mitobronitol and Primary-Myelofibrosis

A 59-year-old woman was diagnosed with essential thrombocythemia in 1988 and had been treated with hydroxyurea, mitobronitol, busulfan, and ranimustine, in that order. Hepatosplenomegaly, low-grade fever, and body weight loss manifested, and a few blasts were noted in the peripheral blood studied in March 2002. A biopsied specimen of the bone marrow showed myelofibrosis but not a leukemia in August 2004. An abnormal karyotype with der(1; 13) appeared for the first time. She was treated with low-dose prednisolone. In January 2005, she experienced left hip joint pain, and magnetic resonance scanning showed a tumoral lesion in the femoral head. Histological diagnosis of the biopsied mass revealed that it was a granulocytic sarcoma, and radiotherapy was performed. In April 2005, bone scintigraphy showed multiple lesions. She became febrile and red blood cell transfusion-dependent with hepatosplenomegaly and a small number of circulating blasts. Intravenous cytarabine (low dose) and etoposide relieved the fever and hepatosplenomegaly; however, she developed a pathologic fracture of the right humerus. An additional karyotypic abnormality (7q22 deletion) was noted. She subsequently died of infection. Granulocytic sarcoma is very rare in essential thrombocythemia, and this patient may be the first reported case of essential thrombocythemia that developed multiple lesions and a pathologic fracture without transformation to overt leukemia.

Topics: Antineoplastic Combined Chemotherapy Protocols; Blood Transfusion; Chromosome Deletion; Chromosomes, Human, Pair 7; Cytarabine; Etoposide; Fatal Outcome; Female; Femoral Fractures; Femoral Neoplasms; Humans; Hydroxyurea; Middle Aged; Mitobronitol; Neoplasm Metastasis; Nitrosourea Compounds; Primary Myelofibrosis; Radiography; Sarcoma, Myeloid; Thrombocythemia, Essential

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.

Topics: Adult; Aged; Aged, 80 and over; Chlorambucil; Female; Humans; Leukemia; Leukemia, Radiation-Induced; Male; Middle Aged; Mitobronitol; Phosphorus Radioisotopes; Polycythemia Vera; Primary Myelofibrosis

Topics: Blood Transfusion; Hematopoiesis; Hepatomegaly; Humans; Mannitol; Mitobronitol; Pancytopenia; Polycythemia; Prednisone; Primary Myelofibrosis; Remission, Spontaneous; Splenomegaly; Thrombocytopenia; Thrombocytosis

Topics: Adult; Aged; Antineoplastic Agents; Azirines; Busulfan; Female; Humans; Leukemia, Erythroblastic, Acute; Male; Middle Aged; Mitobronitol; Organophosphorus Compounds; Primary Myelofibrosis