Acute Panmyelosis With Myelofibrosis ( Apmf ) - 1688 Words

Acute Panmyelosis with Myelofibrosis (APMF) is a rare type of acute myeloid leukemia that manifests with a rapidly fibrotic bone marrow and progressive cytopenias. APMF is usually fatal, a consequence of the disease’s ubiquitous pathologic and disease features, rapidly progressive course, and poor response to traditional treatments. The disease is pathologically recognized for its heavily fibrotic marrow, increased overall cellularity, immaturity of all cell line lineages, abnormal megakaryocyte population, and lack of driver mutations that likely contribute to the rapid and progressive disease course. In this chapter, we focus on how the hematologist and pathologist alike can best identify and manage this disease in light of these clinical challenges. We discuss the background of this unique and deadly disease and what led to the identification of this disease from pathologically similar entities. Morphology and recognition of characteristic disease features, including immun ophenotypic findings, cytogenetics and morphology are discussed. The identification and distinguishing features of this disease are explored in the setting of a comprehensive differential diagnoses that may mimic this pathologic state. Finally, we address disease prognosis and suggestions on management in light of the limited studies available regarding management. Background In 1963, Lewis and Szur described a disorder that they termed ‘malignant myelosclerosis’, characterized by an acute clinicalShow MoreRelatedThe Diverse Presentation Of Acute Leukemia1842 Words   |  8 PagesOverview The diverse presentation of acute leukemia subtypes often makes specific disease subtypes difficult to recognize. Few, however, are as clinically challenging for the practicing hematologic malignancy physician to recognize as acute panmyelosis with myelofibrosis (APMF). APMF is a rare type of acute myeloid leukemia (AML) that manifests with a rapidly fibrotic bone marrow and progressive cytopenias. Early recognition is essential, as APMF can quickly turn fatal, a consequence of the disease’s