急性骨髄線維症に類似の臨床的病理的特徴を持つDiGuglielmo症候群の1例
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概要
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A 58-year-old female was first seen at the Keio University Hospital because of anemia. On admission she had petechiae over her whole body and no hepatosplenomegaly. Blood examination revealed pancytopenia and absence of poikilocytosis. There were numerous erythroblasts with positive PAS stain in peripheral blood and some of them were megaloblastoid. These cells were also found in the hypoplastic bone marrow. Because of the normal level of serum vitamin B12, chromosome aberration and absence of Ph1 chromosome, a diagnosis of DiGuglielmo syndrome was made. The patient died on her 52th hospital day without any antileukemic treatment. Autopsy revealed prominent myelofibrosis and neoplastic cell infiltration in various organs. This case showed many clinical and pathological findings similar to acute myelofibrosis such as pancytopenia, absence of splenomegaly and prominent myelofibrosis. However, many reports concerning acute myelofibrosis stressed on the absence of the invasion of neoplastic cells into other organs and tissues. Myeloproliferative disorders proposed by Dameshek included many clinical entities such as hypoplastic leukemia, myelofibrosis, DiGuglielmo syndrome and sideroblastic anemia, etc. Thus, it is conceived that myeloproliferative disorders could be overlapped each other.
- 一般社団法人 日本血液学会の論文
一般社団法人 日本血液学会 | 論文
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