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Table 5 Distinguishing features of primary myelofibrosis and autoimmune myelofibrosis

From: Fibrosis and bone marrow: understanding causation and pathobiology

Features

PMF

AIMF

I. Bone marrow

 Megakaryocytes

Proliferation and atypia

Lack of clustering/atypia

 Myeloid/erythroid dysplasia

Present

Absent

 Basophilia or eosinophilia

Present/ absent

Absent

 Lymphocytic infiltration

Absent

Present

 Osteosclerosis + /– –

May be present

Absent

II. Laboratory features

 Anemia

 + /–

 + /–

 Leukocytosis

Usually +  + /–

Absent

 Elevated LDH

Usually +  + 

 ± 

 Autoantibodies

 + /–

 + 

III. Clinical features

 Constitutional symptoms

Common

Uncommon

 Splenomegaly

Common

Absent/mild

IV. Other laboratory features

  

 Leukoerythroblastosis

 +  + 

 + /–

 JAK2, CALR, or MPL mutation 

 + (90% of cases)

–

  1.  + , present; –, absent
  2. AIMF Autoimmune myelofibrosis; LDH Lactate dehydrogenase; PMF Primary myelofibrosis