mercaptopurine and Myeloproliferative-Disorders

We describe a patient with leukocytosis with all the stages of neutrophilic series, peripheral dominant myeloblast proliferation, marked dysplasia of myeloid and erythroid series, and extramedullary hematopoiesis of the lymph nodes. A cytogenetic study of the bone marrow cells showed normal karyotype, and molecular analysis of the leukemic cells showed negative for BCR-ABL by RT-PCR. After chemotherapy, the patient went into complete remission with a normal blood and bone marrow profile with no dysplasia. On relapse, the hematological findings showed a typical bone marrow dominant acute myeloid leukemia, with the leukemic cells having a chromosomal abnormality. The patient exhibited the combined features of myeloproliferative disorder, myelodysplastic syndrome, peripheral dominant myeloblast proliferation (so-called peripheral leukemia) and typical acute myeloid leukemia throughout the clinical course. This is thought to be a rare overlapping disease involving these distinct hematological conditions that do not usually occur in the same patient.

Topics: Acute Disease; Antineoplastic Combined Chemotherapy Protocols; Bone Marrow; Cytarabine; Daunorubicin; Disease Progression; Hematopoiesis, Extramedullary; Humans; Leukemia, Myeloid; Male; Mercaptopurine; Middle Aged; Myelodysplastic Syndromes; Myeloproliferative Disorders; Prednisolone; Preleukemia; Remission Induction

Seventeen patients with myeloproliferative disorders and one patient with chronic myelomonocytic leukemia (CMMoL) were treated with ranimustine++ (MCNU), and the efficacy was evaluated. MCNU was given intravenously by drip infusion at an usual dose of 100 approximately 150 mg with intervals arranged according to the counts of peripheral blood cells. A complete remission was achieved in all 10 patients with chronic myelogenous leukemia (CML) in chronic phase. In three of patients with polycythemia vera (PV) the excellent effects were obtained, and the other 2 cases showed moderate effect. An excellent effect was obtained in both 2 patients with essential thrombocythemia (ET). A patient with CMMoL revealed partial remission. The overall efficacy rate was 100%. The cases with CML needed more long term and much more dose of the drug in order to get remission compared with PV and ET. After remission in both PV and ET, well controlled states were maintained for a relatively long period with no additional administration. In CMMoL, MCNU combined with 6-mercaptopurine also showed remarkable anti-tumor effects. It suggests that MCNU may be one of the useful drugs for the treatment of CMMoL. The side effects observed with MCNU were a slight degree of nausea and vomiting (28%), however they showed no trouble on carrying out the therapy.

Topics: Adult; Aged; Antineoplastic Agents; Drug Administration Schedule; Female; Humans; Infusions, Intravenous; Leukemia, Myelomonocytic, Chronic; Male; Mercaptopurine; Middle Aged; Myeloproliferative Disorders; Nitrosourea Compounds; Remission Induction

Topics: Antineoplastic Agents; Aphidicolin; Bone Marrow; Bone Marrow Cells; Cell Differentiation; Cells, Cultured; Cytarabine; Diterpenes; DNA Replication; Formamides; Hematopoietic Stem Cells; Humans; Leukemia, Myeloid; Mercaptopurine; Myeloproliferative Disorders; Reference Values; Tretinoin

On the basis of five observations of adult patients with the clinical feature of mature cellular leukaemia which proved to be therapy-refractory and which was characterized by a rapid course is referred to the necessity of the differentiation of such cases from the classical myeloic leukaemia. The cardinal symptoms of this type of disease, which probably is identified with the cases described in literature as atypical chronic myelosis, as paraneutrophil leukaemia or as acute myelofibrosis, and also shows common features with the juvenile chronic myelosis, are, apart from the mature cellular differential blood picture a short life expectancy (less than 1 year), an initial thrombocytopenia, a normal or increased activity of the alkaline granulocyte phosphatase, the lack of Ph1-chromosome as well as the bad therapeutic reaction to busulfan. The observation of the simultaneously existing fibroses of the bone marrow as well as of the final increase of immature blasts induced the classification of the clinical picture as a special form of the myeloproliferative syndrome.

Topics: Acute Disease; Adult; Azathioprine; Bone Marrow Examination; Fetal Hemoglobin; Hematopoietic Stem Cells; Humans; Leukemia, Myeloid; Mercaptopurine; Myeloproliferative Disorders; Primary Myelofibrosis

A 3 months old girl presented with significant enlargement of liver, spleen and lymphnodes, with moderate anemia, thrombopenia and leucocytosis. In the differential count there was a shift to the left and an increase of monocyte-like cells (35%). Differential diagnosis included leucemoid reaction, infectious mononucleosis, myelo-proliferative disorder with a missing C chromosome and chronic myeloid leucemia. Clinical symptoms, cytochemistry and caryotype of bone marrow cells suggested infantile chronic myeloic leucemia and normal ALP index and possibly normal HbF. Treatment with 6-mercaptopurine was followed by partial remission. The therapeutic consequences of exact differential diagnosis are discussed.

Topics: Anemia; Chromosome Aberrations; Chromosome Disorders; Diagnosis, Differential; Humans; In Vitro Techniques; Infant; Infectious Mononucleosis; Leukemia, Myeloid; Leukocytosis; Liver Diseases; Lymphatic Diseases; Mercaptopurine; Myeloproliferative Disorders; Splenic Diseases; Thrombocytopenia

Topics: Adrenal Insufficiency; Adrenocorticotropic Hormone; Allopurinol; Aminopterin; Busulfan; Humans; Leukemia, Myeloid; Mercaptopurine; Myeloproliferative Disorders; Pituitary-Adrenal System

Topics: Adult; Blood Coagulation Tests; Female; Humans; Injections, Intravenous; Mercaptopurine; Myeloproliferative Disorders; Phosphorus Isotopes; Primary Myelofibrosis

Topics: Adolescent; Adult; Aged; Child; Child, Preschool; Female; Glucocorticoids; Humans; Leukemia, Myeloid, Acute; Male; Mercaptopurine; Middle Aged; Myeloproliferative Disorders; Thrombocythemia, Essential

Topics: Allopurinol; Bone Marrow Diseases; Drug Synergism; Enzyme Therapy; Humans; Leukemia, Myeloid, Acute; Mercaptopurine; Myeloproliferative Disorders; Xanthine Oxidase