Myelodysplasia with Marrow Fibrosis

 

NOTE: This may represent an overlap syndrome involving myelodysplasia and primary myelofibrosis (myelodysplasia-myeloproliferative syndrome).

 

Features:

(1) trilineage dysplasia, often with increased numbers of megakaryocytes

(2) diffuse marrow fibrosis

(3) pancytopenia

(4) variable hepatosplenomegaly

(5) variable leukoerythroblastic reaction (blasts and immature cells in the peripheral blood with teardrop cells)

 

where:

• Immunostains using antibodies to CD61 can help to identify megakaryocytes.

• A slight increase in reticulin fibers in a patient with myelofibrosis probably does not have clinical significance.

 

Significance:

(1) early marrow failure

(2) poor prognosis

(3) shorter time to leukemia transformation