Acute Megakaryocyte Myelofibrosis: Case Report of an Unusual Myeloproliferative Syndrome

Estévez, José Manuel Gálligo; Urueta, Enrique E.; Moran, Thomas J.

doi:10.1093/ajcp/62.1.52

Cited by 21 publications

(3 citation statements)

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“…A number of subsequent studies have described a similar disorder that has been termed 'acute myelofibrosis,' 'acute myelosclerosis,' 'acute megakaryocytic myelofibrosis,' and 'acute myelodysplasia with myelofibrosis.' [2][3][4][5] Most of these cases are now believed to correspond to the entity presently defined as acute panmyelosis with myelofibrosis (APMF) in the WHO classification system of hematopoietic malignancies. 6 APMF is exceedingly rare and corresponds to less than 1% of the cases of acute myeloid leukemias (AML), although this may be an underestimation due to missed diagnosis.…”

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Acute panmyelosis with myelofibrosis: an entity distinct from acute megakaryoblastic leukemia

et al. 2005

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The WHO criteria for diagnosing acute panmyelosis with myelofibrosis are somewhat distinct from those for acute megakaryoblastic leukemia. However, clinical and hematopathologic findings partially overlap. This has raised questions as to whether these are indeed separate, definable entities. To determine the potential importance of bone marrow biopsy supplemented by immunohistochemistry in distinguishing between these two conditions, we studied 17 bone marrow biopsies of well-characterized cases of acute panmyelosis with myelofibrosis (six cases) and acute megakaryoblastic leukemia (11 cases). We compared blast frequency, reticulin content, CD34 expression, and the degree of megakaryocytic differentiation of the blast cells in these two conditions. Our results demonstrate important differences. Acute panmyelosis with myelofibrosis is characterized by a multilineage myeloid proliferation with a less numerous population of blasts than acute megakaryoblastic leukemia (Po0.01). In the former condition, blasts are always positive with CD34, while in acute megakaryoblastic leukemia they express CD34 in 60% of the cases. The blasts in acute panmyelosis with myelofibrosis only rarely express megakaryocytic antigens. By contrast, acute megakaryoblastic leukemia has a significantly higher proportion of blasts expressing megakaryocytic antigens (Po0.01 with CD42b). Our results confirm that histology supplemented by immunohistochemistry permits the distinction of these conditions in routinely processed bone marrow biopsies.

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Acute panmyelosis with myelofibrosis: an entity distinct from acute megakaryoblastic leukemia

et al. 2005

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“…These were formerly called malignant myelosclerosis ( 14, 1 5), acute myelofibrosis ( 16), or acute megakaryocytic myelofibrosis (17). Methods of phenotyping megakaryoblasts indicated that these cases were variants of AML,rather than myelofibrosis, and had been designated as acute megakaryoblastic leukemia, referred to as M7 by the FAB classification (18,19).…”

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Myelodysplastic Syndrome Associated with Myelofibrosis, A Report of 3 Cases.

1993

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“…performed at the Institute of Pathology, Arhus Amtssygehus. in a 6 year period ( I 970-1 976) were selected, 55 had leukaemia (43 acute and I2 chronic) and 16 had multiple myeloma. Haematoxylin-eosin stained sections of lungs from the files were screened for megakaryocytes; 25 megakaryocytes per cm2 of lung tissue were defined as the upper normal limit for the number of pulmonary megakaryocytes (Aabo and Hansen ( I 977)).…”

Section: Autopsies With Leukaemia and Multiple Myelomamentioning

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Megakaryocytes in Pulmonary Blood Vessels

Hansen¹,

Aabo²

1978

Acta Pathologica Microbiologica Scandinavica Section A Patholog

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In a study of 71 autopsies in patients with malignant haematological disorders (55 leukaemias and 16 multiple myelomas) we found an increased number of megakaryocytes in the lung capillaries in only one of 55 cases of leukaemia (43 acute and 12 chronic leukaemias) with a mean value of 3 megakaryocytes per cm2. The incidence of pulmonary megakaryocytes in 16 cases of multiple myeloma was identical to that in an unselected, consecutive series of hospital autopsies. The discrepancy between the increased megakaryocytopoiesis and previously reported high number of circulating megakaryocytes in chronic myeloid leukaemia, and the few megakaryocytes in the pulmonary blood vessels of histological sections of autopsy specimens is discussed.

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Acute Megakaryocyte Myelofibrosis: Case Report of an Unusual Myeloproliferative Syndrome

Cited by 21 publications

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Acute panmyelosis with myelofibrosis: an entity distinct from acute megakaryoblastic leukemia

Acute panmyelosis with myelofibrosis: an entity distinct from acute megakaryoblastic leukemia

Myelodysplastic Syndrome Associated with Myelofibrosis, A Report of 3 Cases.

Megakaryocytes in Pulmonary Blood Vessels

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