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Volume 29, Issue 9
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Jyunichi MIMAYA, Takeo FUJIMOTO, Takeo TAKEDA, Masanori YANAI, Michio ...1988 Volume 29 Issue 9 Pages 1357-1364
Published: 1988
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSFrom 1981 to 1984, 99 previous untreated patients with childhood ALL were stratified into poor prognosis group by initial age and WBC and were entered on high risk ALL protocol. Results and conclusions of long-term follow-up were obtained as follows:
1) Significant difference was observed for remission induction rate of group A (95.2%) in which early consolidation with HD-MTX-CF therapy+ADR was induced and group B (78.9%) in which CNS preventive therapy including 18Gy cranial irradiation and intrathecal MTX-HDC and cyclic maintenance therapy with ADR, VCR, CPM, PRD, MTX and 6MP.
2) The relapse rates were 59.3% in group A and 40% in group B, respectively. For the initial relapse site, simultaneous relapse at BM+CNS was observed in 31% of group A, while no simultaneous relapse was observed in group B. Testicular relapse was observed in only 2 cases in group A.
3) The rates of non-response and relapse were observed 90.9% in T-ALL (10/11). T-ALL group were extremely poor prognosis compared with unclassifid, common ALL (including pre-B) and B-ALL.
4) Significant difference was observed (p<.01) for disease free survival according to Kaplan-Meier method between group A 24.8±7.9% (76 months) and group B 51.5±12.5% (75 months). Significant difference was observed for event free survival between group A 23.4±7.5% (76 months) and group B 41.7±11% (75 months).
5) From the above findings, results of present long-term follow-up based on high risk ALL 811 protocol were never satisfactory. We should consider another treatment protocol as super high risk ALL for T-ALL, as well as modification HD-MTX-CF therapy schedule and induce of cranial irradiation in CNS preventive therapy.View full abstractDownload PDF (631K) -
Atsushi KOMIYAMA, Akira ISHIGURO, Taro AKABANE, Masahiro TSUCHIDA, Ich ...1988 Volume 29 Issue 9 Pages 1365-1370
Published: 1988
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSClinical effects of P-100 (human urinary colony-stimulating factor: CSF-HU) were studied in 28 patients with several types of chronic neutropenia of childhood, ranging in age from 3 months to 18 years. The patients were given P-100 at a dose of 2×105 U/kg/day for seven consecutive days, and their neutrophil counts were followed. In addition, changes in the bone marrow morphology and granulocyte-macrophage colony-forming cells (GM-CFU) were studied. Increases in neutrophil counts occurred after P-100 administration in 10 of the 19 patients who were enrolled for the estimation. Transient increases in neutrophil counts occurred during the first 1∼3 weeks in five patients. Repeated increases were seen in a cyclic fashion in five patients; three of them eventually recoverd from the neutropenia. The percentage of bone marrow neutrophilic cells and ratio of the mature neutrophils to younger forms increased, but the number of the GM-CFU did not increase 2 weeks after P-100 administration. Low-grade of fever and redness at the injection site occurred in two and in one of the patients, respectively, but they disappeared without any particular treatments. P-100 can increase neutrophil counts probably through its effects to increase the number and maturity of the bone marrow neutrophil precursors in some types of chronic neutropenia of childhood.View full abstractDownload PDF (450K) -
Yukiyoshi MORIUCHI, Shimeru KAMIHIRA, Masao TOMONAGA, Keizo YAMAGUCHI, ...1988 Volume 29 Issue 9 Pages 1371-1375
Published: 1988
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESS56 febrile episodes during neutropenic (<500/μl) phase of chemotherapy for acute leukemia were treated with an empiric antibiotic regimen “CAP” [third generation cephalosporin, aminoglycoside and piperacillin (or ticarcillin)]. Effect of the regimen was evaluated 48 or 72 hours after the start of the administration.
In case of no-effect empiric drip infusion therapy of amphotericin B was started, and patients who had abdominal complaints (diarrhea, abdominal pain etc.) were selectively treated wih regimen “EP” (ampicillin, high dose amikacin, cefoperazone and minocyclin). (“EP” designated for enterococcus and pseudomonas). Patients who had no abdominal complaints were alternatively treated with regiman “P” [high dose amikacin, cefoperazone and cefsulodin (or piperacillin or ticarcillin)]. (“P” for pseudomonas)
50 out of 56 episodes (89.3%) responded well; in 48 of the them (85.7%) patients became afebrile before neutrophil count recovered to more than 500/μl.
16 out of 20 patients (80.0%) who received the first inducation chemotherapy and encountered febrile episodes responded to this regimen.View full abstractDownload PDF (425K) -
—The Effect of Exchange Transfusion for Induction Therapy—Shinjiro TODO, Tesuo HASHIDA, Kentaro TSUNAMOTO, Noriko ESUMI, Fumihir ...1988 Volume 29 Issue 9 Pages 1376-1382
Published: 1988
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSHyperleukocytosis in acute leukemia has been reported to be associated with an increased risk of fatal complications such as intracranial hemorrhage, respiratory failure, or metabolic derangement through tumor lysis. We treated seven patients with acute leukemia (3 ALL & 4 ANLL) by intensive chemotherapy after leucocyte reduction by an exchange transfusion. The patients received exchange transfusions 1∼4 times until white cell counts (WBC) reduced to 20,000∼40,000/μl prior to induction chemotherapy. Although five of the seven cases obtained sufficiently decreased WBC and complete remission (CR), the duration of continuous complete remission was only three to ten months. During the transfusions, respiratory failure progressed in one patient, which caused to death, but none of other complications were seen in our patients.
An exchange transfusion is thought to be useful as supportive therapy to prevent the fatal complications in the pediatric acute leukemia patients with extremely elevated WBC at diagnosis. However, more intensive chemotherapy is necessary combined with cytoreduction procedure to improve the ultimate prognosis.View full abstractDownload PDF (557K) -
Takashi YOSHIDA, Shinobu NAKAMURA, Shigeki OHTAKE, Keiko ITOH, Kazumi ...1988 Volume 29 Issue 9 Pages 1383-1387
Published: 1988
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSIn a prospective, and randomized tiral, ABEP regimen (aclacinomycin, n4-behenoyl-1-β-D-arabinofuranosylcytosine, etoposide, prednisolone) was compared with ACOPE regimen (adriamycin, cyclophosphamide, vincristine, prednisolone, etoposide) in previously untreated patients with non Hodgkin's lymphoma CS IV.
Forty-one patients were evaluated for response and survival. Patients treated with ABEP had a complete remission rate of 60% in contrast to 47.6% for those receiving ACOPE. Four-year survival rate was 62.3% versus 30.2% (Cox Mantle Test: P<0.05). And four year disease free survival rate was 82.5% versus 40%. Hematological toxocity was included with a higher frequency in ABEP. These results suggest that ABEP treatment of non-Hodgkin's lymphoma CS IV is more effective than ACOPE.View full abstractDownload PDF (409K) -
Fumihiro FUJIWARA, Takao YOSHIHARA, Satoshi IKUSHIMA, Noriko ESUMI, Sh ...1988 Volume 29 Issue 9 Pages 1388-1393
Published: 1988
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSA retrospective review of 30 pediatric patients with hematological malignancies treated with BH-AC/ACM therapy demonstrated the occurrence of urological hemorrhage as the most common side effect of ACM. Of 85 treatment courses in 30 patients, hematuria occurred during 16 courses (18.8%) in 10 patients (33.3%). Two of the patients developed renal dysfunction, associated with pelvic dilatation and clot formation in the pelvis confirmed by echograms. None of age, sex, urine volume, hydration and platelet count was significantly related to the hemorrhagic episodes. Our experience and the review of the literature showed that hematuria was mostly observed within two weeks after starting ACM. Although the results were preliminary, 2-mercaptoethane sulfate (mesna) was not effective for urotoxicity of ACM. These data indicate that early detection of hematuria through frequent urinalysis is required to prevent serious urological toxicity in patients under BH-AC/ACM treatment.View full abstractDownload PDF (480K) -
Shigeyoshi MAKINO, Yasuhiko ARAKI, Kazuo TAMURA, Masashi SEITA1988 Volume 29 Issue 9 Pages 1394-1398
Published: 1988
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSTen patients with acute adult leukemia in relapse or refractory to the initial induction therapy were treated with “A-Triple-V” combination consisting of cytosine arabinoside, etoposide, vincristine and vinblastine. Eight patients with acute myelogenous leukemia (AML) including 3 patients in first relapse, 1 in second relapse and 4 in refractory leukemia, and two with acute lymphoblastic leukemia (ALL) in relapse were entered to this pilot study.
Five of 8 patients with AML achieved complete remission (CR) lasting for 1, 1, 3+, 3.5 and 12+ months. In addition, three other patients obtained partial remission. Two patients with ALL, however, failed to respond to this regimen.
Nadir leukocyte counts were below 500/μl in all patients, and they retured to above 1,000/μl within 18 days in average. Alopecia, gastrointestinal toxicity such as nausea and vomiting, and neurotoxicity were frequently seen, but they were mild and well tolerated. These results indicate that “A-Triple-V” treatment appears to be effective against the relapsed or refractory adult AML, although the remission duration is rather short.View full abstractDownload PDF (397K) -
Hideo NAKAMURA, Naoki SADAMORI, Ippei SASAGAWA, Takahiro ITOYAMA, Tomo ...1988 Volume 29 Issue 9 Pages 1399-1407
Published: 1988
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSChromosomal banding studies were performed on 13 patients consisting of 11 with acute myelomonocytic leukemia (M4) and two with acute monocytic leukemia with maturation (M5b).
Inv (16)(p13q22) was revealed in 5 out of the 11 patients with M4. All the M4 patients with inv (16) had bone marrow eosinophilia (>5%) with distinct dysplastic changes, and easily achieved complete remission after induction chemotherapy. The hemoglobin level of the peripheral blood was significantly lower in patients with inv (16) than in the other M4 patients.
Chromosome# 11 anomalies at band 11q23 were observed in one of the other M4 patients and one of the two M5b patients. The progress of disease in the two patients was aggressive resulting in a very poor prognosis.
It was suggested based on the data of our patients and those in the literature that two major chromosome subgroups may be present among patients diagnosed as having M4 or M5: one subgroup with inv (16) and the other with chromosome# 11 anomalies at band 11q23. However, the remaining patients may include those having micromegakaryocytes in the bone marrow and being refractory to chemotherapy, who can also form a distinct subgroup.View full abstractDownload PDF (1282K) -
Naoko ISHITOYA, Itizou GOMIBUCHI, Kohji FUJISAWA, Jun-ichi AKATSUKA1988 Volume 29 Issue 9 Pages 1408-1412
Published: 1988
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSSecondary hemosiderosis and death from chronic iron overload may result from repeated blood transfusions in refractory anemia. We tried iron chelation therapy with continuous subcutaneous infusion of deferoxamine in a 5-year old boy with Blackfan-Diamond syndrome which was diagnosed at 9 months old and received a total of 68 units of red blood cells.
His physical examination on admission revealed skin pigmentation and hepatomegaly, and serum ferritin level was as high as 2,854 ng/ml and transferrin saturation, 98%. However, neither liver dysfunction nor endocrinologic abnormality was found. Continuous subcutaneous deferoxamine therapy was done for 14 weeks, by increasing the dose of deferoxamine from 55,70 to 80 mg/kg. Urinary iron excretion was not increased despite of stepwise increase in the dose of the drug and remained about 10 mg/day throughout. Iron excretion exceeded iron loading caused by transfusion, and this chelation therapy was thought to be effective without any severe side effect.View full abstractDownload PDF (345K)
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Sumiko IMAI, Hiroko YAMAMOTO, Yukiko SIMAMOTO, Norio ENDO1988 Volume 29 Issue 9 Pages 1413-1416
Published: 1988
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSWe occasionaly see leukemia infiltration into heart at the terminal stages. However, there are only few reports that cardiac tanponade caused by leukemia infiltration into heart is the initial symptom of ALLs.
A 6 yeres old boy complained of wheezing and abdominal distention. His ECG and Echo cardiagram suggested cardiac tamponade. Leukemic cells were found in peripheral blood, in bone marrow and in pericardial effusion. Histologically pericardial invasion with leukemic cells was observed.View full abstractDownload PDF (472K) -
Takayuki ISHIKAWA, Hiroki YABE, Kenichi NAGAI, Shiro NAKAYAMA, Toshio ...1988 Volume 29 Issue 9 Pages 1417-1421
Published: 1988
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSA case of HTLV-I negative helper/inducer T-CLL associated with OKT3 epitope deficiency is reported.
A 78-year-old man was admitted to our hospital in November 1985 because of gradual progression of leukocytosis which was first found 3 years ago. Physical examination on admission revealed no hepatosplenomegaly. Hemoglobin 13.2 g/dl, platelets 14.6×10/μl and WBC 62,800/μl consisted of 92% uniform, mature lymphoid cells including a small percentage of flower-like cells with convoluted nuclei. Bone marrow showed 46% lymphoid cells. Immunological phenotype of peripheral lymphoid cells gave positive reactions for OKT1, T4 and T11-1, but negative for OKT3, T8, T9, T10, T11-0, Ia, Tac, WT-31 and Mol. Levels of both BUN and creatinin were slightly elevated, but other blood chemistry findings including LDH and Ca were within normal range. Anti-HTLV-I antibody in his serum and integration of the HTLV-1 into leukemic cells were negative. He was diagnosed as variant T-CLL with OKT3 negative helper/inducer phenotype, and followed in out-patient clinic without any therapy, remaining clinically and hematologically unchanged up to date.
Since some ATL cases develop blood pictures similar to CLL and express a reduced number of T3 cells in comparison with that of T4 and T11 cells, it is possible to speculate in our case that structure or expression of T3 molecule may be altered by malignant transformation or by infection of some kinds of viruses other than HTLV-I.View full abstractDownload PDF (405K) -
Atae UTSUNOMIYA, Takeshi SAITO, Shuichi HANADA, Enan TIN, Tamao OSHIGE ...1988 Volume 29 Issue 9 Pages 1422-1426
Published: 1988
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSA 28-year-old woman diagnosed as having Chediak-Higashi syndrome is reported. Her hair was grey-white color. The dark brown skin pigmentation was observed in the face, bilateral upper and lower extremities, and the big skin ulceration was noticeble in the right lower extremity. She had mental retardation, systemic muscle weakness, disturbance of sensory motor and cerebellar ataxia. In granulocytic function studies, phagocytic function was normal, but intracellular bacteriocidal capacity was found to be diminished. On the other hand, leukocyte migration using the skin window technique was not decreased. In immunologic studies, natural killer activity was markedly decreased and antibody-dependent cell mediated cytotoxicity was slightly decreased, while T4/T8 ratio in the peripheral blood was not inverted.View full abstractDownload PDF (586K) -
Masahiro SUGAWARA, Seiichi HORIE, Soutaro ABE, Taijiro ISHIYAMA, Masak ...1988 Volume 29 Issue 9 Pages 1427-1431
Published: 1988
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSPathologic rupture of the spleen is an unusual complication of malignant lymphoma.
A 66-year-old female with diffuse pleomorphic T cell lymphoma was admitted because of fever and lymphadenopathy of one month duration. Eight days after admission she died suddenly. Autopsy revealed rupture of the spleen with malignant lymphoma which also involved many other organs. Sixteen cases with malignant lymphoma resulting in spleen rupture had been already reported. In this paper, seventeen cases, including our case, were reviewed. The mean age was 46 years, male to female ratio 10.7, and the mean splenic weight 1,321 g.View full abstractDownload PDF (540K) -
Tetsuji KOYAMA, Noriko ONO, Mitsuhiro HIGUCHI, Akihisa KANAMARU, Eizo ...1988 Volume 29 Issue 9 Pages 1432-1436
Published: 1988
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSA 77-year-old male suffering from bleeding tendency was admitted to our hospital in October 1986. He had anemia, thrombocytopenia and leukoerythroblastosis on peripheral blood smear. A bone marrow biopsy revealed myelofibrosclerotic changes without the metastasis of malignant cells. Hypertrophic prostate showed an irregular region in part, and the serum prostatic acid phosphatase (PAP) was high. Plasma levels of fibrinogen and α2-plasmin inhibitor were both low, but D-dimer remained within normal range in spite of the increase in fibrin and fibrinogen degradation products (FDP). These data indicated that bleeding tendency might be induced by a primary fibrinogenolysis and thrombocytopenia due to secondary myelofibrosis. Histopathological examination on prostate could not be done, but this myelofibrosis was strongly suggested to be based on prostatic carcinoma by the fact that the myelofibrosis and hematological data were markedly improved by the treatment with diethylstilbestrol.View full abstractDownload PDF (634K) -
Yoshikata MISAKI, Akimichi OHSAKA, Youichi SAKATA, Minoru YOSHIDA, Yuk ...1988 Volume 29 Issue 9 Pages 1437-1441
Published: 1988
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSA 65-year-old woman was admitted to Jichi Medical School Hospital because of fever, hemorrhagic diathesis and stomatitis on April 9, 1986. Physical examination revealed tumors of lip and tongue, which were histologically diagnosed as infiltration of leukemic cells. White blood cell count was 32.5×104/μl with 98.0% leukemic cells. Bone marrow was hypercellular with 97.2% leukemic cells. Most of the leukemic cells in peripheral blood contained few granules, whereas leukemic cells in bone marrow contained abundant azurophilic granules and a few Auer rods, but were devoid of “faggots”. Histochemistry of leukemic cells were positive for peroxidase and Sudan Black B stainings. Cell surface marker analysis of leukemic cells disclosed that they were positive for My7, My9, and OKT10, but not OKDR, suggesting that leukemic cells expressed some antigens compatible with promyelocytes. Leukemic cells in peripheral blood were also identified as promyelocytes by means of electron microscopy. Chromosomal abnormality t(15,17), which was typically detected in hypergranular acute promyelocytic leukemia (APL), was not seen. DIC was clinically observed. Therefore, this case was diagnosed as an atypical form of APL associated with hyperleukocytosis and oral involvement. These appear to be rare in APL.View full abstractDownload PDF (717K) -
Kinuko MITANI, Yukio KOBAYASHI, Yutaka KAWAMURA, Kensuke USUKI, Akira ...1988 Volume 29 Issue 9 Pages 1442-1446
Published: 1988
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSA 58-year-old male was admitted to our hospital because of fever and swelling of submandibular lymph nodes. The leukocyte count of the peripheral blood was 21,900/μl. Ninety-nine percent of the cells were blasts with lymphoid appearance. Bone marrow was occupied with 90 percent blasts cytochemically negative for peroxidase. This case was diagnosed as acute lymphocytic leukemia (ALL, L1) according to the criteria of FAB classification. Southern analysis showed clonal rearrangements of both T cell receptor gene (β) and immunoglobulin heavy chain gene.
However, the leukemic cells were positive for both lymphoid and myeloid markers (CD7, CD13, and CD33) on cell surface marker analysis, suggesting that this case could be classified as hybrid (mixed lineage) leukemia.
After L-AdVP therapy, complete remission was attained. One year later, he was readmitted with the relapse and L-AdVP therapy was again successful.View full abstractDownload PDF (425K) -
Kazue KAJIYAMA, Kazuyoshi TAKAHASHI, Shigeru ITAKURA, Fumi MIZOROGI, M ...1988 Volume 29 Issue 9 Pages 1447-1453
Published: 1988
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSA 65-year-old Japanese male developed a left neck tumor in 1979, and the lesion was interpreted as follicular lymphoma by lymph node biopsy. None were conducted over the next six years for lack of symptoms. In February 1986, he was admitted to our hospital because of marked hepatosplenomegaly and generalized lymphademopathy, and a lymph node biopsy determined that he had non-Hedgkin's lymphoma, follicular mixed cell type. A peripheral smear and a bone marrow biopsy revealed the involvements of peripheral blood and bone marrow. He was treated with a variety of chemotherapies, namely, cyclophosphamide, vincristine, vindesine and prednisolone, resulting in partial remission. However, he developed high fever and ascites at the close of the year. The CHOP therapy was started, but in Feb 1987 the patient died three weeks after the last lymph node biopsy. In the three lymph node biopsies, the neoplastic follicles were evenly distributed in a back-to-back pattern, comprised of large and small cleaved and non-cleaved type cells. Histological transformation was not observed in the biopsies, but it was noticed that the population of non-cleaved cells and mitotic figures increased with time and that the follicles merged with one another in reticulin impregnation of the last biopsy.View full abstractDownload PDF (2676K) -
Keisuke MIYAZAWA, Osamu YOSHIKAWA, Yoshio TAKAKUWA, Akemi TUDA, Masahi ...1988 Volume 29 Issue 9 Pages 1454-1459
Published: 1988
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSWe report the sixth case of congenital dyserythropoietic anemia type I in Japan, treated with deferoxamine. The patient was a 36-year-old male who had splenomegaly, jaundice and brownish skin color, and often required blood transfusions due to severe anemia (Hb 5.6 g/dl). The laboratory data revealed hemolysis and iron overload, and acidified serum test was negative. Studies of ferrokinetics showed the pattern of ineffective erythropoiesis. The morphological aberrations of erythroblasts by light microscopic and ultrastructural study were compatible with congenital dyserythropoietic anemia type-I (CDA-I).
When deferoxamine was given for a total of 19 months, anemia was ameliorated around the level of Hb 10 g/dl without any blood transfusion accompanied by a decrease of serum ferritin. Splenomegaly was reduced in its size, and skin color, serum LDH and indirect bilirubin levels were normalized.
Iron overload and secondary hypersplenism seem to play an important role in the etiology of anemia in this case. It is suggested that removal of an excess iron with deferoxamine is important in the treatment with CDA-I.View full abstractDownload PDF (556K) -
Masanori SAITO, Kazuaki ODAKA, Shigeki OTAKE, Takashi YOSHIDA, Shinobu ...1988 Volume 29 Issue 9 Pages 1460-1464
Published: 1988
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSA 53 year old woman with non-Hodgkin's lymphoma (NHL) clinical stage III B was admitted in September 1984. She first noticed right cervical lymph nodes swelling in 1981, thereafter the symptoms of dry eyes and xerostomia appeared in 1984. She received ACOP (adriamycin, cyclophosphamide, vincristine, prednisolone) treatment for NHL and obtained complete remission in October 1984 with some relief of sicca symptoms. But, she was readmitted in May 1985 because of the recurrence of NHL and exacerbation of sicca syndrome, associated with nodular goiter. A diagnosis was made of papillary adenocarcinoma of thyroid gland by needle biopsy. Labolatory examinations on 2nd admission revealed hypergammaglobulinemia and auto-antibodies such as anti nuclear and anti-SS-B antibodies. It appeared that underlying immunological abnomalities caused by NHL and chemotherapy might be related to the developement of thyroid cancer.View full abstractDownload PDF (523K) -
Tsutomu SAKUMA, Takashi SATOH, Ryoichi SATODATE, Chieko KATOH, Takeo M ...1988 Volume 29 Issue 9 Pages 1465-1470
Published: 1988
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSWe reported a 48-year-old man who complained of abdominal fullness and had ATL. There was abdominal distention caused by accumulation of ascites, but neither skin eruption nor lymphadenopathy was observed. CT scan disclosed hepatosplenomegaly, but no swelling of the retroperitoneal lymph nodes. Peripheral leukocyte count was 12,500/μl with 31.4% in lymphocytes including abnormal lymphocytes. Abnormal lymphocytes measured 8∼15 μm in diameter and had an indented nucleus. Peripheral lymphocytes were 81.6% OKT4+, 6.1% OKT8+ and 51.8% IL2-R+. Anti-ATLA antibody was positive. Nucleated cell count in ascites was 32,000/μl with 74% in blasts. The blasts were 20∼50 μm in diameter, had an indented nucleus, and were occasionally multinucleated. The blasts were 96.9% OKT4+, 0.1% OKT8+ and 81.9% IL2-R+. The blasts of ascites were considered to be derived from the leukemic cells and were transformed in ascites.
In addition, his elder sister who died of Sézary syndrome in 1976 was retrospectively considered to have ATL, because the siblings came from an endemic area of HTLV-I and his brother (the case reported here) developed ATL. And they were suggested to be infected HTLV-I by mother-to-child transmission, although anti-ATLA antibody of their mother was not examined.View full abstractDownload PDF (1020K) -
Makoto OITA, Mihiro OKABE, Noriko TACHIBANA, Makoto SUKEGAWA, Takayuki ...1988 Volume 29 Issue 9 Pages 1471-1475
Published: 1988
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSA 23-year-old male was admitted bacause of fever and abdominal pain on October 3, 1986. His peripheral blood examination revealed elevated WBC count 123.8×109/L with 37% monocytoid cells. The bone marrow aspirate revealed hypercellular material with granulocytic and monocytic hyperplasia. The proliferating monocytoid cells were positive for non-specific esterase stain and showed interleukin-1 (IL-1) producibility in the similar degree of normal monocytes. A splenomegaly with massive cystic lesion was seen by CT scan and ultrasonography. As splenomegaly was progressed, splenectomy was carried out for the purpose of prevention of rupture. The resected spleen showed wide spaced necrosis and involved brown fluid. Neither bleeing nor hematoma was found. The microscopic study revealed diffuse infiltration by monocytoid cells, numerous thrombi and necrotic tissue due to infarction. At present, he is going well without progression, followed by treatment with 6-mercaptopurine.View full abstractDownload PDF (734K) -
Tetsuro FUJIMOTO, Nobutaka IMAMURA, Osamu SAITO, Kingo FUJIMURA, Kosuk ...1988 Volume 29 Issue 9 Pages 1476-1481
Published: 1988
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSAdult T-cell leukemia (ATL) was succesfully treated with α-interferon alone. A 52 years old female patient, whose birth place is Kagoshima Prefecture, had skin lesions which made frequent appearances and leukocytosis, since 1978. She was admitted at our hospital in May 1981 because of another episode of skin eruptions, hairloss, lymphnode swelling and ascites. Later in the course, the ascites increased prominently and hepatosplenomegaly was also observed. The white blood cell count was 113,000/μl with the seventy-nine percent of atypical lymphocytes which had convoluted nucleus. Antibody to the ATL-associated antigen in serum was positive. Lymph node biopsy revealed the finding of pleomorphic type in diffuse lymphoma, according to the LSG classification. Under electron microscope, release of c-type virus particles from these convoluted lymphocyte culture cells were seen. A diagnosis of ATL was then made from above findings. Alpha-interferon (α-IFN) was, for the first time, administered in November 1981, resulting in the decrease of white blood cell counts and disappearance of the ascites, hepatosplenomegaly and skin lesions. This α-IFN therapy was effective for 3 years and 9 months, but after then, the white blood cell count increased again, despite continuous administration of the α-IFN, and the patient died due to pneumonia, in April 1986.View full abstractDownload PDF (468K) -
Chiyuki KAWASAKI, Shin HAYASHI, Tsunefumi SHIBUYA, Teruhisa OTSUKA, Se ...1988 Volume 29 Issue 9 Pages 1482-1487
Published: 1988
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSA 66-year-old man was admitted to our hospital because of general fatigue and disturbed consciousness. Abnormal lymphocytes with convoluted nuclei were detected in peripheral blood and pleural effusion. In addition to antibody to adult T-cell leukemia (ATL) virus-associated antigen in serum, the monoclonal integration of HTLV-I proviral DNA was demonstrated in these abnormal lymphocytes. From these findings, the patient was diagnosed as ATL.
Despite the absence of edema or ascites and normal renal and adrenal function, profound hyponatremia was detected while osmolality was low in serum and high in urine. So diagnosis of syndrome of inappropriate secretion of antidiuretic hormone (SIADH) was established. The head MRI revealed mass lesion in the pontine area and periventricular hyperintensity. Nucleated cell counts were increased in cerebrospinal fluid and abnormal lymphocytes were detected. Chest and abdominal computed tomography revealed no mass lesions.
The patient was treated with intrathecal methotrexate and cytarabine and intravenous high dose methotrexate. This treatment was effective because the serum sodium level recovered to normal with the disappearance of pontine lesion and periventricular hyperintensity in the MRI examination. From these observations, it is highly suggested that the SIADH in this patient might be caused by the invasion of ATL cells into the central nervous system.View full abstractDownload PDF (779K) -
Keisuke MIYAZAWA, Junko H. OHYASHIKI, Tetsuzou TAUCHI, Hiromi FUJIEDA, ...1988 Volume 29 Issue 9 Pages 1488-1492
Published: 1988
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSThe patient was a 23-year-old female who had been followed as Ph1-positive chronic myeloid leukemia (CML) in the chronic phase over 12 years. The patient manifested hemorrhagic tendency and was diagnosed as having blastic crisis by the hematologic examination. At this time, an increasing number of Gaucher-like cells was observed in her bone marrow. According to the electron microscopic study, these Gaucher-like cells had abundant cytoplasm containing tubular structural inclusion bodies. Chromosome analysis of the bone marrow cells at this time revealed that cells without a Ph1-chromosome were present. Surface marker analysis of the blasts revealed to be of B-lymphoid characteristics; B4 88.3%, J5 93.1% and OKIal 87.0%, though they contained myeloperoxidase positive granules by the electron microscopic examination. DNA analysis showed that the bone marrow cells in the blastic crisis had JH rearrangement, indicating the blasts had biphenotypic nature.View full abstractDownload PDF (829K) -
Hiroshi KAMANO, Yoshitugu KUBOTA, Hiroaki OHNISHI, Teruhisa TAOKA, Ter ...1988 Volume 29 Issue 9 Pages 1493-1498
Published: 1988
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSA 45-year-old female was admitted to our hospital because of fever. On admission her conjunctiva was anemic, and auscultation of the heart disclosed a grade 2 systolic murmur at the apex of the heart.
Peripheral blood showed RBC 430×104/μl, Hb 12.6 g/dl, platelets 7.7×104/μl, WBC 72,200/μl and 91.5% blast cells. Examination of the bone marrow showed 86.4×104/μl nucleated cells with 92.5% blast cells. There were no azurophil granules or Auer bodies in these blast cells. Leukemia cells were positive for peroxidase but negative for PAS, naphthol AS-D chloroacetate esterase and α-naphthyl butyrate esterase. Surface marker analyses by indirect 125I-protein A binding assay showed that only OKIal monoclonal antibody positively reacted with leukemia cells. Cytogenetic analyses from peripheral blood revealed a karyotype of 46, XX, t(11;19)(q23.3;p13.3) in all metaphases examined.
From these findings, diagnosis of AML (FAB M1) was made. A complete remission has been achieved by BH-AC DMP therapy and maintained by cyclic intensification therapy with BH-AC DMVP. As far as we know, this is the third case of AML with the karyotype of t(11;19)(q23;p13).View full abstractDownload PDF (507K) -
Masashi KOBAYASHI, Yutaka OGUMA, Kaoru TAKAHASHI, Wataru TATEWAKI, Hoy ...1988 Volume 29 Issue 9 Pages 1499-1503
Published: 1988
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSA case of type I von Willebrand disease (vWD) accompanied with idiopathic thrombocytopenic purpura (ITP) is reported.
The case is a 43-year-old female who had suffered from many episodes of epistaxis, ecchymoses, and prolonged bleeding at the time of shedding of teeth since her infancy. However, she had received no special treatments for these hemorrhagic diatheses. Thrombocytopenia was pointed out in her pregnancies at the ages of both 24 and 29, and she delivered her babies by planned Cesarean sections receiving platelet transfusions. Her older brother and nephew also are diagnosed as vWD. As for her laboratory data, F. VIII: C was 48%, vWf: Ag 15% and RCof 31%, and the multimeric composition of vWf was normal on both of crossed immunoelectrophoresis and SDS-agarose gel electrophoresis. On the other hand, platelet count in the peripheral blood and megakaryocyte count in the bone marrow aspirate were 32,000/μl and 187/μl, respectively. Shortening of platelet life span (half life 16.2 hr) was recognized. Thus, she was diagnosed as type I vWD accompanied with ITP. And her dental extractions were successfully performed under infusion of platelet concentrates and factor VIII concentrates without prolonged bleeding.View full abstractDownload PDF (472K) -
Hideyo NATORI, Kazuyuki NATORI, Katsunobu NAITO, Eiji NAKAMURA, Ken TA ...1988 Volume 29 Issue 9 Pages 1504-1510
Published: 1988
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSA 66-year-old male was admitted to our hospital because of petechiae. The lymph nodes, liver and spleen were not palpable.
Haematological examinations revealed that the number of leukocytes was 4,500/μl with 7% leukemic cells and the number of platelets was 5,000/μl. Bone marrow smears was hypercellular marrow with 31% erythroblasts, 21% leukemic cells and abundant megakaryocytes. Pseudo-Pelger-Hüet anomalies of neutrophils and nuclear fragmentation of erythroblasts were shown.
The patient was diagnosed as refractory anemia with excess of blasts in transformation (RAEB in T).
The most important abnormalities were observed in megakaryocytes. Thus, emperipolesis of segmented neutrophils and/or erythrocytes by immature megakaryocytes, so-called megakaryoblasts were found.
Myelodysplastic syndrome was diagnosed morphologically by abnormalities of the haematopoietic cell lines. Particularly, dysmegakaryopoiesis, i.e. megakaryoblasts, micromegakaryocytes and abnormal lobulation of nuclei were present.
Emperipolesis of megakaryocytes was regarded as an abnormality of megakaryocytes in RAEB in T.View full abstractDownload PDF (873K) -
Taiji KUNITOMI, Hirotoshi TAKIGAWA, Shin KANZAKI, Nobuyuki KODANI, Kei ...1988 Volume 29 Issue 9 Pages 1511-1515
Published: 1988
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSA case of acute lymphoblastic leukemia (ALL) complicated with intestinal perforation during remission course was reported. The patient was one year-old boy with hematuria and petechiae. A diagnosis of ALL was mede. He was successfully treated with chemotherapeutic agents. He had been on daily 6-mercaptopurine and weekly methotrexate and cyclophosphamide.
After 3 months of remission he presented an acute abdominal distension sign with a high grade fever. At laparotomy, a perforation hole of 10mm in diameter, at the site of 1m proximal to the ileocecal junction, was discovered. The specimen obtained from the perfortion tissue was revealed a diffuse infiltration of leukemic cells in the submucosa and the lamina propria, but aspiration and biopsy specimens of bone marrow showed no signs of aggravation of leukemia.View full abstractDownload PDF (467K) -
Yoshitaka MORI, Akira DEGUCHI, Hikoji SUZUKI, Itsuko OHKUBO, Masayuki ...1988 Volume 29 Issue 9 Pages 1516-1521
Published: 1988
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSIn the past 19 years, 5 hemophiliacs aged from 2 to 29 years were admitted to our hospital for intracranial hemorrhage caused by central nervous system trauma. Three had hemophilia A, and two had hemophilia B. One patient had intracerebral hematoma, one had epidural hematoma and the other three had subdural hematoma. One case was associated with arachnoid cyst. Substitution therapy was started between 1 and 72 hours after the trauma. Four patients were operated at 0, 1, 3, and 4 day after admission. However, three of them died despite intensive care. Anti-hemophilic factor (36, 12, 85, 20-80, and 30 U/kg×3/day) was given on admission. Disseminated intravascular coagulation (DIC) was observed in two hemophilia B patients. Postoperative hemostatic data in these patients improved by decreasing the prothrombin complex cocentration, and treatment with gabexate mesilate and heparin. The present cases indicate the importance of carefully observing computed tomography findings and clinical symptoms because intracranial hemorrhage develops after a relatively long latency following head trauma. Furthermore, early operation and frequent coagulation studies are important to prevent DIC syndrome.View full abstractDownload PDF (639K) -
Susumu OKUBO, Masahiro YAMANO, Kojiro YASUNAGA1988 Volume 29 Issue 9 Pages 1522-1526
Published: 1988
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSAlthough the plasma F.XIII Levels were lowered in some SLE patients, we experienced one case of a 20 year-old female with SLE, showing an acquired low F.XIII level (20%) followed by the severe and prolonged oozing characteristic of F.XIII deficiency. The patient had been diagnosed 3 years earlier as SLE with thrombocytopenia, and a therapy including prednisolone (PSL) was begun. At that time, she suffered a rectal perforation, which was successfully operated. On Apr. 25, 1987, when she was admitted to our hospital for the fourth time, she complained of proteinuria, the laboratory findings on liver function and hemostatis being all within normal range. After four months of therapy including PSL, subcutaneous bleeding appeared, which rapidly grew into a huge hematoma covering her back. At this time, too, PT, APTT, Fbg, AT III, and each clotting factor activity were within normal range, except for a low platelet count (92,000/μl) and a low F.XIII level (20%). At least, obvious DIC could be denied. The administration of an F.XIII concentrated product (Fibrogammin) for five days resulted in partial improvement of her huge hematoma and exfoliative wound. Nevertheless, 4 days thereafter obvious DIC occurred resulting in her death. The decrement of F.XIII in this SLE patient may be attributable to pre-DIC or may reflect latent vascular injury due to SLE. The monitoring of plasma F.XIII in SLE patients is very important in order to avoid and to treat hemorrhagic complications.View full abstractDownload PDF (505K)
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1988 Volume 29 Issue 9 Pages 1527-1548
Published: 1988
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSDownload PDF (1959K)
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1988 Volume 29 Issue 9 Pages 1549-1555
Published: 1988
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSDownload PDF (652K)
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