Erythremia
The Erythrämien (from ancient Greek ἐρυθρός , erythros "red" and αίμα , Haema "blood") is a malignant haematological malignancies caused by increased proliferation (proliferation) nucleated red precursors (i.e. erythropoietic cells) in the blood forming organs and by their appearance in the peripheral blood are characterized without the granulocytopoiesis being significantly changed. A distinction is made between an acute form with smooth transitions to acute erythroleukemia (= acute myeloid leukemia of the FAB classification M6) and a chronic form, which corresponds in its course to chronic myeloid leukemia .
Molecular causes
The cause and pathogenesis of the disease have not yet been clarified. Erythremia is caused by a degeneration of erythropoiesis , that is, the formation process of red blood cells. Immature erythrocytes with a shortened lifespan, so-called erythroblasts , which are also called megaloblasts if they are abnormally large, predominate . The differences in size of the red blood cells or their precursors and their unusual shape compared to healthy cells are striking.
Symptoms
- Acute erythremia is characterized by fever and swelling of the liver and spleen . The lack of blood platelets ( thrombocytes ) causes a tendency to bleed. It is not uncommon for severe anemia to occur . Since the number of granulocytes is usually reduced, there is a susceptibility to infections. If left untreated, acute erythremia leads to death within a few months.
- Chronic erythremia (or chronic erythroblastosis ) is rare and is less severe than acute. Sometimes it is difficult to differentiate from the sideroblastic anemia that occurs with iron utilization disorder. The erythroblasts show less pronounced changes .
diagnosis
The diagnosis is made using a blood count and examination of the bone marrow . Here one can see a left shift (see nuclear shift ) of the erythropoiesis. The differentiation from other subtypes of leukemia is often not easy.
therapy
Erythremia is treated with cytostatics (tumor-inhibiting substances). In addition to blood transfusions used.
literature
- Erythremia and erythroleukemia . In: Microsoft Encarta 2008
- Roche Lexicon Medicine . 4th edition, Urban & Fischer, Munich 1999
- Ludwig Heilmeyer , Herbert Begemann: blood and blood diseases. In: Ludwig Heilmeyer (ed.): Textbook of internal medicine. Springer-Verlag, Berlin / Göttingen / Heidelberg 1955; 2nd edition ibid. 1961, pp. 376–449, here: p. 416 ( Die Erythrämien ) and 430.
Individual evidence
- ↑ Michael Begemann: Practical Hematology . Thieme Verlag, Stuttgart, New York 1999, ISBN 3-13-306211-5 , pp. 145 f.
