Megakaryoblastic Leukaemia (Acute Myelofibrosis): a Report of Three Cases

Ottolander, G. J. den; Velde, J. te; Brederoo, P.; Geraedts, J. P. M.; Slee, P. H. Th. J.; Willemze, R.; Zwaan, F. E.; Haak, H. L.; Müller, H; Bieger, R.

doi:10.1111/j.1365-2141.1979.tb03693.x

Cited by 126 publications

(21 citation statements)

References 26 publications

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“…Some believe that it is a variant of AML, and consider it to be equivalent to AMKL. 8,[18][19][20][21] Yet others believe that it is an acute variant of MDS, 22-24 a group of disorders in which myelofibrosis can also be observed. 25,26 The primary differential diagnosis of APMF is, however, with AMKL.…”

Section: Discussionmentioning

confidence: 99%

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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Section: Discussionmentioning

confidence: 99%

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

et al. 2005

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“…There is also some controversy about the relationship of APMF to acute megakaryoblastic leukemia. 58,59 The WHO committee recognized this problem. If the leukemia is predominantly megakaryoblastic with myelofibrosis, we suggest the term "acute megakaryoblastic leukemia with myelofibrosis."…”

Section: Org Frommentioning

confidence: 99%

The World Health Organization (WHO) classification of the myeloid neoplasms

Vardiman

Harris²,

Brunning³

2002

Blood

1,906

1,288

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A World Health Organization (WHO) classification of hematopoietic and lymphoid neoplasms has recently been published. This classification was developed through the collaborative efforts of the Society for Hematopathology, the European Association of Hematopathologists, and more than 100 clinical hematologists and scientists who are internationally recognized for their expertise in hematopoietic neoplasms. For the lymphoid neoplasms, this classification provides a refinement of the entities described in the Revised European-American Lymphoma (REAL) Classification-a system that is now used worldwide. To date, however, there has been no published explanation or rationale given for the WHO classification of the myeloid neoplasms. The purpose of this communication is to outline briefly the WHO classification of malignant myeloid diseases, to draw attention to major differences between it and antecedent classification schemes, and to provide the rationale for those differences. ( IntroductionRecently, the World Health Organization (WHO), in conjunction with the Society for Hematopathology and the European Association of Hematopathology, published a new classification for hematopoietic and lymphoid neoplasms. 1 The concepts that underlie this classification were derived from numerous published clinical and scientific studies and from the experience of more than 100 pathologists, clinicians, and scientists from around the world who collaborated to develop this consensus classification. 2 A basic principle of the WHO system is that the classification of hematopoietic and lymphoid neoplasms should utilize not only morphologic findings but also all available information, including genetic, immunophenotypic, biologic, and clinical features to define specific disease entities. Essentially, the WHO classification attempts to incorporate those disease characteristics that have proved to have clinical and biologic relevance into a useful, working nomenclature.For the lymphoid neoplasms, the WHO classification provides refinement of the entities defined in the Revised EuropeanAmerican Lymphoma (REAL) Classification-a system that is now widely used by pathologists and clinicians. 3 The WHO classification of myeloid neoplasms includes many of the criteria of the French-American-British (FAB) Cooperative Group classifications of acute myeloid leukemia (AML) 4 and myelodysplastic syndromes (MDS) 5 as well as guidelines of the Polycythemia Vera Study Group (PVSG) for the chronic myeloproliferative diseases (CMPDs), 6,7 but there are some significant differences. The purpose of this communication is to outline briefly the WHO classification of the myeloid neoplasms, to draw attention to major differences between the WHO and previous classifications of these disorders, and to provide the rationale for these differences. In addition, we wish to address and clarify specific issues concerning the classification that have appeared both prior and subsequent to the publication of the WHO monograph. The WHO classification of the myeloid neop...

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“…Several investi gators have described single cases of myelofibrosis with a unique terminal picture with thrombocytosis, giant plate lets and abnormal mononuclear cells in the circulation difficult to characterize on a morphological basis only. Published pictures of those 'atypical blastic cells', are con sistent with megakaryocytic precursors and micromega karyocytes [4,10,11,16].…”

Section: Discussionmentioning

confidence: 99%

“…Myelofibrosis with myeloid metaplasia has been fre quently described as a complication of patients with essential thrombocythemia and this probably represents the natural course of myeloproliferative disorders involv ing the megakaryocytic lineage [4][5][6][7][8][9][10][11][12][13][14][15][16][17], Reilly [18] has recently reviewed the pathogenesis of the so-called idio pathic myelofibrosis and concluded that the fibroblast proliferation is a reactive process secondary to the un derlying clonal hematopoiesis and not, as previously thought, a primarily malignant fibroblastic disorder. Us ing G6PD isoenzyme analysis it has been demonstrated that the fibroblast proliferation is a polyclonal process in response to megakaryocytic or myeloid-produced factors [19].…”

Section: Discussionmentioning

confidence: 99%

“…These authors proposed the designation of chronic myelosclerosis as a subtype of myeloproliferative disorder. Ottolanderet al [4] in 1979 described 3 patients with acute myelofibrosis and megakaryoblastic leukemia defined by morphological criteria under light and electron microscopy. Simultaneously, Bearman et al [5] published a detailed review of 49 cases reported under the designa tion of acute 'malignant' myelosclerosis presenting with pancytopenia, fibrotic bone marrow and with particular prominence of megakaryocytes and their precursors.…”

Section: Introductionmentioning

confidence: 99%

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Leukemia with Megakaryocytic Differentiation following Essential Thrombocythemia and Myelofibrosis

Patiño-Sarcinelli

Knecht²,

Pechet³

et al. 1996

Acta Haematol

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Leukemias of megakaryocytic lineage are rare and heterogeneous clinical entities. The nomenclature published in the literature is confusing and perhaps inappropriate to designate these primary myeloproliferative disorders. We describe a patient with essential thrombocythemia who evolved through myelofibrosis and myeloid metaplasia to a final picture of leukemia with megakaryocytic differentiation in the peripheral blood. This case illustrates different aspects of a chronic myeloproliferative disorder where myelofibrosis and myeloid metaplasia are frequent but secondary events. We have reviewed the literature focusing on the role of clonal megakaryocytic proliferation in myelofibrosis and on the clinical characterization of leukemias with megakaryocytic phenotype. We also present our interpretation of the literature which indicates that a formal review of the nomenclature is urgently needed.

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Megakaryoblastic Leukaemia (Acute Myelofibrosis): a Report of Three Cases

Cited by 126 publications

References 26 publications

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

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

The World Health Organization (WHO) classification of the myeloid neoplasms

Leukemia with Megakaryocytic Differentiation following Essential Thrombocythemia and Myelofibrosis

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