記述言語：英語   掲載種別：研究論文（学術雑誌）  

Letermovir is used to prevent cytomegalovirus infection in hematopoietic stem cell transplantation (HSCT) recipients. Although this agent decreases voriconazole exposure in healthy individuals, the effect of coadministration of letermovir and voriconazole in HSCT recipients is unknown. This retrospective, observational, single-center study was conducted between January 2016 and July 2019 to examine the voriconazole concentration-to-dose ratio over three periods: (A) (days -7 to -1 [day 0: day of HCST]), (B) (days 4-10), and (C) (days 11-17). Forty-two HSCT recipients administered voriconazole were divided into the following two groups based on letermovir coadministration: letermovir (n = 15) and control (n = 27). The percent change (-33.2%, p < 0.05) in the voriconazole concentration-to-dose ratio from periods A to C in the letermovir group was significantly lower than that in the control group. Therefore, frequent therapeutic drug monitoring of voriconazole concentrations and subsequent dose adjustments should be performed regularly in HSCT recipients.

DOI： 10.1038/s41409-020-01093-w

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

HLA haploidentical hematopoietic stem cell transplantation (HSCT), i.e., HSCT from a 1-HLA-haplotype-mismatched family donor, has been successfully performed even as a second transplantation for posttransplant relapse. Is the haploidentical the limit of HLA mismatches in HSCT? In order to explore the possibility of HLA-mismatched HSCT from family donors beyond haploidentical relatives, we conducted a prospective phase I/II study of 2-HLA-haplotype-mismatched HSCT (2-haplo-mismatch HSCT). We enrolled 30 patients with posttransplant relapse (acute myeloid leukemia: 18, acute lymphoblastic leukemia: 11, non-Hodgkin lymphoma: 1). 2-haplo-mismatch HSCT was performed as the second to sixth transplantations. The donors were siblings (n = 12), cousins (n = 16), and second cousins (n = 2). The conditioning regimen consisted of fludarabine, cytarabine, melphalan, low-dose anti-thymocyte globulin, and 3 Gy of total body irradiation. Graft-versus-host disease (GVHD) prophylaxis consisted of tacrolimus, methylprednisolone, and mycophenolate mofetil. All patients achieved neutrophil engraftment, except for a case of early death. The cumulative incidences of grades II-IV and III-IV acute GVHD were 36.7% and 16.7%, respectively. The overall survival at 1 year, relapse, and non-relapse mortality rates was 30.1%, 38.9%, and 44.3%, respectively. Considering the poor prognosis of posttransplant relapse, 2-haplo-mismatch HSCT can be an alternative option in a second or third transplantation.

DOI： 10.1038/s41409-020-0980-8

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: Hepatotoxicity and visual symptoms are common adverse effects (AEs) of voriconazole therapy. OBJECTIVE: To retrospectively evaluate the effects of treatment modification based on therapeutic drug monitoring on AEs in patients undergoing voriconazole therapy. METHODS: The target voriconazole trough concentration (Cmin ) was 1-5 µg/mL. Receiver operating characteristic curves were used to determine Cmin cut-offs for AEs. RESULTS: A total of 401 patients were included. Among 108 patients with high initial Cmin , voriconazole was discontinued in 32 and the dose was reduced in 71. Among 44 patients with low initial Cmin , voriconazole was discontinued in 4 and the dose was increased in 19. Hepatotoxicity occurred in 6.0% of patients, after a median of 10 days. Visual symptoms were evident in 9.5% of patients after a median of 4 days. Initial Cmin was significantly associated with visual symptoms but not hepatotoxicity, which suggested the effect of treatment modification on hepatotoxicity. However, both hepatotoxicity and visual symptoms were significantly correlated with Cmin at the onset of AEs, and the Cmin cut-offs were 3.5 μg/mL for hepatotoxicity and 4.2 μg/mL for visual symptoms. Voriconazole was discontinued after the occurrence of AEs in 62.5% of patients with hepatotoxicity but only 26.3% of patients with visual symptoms. With dose adjustment, treatment was completed in 8/9 patients with hepatotoxicity and 27/28 patients with visual symptoms. CONCLUSIONS: A significant preventive effect was demonstrated on hepatotoxicity, but not on visual symptoms because of earlier occurrence. With treatment modification after the occurrence of AEs, most patients completed therapy.

DOI： 10.1111/myc.13129

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

We evaluated the impact of FLT3-ITD, NPM1 mutations, and double mutant CEBPa (dmCEBPa) on overall survival (OS) after relapse in patients with cytogenetically intermediate-risk acute myeloid leukemia (AML) who were treated with chemotherapy alone in the first remission (CR1). Patients aged 16-65 years diagnosed with cytogenetically intermediate-risk AML, and who achieved CR1 were included. We retrospectively analyzed FLT3-ITD, NPM1 mutations and CEBPa using samples obtained at diagnosis, which therefore did not affect the therapeutic decisions. Among 235 patients who had achieved CR1, 152 relapsed, and 52% of them achieved second CR. The rate of achieving second CR was significantly higher (85%) in those with dmCEBPa. Patients with FLT3-ITD had significantly worse OS after relapse than those without (19% vs 41%, p = 0.002), while OS was comparable between patients with and without NPM1 mutations (37% vs 34%, p = 0.309). Patients with dmCEBPa had improved OS than those without (61% vs 32%, p = 0.006). By multivariate analysis, FLT3-ITD was independently associated with worse OS after relapse [hazard ratio (HR) 1.99, 95% CI 1.27-3.12, p = 0.003], and dmCEBPa with improved OS (HR 0.40, 95% CI 0.17-0.93, p = 0.033). Our data show that screening for these mutations at diagnosis is useful for facilitating effective therapeutic decision-making even after relapse.

DOI： 10.1007/s12185-020-02894-x

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: Acute kidney injury (AKI) and chronic kidney disease (CKD) are considered common complications after allogeneic hematopoietic stem cell transplantation (allo-HSCT). OBJECTIVES AND METHOD: In this study, 114 patients who had undergone allo-HSCT were retrospectively analyzed to investigate the risk factors for onset of posttransplant AKI and CKD as defined by the new Kidney Disease Improving Global Outcomes criteria. RESULTS: Seventy-four patients (64.9%) developed AKI and 25 (21.9%) developed CKD. The multivariate analysis showed that the risk factors for developing stage 1 or higher AKI were age ≥46 years at the time of transplant (p = 0.001) and use of ≥3 nephrotoxic drugs (p = 0.036). For CKD, the associated risk factors were disease status other than complete remission at the time of transplantation (p = 0.018) and onset of AKI after transplant (p = 0.035). The 5-year overall survival (OS) was significantly reduced by development of AKI (p < 0.001), but not CKD. Posttransplant AKI significantly increased the 5-year nonrelapse mortality (p < 0.001), whereas posttransplant CKD showed an increasing tendency, but the difference was not significant. CONCLUSIONS: Posttransplant AKI impacts OS, significantly increases the risk of CKD, and is significantly associated with disseminated intravascular coagulation and use of ˃3 nephrotoxic drugs.

DOI： 10.1159/000504354

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Letermovir (LMV) is a new antiviral drug used to prevent cytomegalovirus infection in hematopoietic stem cell transplantation (HSCT) recipients. It has been reported to increase tacrolimus (TAC) exposure and decrease voriconazole (VRCZ) exposure in healthy participants. However, VRCZ inhibits the metabolism of TAC. Thus, the effects of LMV on TAC exposure in patients receiving VRCZ are unknown. This retrospective, observational, single-center study was conducted between May 2018 and April 2019. The TAC concentration/dose (C/D) ratio, VRCZ concentration, and VRCZ C/D ratio for 7 days before and for the first and second 7-day periods after the initiation of LMV administration were evaluated. Fourteen HSCT recipients receiving VRCZ were enrolled. There was no significant difference in the TAC C/D ratio for 7 days before and for the first and second 7-day periods after initiating LMV administration (median: 866 [IQR: 653-953], 842 [IQR: 636-1031], and 906 [IQR: 824-1210] [ng/mL]/[mg/kg], respectively). In contrast, the VRCZ C/D ratio and concentration for the first and second 7-day periods after LMV initiation were significantly lower than those before initiating LMV administration (mean 1.11 ± 0.07, 0.12 ± 0.08, and 0.22 ± 0.12 [μg/mL]/[mg/kg] and 0.7 ± 0.5, 0.8 ± 0.5, and 1.3 ± 0.7 μg/mL, respectively; n = 12). This can be explained by the increase in TAC concentration caused by CYP3A4 inhibition due to LMV and by the decrease in TAC concentration ascribed to the decrease in VRCZ concentration by CYP2C19 induction due to LMV. These results suggest that it is unnecessary to adjust the dose of TAC based on LMV initiation; however, it is necessary to adjust the dose of TAC based on conventional TAC concentration measurements.

DOI： 10.7150/ijms.42011

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

The clinical impact of KIT mutations in core binding factor acute myeloid leukemia (CBF-AML) is still unclear. In the present study, we analyzed the prognostic significance of each KIT mutation (D816, N822K, and other mutations) in Japanese patients with CBF-AML. We retrospectively analyzed 136 cases of CBF-AML that had gone into complete remission (CR). KIT mutations were found in 61 (45%) of the patients with CBF-AML. D816, N822K, D816 and N822K, and other mutations of the KIT gene were detected in 29 cases (21%), 20 cases (15%), 7 cases (5%), and 5 cases (4%), respectively. The rate of relapse-free survival (RFS) and overall survival (OS) in patients with D816 and with both D816 and N822K mutations was significantly lower than in patients with other or with no KIT mutations (RFS: p < 0.001, OS: p < 0.001). Moreover, stratified analysis of the chromosomal abnormalities t(8;21)(q22;q22) and inv(16)(p13.1q22), t(16;16)(p13.1;q22) showed that D816 mutation was associated with a significantly worse prognosis. In a further multivariate analysis of RFS and OS, D816 mutation was found to be an independent risk factor for significantly poorer prognosis. In the present study, we were able to establish that, of all KIT mutations, D816 mutation alone is an unfavorable prognostic factor.

DOI： 10.1007/s00277-017-3074-y

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Acute lymphoblastic leukemias (ALL) positive for KMT2A/AFF1 (MLL/AF4) translocation, which constitute 60% of all infant ALL cases, have a poor prognosis even after allogeneic hematopoietic stem cell transplantation (allo-HSCT). This poor prognosis is due to one of two factors, either resistance to TNFα, which mediates a graft-versus-leukemia (GVL) response after allo-HSCT, or immune resistance due to upregulated expression of the immune escape factor S100A6. Here, we report an immune stimulatory effect against KMT2A/AFF1-positive ALL cells by treatment with the anti-allergy drug amlexanox, which we found to inhibit S100A6 expression in the presence of TNF-α. In KMT2A/AFF1-positive transgenic (Tg) mice, amlexanox enhanced tumor immunity and lowered the penetrance of leukemia development. Similarly, in a NOD/SCID mouse model of human KMT2A/AFF1-positive ALL, amlexanox broadened GVL responses and extended survival. Our findings show how amlexanox degrades the resistance of KMT2A/AFF1-positive ALL to TNFα by downregulating S100A6 expression, with immediate potential implications for improving clinical management of KMT2A/AFF1-positive ALL. Cancer Res; 77(16); 4426-33. ©2017 AACR.

DOI： 10.1158/0008-5472.CAN-16-2974

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

In recent years, it has been reported that the frequency of DNA-methylation regulatory gene mutations - mutations of the genes that regulate gene expression through DNA methylation - is high in acute myeloid leukemia. The objective of the present study was to elucidate the clinical characteristics and prognosis of acute myeloid leukemia with associated DNA-methylation regulatory gene mutation. We studied 308 patients with acute myeloid leukemia. DNA-methylation regulatory gene mutations were observed in 135 of the 308 cases (43.8%). Acute myeloid leukemia associated with a DNA-methylation regulatory gene mutation was more frequent in older patients (P<0.0001) and in patients with intermediate cytogenetic risk (P<0.0001) accompanied by a high white blood cell count (P=0.0032). DNA-methylation regulatory gene mutation was an unfavorable prognostic factor for overall survival in the whole cohort (P=0.0018), in patients aged ≤70 years, in patients with intermediate cytogenetic risk, and in FLT3-ITD-negative patients (P=0.0409). Among the patients with DNA-methylation regulatory gene mutations, 26.7% were found to have two or more such mutations and prognosis worsened with increasing number of mutations. In multivariate analysis DNA-methylation regulatory gene mutation was an independent unfavorable prognostic factor for overall survival (P=0.0424). However, patients with a DNA-methylation regulatory gene mutation who underwent allogeneic stem cell transplantation in first remission had a significantly better prognosis than those who did not undergo such transplantation (P=0.0254). Our study establishes that DNA-methylation regulatory gene mutation is an important unfavorable prognostic factor in acute myeloid leukemia.

DOI： 10.3324/haematol.2016.143073

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

We performed a decision analysis comparing allogeneic hematopoietic cell transplantation (allo-HCT) versus chemotherapy in first complete remission for patients with cytogenetically intermediate-risk acute myeloid leukemia, depending on the presence or absence of FLT3-internal tandem duplication (ITD), nucleophosmin (NPM1), and CCAAT/enhancer binding protein alpha (CEBPA) mutations. Adjusted means of the patient-reported EQ-5D index were used as quality-of-life (QOL) estimates. In 332 patients for which FLT3-ITD status was available, FLT3-ITD was present in 60. In 272 patients without FLT3-ITD, NPM1 mutations were present in 83. CEBPA biallelic mutations were detected in 53 patients. For patients harboring FLT3-ITD, allo-HCT improved life expectancy (LE) (52 versus 32 months during 10-year observation) and QOL-adjusted life expectancy (QALE, 36 versus 21). Monte-Carlo simulation identified allo-HCT as the favored strategy in 100% of simulations. In patients without FLT3-ITD, allo-HCT improved LE/QALE with or without NPM1 mutations. However, sensitivity analyses showed that the results were not robust enough. For patients harboring CEBPA biallelic mutations, chemotherapy was favored (LE, 53 versus 84; QALE, 37 versus 59), whereas, for patients with monoallelic mutations or wild-type CEBPA, allo-HCT was favored (LE, 68 versus 54; QALE, 48 versus 37). Sensitivity analyses did not change the results in either group. In conclusion, based on a Markov decision analysis, allo-HCT was a favored postremission strategy in patients with FLT3-ITD, and chemotherapy was favored in patients with biallelic CEBPA mutations. A prospective study is warranted to determine the value of allo-HCT, especially in FLT3-ITD-negative patients.

DOI： 10.1016/j.bbmt.2016.03.015

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Objective In hematological malignancy patients, the complication of acute respiratory failure often reaches a degree of severity that necessitates mechanical ventilation. The objective of the present study was to investigate the therapeutic outcomes of mechanical ventilation in hematological malignancy patients with respiratory failure and to analyze the factors that are associated with successful treatment in order to identify the issues that should be addressed in the future. Methods The present study was a retrospective analysis of 71 hematological malignancy patients with non-cardiogenic acute respiratory failure who were treated with mechanical ventilation at Nippon Medical School Hospital between 2003 and 2014. Results Twenty-six patients (36.6%) were treated with mechanical ventilation in an intensive care unit (ICU). Non-invasive positive pressure ventilation (NPPV) was applied in 29 cases (40.8%). The rate of successful mechanical ventilation treatment with NPPV alone was 13.8%. The rate of endotracheal extubation was 17.7%. A univariate analysis revealed that the following factors were associated with the successful extubation of patients who received invasive mechanical ventilation: respiratory management in an ICU (p=0.012); remission of the hematological disease (p=0.011); female gender (p=0.048); low levels of accompanying non-respiratory organ failure (p=0.041); and the non-use of extracorporeal circulation (p=0.005). A subsequent multivariate analysis revealed that respiratory management in an ICU was the only variable associated with successful extubation (p=0.030). Conclusion The outcomes of hematological malignancy patients who receive mechanical ventilation treatment for respiratory failure are very poor. Respiratory management in an ICU environment may be useful in improving the therapeutic outcomes of such patients.

DOI： 10.2169/internalmedicine.55.5822

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記述言語：日本語   出版者・発行元：日本感染症学会・日本化学療法学会  

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記述言語：日本語   出版者・発行元：一般社団法人 日本血液学会  

DOI： 10.11406/rinketsu.64.1395

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記述言語：日本語   出版者・発行元：(一社)日本集中治療医学会  

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記述言語：日本語   出版者・発行元：金原出版(株)  

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記述言語：日本語   出版者・発行元：(一社)日本輸血・細胞治療学会  

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記述言語：日本語   出版者・発行元：(一社)日本臨床微生物学会  

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記述言語：英語   出版者・発行元：(一社)日本血液学会  

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記述言語：英語   出版者・発行元：(一社)日本血液学会  

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記述言語：日本語   出版者・発行元：(株)ヴァンメディカル  

＜文献概要＞血液内科領域における真菌感染は原疾患の治療中に発生することが多く,コロナパンデミックが直接影響することは少ない。本稿では最近改訂作業が行われている『侵襲性カンジダ症に対するマネジメントのための臨床実践ガイドライン』の内容を中心に,好中球減少時の抗真菌薬マネジメントについて概説する。

DOI： 10.34426/kk.0000000196

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その他リンク： https://search.jamas.or.jp/default/link?pub_year=2021&ichushi_jid=J03177&link_issn=&doc_id=20210617020008&doc_link_id=10.34426%2Fkk.0000000196&url=https%3A%2F%2Fdoi.org%2F10.34426%2Fkk.0000000196&type=%E5%8C%BB%E6%9B%B8.jp_%E3%82%AA%E3%83%BC%E3%83%AB%E3%82%A2%E3%82%AF%E3%82%BB%E3%82%B9&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00024_2.gif

記述言語：日本語   出版者・発行元：東京 : ヴァンメディカル  

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その他リンク： https://ndlsearch.ndl.go.jp/books/R000000004-I031571185

記述言語：日本語   出版者・発行元：(一社)日本リンパ網内系学会  

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記述言語：日本語   出版者・発行元：(一社)日本リンパ網内系学会  

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記述言語：日本語   出版者・発行元：(一社)日本医真菌学会  

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記述言語：日本語   出版者・発行元：(一社)日本医真菌学会  

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記述言語：日本語   出版者・発行元：(公社)日本化学療法学会  

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記述言語：日本語   出版者・発行元：(一社)日本リンパ網内系学会  

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記述言語：日本語   出版者・発行元：(一社)日本輸血・細胞治療学会  

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記述言語：日本語   出版者・発行元：日本医真菌学会  

DOI： 10.11534/jsmm.61.suppl1.0_45_2

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記述言語：日本語   出版者・発行元：(一社)日本エイズ学会  

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記述言語：日本語   出版者・発行元：(一社)日本輸血・細胞治療学会  

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記述言語：日本語   出版者・発行元：(一社)日本組織適合性学会  

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記述言語：日本語   出版者・発行元：(公社)日本化学療法学会  

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記述言語：日本語   出版者・発行元：(一社)日本環境感染学会  

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記述言語：日本語   出版者・発行元：東京 : 科学評論社  

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その他リンク： https://ndlsearch.ndl.go.jp/books/R000000004-I029360075

記述言語：英語   出版者・発行元：(一社)日本血液学会-東京事務局  

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記述言語：英語   出版者・発行元：(一社)日本血液学会-東京事務局  

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記述言語：英語   出版者・発行元：(一社)日本血液学会-東京事務局  

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記述言語：英語   出版者・発行元：(一社)日本血液学会-東京事務局  

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記述言語：英語   出版者・発行元：(一社)日本血液学会-東京事務局  

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記述言語：日本語   出版者・発行元：応用統計学会  

生存時間解析では，観測開始時点で記録される共変量の値が，目標事象発生（打切りあるいは死亡）まで一定であるとして解析してきた．しかし，患者は観測期間中モニタリングされ，目標事象までに種々のイベント発生の検査値が記録される．本稿では，共変量の値が時間に依存して変化する骨髄移植（同種造血幹細胞移植）データを取上げる．イベント発生ごとに変化する共変量の時系列情報を取込むため，自然言語処理の分野で急激な発展を遂げているリカレントニューラルネットワーク（Recurrent Neural Network；RNN）を活用する．具体的には，ブートストラップ法を援用し，最適な隠れユニット数の決定，影響分析による外れ値の検出，モデル改善の検証，およびモデルの適合度検定などを目途とする．部分ロジスティックモデルを拡張したフィードフォワードニューラルネットワーク（Feed-Forward Neural Network；FFNN），更にそれに帰還路を加えたRNNを活用することにより，非線形なイベントヒストリー解析が可能になる．RNNを活用し，観測期間中のイベント発生時点における，ある共変量のもとでの半年後条件付き生存確率を予測すれば，従来のFFNN，部分ロジスティックモデルおよびCoxの比例ハザードモデルに比べて，イベントの発生現象を的確に表現できる．

DOI： 10.5023/jappstat.47.71

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その他リンク： https://ndlsearch.ndl.go.jp/books/R000000004-I029502268

記述言語：日本語   出版者・発行元：日本内科学会-関東地方会  

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記述言語：英語   掲載種別：研究発表ペーパー・要旨（国際会議）   出版者・発行元：AMER SOC HEMATOLOGY  

Web of Science

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記述言語：英語   掲載種別：研究発表ペーパー・要旨（国際会議）   出版者・発行元：AMER SOC HEMATOLOGY  

Web of Science

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記述言語：日本語   出版者・発行元：日本内科学会-関東地方会  

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記述言語：日本語   出版者・発行元：株式会社医学書院  

71歳,女性.初診時,顔面・躯幹に紅斑,上肢関節部に結節,指の腫脹などの多彩な皮疹があった.皮膚病理組織では真皮上層から皮下までびまん性にリンパ球様細胞が浸潤していた.免疫染色から皮膚T細胞リンパ腫を疑い,母に成人T細胞白血病/リンパ腫(adult T-cell leukemia/lymphoma:ATLL)の既往があることを聴取した.抗HTLV-1抗体陽性で,皮疹部の遺伝子解析でHTLV-1プロウイルスのモノクローナルな組み込みがあり,ATLLと診断した.病型はくすぶり型であり,臨床症状と病理組織学的所見より結節腫瘤型と分類した.経過中に皮疹の増悪,表在リンパ節の腫脹,血液検査で異型リンパ球の出現がみられた.急性転化したため化学療法を施行し,現在症状は改善している.結節腫瘤型のATLLは短期間で急性転化する場合があるため,定期的な経過観察と急性転化時の早急な対応が必要である.(著者抄録)

DOI： 10.11477/mf.1412204820

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その他リンク： https://ndlsearch.ndl.go.jp/books/R000000004-I027454585

記述言語：日本語   出版者・発行元：老年者造血器疾患研究会  

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記述言語：日本語   出版者・発行元：(一社)日本血液学会-東京事務局  

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記述言語：日本語   出版者・発行元：(公社)日本皮膚科学会  

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記述言語：日本語   出版者・発行元：(株)メディカル葵出版  

組織学的および遺伝子学的解析で多クローン性B細胞増殖を呈したが、眼窩から眼内浸潤と脳播種をきたし、臨床的には悪性リンパ腫を疑わせた1例を報告する。症例は43歳、男性。右眼の疼痛と視力低下を主訴に近医で特発性視神経炎と診断、ステロイドパルスで視力回復後、左眼の疼痛と視力低下が出現した。血漿交換まで施行したが、左眼は光覚消失となり日本医科大学眼科紹介受診した。MRI(磁気共鳴画像)で左眼窩先端部と海綿静脈洞に増強効果を認め、左眼網膜下黄白色病変が出現した。硝子体生検はIL-10/IL-6比1以下、B細胞遺伝子再構成PCR(polymerase chain reaction)で多クローン性B細胞増殖を認めた。デキサメタゾン全身投与と放射線療法で眼病変は沈静化したが、左側頭葉播種性病変が出現した。脳生検も多クローン性B細胞増殖組織であったが、臨床経過より悪性リンパ腫を考え、メトトレキセート全身投与ならびに全脳照射を行った。悪性リンパ腫の確定診断に至らなかった理由は、腫瘍反応リンパ球浸潤や頻回治療による浸潤細胞数減少などが考えられた。(著者抄録)

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記述言語：日本語   出版者・発行元：(一社)日本内科学会  

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記述言語：英語   掲載種別：研究発表ペーパー・要旨（国際会議）   出版者・発行元：AMER SOC HEMATOLOGY  

Web of Science

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記述言語：日本語   出版者・発行元：(一社)日本血液学会-東京事務局  

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記述言語：日本語   出版者・発行元：金原出版(株)  

症例は64歳男性で、顔面と胸部の紅斑、手足背側の圧痕性浮腫、上下肢の関節痛を主訴に、近医にてセレスタミン投与を受け一時軽快したが、再燃を繰り返した。血液検査にて異型リンパ球数の上昇、HTLV-1抗TAIの陽性を指摘され、当院受診となった。皮膚生検、血液学的検査、骨髄穿刺所見、PET-CT所見より、RS3PE症候群を伴ったくすぶり型の成人T細胞白血病/リンパ腫(ATLL)と診断した。ATLLに対し内服PUVA療法と副腎皮質ステロイド軟膏の外用療法、RS3PE症候群に対しプレドニゾロンの内服療法を開始したが皮疹は改善せず、ATLLは入院1ヵ月後にくすぶり型から急性型へ移行した。

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記述言語：日本語   出版者・発行元：(一社)日本内科学会  

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記述言語：日本語   出版者・発行元：公益社団法人 日本皮膚科学会  

42歳男性．29歳時頃，健診で蛋白尿を指摘され，同時期より体幹に暗赤褐色斑が出現した．3年後，胸部異常陰影を指摘され，Castleman病を疑われ，当科を紹介された．血液検査で多クローン性高γグロブリン血症，貧血，CRP高値，血沈亢進，血清IL-6高値を認めた．皮膚生検では真皮内に胚中心の形成を伴うリンパ濾胞が多発し，多クローン性の形質細胞が高度に浸潤していた．皮膚組織より施行した遺伝子再構成はサザンブロット法，PCR法ともIgH鎖はgerm lineであった．CTでは両側肺野末梢に小粒状影の散在，両側腋窩，気管近傍，縦隔リンパ節腫脹，肝・腎腫大を認めた．Multicentric typeのCastleman病と診断し，プレドニゾロン，シクロホスファミドによる治療を試みた．症状は緩徐に進行したため，38歳時よりトシリズマブを投与したところ，臨床症状，検査所見ともに改善した．しかし投与を開始して2年8カ月後より右内深頸リンパ節腫大が出現し，生検の結果，Hodgkinリンパ腫（混合細胞型）と診断された．トシリズマブを中止し，当院血液内科で化学療法を施行したところ，効果判定はComplete remission（CR）であった．

DOI： 10.14924/dermatol.122.1571

CiNii Books

CiNii Research

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その他リンク： https://ndlsearch.ndl.go.jp/books/R000000004-I023637237

記述言語：日本語   出版者・発行元：(一社)日本血液学会-東京事務局  

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記述言語：日本語   出版者・発行元：(一社)日本血液学会-東京事務局  

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記述言語：日本語   出版者・発行元：(公社)日本皮膚科学会  

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記述言語：日本語   出版者・発行元：(一社)日本医学教育学会  

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記述言語：日本語   出版者・発行元：日本医科大学医学会  

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