myeloid metaplasia
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Pathology
- occurs in association with myelofibrosis
- bone marrow
- panhypoplasia (85%)
- fibroblast proliferation (may be primary event)
- myelofibrosis
- osteosclerosis
- splenomegaly
- hepatomegaly
- engorgement with blood
- extramedullary hematopoiesis
- hemosiderosis
- anemia secondary to:
- ineffective erythropoiesis
- expanded plasma volume
- blood loss
- hemolysis
Clinical manifestations
- splenomegaly (100%)
- hepatomegaly (70%)
Laboratory
- complete blood count (CBC) shows anemia
- peripheral smear
- leukoerythroblasts (96%)
- dacrocytes (tear-drop cells)
- bone marrow biopsy - myelofibrosis
- serum vitamin B12 & serum folate
- fecal occult blood
Complications
- esophageal varices
- microinfarcts in the gut
Management
- also see myelofibrosis
- 80% of asymptomatic patients will remain asymptomatic at 5 years if untreated
- transfusion with packed RBC for symptomatic anemia
- androgens for ineffective erythropoiesis
- testosterone enanthate 400-600 mg/week
- fluoxymesterone 10 mg TID
- favorable response in 40% of males & 32% of females
- corticosteroids for hemolysis
- treat vitamin B12 deficiency & folate deficiency if indicated
- allogeneic stell cell transplantation for patients < 55 years of age
- pressure symptoms from splenomegaly
- hydroxyurea
- splenic irradiation
- splenectomy is associated with hemorrhagic & thrombotic complications, increased risk of progression to AML & has no benefit for survival[2]
- overall 5 year survival is 60%