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acute panmyelosis with myelofibrosis
poorly defined disorder
Etiology:
- clonal disorder
- toxic exposure to the bone marrow
Epidemiology:
- rare
- mostly adults
Microscopic pathology:
- proliferative process involves all major myeloid lines: erythroid, granulocytic, megakaryocytic
- abnormal megakaryocytes
- macrocytic erythropoiesis
- defects in neutrophil production
- myelofibrosis
Immunophenotype:
- phenotypic heterogeneity
- one or more myeloid antigens: CD13, CD33, CD117, myeloperoxidase
Genetics:
- complex abnormalities
- frequent involvement of chromosomes 5 and/or 7
Clinical manifestations:
- weakness
- fatigue
- marked pancytopenia
- no or minimal splenomegaly
- rapidly progressive course
Laboratory:
- bone marrow biopsy
- complete blood count
- pancytopenia
- very few blasts
- flow cytometry
- CD34-positive leukocytes
Differential diagnosis:
- AML with multilineage dysplasia
- acute megakaryoblastic leukemia
- other types of acute leukemia with marrow fibrosis
- metastatic neoplasm with desmoplasia
- chronic idiopathic myelofibrosis
Management:
- stem cell transplantation has been used
- prognosis: median survival: 8 months
Notes:
- currently classified as a form of acute myeloid leukemia (AML)
- lack of blasts in the peripheral blood would seem to preclude AML
- classification as a form of myelodysplastic syndrome seems more appropriate
General
panmyelosis
myelofibrosis
myelodysplastic syndrome (MDS)
acute myeloid leukemia (AML)
References
- Wikipedia: Acute panmyelosis with myelofibrosis
http://en.wikipedia.org/wiki/Acute_panmyelosis_with_myelofibrosis
- WHO Classification Tumours of Haematopoietic and Lymphoid
Tissues. IARC Press 2001
- Bae E, Park CJ, Cho YU et al
Differential diagnosis of myelofibrosis based on WHO 2008
criteria: acute panmyelosis with myelofibrosis, acute
megakaryoblastic leukemia with myelofibrosis, primary
myelofibrosis and myelodysplastic syndrome with myelofibrosis.
Int J Lab Hematol. 2013 Dec;35(6):629-36.
PMID: 23693053