Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.1111/ped.14583

PubMed

researchmap

Language：English   Publishing type：Research paper (scientific journal)  

Lymphomatoid papulosis (LyP) is a chronic, recurrent benign skin disease characterized by histological features of a CD 30-positive cutaneous T-cell lymphoproliferative disorder. It is rare, with an annual, worldwide incidence of 1.2 - 1.9 per million, and accounts for 16-47% of pediatric cutaneous lymphoproliferative disorders. It often occurs on the extremities or the trunk and rarely affects the face or genitals. Its onset may be triggered by irradiation therapy, immunomodulating agents, infection or atopic dermatitis. It has a benign course but is associated with certain hematological malignancies. Mycosis fungoides and primary cutaneous anaplastic large cell lymphoma are the most commonly associated hematological malignancies. The incidence of lymphoma in children with LyP has been reported to be 8.5% at most. Most patients who develop lymphomas do so within 4 years of the LyP onset; therefore, patients with LyP should be carefully followed up. Herein, we report a case in which tumors appeared in the left scrotum and under the left lip during maintenance therapy for precursor B-cell acute lymphoblastic leukemia. We needed to distinguish the tumor from extramedullary recurrence of ALL or <i>de novo</i> other cutaneous lymphoma; however, the histological findings of a tumor biopsy resulted in a diagnosis of LyP.

DOI： 10.14740/jmc3718

PubMed

researchmap

Publishing type：Research paper (scientific journal)  

DOI： 10.1038/s41409-020-01076-x

PubMed

researchmap

Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.1186/s40792-020-00873-2

researchmap

Language：English   Publishing type：Research paper (scientific journal)  

Allogeneic hematopoietic stem cell transplantation (HSCT) is the only curative therapy for the hematologic manifestations of Diamond-Blackfan anemia (DBA). However, data regarding the optimal conditioning regimen for DBA patients are limited. We retrospectively compared the outcomes of DBA patients who underwent HSCT using either myeloablative conditioning (MAC) or reduced-intensity conditioning (RIC) regimens. The patients belonged to a cohort treated at our hospitals between 2000 and 2018. HSCT was performed in 27 of 165 patients (16.4%). The median age at the time of HSCT was 3.6 years. Stem cell sources included bone marrow for 25 patients (HLA-matched sibling donors, n = 5; HLA-mismatched related donors, n = 2; HLA-matched/mismatched unrelated donors, n = 18) or cord blood for 2 patients. MAC or RIC regimens were used in 12 and 15 patients, respectively. Engraftment was successful in all 27 patients who underwent HSCT. Three patients who underwent HSCT using MAC regimens developed sinusoidal obstruction syndrome. The 3-year overall survival (OS) and failure-free survival rates (FFS) post-transplantations were 95.2% and 88.4%, respectively, with no significant differences between MAC and RIC regimens. Our data suggest that HSCTs using RIC regimens are effective and obtain engraftment with excellent OS and FFS for young DBA patients.

DOI： 10.1038/s41409-020-01056-1

PubMed

researchmap

Authorship：Corresponding author   Language：English   Publishing type：Research paper (scientific journal)  

<h4>Background</h4>Philadelphia-negative (Ph-negative) myeloproliferative neoplasms (MPNs), including polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF), are exceptionally rare during childhood. Thus, clinical features of pediatric Ph-negative MPNs remain largely unknown. This study was therefore performed to address this.<h4>Methods</h4>We performed a retrospective study to collect clinical information of children diagnosed with Ph-negative MPNs from 2000 to 2016 using questionnaires in qualified institutions in Japan. The results obtained from the questionnaire survey were then combined with those from the national registry data.<h4>Results</h4>Among 50 children identified, five had PV, 44 had ET, and one had PMF. Median age at diagnosis was 14.0, 9.0, and 0 years, respectively. Male to female ratio was 4:1, 21:23, and 1:0, respectively. Detection rates of the <i>JAK2</i> V617F variant were 0/5 in PV and 9/39 in ET. Frequencies of complications, such as thrombosis and subsequent leukemia, were lower than complication frequencies in adults. We identified two children who developed subsequent leukemia, which has not been reported previously, and one of them died.<h4>Conclusion</h4>This is the first nationally representative survey of pediatric Ph-negative MPNs. Given its rarity, an international collaboration with comprehensive genetic analyses might be needed to fully elucidate the clinical and genetic features.

DOI： 10.1002/jha2.39

PubMed

researchmap

Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.1097/mph.0000000000001743

researchmap

Authorship：Corresponding author   Language：Japanese   Publishing type：Research paper (scientific journal)  

researchmap

Language：Japanese   Publishing type：Research paper (scientific journal)  

症例は11歳女児で、8歳時に咳と発熱を主訴に近医を受診、胸部X線検査で右下肺野の浸潤影と縦隔側の腫瘤影を認めた。肺炎の診断で内服治療を行い症状は改善したが、再検の胸部X線検査でも腫瘤影は残存していた。腫瘤性病変疑いで前医を紹介受診し、胸部CTで前縦隔に腫瘤を認め、精査加療目的で当院を紹介入院した。その後、外来経過観察していたが、胸部X線検査で腫瘤影の増大を認め、11歳時の造影CTで腫瘤影の増大と上大動脈の圧排所見を認め、摘出術の方針で入院となった。胸骨正中切開で開胸し、腫瘤を確認すると胸腺との境界が不明瞭であり胸腺合併切除(全摘術)の方針とした。胸腺と腫瘍は境界比較的明瞭で、腫瘍間質のリンパ組織内にもCK5/6陽性細胞は確認できなかったが、一部胸腺から移行するように腫瘍が認められることから、胸腺原発の血管脂肪腫の診断に至った。術後経過は良好で、術後11日目に退院し外来フォロー中である。

researchmap

Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.1111/ped.13749

researchmap

Language：English   Publishing type：Research paper (scientific journal)  

Survival rates in adolescent/young adult (AYA) patients with malignant diseases have improved with the introduction of pediatric-type chemotherapy; however, the higher frequency of treatment-related complications, including infections, remains a major challenge. We hypothesized that the efficacy of antibiotics may differ between AYA and younger children. We aimed to evaluate differences in the efficacy of antibiotics between them by retrospectively analyzing patients registered in previous first-line antibiotic comparative studies on febrile neutropenia (FN). Patients were classified into two groups: patients younger than 15 years of age (children group) and those aged 15 years or older (AYA group). The efficacy of antibiotic therapy was compared between groups. Success of therapy was defined as resolution of febrile episodes and clinical signs of infection within 120 h of the initiation of antibiotic therapy. A total of 818 febrile episodes in 204 patients were analyzed. Antibiotic therapy success rates were lower in the AYA group than in the children group (53.8 vs. 63.7%, P = 0.028), even when patients were restricted to those with bacteremia (11.8 vs. 41.4%, P = 0.025). However, mortality rates did not differ (0 vs. 0.5%, P = 1.000). The efficacy of first-line antibiotic therapy for FN was poorer in AYA patients than in child patients.

DOI： 10.1007/s12185-018-2503-6

researchmap

Language：English   Publishing type：Research paper (scientific journal)  

A 10-year-old girl with persistent erythrocytosis and ruddy complexion was diagnosed with primary familial congenital polycythemia (PFCP) involving a novel heterozygous mutation of c.1220C>A, p.Ser407X in exon 8 of the erythropoietin receptor gene (EPOR). This mutation causes truncation of EPOR, resulting in loss of the cytoplasmic region, which is necessary for negative regulation of erythropoietin signal transmission. Genetic analysis showed that the mutated EPOR was inherited from her mother. Her mother had polycythemia and had undergone venesection several times when she was young, but her polycythemic state appeared to have resolved. Venesection was not needed to maintain Hct levels within normal range. For the case reported here, venesection was also conducted to maintain the blood Hct level below 50%. We observed that after the patient experienced menarche, the volume and frequency of venesection needed to maintain Hct level < 50% were clearly reduced compared with those before menarche. These findings suggest that, in female patients with this type of EPOR mutation, menstruation might reduce blood volume in a manner similar to venesection. Spontaneous remission of erythrocytosis may thus occur after the start of menstrual bleeding.

DOI： 10.1007/s12185-018-2435-1

researchmap

Language：English   Publishing type：Research paper (scientific journal)  

OBJECTIVE: This multicenter cooperative study aimed to analyze the adverse events (AEs) associated with tyrosine kinase inhibitors (TKIs) used as initial treatment for chronic-phase chronic myeloid leukemia (CML-CP) and their impact on outcome. METHODS: We retrospectively evaluated 450 patients with CML-CP who received TKIs between 2004 and 2014. RESULTS: The 5-year overall survival (OS) and event-free survival (EFS) rates were 95.1% and 89.0%, respectively. Patients with comorbidities (46.4%) and aged >/=60 years (50.4%) at diagnosis had significantly inferior OS to those without comorbidities and aged <60. Patients achieved higher rates of major molecular response (MMR) at 6 and 12 months after initial treatment with dasatinib or nilotinib compared to imatinib, but final MMR rates were almost the same. Sixty-six percent of patients required treatment modifications from first-line TKI therapy; the main reasons were AEs (48.4%) and failure (18%). Grade III-IV AEs in first-line TKI therapy were significantly correlated to inferior OS/EFS compared to grade 0-II AEs. CONCLUSION: Although long-term outcomes were similar in CML-CP patients treated with each TKI regardless of first-line TKI selection, severe AEs in first-line TKI therapy decreased their survival rates. Early change in TKIs is recommended, when faced with severe AEs of specific TKIs.

DOI： 10.1111/ejh.13081

researchmap

Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.1097/mph.0000000000000865

researchmap

Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.1002/pbc.26234

researchmap

Authorship：Lead author   Language：English   Publishing type：Research paper (scientific journal)  

Mature B-cell acute lymphoblastic leukemia (B-ALL) is typically associated with French-American-British (FAB)-L3 morphology and MYC gene rearrangement. However, rare cases of mature B-ALL with non-L3 morphology and MLL-AF9 fusion have been reported, and such cases are characterized by a rapid and aggressive clinical course. We here report three such cases of pediatric mature B-ALL in female patients respectively aged 15 months, 4 years, and 4 months. Bone marrow smears at diagnosis showed FAB-L1 morphology in all patients. Immunophenotypically, they were positive for cluster of differentiation (CD)10, CD19, CD20 (or CD22), Human Leukocyte Antigen-DR, and surface immunoglobulin lambda. No evidence of MYC rearrangement was detected in any of the cases by fluorescent in situ hybridization (FISH) analysis. However, MLL rearrangement was detected by FISH, and MLL-AF9 fusion was confirmed by reverse transcriptase-polymerase chain reaction. All patients achieved complete remission after conventional chemotherapy and subsequently underwent hematopoietic stem cell transplantation as high-risk ALL; patient 3 for infantile ALL with MLL rearrangement and the others for ALL with MLL rearrangement and hyperleukocytosis (white blood cell count at diagnosis >50 x 109/L). At the latest follow-up for each case (12-98 months post-transplantation), complete remission was maintained. Moreover, we discuss the clinical, genetic, and immunophenotypic features of this rare disease.

DOI： 10.1007/s12185-016-1971-9

researchmap

Authorship：Corresponding author   Language：English   Publishing type：Research paper (scientific journal)  

In childhood acute myelogenous leukemia, extramedullary tumor is an occasional clinical symptom. However, extramedullary acute megakaryocytic leukemia is extremely rare. Here, we report an extremely rare case of acute megakaryocytic leukemia in a patient who presented with extramedullary tumor of cerebral falx as a first manifestation before the diagnosis of systemic bone marrow leukemia.

DOI： 10.1097/mph.0000000000000153

researchmap

Authorship：Lead author   Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: Febrile neutropenia (FN) is a common and serious complication of cancer chemotherapy associated with significant morbidity and mortality. Cefozopran (CZOP) is a potential candidate for empirical monotherapy in FN. However, studies on the use of CZOP as empirical treatment for pediatric patients with FN are quite limited. The purpose of this study was to compare the efficacy and safety of CZOP with cefepime (CFPM) empirical monotherapy in pediatric cancer patients with FN. PROCEDURES: A total of 64 patients with 224 episodes of FN were randomly assigned to receive antibiotic therapy with either CZOP (100 mg/kg/day) or CFPM (100 mg/kg/day). Of these episodes, 223 were considered eligible for the study. Success was defined as resolution of febrile episodes and clinical signs of infection within 120 hr following the start of antibiotic therapy. RESULTS: The success rate was not significantly different between the CZOP (64.0%) and CFPM (56.3%) groups (P = 0.275). Duration of fever, duration of antibiotic therapy, and the success rate in patients with blood stream infection did not differ between the two groups. There was no infection-related mortality in the study period. CONCLUSION: Both CZOP and CFPM as monotherapy appear to be effective and safe in pediatric patients. This study suggests that CZOP has satisfactory efficacy and is well tolerated as initial empirical therapy for pediatric cancer patients with FN.

DOI： 10.1002/pbc.25148

researchmap

Authorship：Corresponding author   Language：English   Publishing type：Research paper (scientific journal)  

There have been several reports on the reactivation of human herpesvirus 6 (HHV6) after stem cell transplantation (SCT) in adults, which sometimes induces severe illness. Few reports exist on pediatric patients; therefore, we retrospectively examined HHV6 reactivation after SCT in children. We reviewed 80 patients with a median age of 6 years. We analyzed HHV6 DNA serum samples from the patients before SCT and at 20 and 40 days after SCT using polymerase chain reaction. We also analyzed the relationship between HHV6 reactivation and the syndrome of inappropriate antidiuretic hormone secretion (SIADH). At 20 days after SCT, 35.0% of serum samples were positive for HHV6 DNA. The median viral load was 3.1x10 copies/mL serum. Multivariate analysis showed cord blood transplantation as the only risk factor for HHV6 reactivation. HHV6 reactivation occurs in 59.4% of 32 patients who underwent cord blood transplantation and in 18.8% of 48 patients who underwent SCT from other sources. Among the 14 patients with SIADH, 78.6% experienced HHV6 reactivation. Among the 66 patients without SIADH, only 25.8% had HHV6 reactivation. This result was statistically significant (P<0.001). This analysis revealed that HHV6 reactivation occurs in many children. In addition, HHV6 reactivation plus SIADH should prompt evaluation for central nervous system disease.

DOI： 10.1097/mph.0b013e3182a11676

researchmap

Authorship：Lead author   Language：English   Publishing type：Research paper (scientific journal)  

Bloodstream infection (BSI) is a recognized cause of morbidity and mortality in children after hematopoietic stem cell transplantation (HSCT). However, there are limited reports on BSI after HSCT in pediatric patients in multiple centers. This study was a retrospective cohort analysis of consecutive patients who underwent allogeneic and autologous HSCT at the Department of Paediatrics, Hokkaido University Hospital, between 1988 and 2009; the Department of Paediatrics, Sapporo Hokuyu Hospital, between 2007 and 2009; and the Department of Paediatrics, Asahikawa Medical University, between 1989 and 2009. A total of 277 patients underwent HSCT during the study period. In this multicenter analysis, cases of BSI after HSCT were recorded in the early posttransplant period (within the first 100 d), and BSI was observed in 24 of 277 HSCT patients. Multivariate analysis showed that nonmalignant disease was an independent factor associated with BSI after HSCT (hazard ratio 6.3 for aplastic anemia or Wiskott-Aldrich syndrome patients; confidence interval, 1.4-12.8; P = 0.012). We conclude that aplastic anemia and Wiskott-Aldrich syndrome were the novel risk factors for BSI in pediatric patients after HSCT.

DOI： 10.1097/mph.0b013e3182677f35

researchmap

Authorship：Corresponding author   Language：English   Publishing type：Research paper (scientific journal)  

We report the case of a female Japanese infant who was diagnosed with incontinentia pigmenti (IP) on the basis of the clinical and pathological findings of characteristic skin lesions and the detection of deletion in the nuclear factor-kappa B essential modulator gene at Xq28. The patient developed repetitive seizures at the age of 7 months when she was diagnosed with acute disseminated encephalomyelitis (ADEM), an inflammatory demyelinating disease of the central nervous system that often occurs after vaccination or infection. The causative infectious agent in this patient seemed to be Mycoplasma pneumoniae because of the increased titer of its serum antibody and the detection of its DNA in the initial cerebrospinal fluid sample. This patient showed significant improvement on receiving immunosuppressive therapy with corticosteroids. This is the second case report presenting an IP patient susceptible to ADEM, and therefore, ADEM should be considered early in the differential diagnosis of acute neurological illness for IP patients.

DOI： 10.1016/j.braindev.2008.08.010

researchmap

Event date： 2024.9

Language：Japanese   Presentation type：Oral presentation (general)  

researchmap

Event date： 2024.7

Language：Japanese   Presentation type：Oral presentation (general)  

researchmap

Event date： 2024.3

Language：Japanese   Presentation type：Oral presentation (general)  

researchmap

Event date： 2023.9 - 2023.10

Language：Japanese   Presentation type：Poster presentation  

researchmap

Event date： 2023.9 - 2023.10

Language：Japanese   Presentation type：Oral presentation (general)  

researchmap

Event date： 2023.9 - 2023.10

Language：Japanese   Presentation type：Poster presentation  

researchmap

Event date： 2023.9 - 2023.10

Language：Japanese   Presentation type：Oral presentation (general)  

researchmap

Event date： 2023.9

Language：Japanese   Presentation type：Oral presentation (general)  

researchmap

Event date： 2023.8 - 2024.8

Language：Japanese   Presentation type：Oral presentation (general)  

researchmap

Event date： 2023.5

Language：English   Presentation type：Oral presentation (general)  

researchmap

Event date： 2023.4

Language：Japanese   Presentation type：Poster presentation  

researchmap

Event date： 2022.3

Language：English   Presentation type：Oral presentation (general)  

Venue：札幌京王プラザホテル/日本新薬旭川営業所  

researchmap

Event date： 2022.2

Language：English   Presentation type：Oral presentation (general)  

Venue：アートホテル旭川  

researchmap

Event date： 2021.10

Language：Japanese   Presentation type：Oral presentation (general)  

Venue：旭川トーヨーホテル  

researchmap

Event date： 2019.3

Language：Japanese   Presentation type：Poster presentation  

Venue：大阪市  

researchmap

Event date： 2019.2

Language：Japanese   Presentation type：Oral presentation (invited, special)  

Venue：札幌市  

researchmap

Event date： 2018.11

Language：Japanese   Presentation type：Poster presentation  

Venue：京都市  

researchmap

Event date： 2018.10

Language：Japanese   Presentation type：Oral presentation (invited, special)  

Venue：札幌市  

researchmap

Event date： 2018.8

Language：Japanese   Presentation type：Oral presentation (general)  

Venue：札幌市  

researchmap

Event date： 2018.3

Language：Japanese   Presentation type：Oral presentation (general)  

Venue：旭川市  

researchmap

Event date： 2018.3

Language：Japanese   Presentation type：Oral presentation (general)  

Venue：旭川市  

researchmap

Event date： 2018.3

Language：Japanese   Presentation type：Oral presentation (general)  

Venue：札幌市  

researchmap

Event date： 2018.2

Language：Japanese   Presentation type：Oral presentation (general)  

Venue：札幌市  

researchmap

Event date： 2018.2

Language：Japanese   Presentation type：Oral presentation (general)  

Venue：札幌市  

researchmap

Event date： 2018.2

Language：Japanese   Presentation type：Poster presentation  

Venue：札幌市  

researchmap

Event date： 2017.11

Language：Japanese   Presentation type：Poster presentation  

Venue：松山市  

researchmap

Event date： 2017.11

Language：Japanese   Presentation type：Poster presentation  

Venue：松山市  

researchmap

Event date： 2017.10

Language：Japanese   Presentation type：Oral presentation (general)  

Venue：札幌市  

researchmap

Event date： 2017.10

Language：Japanese   Presentation type：Poster presentation  

Venue：東京都  

researchmap

Event date： 2017.10

Language：English   Presentation type：Poster presentation  

Venue：Washington DC, USA  

researchmap

Event date： 2017.8

Language：Japanese   Presentation type：Oral presentation (general)  

Venue：札幌市  

researchmap

Event date： 2017.5

Language：Japanese   Presentation type：Oral presentation (general)  

Venue：札幌市  

researchmap

Event date： 2016.12

Language：Japanese   Presentation type：Oral presentation (general)  

Venue：旭川市  

researchmap

Event date： 2016.10

Language：English   Presentation type：Poster presentation  

Venue：Dublin, Ireland  

researchmap

Event date： 2016.9

Language：Japanese   Presentation type：Oral presentation (general)  

Venue：札幌市  

researchmap

Event date： 2016.5

Language：Japanese   Presentation type：Poster presentation  

Venue：札幌市  

researchmap

Event date： 2016.2

Language：Japanese   Presentation type：Oral presentation (general)  

Venue：札幌市  

researchmap

Event date： 2015.11

Language：English   Presentation type：Oral presentation (general)  

Venue：甲府市  

researchmap

Event date： 2015.4

Language：Japanese   Presentation type：Poster presentation  

Venue：大阪市  

researchmap

道内のがん診療体制について話し合い施設などを整備していく。

researchmap

道内のがん診療施設においてがん地域連携クリティカルパスの利用実績を調査し、より有用なクリティカルパスの運用を検討する。

researchmap

2020年2月5日 委員会出席

researchmap

血液・腫瘍疾患をもつ小児が抱える問題点を一般向けに講演

researchmap

International Journal of Hematologyからの論文査読を担当。

researchmap

researchmap

和文雑誌「臨床小児医学」の論文査読を担当した。

researchmap

International Journal of Hematologyの査読を1編行った。

researchmap

上記研究グループの再発ALL委員会委員

researchmap

International Journal of Hematologyからの論文査読を担当。

researchmap

臨床小児医学からの論文査読を担当

researchmap

第37回北海道小児血液研究会の一般演題について座長を務めた。

researchmap

OncoTargets and Therapyからの論文査読を担当。

researchmap

2019年6月1日 同研究会
2019年7月19日 同世話人会

researchmap

2020年1月22日 査読1件

researchmap

2019年3月30日 同協議会

researchmap

第304回日本小児科学会北海道地方会の一般演題の座長を務めた。

researchmap

researchmap

第4回造血細胞移植セミナーの特別講演の座長を務めた。

researchmap

第35回北海道小児血液研究会の一般演題について座長を務めた。

researchmap

researchmap

researchmap

researchmap

第42北海道小児がん研究会の一般演題4演題について座長を務めた。

researchmap

2019年8月24日 同世話人会及び研究会

researchmap

第23回北海道血友病研究会の一般演題2演題の発表について座長を務めた。

researchmap

researchmap

researchmap

researchmap

International Journal of Hematologyからの論文査読を担当。

researchmap

小児がん患者における末梢挿入型中心静脈カテーテルの安全性と有用性について論じられた論文について査読した。

researchmap