission, treatment with plasma exchange was instituted (total plasma exchange volume was 18.1
l), and his clinical and hematological conditions improved markedly. Since then, he has been in a remission state for about three years. Laboratory examinations during the acute phase showed increase of vWf: Ag, decrease of RCof/vWf: Ag, increase of vWf large multimers and a high endothelial cell injury activity by the patient's serum. In the next day following the plasma exchange therapy, RCof/vWf: Ag improved, but not to the normal range. One and a half years later, while in the remission phase, the vWf multimers and endothelial cell injury activity normalized. Thus, these findings show further evidence on the involvement of endothelial cell injury and vWf in the pathogenesis of TTP.
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Hiroshi KOJIMA, Yuichi HASEGAWA, Masaki NAKAZAWA, Toshiro NAGASAWA, Ya ...
1989 Volume 30 Issue 12 Pages
2210-2214
Published: 1989
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A 32-year-old man with an 18-year history of protein losing enteropathy (PLE) was admitted to hospital for abdominal distention. On physical examination, he had massive pleural effusion, ascites and edema of the right leg, but no superficial lymphadenopathy or organomegaly. Laboratory studies revealed mild microcytic anemia and hypoproteinemia. α1-antitrypsin clearance was elevated (316 m
l/day). Examination of ascites disclosed numerous lymphoblastoid cells of B cell phenotype with μ chain and λ light chain of immunoglobulin (Ig) in the cytoplasm. Southern blot analysis showed monoclonal rearrangement of μ chain and λ chain genes. No evidence of lymphomatous involvement of lymph nodes and non-lymphoid organs was found by CT scan, ultrasound echography and gallium scan of the chest and abdomen. Bone marrow biopsy was negative. Thus, a diagnosis of non-Hodgkin lymphoma (NHL) stage IVB limited in the pleural and peritoneal cavities was made. He was treated with the combination chemotherapy of BACOD with high dose ara-C or methotrexate followed by 4 doses of autologous LAK cell infusion resulting in no significant response. The massive pleural effusion, ascites and edema of the leg have not been improved. We consider this to be a rare case of NHL associated with PLE which is extremely resistant to chemotherapy or LAK therapy.
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Taijiro MORI, Takashi ABE, Shinpei NAKAZAWA, Rintaro HASHIMOTO, Kayo M ...
1989 Volume 30 Issue 12 Pages
2215-2219
Published: 1989
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A 16 year-old boy of non-Hodgkin's lymphoma (NHL) was reported. Although Hodgkin's disease was suspected by the presence of Reed-Sternberg-like cells and lacunar cells histologically, a diagnosis of NHL was made because of atypism and monoclonality of the background's cells as well as the morphology of invasive cells in the bone marrow. The tumor cells expressed, CD2, CD3, CD4, CD5 and CD7 antigens, which corresponded to the phenotype of helper-inducer T-lymphocytes. In the analysis of their karyotypes, 16 out of 24 cells revealed normal karyotype, while all the rest showed near-triploidy. Common abnormality was identified as trisomies of No. 1, 3, 5, 16, 21 chromosomes, tetrasomies of No. 10, 19, 20 chromosomes, and 4q
+, 7q
+, 14p
+.
Multimodal chemotherapy was successful to induce the patient promptly into complete remission. He has been free from the disease for approximately 12 months.
Thus far, triploid clones in hematopoietic malignancies have rarely been described. More importantly, the appearance of them in pediatric lymphoid neoplasms has not yet been reported.
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Hideo NAKAMURA, Tohru JUBASHI, Itsuro JINNAI, Ippei SASAGAWA, Takahiro ...
1989 Volume 30 Issue 12 Pages
2220-2224
Published: 1989
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In February 1986, a 68-year-old woman was diagnosed as having acute myeloblastic leukemia (FAB-M1). At the time of diagnosis, 86.0% of the bone marrow cells were myeloblastoid, and 15% of these myeloblastoid cells were positive to myeloperoxidase. Surface marker analysis by flow cytometry disclosed granulocyte-associated antigen (MY7) and also lymphocyte-associated antigen (CALLA) on the leukemic cells. Chromosomal banding studies of bone marrow cells revealed trisomy 11 in 6 of 19 metaphases examined and normal karyotype in the others. Complete remission was attained after intensive combination chemotherapy, and has remained for 38 months.
Only 19 patients with trisomy 11-associated acute nonlymphocytic leukemia (ANLL) including the present case have been reported. Morphologic analyses have revealed that the frequency of FAB-M1 is high. However, except for the present case, surface marker findings were apparent in only one M5a patient, in whom monocyte-macrophage-associated antigen was detected. Accordingly, careful surface marker studies will be needed to clarify the frequency of acute mixed lineage leukemia in such patients.
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Miho TAKECHI, Kimio TANAKA, Chiharu SIGETA, Nobuo OGUMA, Nanao KAMADA, ...
1989 Volume 30 Issue 12 Pages
2225-2229
Published: 1989
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A case of acute myelocytic leukemia (AML-M2) with a late appearance of Philadelphia chromosome (Ph
1) is presented. Chromosome analysis revealed a normal karyotype at the time of diagnosis and for 23 months, when hematological relapse occurred, accompanied by abnormal clones, 46, XX, t(9;22)(q34;q11) (78%) and 45, XX, -16, t(9;22)(q34;q11), del(5)(q13q31)(22%). The patient died of GVHD after bone marrow transplantation. Molecular analysis confirmed
bcr gene rearrangement in the cells with Ph
1 coromosome. Acquisition of Ph
1 chromosome during the course of hematological malignancies other than CML is extremely rare. This case is undoubtedly important for the understanding of leukemogenesis and the evolution of leukemia clones. The authors discussed possible mechanisms of Ph
1 acquisition in the late stages of AML.
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