The Myeloproliferative Disorders an Historical Appraisal and Personal Experiences
| Autor: | Jan Jacques Michiels |
|---|---|
| Rok vydání: | 1996 |
| Předmět: |
Male
Cancer Research Pathology medicine.medical_specialty Myeloid Myeloproliferative Disorders Polycythemia vera Bone Marrow Erythromelalgia hemic and lymphatic diseases medicine Humans Paresthesia Vascular Diseases Hemorrhagic thrombocythemia Myelofibrosis Polycythemia Vera Essential thrombocythemia business.industry Thrombosis Hematology History 20th Century medicine.disease Combined Modality Therapy medicine.anatomical_structure Oncology Female business Cell Division Busulfan Thrombocythemia Essential medicine.drug |
| Zdroj: | Leukemia & Lymphoma. 22:1-14 |
| ISSN: | 1029-2403 1042-8194 |
| DOI: | 10.3109/10428199609074356 |
| Popis: | According to strict morphological, biochemical and cytogenetic criteria Philadelphia chromosome positive essential thrombocythemia and chronic granulocytic leukemia constitute a separate malignant and individual disease entity, whereas Philadelphia chromosome negative essential thrombocythemia, polycythemia vera and agnogenic or megakaryocytic myeloid metaplasia form a chronic proliferation of three hematopoietic cell lines. Histopathology from bone marrow biopsies permits the characterization and diagnostic differention of the various myeloproliferative disorders and appears to be a main and specific diagostic criterion for polycythemia vera and essential thrombocythemia. Hemorrhagic thrombocythemia is a clinical syndrome of recurrent spontaneous mucocutaneous and secondary hemorrhages often preceded by thromboses, extremely high platelet counts, pseudohyperkalemia, increased bone marrow cellularity and frequently splenomegaly. The diagnostic criteria of essential thrombocythemia with paradoxical occurrence of thrombotic events and hemorrhagic manifestations are a platelet count in excess of 1000 x 10(9)/L and increased bone marrow cellularity in the majority of the cases. Erythromelalgia and other microcirculatory ischemic or thrombotic events or accidents in essential thrombocythemia and polycythemia vera already occur at platelet counts in excess of the upper limit of normal. First line treatment options in essential thrombocythemia and polycythemia vera are control of platelet function with low-dose aspirin and reductive control of platelet count and erythrocytes by bloodletting, interferon and busulfan or hydroxyurea monochemotherapy. |
| Databáze: | OpenAIRE |
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