TITLE:
Acute Panmyelosis with Myelofibrosis: A Rare Subtype of Acute Myeloid Leukemia
AUTHORS:
Alioune Badara Diallo, Sokhna Aissatou Touré, Moussa Seck, Mouhamed Keita, Elimane Seydi Bousso, Blaise Felix Faye, Saliou Diop
KEYWORDS:
Acute Panmyelosis with Myelofibrosis, Bone Marrow Fibrosis, Cytopenias
JOURNAL NAME:
Open Journal of Blood Diseases,
Vol.11 No.3,
September
18,
2021
ABSTRACT: Acute panmyelosis with myelofibrosis (APMF) is a
subtype of acute myeloid leukemia (AML) classified among the category of “AML,
not otherwise specified” in the WHO 2016 classification of hematopoietic
tumors. It is a rare, fatal hematological neoplasm that is characterized by
acute onset of cytopenias and bone marrow fibrosis in the absence of
splenomegaly or fibrosis related morphological changes in the red blood cells.
The difficulty of diagnosis and management explains why APMF is rarely reported
in Africa. We report here the case of a 30-year-old man who presented with
dizziness, palpitations and dyspnea. Diagnosis of APMF was retained on bone
marrow histology and immunohistochemistry which showed bone marrow fibrosis and
high cellularity with majority of myeloid blast cells. The patient was treated
by low dose cytarabine monotherapy 30 mg/m2 per week. At 3 months of
treatment, the patient was transfusion-independent, with normalization of
hemoglobin and platelets counts. However, the death occurred after 8 months.
This case highlights the diagnosis specificity and management of AMPF, knowing
the number of potential differential diagnoses and difficulties of its
therapeutic management.