シンポジウム3．
慢性骨髄増殖性症候群―各疾患相互の関係と位置づけ―
慢性骨髄増殖性症候群―各疾患相互の関係と位置づけ―
慢性骨髄増殖性症候群・各疾患の自験例にみる臨床経過とその終末像
1988 年 29 巻 7 号 p. 950-959
詳細
1988 年 29 巻 7 号 p. 950-959
One hundred and seventeen patients with chronic myeloproliferative syndrome, consisting of 40 patients with chronic myelogenous leukemia (CML), 46 patients with polycythemia vera (PV), 17 patients with primary myelofibrosis (PMF), and 14 patients with essential thrombocythemia (ET) have been observed between 1970 and 1982.
The results of the repeated hematological investigation were: (1) at diagnosis, significant difference were noted in hematologic and chemical data among these diseases. However, some cases were difficult to diagnosis. (2) Median survival times of the patients with CML, PV, and PMF were 35 months, 135 months, and 70 months, respectively. (3) The incidences of acute leukemic transformation in CML, PV, and PMF were 89.6% (26/29), 23.8% (5/21), and 18.8% (3/16), respectively. Moreover, one patient with ET transformed into acute leukemia. (4) The preleukemic phase, characterized by rapidly evolving spontaneous pancytopenia, was often observed in PV, PMF, and ET. (5) Extensive overlap in hematologic and pathological features was noted between PMF and the terminal phase of PV.
