thioguanine-anhydrous and Primary-Myelofibrosis

Topics: Anemia; Ascites; Blood Coagulation Disorders; Bone Marrow; Busulfan; Chlorambucil; Humans; Hypertension, Portal; Primary Myelofibrosis; Prognosis; Spleen; Splenectomy; Thioguanine

Acute myelofibrosis is a rare and still ill-defined disease. Based on morphological observation, immunophenotyping and ultrastructural analysis, we support the assumption that acute myelofibrosis is a malignant disorder mainly of the megakaryocytic lineage and is closely related to acute megakaryocytic/blastic leukaemia. Consequently, the 11 patients reported here were treated with aggressive polychemotherapy with combinations including daunorubicin and cytosine arabinoside and 6-thioguanin or VP16-213. 4 complete remissions, 2 partial remissions and 1 minor response were observed. Duration of aplasia was not significantly prolonged. These findings indicate that the use of aggressive polychemotherapy is feasible in acute myelofibrosis and results in a significant number of remissions.

Topics: Acute Disease; Adult; Aged; Bone Marrow; Cytarabine; Daunorubicin; Drug Therapy, Combination; Erythrocyte Count; Female; Humans; Infusions, Intravenous; Injections, Intravenous; Male; Megakaryocytes; Middle Aged; Primary Myelofibrosis; Thioguanine

12 patients with symptomatic chronic myelofibrosis were treated with either busulphan or 6-thioguanine. Therapy was without significant side effects and resulted in a reduction in the size of liver and spleen and an improvement in well-being in all cases. In 7 patients the Hb value rose and the extent of marrow fibrosis was reduced in the 5 patients in whom bone marrow examination was repeated after treatment. Chemotherapy is an effective and safer alternative to splenectomy in patients with myelofibrosis with symptomatic anaemia, symptomatic splenomegaly or constitutional symptoms such as fever, weight loss and night sweats.

Topics: Aged; Biopsy; Bone Marrow; Busulfan; Female; Humans; Liver; Male; Middle Aged; Organ Size; Primary Myelofibrosis; Spleen; Thioguanine

Bone marrow fibre content was studied in 34 patients with acute myeloid leukaemia at presentation and subsequently after chemotherapy and at complete remission. The findings indicate that: some degree of marrow fibrosis is present in about one third of the patients with acute myeloid leukaemia at presentation; effective antileukaemia treatment may result in reversal of marrow fibrosis; and increase in marrow fibre content at diagnosis does not affect haemopoietic regeneration after treatment and achievement of complete remission.

Topics: Antineoplastic Combined Chemotherapy Protocols; Bone Marrow; Collagen; Cytarabine; Daunorubicin; Humans; Leukemia, Myeloid, Acute; Podophyllotoxin; Primary Myelofibrosis; Reticulin; Thioguanine

Topics: Aged; Anemia; Blood Cell Count; Bone Marrow Examination; Cytarabine; Diagnosis, Differential; Female; Humans; Leukemia, Myeloid, Acute; Primary Myelofibrosis; Thioguanine

Staining with naphthol AS phosphate and Fast Blue BB salt has been used for the estimation of neutrophil alkaline phosphatase (NAP) scores in patients with chronic granulocytic leukaemia (CGL). The very low scores found at diagnosis rise when the disease is treated, and there is some inverse correlation between the NAP score and the absolute neutrophil count. Patients treated intensively developed high NAP scores. Elective splenectomy performed during the chronic phase of CGL is followed by a pronounced but transient neutrophilia and a concurrent striking rise in the NAP score. Similar changes were observed in patients without CGL who underwent splenectomy. These observations can be explained by assuming that newly formed neutrophils in CGL have a normal content of NAP but are rapidly sequestered in non-circulating extramedullary pools, whereas the circulating neutrophil with a typically low NAP content is a relatively aged cell which has lost enzyme activity. In subjects with or without CGL, removal of the spleen, a major site of such pooling, temporarily permits the circulation of newly formed neutrophils but eventually other organs assume the sequestering functions of the spleen. Thus the aberrations of NAP score seen in CGL might be attributable not to an intrinsic cellular defect but to an exaggeration of the granulocyte storage phenomena which also occur in subjects without CGL.

Topics: Alkaline Phosphatase; Bone Marrow Cells; Busulfan; Chronic Disease; Clinical Enzyme Tests; Histocytochemistry; Leukemia, Myeloid; Leukemoid Reaction; Neutrophils; Polycythemia; Primary Myelofibrosis; Splenectomy; Thioguanine; Time Factors