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The Diverse Presentation Of Acute Leukemia

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Overview
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 ubiquitous pathologic features, aggressive clinical course, and poor response to traditional treatments. Pathologically this AML subtype is recognizable for its heavily fibrotic marrow, increased overall cellularity, immaturity of all cell-line lineages, abnormal megakaryocyte population, and lack of driver mutations. Clinical features include lack of spleen enlargement, bony pain, fevers and other features associated with systemic inflammation.
In this chapter, we focus on how the hematologist and pathologist alike can best identify and manage the disease in light of the clinical challenges that accompany its rapid and progressive course. 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 immunophenotypic findings, cytogenetics and morphology are discussed. The identification and distinguishing features of

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