Acute panmyelosis with myelofibrosis: clinical, immunophenotypic and cytogenetic study of twelve cases.
The clinical, cytogenetic, and immunophenotypic features in 12 adult patients with acute panmyelosis with myelofibrosis (APMF; ICD-0-3: 9931/3; C42.1) are reported (median age: 57 years; f/m = 1.4). The white cell count (WBC) was normal in 3 patients; 9 had leucopenia. The median hemoglobin value was 64.5 g/l, and median platelet count 12 x 10(9)/l. Bone marrow biopsy showed a hypercellular marrow in 10/12 patients with a significant infiltration of pathological blasts (range: 30 - 60%). All the cases had marked reticulin fibrosis. Immunophenotyping of bone marrow blast cells showed the expression of early (CD34) and lineage-unspecified antigens (HLA-DR) in 6/7, and 7/7 patients, respectively. "Early" myeloid antigens (CD13, CD33) were seen in 6/7 and 4/6 patients respectively. Monocyte antigen (CD14) was expressed in 3/7 patients. Megakaryocyte antigen (CD61) and erythroid cell antigen (GpA) were each expressed in only 1 patient. Two patients had expression of CD34, HLA-DR and "early" myeloid antigens by their bone marrow blast cells and 1 of these also had a co-expression of the antigens from a differentiated monocytic cell proliferation (lysozyme+, CD68+). Nonspecific chromosomal aberrations were recorded in 8/10 patients. The median survival was 2 months. These findings suggest an immature myeloid phenotype of blast cells in APMF. In 6/9 patients a leukemic cell differentiation into monocytic, megakaryocytic or erythroid lineage was also demonstrated.
Suvajdzic
N
N
Institute of Hematology, Clinical Center of Serbia, Belgrade, Union of Serbia and Montenegro. nvukovic@Eunet.yu
Marisavljevic
D
D
Kraguljac
N
N
Pantic
M
M
Djordjevic
V
V
Jankovic
G
G
Cemerikic-Martinovic
V
V
Colovic
M
M
D000293:Adolescent; D000328:Adult; D000368:Aged; D005260:Female; D006801:Humans; D016130:Immunophenotyping; D007621:Karyotyping; D008297:Male; D008875:Middle Aged; D055728:Primary Myelofibrosis / Q000097:blood / Q000235:genetics* / Q000276:immunology / Q000473:pathology*; D015996:Survival Rate; D016896:Treatment Outcome