Is it chronic idiopathic myelofibrosis, myelofibrosis with myeloid metaplasia, chronic megakaryocytic–granulocytic myelosis, or chronic megakaryocytic leukemia? Further thoughts on the nosology of the clonal myeloid disorders

Chronic megakaryocytic leukemia, misnamed chronic idiopathic myelofibrosis, has neoplastic not hyperplastic megakaryocytopoiesis

“…Philadelphia-negative clonal hematopoiesis is a significant feature of dasatinib therapy for chronic myeloid leukemia Descriptions of chromosomally abnormal Philadelphia-negative (Ph Ϫ ) clones in chronic myeloid leukemia (CML) patients treated with chemotherapy, 1 interferon-alpha, 2 and, most recently, imatinib 3,4 suggest that such events arise indirectly as a result of suppression of the BCR-ABL-positive cell population. However, the incidence and significance of karyotypic abnormalities in Ph Ϫ cells of patients receiving alternative tyrosine kinase inhibitors (TKIs) to imatinib have not yet been explored.…”

“…4 The members of the WHO committee who are responsible for nomenclature of the clonal myeloid diseases should permit nosology to advance with the science it is meant to encapsulate. The paper by Ciurea et al 1 adds important evidence to the centrality of neoplastic megakaryocytes in this disorder.…”

Is it primary myelofibrosis or chronic megakaryocytic leukemia?

“… 3 Remarkably, the first three designations cited above were used in the title of three papers on the topic by the same senior author. 4 The current “official’ designation of primary myelofibrosis is a profound pathobiological misnomer.…”

Idiopathic myelofibrosis mimicking hemolytic anemia

“…2,3 Myelofibrosis was first described in 1879 by Gustav Heuck. 4 There is stepwise evolution of the disease process with an intial prefibrotic stage that is characterized by hypercellular bone marrow (BM) with minimal reticulin fibrosis that merges into fibrotic stages which demonstrates marked reticulin or collagen fibrosis in bone …”