AI Article Synopsis
- Myelofibrosis with myeloid metaplasia is a rare type of myeloproliferative syndrome, making diagnosis straightforward in its classical form due to specific symptoms like splenomegaly and unique blood cell appearances.
- The disease has a variable progression, with an average survival of 40 to 60 months, and there are several factors, particularly anemia, that can affect prognosis.
- Currently, no treatments have been proven to extend survival either, except for an allogenic bone marrow transplant, leading to challenges in establishing standard approaches for diagnosis, prognosis, and therapy due to the disease's rarity and complexity.
Article Abstract
Myelofibrosis with myeloid metaplasia is the rarest myeloproliferative syndrom. Diagnosis is often easy in classical form, characterised by splenomegaly, leukoerythroblastic blood reaction and tear-drop erythrocytes on blood count and myelofibrosis on bone marrow biopsy. Evolution is highly variable with a median overal survival of 40 to 60 months and numerous prognostic factors especially anemia. No treatment has been demonstrated to improve survival (apart from allogenic bone marrow transplant). The rarity and the complexity of the disease are the most important difficulties for the definition of standardized diagnostic, prognostic and therapeutic criteria.
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