Important Pathologic Considerations for Establishing the Diagnosis of Myelofibrosis

Diagnostic criteria for primary myelofibrosis as defined by the 2017 revised World Health Organization (WHO) classification system incorporate clinical and laboratory findings, including driver mutational status (JAK2, MPL, CALR. and triple negative). The WHO emphasized the role of histopathology in making an accurate diagnosis of primary myelofibrosis and successfully incorporated a fibrosis scoring system and scoring schemas for collagen fibrosis and osteosclerosis. These steps represent a significant addition to the standardization of myelofibrosis evaluation and minimize the risk for misdiagnosis. This article reviews important pathologic considerations along with highlights of potentially relevant pitfalls relevant to histopathological diagnosis of myelofibrosis.