Course and transformation of polycythaemia vera in relation to therapy.
The fate of the polycythaemic patient depends on the treatment employed which may determine the nature of the transformation commonly occurring late in the course of the disease. Treatment is, on the other hand, aimed at prevention of the most frequent complications, that is of thromboembolic processes. In the last 30 years the authors treated a total of 118 PV patients, of whom 60 have died. Initially 32P treatment was applied, which was modified later, because of acute leukaemia that had occurred in 9% of the treated cases, to a single 5 mC 32P+Myelobromol (DBM) treatment. Still later only DBM was administered in the form of stosstherapy (2500 mg per day over a period of 4 days). In the latter two groups, acute leukaemia occurred as few as two cases. The course of untreated polycythaemia vera is characterized by transformation into another myeloproliferative disease. This phenomenon occurs in 50% of the cases on drastic treatment and in patients treated with 32P. Of the patients who were alive when the report was finished 35% had been free of complications, while 5.2% were suffering from chronic granulocytic leukaemia (CGL), 34.5% from sclerotic osteo-myelofibrosis (OMF-SC) and 3.4% from chronic megakaryocytic granulocytiv leukaemia (CMGL). Of the 60 patients having died, 15% had suffered from other complications being predominantly of vascular nature. 11.8% of them died of AML, 10% of CGL, 26.7% of OMF-SC and 26.7% of CMGL. The terminal stage was characterized, in the majority of cases, by blastic crisis. Based on their own results and literary data authors recommend DBM treatment besides the indispensable phlebotomy.
Burger
T
T
2nd Department of Medicine, University Medical School, Pécs, Hungary.
Schmelczer
M
M
Molnár
L
L
Pajor
L
L
Koszorus
S
S
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002699:Chlorambucil / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007938:Leukemia / Q000139:chemically induced / Q000209:etiology; D007953:Leukemia, Radiation-Induced / Q000209:etiology; D008297:Male; D008875:Middle Aged; D008927:Mitobronitol / Q000627:therapeutic use; D010761:Phosphorus Radioisotopes / Q000627:therapeutic use; D011087:Polycythemia Vera / Q000150:complications* / Q000188:drug therapy / Q000532:radiotherapy; D055728:Primary Myelofibrosis / Q000139:chemically induced / Q000209:etiology
D010761:Phosphorus Radioisotopes; D002699:Chlorambucil; D008927:Mitobronitol