title: [Chronic myeloproliferative diseases. Diagnosis and therapy].
abstract: Chronic myeloproliferative disorders (CMPD) are neoplastic disorders of the hematopoietic stem cell. Four different entities are defined: chronic myeloid leukemia (CML), polycythemia vers, essential thrombocythaemia, and idiopathic myelofibrosis. In addition, overlapping entities within the CMPDs and between CMPDs and myelodysplastic syndrome have been described. Diagnostic measures are performed to classify the subtype exactly and to assess risk factors and prognosis. Cytogenetic and molecular analyses are mandatory for the characterization of the malignant clone. Hydroxyurea and interferon-alpha have proven effective in all CMPE. In CML, specific inhibition of the elevated ABL tyrosine kinase activity with imatinib is associated with high response rates. Allogeneic stem cell transplantation is the only curative treatment option for all entities. In CML, the decision-making analysis should be based on established scores. In BCR-ABL negative CMPDs an allogeneic stem cell transplantation should only be performed in patients with unfavorable prognosis.
authors.affiliation: II. Medizinische Klinik Mannheim, Universität Heidelberg. eva.lengfelder@med3.ma.uni-heidelberg.de
authors.forename: E
authors.initials: E
authors.lastname: Lengfelder
authors.forename: U
authors.initials: U
authors.lastname: Berger
authors.forename: A
authors.initials: A
authors.lastname: Reiter
authors.forename: A
authors.initials: A
authors.lastname: Hochhaus
authors.forename: R
authors.initials: R
authors.lastname: Hehlmann
mesh_terms: D000970:Antineoplastic Agents / Q000627:therapeutic use; D001549:Benzamides; D001853:Bone Marrow / Q000473:pathology; D016026:Bone Marrow Transplantation; D002908:Chronic Disease; D003937:Diagnosis, Differential; D004791:Enzyme Inhibitors / Q000627:therapeutic use; D016044:Fusion Proteins, bcr-abl; D015971:Gene Expression Regulation, Enzymologic / Q000187:drug effects; D006801:Humans; D006918:Hydroxyurea / Q000627:therapeutic use; D000068877:Imatinib Mesylate; D000077190:Interferon alpha-2; D016898:Interferon-alpha / Q000627:therapeutic use; D009190:Myelodysplastic Syndromes / Q000175:diagnosis / Q000235:genetics / Q000473:pathology / Q000628:therapy; D009196:Myeloproliferative Disorders / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology / Q000628:therapy; D010879:Piperazines / Q000627:therapeutic use; D011379:Prognosis; D011505:Protein-Tyrosine Kinases / Q000037:antagonists & inhibitors / Q000235:genetics; D011743:Pyrimidines / Q000627:therapeutic use; D011994:Recombinant Proteins
chemical_list: D000970:Antineoplastic Agents; D001549:Benzamides; D004791:Enzyme Inhibitors; D000077190:Interferon alpha-2; D016898:Interferon-alpha; D010879:Piperazines; D011743:Pyrimidines; D011994:Recombinant Proteins; D000068877:Imatinib Mesylate; C426236:ARG tyrosine kinase; D011505:Protein-Tyrosine Kinases; D016044:Fusion Proteins, bcr-abl; D006918:Hydroxyurea