Acute Panmyelosis with Myelofibrosis: Clinical, Immunophenotypic and Cytogenetic Study of Twelve Cases
Institute of Hematology, Clinical Center of Serbia, Belgrade, Union of Serbia and Montenegro.Leukemia and Lymphoma (Impact Factor: 2.89). 10/2004; 45(9):1873-9. DOI: 10.1080/10428190410001683688
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.
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ABSTRACT: In a retrospective study of 236 patients with primary myelodysplastic syndromes (MDS), 130 cases (55.1%) revealed myelofibrosis in bone marrow biopsies. It was observed that fibrosis mostly occurs focally or patchy, and collagen deposits were found very rarely (only four patients). The histopathology of bone marrow biopsies revealed several differences between fibrotic and non-fibrotic MDS: cellularity is significantly higher, dysmegakaryopoiesis is more pronounced, plasmocytes and mast cells are more often increased, and disturbance of marrow topography (particularly of the MK- and G-line) can be found more frequently in MDS with myelofibrosis. Reticulin fibrosis occurred in all subtypes of MDS; however, there was a higher incidence in chronic myelomonocytic leukemia. The frequency of abnormal growth of GM-progenitors was significantly higher in the MDS cases with myelofibrosis, compared to the cases without fibrosis. Clinical data showed significantly higher WBC, more frequent presence of immature granulocytes, and higher percentage of myeloblasts in peripheral blood and bone marrow in MDS with myelofibrosis compared to cases without myelofibrosis. Life expectancy was reduced to 13 mo, compared with 35 mo in MDS without fibrosis (p=0.00055). Time to leukemic transformation was 32 mo in MDS with fibrosis, compared with >56 mo in MDS without fibrosis (p=0.015). Myelofibrosis therefore seems to herald a poor prognosis.
Chapter: Acute Myeloid Leukemia[Show abstract] [Hide abstract]
ABSTRACT: Acute myeloid leukemia (AML) represents a group of hematopoietic neoplasms derived from the bone marrow precursors of myeloid lineage. The neoplastic process is the result of clonal proliferation of an aberrant, committed stem cell at the level of CFU-S or later stages of differentiation leading to the accumulation of immature forms without, or with limited, maturation. Other terms used to denote AML include acute nonlymphoid leukemia (ANLL), acute myelogenous leukemia, and acute myeloblastic leukemia. According to this classification, AML is divided into four major categories as: AML with recurrent genetic abnormalities, AML with multilineage dysplasia, AML and myelodysplastic syndromes (MDS), therapy related, AML not otherwise categorized. Three major environmental insults have been implicated in the increased incidence of AML: ionizing radiation, chemotherapeutic agents, and occupational exposure to chemicals. Ionizing radiation induces DNA damage leading to chromosomal breaks which may cause mutations, deletions, and translocations. The extent of this damage depends on the type of radiation, the amount and rate of absorption, distribution of the absorbed energy in the tissue, and the intervals between the radiation exposures. Therapy-related AML (t-AML) and therapy-related MDS (t-MDS) represent spectrums of a progressive clonal hematopoietic disorder that is evolved following cytotoxic chemotherapy and/or irradiation. The reason for chemotherapy or irradiation is usually a primary malignancy.
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