期刊
INTERNATIONAL JOURNAL OF LABORATORY HEMATOLOGY
卷 35, 期 6, 页码 629-636出版社
WILEY
DOI: 10.1111/ijlh.12101
关键词
Myelofibrosis; acute megakaryoblastic leukemia; acute panmyelosis with myelofibrosis; myelodysplastic syndrome; primary myelofibrosis
类别
资金
- Asan Institute for Life Sciences [2010-109]
IntroductionThe aim of this study was to characterize clinicopathological features of acute panmyelosis with myelofibrosis (APMF), acute megakaryoblastic leukemia with myelofibrosis (AMKL-MF), primary myelofibrosis (PMF) and myelodysplastic syndrome with myelofibrosis (MDS-MF) in order to provide the keys to the differential diagnosis of bone marrow (BM) fibrosis. MethodsWe compared age, gender, splenomegaly, serum lactate dehydrogenase level, blood cell counts, blast counts in peripheral blood (PB) and BM, megakaryocyte counts, BM cellularity, dysplasia, and the karyotypes of patients with APMF (n=6), AMKL-MF (n=7), PMF (n=44), and MDS-MF (n=44). ResultsAPMF showed hyperplasia of all three lineages, increase in megakaryocyte count with dysplasia and frequent abnormal karyotypes. AMKL-MF was associated with elevated BM blast counts, decreased BM megakaryocyte count with rare megakaryocytic dysplasia and chromosome 21 abnormality. PMF patients displayed splenomegaly, rare blasts in PB/BM, and JAK2 V617F mutation. MDS-MF patients showed pancytopenia, dysplasia in all three lineages and recurrent chromosomal abnormalities involving chromosome 5,7,12, and 17. ConclusionsAlthough differential diagnosis among APMF, AMKL-MF, PMF, and MDS-MF is very challenging due to the overlapping clinical and morphological features, meticulous investigation of the patient with respect to splenomegaly, blood cell count, PB and BM findings, and karyotype will serve as a guide to correct diagnosis.
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