mercaptopurine and Primary-Myelofibrosis

Acute myelofibrosis (AMF), as defined by an acute panmyelopathy associated with marked megakaryocytic hyperplasia and marrow fibrosis, appears to be a stem cell disorder. Even though it is most difficult to distinguish from various myeloproliferative and myelodysplastic disorders as well as acute myelogenous leukemia, it has rarely been reported to terminate as acute lymphoblastic leukemia (ALL). Only five cases have been reported in the literature; two from the pediatric literature and only three from the adult literature. Of the three adult cases, two were defined by light microscopy alone. Among the cases with follow-up (3/5), all died within 2 weeks to 2 months of diagnosis. We report an additional case in an adult; the ALL was defined by morphology, flow cytometric immunophenotyping, and cytogenetic analysis. The interval from diagnosis of AMF to ALL was 3 months. Our patient was treated with standard therapy for ALL, was in complete remission at last follow-up (3 months off maintenance therapy), and represents the only reported case who attained a complete remission. There are too few cases to determine the prognostic significance of termination of AMF in an acute leukemia of lymphoid origin vs. myeloid origin.

Topics: Adult; Antineoplastic Combined Chemotherapy Protocols; Asparaginase; Blast Crisis; Bone Marrow; Chromosome Deletion; Chromosomes, Human, Pair 5; Combined Modality Therapy; Cranial Irradiation; Cyclophosphamide; Cytarabine; Daunorubicin; Disease Progression; Flow Cytometry; Humans; Immunophenotyping; Male; Mercaptopurine; Methotrexate; Neoplastic Stem Cells; Precursor B-Cell Lymphoblastic Leukemia-Lymphoma; Precursor Cell Lymphoblastic Leukemia-Lymphoma; Prednisone; Primary Myelofibrosis; Remission Induction; Vincristine

We report two additional patients with acute myeloid leukemia (AML) and a translocation between chromosomes 7 and 11: t(7;11)(p15;p15). One patient was diagnosed as having AML-M2 and the other as AML with myelofibrosis. Both patients had low-level neutrophil alkaline phosphatase (NAP) scores. In the literature, only 15 AML patients with t(7;11)(p15;p15) have been reported; nine of them had an AML-M2 morphology, and all had a decreased NAP score. Moreover, mean survival of the reported AML patients with t(7;11)(p15;p15) was 15 months, although 85% of them obtained complete remission, indicating that this type of leukemia frequently tends to relapse. These findings indicate a strong association between the chromosome abnormality and hematologic manifestations of this disease.

Topics: Acute Disease; Adult; Alkaline Phosphatase; Antineoplastic Combined Chemotherapy Protocols; Bone Marrow Transplantation; Chromosomes, Human, Pair 11; Chromosomes, Human, Pair 7; Cytarabine; Daunorubicin; Female; Humans; Leukemia, Myeloid; Leukemia, Myeloid, Acute; Male; Mercaptopurine; Middle Aged; Neutrophils; Prednisolone; Primary Myelofibrosis; Remission Induction; Translocation, Genetic

Topics: Aged; Antineoplastic Combined Chemotherapy Protocols; Bone Marrow; Combined Modality Therapy; Cytarabine; Fatal Outcome; Female; Humans; Mercaptopurine; Middle Aged; Nitriles; Primary Myelofibrosis; Pyrazoles; Pyrimidines; Retreatment; Treatment Outcome

Topics: Aged; Antineoplastic Combined Chemotherapy Protocols; Female; Humans; Hydroxyurea; Idarubicin; Leukemia, Promyelocytic, Acute; Mercaptopurine; Methotrexate; Mitoxantrone; Primary Myelofibrosis; Remission Induction

A 62 year old male patient was previously admitted to another hospital in February 1989 because of palpitation. Peripheral blood examination revealed pancytopenia. Although the number of nucleated cells in the bone marrow aspirate was in the normal range. It contained 2.2% of blastic cells and dysplastic cells. He was diagnosed to be myelodysplastic syndrome with refractory anemia. He subsequently received repeated blood transfusions and other symptomatic treatments as an outpatient until 1990. When he was admitted to our hospital because of severe pancytopenia. The numbers of WBC, RBC, and platelets were 2,000/microliters, 134 x 10(4)/microliters, 2.9 x 10(4)/microliters respectively. Bone marrow aspiration resulted in dry tap, and biopsy at the iliac bone showed remarkable fibrosis with marked decrease of normal hematopoietic cells. Chromosome analysis revealed multiple aberrations such as 47XY, +8, 13q-, 14p+, 48XY, +8, +9, 13q+. The patient was treated with BHAC-AMP combination chemotherapy. After 3 cycles of the therapy, pancytopenia was improved and chromosomal aberration disappeared. This case was considered to be an acute myelofibrosis developed from myelodysplastic syndrome and worth to reporting with a review of literature, because drastic combination chemotherapy was extremely effective.

Topics: Aclarubicin; Acute Disease; Antineoplastic Combined Chemotherapy Protocols; Bone Marrow; Chromosome Aberrations; Cytarabine; Humans; Male; Mercaptopurine; Middle Aged; Myelodysplastic Syndromes; Prednisolone; Primary Myelofibrosis

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

Topics: Adult; Aged; Antineoplastic Agents; Azathioprine; Busulfan; Female; Humans; Male; Mannitol; Mercaptopurine; Middle Aged; Osteosclerosis; Primary Myelofibrosis

Topics: Adult; Afibrinogenemia; Aged; Anemia; Antineoplastic Agents; Child; Cytarabine; Daunorubicin; Humans; Hydroxyzine; Leukemia, Myeloid; Leukemia, Myeloid, Acute; Leukopenia; Mercaptopurine; Methotrexate; Middle Aged; Polycythemia Vera; Prednisone; Primary Myelofibrosis; Splenomegaly; Vincristine

Mitochondrial DNA's from the peripheral blood of 14 patients with granulocytic leukemia contained a circular dimer form. No such structure could be found in M DNA's from three patients with nonmalignant proliferations of granulocytes. The frequency of the circular dimer form is reduced upon treatment with antileukemic drugs. The above results suggest that a significant relation exists between the formation and presence of the circular dimer M DNA form and granulocytic leukemia in man.

Topics: Busulfan; DNA; Humans; Leukemia, Myeloid; Leukemoid Reaction; Leukocytes; Mercaptopurine; Methods; Methotrexate; Microscopy, Electron; Mitochondria; Primary Myelofibrosis

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

Topics: Adult; Aged; Humans; Leukemia; Mercaptopurine; Middle Aged; Primary Myelofibrosis; Vincristine

Topics: Alkylating Agents; Aminopterin; Busulfan; Hodgkin Disease; Humans; Leukemia; Leukemia, Lymphoid; Leukemia, Myeloid; Lymphoma; Lymphoma, Large B-Cell, Diffuse; Lymphoma, Non-Hodgkin; Mechlorethamine; Mercaptopurine; Methotrexate; Multiple Myeloma; Neoplasms; Pathology; Primary Myelofibrosis; Sarcoma; Triethylenemelamine; Tuberculosis; Tuberculosis, Pulmonary; Urethane

Topics: Blood Platelet Disorders; Busulfan; Child; Humans; Leukemia; Leukemia, Myeloid; Leukocytosis; Lymph Nodes; Mercaptopurine; Prednisone; Primary Myelofibrosis; Radiotherapy; Thrombocytopenia; Triamcinolone