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Volume 56, Issue 3
Displaying 1-17 of 17 articles from this issue
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Picture in Clinical Hematology
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[in Japanese], [in Japanese], [in Japanese], [in Japanese], [in Japane ...2015 Volume 56 Issue 3 Pages 243
Published: 2015
Released on J-STAGE: April 15, 2015
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Feature Articles: Update in hematology 2015 —focusing on the biology (Lymphoid diseases)—
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Ayako ARAI2015 Volume 56 Issue 3 Pages 245
Published: 2015
Released on J-STAGE: April 15, 2015
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Mamiko SAKATA-YANAGIMOTO2015 Volume 56 Issue 3 Pages 246-252
Published: 2015
Released on J-STAGE: April 15, 2015
JOURNAL RESTRICTED ACCESSAngioimmunoblastic T-cell lymphoma (AITL) is a distinct subtype of peripheral T-cell lymphoma. The origins of AITL tumor cells were thought to be follicular helper T (TFH) cells based on the common features of these two cell types. Recent findings suggest a multistep model for the development of AITL. The immature blood cells evolve into premalignant (prelymphoma) cells by acquisition of premalignant mutations including TET2 and/or DNMT3A mutations. The premalignant cells finally develop into full-blown tumor cells by accumulation of tumor-specific RHOA mutations. Combinations of premalignant and tumor-specific mutations may induce development of AITL.View full abstractDownload PDF (819K) -
Hayato TAMAI, Koichi INOKUCHI2015 Volume 56 Issue 3 Pages 253-260
Published: 2015
Released on J-STAGE: April 15, 2015
JOURNAL RESTRICTED ACCESSAcute lymphoblastic leukemia (ALL), a clonal expansion of hematopoietic blasts, is a highly heterogeneous disease comprising many entities for which distinct treatment strategies are pursued. Recurrent defects including chromosomal translocations, aneuploidies, and gene-specific alterations generate molecular subgroups of B- and T-ALL with differing clinical courses and distinct responses to therapy. Herein, we review the spectrum of genetic aberrations that promote acute B- and T-ALL, as well as the currently-revealed mechanisms of cell transformation and malignant progression. Although 5-year overall survival of childhood ALL patients has improved to as much as 90% due to progress in chemotherapy and other supporting therapeutic modalities, including allo-HSCT, the prognosis is still poor for the remaining 10% of cases, which consist mainly of MLL-AF4-positive ALL and bcr-abl positive ALL. The prognosis of adults with ALL is not satisfactory and adult ALL remains a challenging disease. The development of novel methodologies, including new molecular therapeutic targets, is also needed to improve the prognosis of ALL.View full abstractDownload PDF (1024K) -
Tetsuya FUKUDA2015 Volume 56 Issue 3 Pages 261-268
Published: 2015
Released on J-STAGE: April 15, 2015
JOURNAL RESTRICTED ACCESSChronic lymphocytic leukemia (CLL) is a mature lymphoid malignancy that has a variable clinical course. Recent genomic studies using next-generation sequencers have revealed recurrent genetic alterations implicated in CLL pathogenesis. Clonal evolution by stepwise acquisition of genetic mutations may result in CLL progression. Among these abnormalities, alteration of B cell receptor (BCR) signaling is critical during CLL development. In addition, signals from the microenvironment support the proliferation and survival of CLL cells. Novel molecular targeted drugs that influence these signals are now becoming clinically available.View full abstractDownload PDF (1076K) -
Ayako ARAI2015 Volume 56 Issue 3 Pages 269-277
Published: 2015
Released on J-STAGE: April 15, 2015
JOURNAL RESTRICTED ACCESSEpstein-Barr virus (EBV) infects human beings and latently persists in B-cells throughout life. EBV infection renders B cells immortal. In immunocompetent individuals, the expansion of EBV-infected B-cells is suppressed by cytotoxic T lymphocytes (CTL). However, once immunosuppression-induced CTL dysfunction or accelerated proliferation of infected cells due to gene mutation accumulation occurs, the infected cells proliferate leading to B-cell neoplasms. The products of the viral genome, i.e. LMP1, EBNA2, EBNA3A, and EBNA3C, are indispensable for transformation of infected B-cells. These viral proteins suppress apoptosis of the infected cells and contribute to the expansion of genetic mutation-bearing clones. It was recently reported that EBV also infects T or NK cells, suppresses their apoptosis, and promotes their survival by inducing CD40 and CD137 expressions. These results indicate that EBV may contribute to the development EBV-positive T- or NK-cell neoplasms. EBV-positive lymphoid neoplasms are generally resistant to chemotherapy and patient outcomes have thus been poor. Clarification of the molecular mechanisms underlying disease development is anticipated to lead to the establishment of novel treatment strategies.View full abstractDownload PDF (864K)
Feature Articles: Update in hematology 2015 —focusing on the biology (Hematopoietic stem cell transplantation)—
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Kazuteru OHASHI2015 Volume 56 Issue 3 Pages 278
Published: 2015
Released on J-STAGE: April 15, 2015
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Hiroshi KOSUGI2015 Volume 56 Issue 3 Pages 279-288
Published: 2015
Released on J-STAGE: April 15, 2015
JOURNAL RESTRICTED ACCESSRecent meta-analyses and systematic reviews focusing on the significance of high dose therapy with autologous hematopoietic stem cell transplantation for newly-diagnosed multiple myeloma patients have shown its benefit in terms of progression-free survival, but no over-all survival benefit versus conventional chemotherapy. New drug-containing regimens, with agents such as iMIDs and proteasome inhibitors, have not yet been established as high-dose therapy, because no phase III RCT has been conducted to date. Although several guidelines recommend maintenance therapy for non-CR patients after transplantation, caution is still warranted because the JSH guideline advocates further careful discussion of this clinical question.View full abstractDownload PDF (962K) -
Kazuhiro IKEGAME, Hiroyasu OGAWA2015 Volume 56 Issue 3 Pages 289-297
Published: 2015
Released on J-STAGE: April 15, 2015
JOURNAL RESTRICTED ACCESSStem cell transplantation from HLA-haploidentical related donors (haploSCT) has been highlighted as an alternative donor source. The regimen consisting of post-transplant cyclophosphamide (PTCY) has been highly prevalent in the US, Europe, and Japan. Considering the status overseas and the current status in Japan, we aim to show our efforts in haploSCT. We initially established the “haplo-full (original)” regimen, which was found to be excessively toxic for general use. Thus, we added ATG to diminish the GVH reaction (haplo-full with ATG). Unfortunately, “haplo-mini (original)” was found to be relatively weak against refractory diseases. Thus, we intensified the preconditioning regimen, which has enabled us to deal with refractory and post-transplant relapse (FAMC-T). One of the characteristics in haploSCT in Hyogo is the use of steroids from the beginning of SCT. The aim of this strategy is to diminish the inflammation affecting GVHD-target organs, and to suppress chemokine release. Since chemokines produced by the preconditioning regimen are well known to induce GVHD, chemokine suppression should effectively suppress GVHD development. The dependency of the GVL effect on chemokines is unclear, possibly serving as a therapeutic window to separate GVHD from the GVL effect.View full abstractDownload PDF (1803K) -
Makoto ONIZUKA2015 Volume 56 Issue 3 Pages 298-303
Published: 2015
Released on J-STAGE: April 15, 2015
JOURNAL RESTRICTED ACCESSThe poor survival of Ph+ALL patients treated with chemotherapy alone has been substantially improved through the use of allogeneic hematopoietic stem cell transplantation in patients experiencing their first complete remission and, more recently, by combining tyrosine kinase inhibitors with induction and post-remission chemotherapy. However, disease relapse after transplantation remains a serious issue for Ph+ALL patients. This review summarizes the increasing knowledge gained by using TKIs for Ph+ALL treatment and considers how patients should be categorized according to their levels of MRD so as to be provided the most suitable therapy, in order to prevent Ph+ALL recurrence and improve survival.View full abstractDownload PDF (650K) -
Noriko DOKI2015 Volume 56 Issue 3 Pages 304-311
Published: 2015
Released on J-STAGE: April 15, 2015
JOURNAL RESTRICTED ACCESSThe need for guidance regarding appropriate systematic long-term follow-up (LTFU) of hematopoietic cell transplantation (HCT) survivors has been recognized. Guidelines for screening and preventive practices for HCT survivors were published in 2006. An international group of transplantation experts updated the recommendations in 2011. Several points regarding the management of late complications must be addressed. Therefore, it is difficult to assess them all at routine visits to the hospital. We herein report the LTFU system employed in the Tokyo Metropolitan Cancer and Infectious Diseases Center, Komagome Hospital. The main purposes include the assessment of chronic GVHD severity, the management of late complications, and patient education for the prevention of infections. We created handbooks for the management of late complications. The questionnaire answered by patients revealed that 93% could understand the necessity for screening of second malignancies and that 84% were satisfied with the LTFU system. The LTFU system is used to provide long-term support according to the patients' social circumstances.View full abstractDownload PDF (1781K)
Case Reports
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Hiroyuki YAMAZAKI, Tadakazu KONDO, Goichi TATSUMI, Shin-ichi KOTANI, Y ...2015 Volume 56 Issue 3 Pages 312-316
Published: 2015
Released on J-STAGE: April 15, 2015
JOURNAL RESTRICTED ACCESSRecombinant human thrombomodulin (rTM) improves the blood coagulation disorder characteristic of disseminated intravascular coagulation (DIC) as well as, or even better than, other anti-DIC drugs. On post-marketing surveillance, its effectiveness has been recognized for hematologic disorders, sepsis and solid tumor subgroups. However, the effect on hemophagocytic syndrome (HPS) complicated by DIC remains unclear. We treated three HPS patients with rTM in addition to chemotherapy for the underlying diseases including nasal NK/T cell lymphoma, angioimmunoblastic T-cell lymphoma and refractory acute myeloid leukemia post cord blood transplantation. Although being refractory to medical management was suspected in our cases, clinical status rapidly came under control including not only amelioration of the blood coagulation disorder but also inflammatory reactions, such as serum ferritin and lactic acid dehydrogenase abnormalities, which represent HPS activity. These observations suggest that rTM might exert marked synergistic effects on HPS with DIC. Given the results obtained in these three cases, administration of rTM appears to offer a promising method of treating HPS complicated by DIC.View full abstractDownload PDF (1007K) -
Yumi NOZAKI, Megumi KOSHIISHI, Yuki SUEKI, Ichirou KAWASHIMA, Takeo YA ...2015 Volume 56 Issue 3 Pages 317-322
Published: 2015
Released on J-STAGE: April 15, 2015
JOURNAL RESTRICTED ACCESSA 60-year-old woman was admitted to our hospital with anemia and thrombocytopenia. Serum testing showed platelet-associated IgG elevation and she was positive on the direct and indirect Coombs tests. Together with bone marrow examination, these findings indicated a diagnosis of Evans syndrome. At diagnosis, she also had an IgM-κ type of monoclonal gammopathy of unknown significance. Initially, we administered steroids and her hemolytic anemia showed improvement. In contrast, only transient recovery of platelet counts was observed and her platelet counts rapidly decreased after steroid dose reduction. Thus, we treated her with a TPO-agonist, romiplostim. During the clinical course, she showed gradual serum IgM elevation. We thus performed another bone marrow biopsy and diagnosed her as having Waldenström's macroglobulinemia (WM). We started treatment with rituximab for WM. Together with the serum IgM reduction, she showed marked improvement of thrombocytopenia. This is a very rare case of WM initially presenting as autoimmune hemolytic anemia and immunethrombocytopenia associated with IgG class auto-antibody. Our experience suggests the usefulness of rituximab and romiplostim for the treatment of immunethrombocytopenia associated with WM.View full abstractDownload PDF (2670K) -
Yasuhiro NAGAMACHI, Naofumi YAMAUCHI, Hirohito MURAMATSU, Naoya MIYAJI ...2015 Volume 56 Issue 3 Pages 323-328
Published: 2015
Released on J-STAGE: April 15, 2015
JOURNAL RESTRICTED ACCESSA 51-year-old man was admitted to our hospital complaining of right upper quadrant abdominal and back pain. Severe hepatomegaly (six fingerbreadths) was detected by liver palpation. Blood test results showed cholestatic liver disease. He was diagnosed with amyloidosis by liver biopsy. Bone marrow aspiration revealed 15% of contents to be plasma cells. BJPκ was detected by urine electrophoresis. Therefore, he was diagnosed with the BJPκ type of multiple myeloma with systemic amyloidosis. The patient was treated with bortezomib, dexamethasone and high-dose melphalan with autologous peripheral blood stem cell transplantation. He achieved VGPR (very good partial response) after transplantation. Hepatomegaly improved but swelling persisted, and he was therefore treated with 1.3 mg/m2/day of bortezomib and 20 mg/day of dexamethasone on day 1 and day 15 in 28-day cycles. Upon finishing 22 cycles in June 2014, his liver had returned to normal size. Restoration of normal liver size after treatment is rare in cases with severe hepatomegaly due to systemic amyloidosis. We thus report our case with a review of the relevant literature.View full abstractDownload PDF (2071K) -
Mizuki WATANABE, Daisuke KAJI, Meiko HASHIMOTO-MAEDA, Go YAMAMOTO, Yuk ...2015 Volume 56 Issue 3 Pages 329-334
Published: 2015
Released on J-STAGE: April 15, 2015
JOURNAL RESTRICTED ACCESSA 52-year-old female presented with stroke-like symptoms after high-dose methotrexate (HDMTX) therapy and MTX intrathecal injection (IT-MTX) as central nervous system (CNS) prophylaxis for intravascular large B-cell lymphoma (IVLBCL). She had been diagnosed as having IVLBCL without CNS involvement 5 months earlier and had received 6 courses of R-CHOP and 2 courses of HDMTX combined with IT-MTX. She experienced acute-onset right hemiparesis involving the face and arm, along with dysarthria, 7 days after the second HDMTX infusion. Brain magnetic resonance imaging (MRI) and cerebrospinal fluid results were normal and suggested neither stroke nor CNS infiltration. Her symptoms gradually resolved within 4 days and follow-up neurologic examination showed no abnormalities. MRI on day 2 (after the onset) showed an area of hyper-intensity on diffusion weighted imaging (DWI). Follow-up MRI performed on day 38 showed resolution of the DWI intensity, while the T2 and FLAIR signals became more evident. Based on her clinical course and these MRI findings, she was diagnosed as having MTX-induced subacute encephalopathy. This syndrome has been reported mainly in children with ALL after HDMTX or IT-MTX, but there have been few reports of adult patients. MTX-induced subacute encephalopathy should be taken into account as a possible cause of neurologic manifestations because early differentiation from stroke and CNS infiltration is essential to successful management.View full abstractDownload PDF (1434K)
Short Reports
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Shojiro HAJI, Yasunobu ABE, Mariko TSUDA, Kosuke TOYODA, Ilseung CHOI, ...2015 Volume 56 Issue 3 Pages 335-338
Published: 2015
Released on J-STAGE: April 15, 2015
JOURNAL RESTRICTED ACCESSCD 20 positive myeloma with small lymphoplasmacytoid morphology is difficult to differentiate from mature B-cell lymphoma. A 71-year-old male was referred to our hospital because of osteolytic vertebral fractures and anemia. Urine was positive for Bence Jones protein, κ type. Bone marrow consisted of approximately 30% small lymphoplasmacytoid cells with scant cytoplasm, and these cells were positive for CD20, CD23 and CD138. FISH analysis revealed t(11;14)(CCND1/IGH). Myeloma with t(11;14) is closely associated with small lymphoplasmacytoid appearance and CD20 and CD23 expressions. The patient was diagnosed as having myeloma based on clinical and cytogenetic findings, and achieved VGPR (very good partial response) after treatment with lenalidomide.View full abstractDownload PDF (1733K)
Introduce My Article
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2015 Volume 56 Issue 3 Pages 339
Published: 2015
Released on J-STAGE: April 15, 2015
JOURNAL RESTRICTED ACCESSDownload PDF (564K)
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