Current problems in the diagnosis of Philadelphia-negative myeloproliferative neoplasms in Japan

• SHIRANE Shuichi

  Department of Hematology, Juntendo University Graduate School of Medicine

• ARAKI Marito

  Department of Transfusion Medicine and Stem Cell Regulation, Juntendo University Graduate School of Medicine

• MORISHITA Soji

  Department of Transfusion Medicine and Stem Cell Regulation, Juntendo University Graduate School of Medicine

• EDAHIRO Yoko

  Department of Hematology, Juntendo University Graduate School of Medicine

• OHSAKA Akimichi

  Department of Transfusion Medicine and Stem Cell Regulation, Juntendo University Graduate School of Medicine

• KOMATSU Norio

  Department of Hematology, Juntendo University Graduate School of Medicine

Bibliographic Information

Other Title

• 日本におけるフィラデルフィア染色体陰性骨髄増殖性腫瘍の診断の現状と問題点
• 臨床研究 日本におけるフィラデルフィア染色体陰性骨髄増殖性腫瘍の診断の現状と問題点
• リンショウ ケンキュウ ニホン ニ オケル フィラデルフィア センショクタイ インセイ コツズイ ゾウショクセイ シュヨウ ノ シンダン ノ ゲンジョウ ト モンダイテン

Abstract

To investigate the current situation and issues regarding the diagnosis of Philadelphia-negative myeloproliferative neoplasms (MPN) in Japan, we retrospectively analyzed an accumulated cohort consisting of 1,081 patients with suspected MPN. Based on WHO2008 diagnostic criteria, we diagnosed 101 of these patients with polycythemia vera, 179 with essential thrombocythemia, 36 with primary myelofibrosis, 45 with unclassifiable MPN, and 4 with myelodysplastic syndromes. Out of 716 patients, 235 were not diagnosed with MPN despite the detection of a JAK2, CALR, or MPL mutation. Among 156 patients with undefined MPN receiving further follow-up, none underwent bone marrow examination and screening for BCR-ABL1 was not performed in 88 cases. Thus, diagnosis was not possible in these cases due to a lack of essential examinations. Since the prognosis and treatment strategy associated with MPN differ among disease types, in addition to mutation analysis, the importance of bone marrow examination and screening for BCR-ABL1 must be re-recognized.

Journal

• Rinsho Ketsueki

  Rinsho Ketsueki 56 (7), 877-882, 2015

  The Japanese Society of Hematology

Keywords

• Philadelphia-negative myeloproliferative neoplasms
• JAK2 mutation
• CALR mutation
• MPL mutation

Details

• CRID: 1390282680011802112
• NII Article ID: 130005093120
• NII Book ID: AN00252940
• DOI: 10.11406/rinketsu.56.877
• ISSN: 18820824
• ISSN: 04851439
• NDL BIB ID: 026636503
• PubMed: 26251152
• Web Site: https://ndlsearch.ndl.go.jp/books/R000000004-I026636503
• Web Site: https://search.jamas.or.jp/link/ui/2015323446
• Text Lang: ja
• Data Source:
  • JaLC
  • NDL
  • PubMed
  • CiNii Articles
  • KAKEN
• Abstract License Flag: Disallowed