Current perspective in agnogenic myeloid metaplasia.
Agnogenic myeloid metaplasia (AMM) carries the worst prognosis among the chronic myeloproliferative disorders. Substantial bone marrow fibrosis, extramedullary hematopoiesis, anemia and hepatosplenomegaly are the characteristic features of the disease. AMM is currently incurable and the available treatment agents are mostly palliative and do not prolong life. Two pathogenetic processes are responsible for the impaired hematopoiesis and the clinical manifestations. The primary disease process is a clonal hematopoietic stem cell disorder which results in chronic myeloproliferation and atypical megakaryocytic hyperplasia. The secondary process of bone marrow fibrosis is the result of non-clonal fibroblastic proliferation and hyperactivity induced by growth factors abnormally shed from clonal megakaryocytes. Therefore, experimental treatment strategies may be directed towards either one or both of these disease processes. This report summarizes the current management options and new therapeutic endeavours.
Tefferi
A
A
Division of Hematology and Internal Medicine, Mayo Clinic, Rochester, MN 55905, USA.
Silverstein
M N
MN
D001856:Bone Marrow Examination; D002999:Clone Cells / Q000473:pathology; D003937:Diagnosis, Differential; D018450:Disease Progression; D005347:Fibroblasts / Q000473:pathology; D006410:Hematopoiesis; D006801:Humans; D006965:Hyperplasia; D007938:Leukemia / Q000453:epidemiology / Q000473:pathology; D008533:Megakaryocytes / Q000473:pathology; D010166:Palliative Care; D010982:Platelet-Derived Growth Factor / Q000502:physiology; D055728:Primary Myelofibrosis / Q000175:diagnosis / Q000209:etiology / Q000401:mortality / Q000473:pathology / Q000628:therapy; D016212:Transforming Growth Factor beta / Q000502:physiology
D010982:Platelet-Derived Growth Factor; D016212:Transforming Growth Factor beta