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Volume 53, Issue 3
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Picture in Clinical Hematology No.52
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[in Japanese]2012 Volume 53 Issue 3 Pages 285
Published: 2012
Released on J-STAGE: April 10, 2012
JOURNAL RESTRICTED ACCESSDownload PDF (1356K)
The 72nd Annual Meeting of the Japanese Society of Hematology
Special Award Received Lecture
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Tohru FUJIWARA2012 Volume 53 Issue 3 Pages 287-294
Published: 2012
Released on J-STAGE: April 10, 2012
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Yong-Mei GUO, Kenichi SAWADA2012 Volume 53 Issue 3 Pages 295-302
Published: 2012
Released on J-STAGE: April 10, 2012
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Clinical Study
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Sho YOKOTA, Yuji URASAKI, Yuichi NAKAMURA, Masami BESSHO2012 Volume 53 Issue 3 Pages 303-309
Published: 2012
Released on J-STAGE: April 10, 2012
JOURNAL RESTRICTED ACCESSIn 114 patients with monoclonal gammopathy of undetermined significance (MGUS) followed-up in Tachikawa Sougo hospital, we retrospectively analyzed progression of their disease to multiple myeloma (MM) or related disorders. The analysis was based on a total of 1,170 person-years of follow-up in a cohort with a median age at diagnosis of MGUS of 68 years 3 months, and with a median follow-up period of 9 years 5 months. Of these 114 patients, 13 (11%) showed progression to MM or related disorders with a median time to progression of 9 years 4 months; and the median age of these 13 patients was 78 years 8 months. The cumulative hazard ratio of progression at 5, 10, 15, and 20 years after diagnosis was 3.0%, 9.0%, 11.4%, and 32.1%, respectively. The risk of progression of MGUS to MM or related disorders in Japanese patients was as high as in Western patients studied previously, demonstrating that MGUS should be carefully monitored as a preneoplastic condition.View full abstractDownload PDF (841K) -
Masao TOMONAGA, Isao KAMAE2012 Volume 53 Issue 3 Pages 310-317
Published: 2012
Released on J-STAGE: April 10, 2012
JOURNAL RESTRICTED ACCESSThe aim of this study was to assess the cost-effectiveness of azacitidine therapy for patients with myelodysplastic syndromes. A Markov model was developed to estimate the total additional direct cost and quality adjusted life years (QALYs) gained with azacitidine therapy versus best-supportive care in patients with high-risk MDS. The cost-effectiveness of azacitidine was evaluated with incremental cost-effectiveness ratio, which represents the additional cost per QALY gained from the more effective treatment. Azacitidine therapy was 1.83 million yen more costly per patient but yielded an additional 0.353 QALYs. The ICER (Increment of Cost-effectiveness Ratio) was 5.18 million yen per QALY. In conclusion, because the ICER was less than the threshold for acceptable cost-effectiveness in Japan, azacitidine therapy for MDS patient was assumed to be cost-effective.View full abstractDownload PDF (1094K) -
Yukinori NAKAMURA, Takehiko MORI, Jun KATO, Yoshinobu AISA, Tomonori N ...2012 Volume 53 Issue 3 Pages 318-322
Published: 2012
Released on J-STAGE: April 10, 2012
JOURNAL RESTRICTED ACCESSA variety of reduced-intensity conditionings have been used in the reported studies of allogeneic hematopoietic stem cell transplantation (HSCT) for elderly patients with myeloid hematological malignancies. This study retrospectively analyzed the outcome of allogeneic HSCT for 10 patients aged 50 years or older with myeloid hematological malignancies after conditioning with fludarabine (125mg/m2), melphalan (140mg/m2) and total body irradiation (TBI; 8Gy). Median age of the patients was 56.5 years, and diagnoses included acute myelogenous leukemia, advance myelodysplastic syndrome, and secondary myelofibrosis. Sources of stem cells were bone marrow from sibling (n=4) or unrelated donor (n=6). Both overall and disease-free survival rates were 40.0% (95% CI: 10.6∼69.4%). Causes of death were relapse (n=2), fungal infection (n=2), and secondary malignancies (n=2). Because of a high incidence of transplant-related mortality, further refinement of this conditioning is required.View full abstractDownload PDF (716K) -
Tomohiko KAMIMURA, Chong YONG, Hideho HENZAN, Katsuto TAKENAKA, Yoshik ...2012 Volume 53 Issue 3 Pages 323-328
Published: 2012
Released on J-STAGE: April 10, 2012
JOURNAL RESTRICTED ACCESSWe retrospectively analyzed outcomes of eight evaluable patients with primary myelofibrosis (PMF) who received allogeneic hematopoietic stem cell transplantation (allo-HSCT), using different graft sources; four patients received peripheral blood stem cells, three bone marrow, and one umbilical cord blood. The median age was 48 years (range, 43∼64 years). Seven patients had an intermediate or high Dupriez score. Three patients underwent myeloablative conditioning, whereas five underwent reduced-intensity conditioning regimens. Engraftment was obtained in all of these recipients. The median days to reach a neutrophil count above 0.5×109/l and platelet count above 20×109/l were 20 and 35 days, respectively. No treatment-related deaths were observed within 100 days after allo-HSCT. Two patients died of sepsis or late-onset non-infectious pulmonary complications. Four patients developed grade I to II acute GVHD, and six patients developed chronic GVHD. The estimated 3-year overall survival was 75% with a median follow up time of 43 months (range, 6∼127). Four of 5 patients who were transfusion-dependent became free from transfusion after allo-HSCT. In six of seven patients, a regression of fibrosis was confirmed by bone marrow biopsy. Despite the small number of cases, our results suggested that allo-HSCT is an encouraging curative strategy for treating PMF.View full abstractDownload PDF (725K) -
Yoko HASHIMOTO, Akihiko YOKOHAMA, Akio SAITOH, Hirotaka NAKAHASHI, Koh ...2012 Volume 53 Issue 3 Pages 329-336
Published: 2012
Released on J-STAGE: April 10, 2012
JOURNAL RESTRICTED ACCESSWe retrospectively investigated pathological types, clinical backgrounds, treatments and prognoses in 726 adult patients with newly diagnosed malignant lymphoma in Gunma Prefecture. They consisted of 679 patients with non-Hodgkin lymphoma (B-cell type, 603; T- and NK-cell type, 76) of which 376 patients had diffuse large B-cell lymphoma (DLBCL) and 47 patients with Hodgkin lymphoma. When comparing the prognosis of DLBCL between patients receiving rituximab (R-CHOP group; n=212) and not using rituximab (CHOP group; n=126), both 3-year overall survival (73.5% vs 61.7%, p=0.010) and 3-year progression-free survival (65.1% vs 45.8%, p<0.001) were statistically better in the R-CHOP group compared to the CHOP group. Our results suggest that more than half of patients were DLBCL and the rituximab-containing regimen results in an improved prognosis for DLBCL patients.View full abstractDownload PDF (1037K)
Case Reports
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Masaki FUKUDA, Ryosei NISHIMURA, Raita ARAKI, Rie KURODA, Shintaro MAS ...2012 Volume 53 Issue 3 Pages 337-341
Published: 2012
Released on J-STAGE: April 10, 2012
JOURNAL RESTRICTED ACCESSEpstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) is characterized by clonal expansion of EBV-infected CD8+T-cells. We have recently demonstrated that detection of a clonally expanded population of EBV-infected CD8+T-cells with CD5 down-regulation was a useful tool to distinguish EBV-HLH from EBV-related disorders such as severe infectious mononucleosis. A 5-year-old girl who presented with fever, pancytopenia and liver dysfunction was diagnosed by this method in addition to conventional diagnostic tests. Further, EBV-infected cells were identified as CD5-HLA-DR+ TCR V β3+ CD8+T cells, an increase or decrease of which over time reflected the disease severity in this patient. Treatment of patients with EBV-HLH varies from steroid alone to intensive chemotherapy or hematopoietic stem cell transplantation. Easy monitoring of EBV-infected cells by using flow cytometry over time may provide useful information to choose an appropriate treatment for each individual patient with EBV-HLH.View full abstractDownload PDF (977K) -
Yukiko HAMASAKI, Akihito MATSUOKA, Masato WAKI, Kimihiro KAWAKAMI2012 Volume 53 Issue 3 Pages 342-346
Published: 2012
Released on J-STAGE: April 10, 2012
JOURNAL RESTRICTED ACCESSA 22-year-old woman was diagnosed with thrombotic thrombocytopenic purpura (TTP). She had a high fever and disorientation without renal dysfunction. She immediately underwent plasma exchange and prednisolone treatment, but they proved ineffective. She subsequently suffered from left major cerebral infarction with right-side hemiplegia. Therefore, 375mg/m2 of rituximab was administered weekly from day 14 with informed consent. Immediate impro vements were noted in not only the hematological and biochemical parameters such as platelet count, hemoglobin level, rate of fragmented red cells, and serum LDH level but also the neurological symptoms and MRI findings. The universal histopathologic findings of TTP are characterized by hyaline thrombi formed by the aggregation of platelets, mostly in small arterioles and capillaries. Therefore, abnormal findings are rarely detected by imaging modalities such as CT and MRI. Moreover, TTP with major stroke is an extremely rare occurrence. In conclusion, we present a patient with refractory TTP with major cerebral infarction, who was effectively treated with rituximab.View full abstractDownload PDF (998K) -
Tori IDA, Shigeo HASHIMOTO, Toshio YANO, Naoko SATO, Tadashi KOIKE2012 Volume 53 Issue 3 Pages 347-351
Published: 2012
Released on J-STAGE: April 10, 2012
JOURNAL RESTRICTED ACCESSWe report a 54-year-old man with acute myeloid leukemia (AML) carrying del(7)(q32) and inv(16)(p13q22). He was diagnosed as having AML M4Eo according to the FAB classification. RT-PCR for CBFβ/MYH11 gene was positive. Karyotype analysis revealed the primary chromosomal abnormality to be del(7)(q32) and inv(16)(p13q22) developed as a secondary abnormality. He achieved complete remission after one course of induction chemotherapy and remained in remission after several courses of consolidation therapy. del(7q) is classified into an intermediate risk group or an adverse risk group, while inv(16)/t(16;16) is classified into a favorable risk group. Some AML cases with inv(16)/t(16;16) exhibit del(7q) as an additional chromosomal abnormality. It was reported that such cases showed good prognosis despite the presence of del(7q). However, AML cases with del(7q) and inv(16)/t(16;16) as secondary chromosomal abnormalities are rare. Further study is needed to clarify the clinical manifestations of such cases.View full abstractDownload PDF (1302K) -
Hiroyoshi ICHIHARA, Shiro KOH, Yasutaka AOYAMA, Takeo KUMURA, Tadanobu ...2012 Volume 53 Issue 3 Pages 352-356
Published: 2012
Released on J-STAGE: April 10, 2012
JOURNAL RESTRICTED ACCESSA 62-year-old man with chronic hepatitis C underwent interferon (IFN)-β therapy. After treatment for a period comprising 29 months and 2 weeks, hematological results showed a decrease in white blood cell, hemoglobin, and platelet counts (WBC 2,300/μl, Hb 7.2g/dl, PLT 4.7×104/μl), and IFN therapy was stopped. Despite therapy discontinuation, the pancytopenia continued to progress with elevation of LDH (LDH 4,898IU/l), and the patient was admitted to our hospital with suspected hematological disease. The patient underwent clinical screening, and pernicious anemia caused by vitamin B12 deficiency was diagnosed. The anemia rapidly improved with vitamin B12 treatment. Interferon is the mainstay of treatment for patients with viral hepatitis. While the adverse effects of interferon therapy are widely recognized, only a few reports have documented pernicious anemia developing during IFN-therapy. We recommend that particular attention be paid to such clinical and laboratory conditions as megaloblastic anemia when administering IFN. We also recommend checking the vitamin B12 level, as a deficiency of this vitamin may lead to the development of megaloblastic anemia.View full abstractDownload PDF (832K) -
Akiko SEKIYA, Eriko MORISHITA, Keiko MARUYAMA, Hidesaku ASAKURA, Shinj ...2012 Volume 53 Issue 3 Pages 357-360
Published: 2012
Released on J-STAGE: April 10, 2012
JOURNAL RESTRICTED ACCESSCongenital factor VII (FVII) deficiency is a bleeding disorder that requires optimal hemostatic management for each case due to its wide variety of bleeding symptoms. We experienced a patient with inherited FVII deficiency who demonstrated different FVII activities depending on tissue thromboplastins used for assays. An 82-year-old woman without any episodes of abnormal bleeding was found to have different FVII activities of 1.4% and 32% when assayed using thromboplastins from rabbit brain and human placenta, respectively. DNA sequencing analysis revealed a homozygous missense mutation of G10828A(FVII Padua) that caused an amino acid substitution of Arg304 to Gln(R304Q). Carriers of 304Q alleles are usually clinically asymptomatic and do not require FVII replacement therapies even in cases of homozygotes. In case a prolonged prothrombin time or reduced FVII activity is detected, re-examination using thromboplastins of other sources can be helpful for preliminary diagnosis of R304Q, in order to prevent unnecessary FVII replacement therapies.View full abstractDownload PDF (686K) -
Shinichi TAMURA, Hiroyuki ISHIDA, Atsushi FUJIKI, Takao YOSHIHARA, Osa ...2012 Volume 53 Issue 3 Pages 361-366
Published: 2012
Released on J-STAGE: April 10, 2012
JOURNAL RESTRICTED ACCESSA 22-year-old man with chronic active Epstein-Barr virus infection underwent allogeneic bone marrow transplantation (allo-BMT) from an HLA two allele-mismatched unrelated donor. Ten months after allo-BMT, he developed protein-losing enteropathy following a respiratory syncytial virus infection. A diagnosis of a recurrent type of acute graft-versus-host disease (GVHD) was made based on the histopathological findings, such as the infiltration of T lymphocytes into the superficial epithelium and crypts, and apoptotic bodies in crypts. Although methylprednisolone (mPSL: 10mg/kg) administration for two consecutive days improved gastrointestinal symptoms, acute pancreatitis and severe depression developed in association with corticosteroid treatment. Reduction of mPSL and administration of infliximab (5mg/kg/dose, 3 times) resulted in rapid improvement of depression and pancreatitis without aggravating intestinal GVHD. Recent studies have demonstrated that tumor-necrosis-factor (TNF)-α is associated with not only GVHD but also depression and acute pancreatitis. In the present case, anti-TNF-α treatment enabled us to reduce corticosteroid dose without aggravating GVHD, which suggests that this approach might be effective for the treatment of depression and acute pancreatitis.View full abstractDownload PDF (2361K)
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