Acute erythremic myelosis (true erythroleukaemia): a variant of AML FAB-M6

Howard, Julie; Wood, Andrew C.; Henry, Kristin; Bain, Barbara J.

doi:10.1136/jcp.54.3.205

Cited by 40 publications

(29 citation statements)

References 16 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Recently, attention has been focused on the heterogeneity of neoplasms of erythroid cells and, in particular, on those that are composed mainly of primitive erythroid precursors with a minimal, if any, myeloblastic component. [47][48][49][50][51] These latter cases may not meet the requirements of the FAB classification for a diagnosis of acute leukemia. …”

mentioning

confidence: 99%

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

Vardiman

Harris²,

Brunning³

2002

Blood

1,903

1,288

View full text Add to dashboard Cite

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...

show abstract

mentioning

confidence: 99%

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

Vardiman

Harris²,

Brunning³

2002

Blood

1,903

1,288

View full text Add to dashboard Cite

show abstract

“…[3][4][5][6] In 1992, KowalVern et al 6 proposed including erythroleukemias with Z30% pronormoblasts, the most immature recognizable erythroid precursors, as a subtype of AML. They also proposed modifying the FAB system by renaming FAB-M6 as M6a, and cases that fit with Di Guglielmo disease as M6b.…”

mentioning

confidence: 99%

Pure erythroid leukemia: a reassessment of the entity using the 2008 World Health Organization classification

Liu

Hasserjian

et al. 2011

Modern Pathology

View full text Add to dashboard Cite

Pure erythroid leukemia (PEL) is rare, characterized by a neoplastic proliferation of erythroblasts. Given recent incorporation of molecular genetic findings and clinical features in the revised 2008 World Health Organization classification scheme of acute myeloid leukemia, we questioned if PEL still remains as a distinct subtype of acute myeloid leukemia. In this retrospective study, we identified 18 cases of acute leukemia with morphologic and immunophenotypic features of PEL. Following the current World Health Organization classification algorithm, these cases were classified as: 13 acute myeloid leukemia with myelodysplasia-related changes, 3 therapy-related acute myeloid leukemia, and 1 chronic myelogenous leukemia blast crisis, and one unclassifiable due to insufficient clinical information. All 16 cases with cytogenetic data harbored an extremely complex karyotype and the median overall survival of the 18 patients was 3 months (range, 1-7 months). This survival was significantly shorter than that of patients with acute erythroid leukemia, erythroid/myeloid subtype, or acute myeloid leukemia with myelodysplasia-related changes with erythroid predominance (Po0.001). PEL is characterized as a neoplastic erythroid hyperproliferation with maturation arrest. E-cadherin emerged as the most sensitive and specific marker for immature erythroblasts, and was helpful in distinguishing PEL from other erythroid proliferations. Our study showed that the criteria for acute myeloid leukemia in the 2008 World Health Organization system facilitate reclassification of PEL cases into other acute myeloid leukemia categories, mainly of acute myeloid leukemia with myelodysplasia-related changes. These new assigned categories fail to capture the distinct features of PEL, where the phenotype of PEL correlates with a very complex karyotype and an extremely aggressive clinical course.

show abstract

“…In the era before the definition of PEL, Hasserjian et al (21) summarized the previously reported "true erythroleukemia" cases, and noted that about one-third of cases had clinically recognized hepatosplenomegaly. They showed hepatic infiltration by the neoplastic erythroid cells by liver biopsy in a case of acute erythremic myelosis (21).…”

Section: Discussionmentioning

confidence: 99%

“…They showed hepatic infiltration by the neoplastic erythroid cells by liver biopsy in a case of acute erythremic myelosis (21). After the establishment of the PEL criteria, Wang et al (9) reported that PEL could present as a myeloid sarcoma in the form of ovarian masses.…”

Section: Discussionmentioning

confidence: 99%

Therapy-Related Pure Erythroid Leukemia with Hepatic Infiltration and Hemophagocytic Syndrome

et al. 2011

View full text Add to dashboard Cite

Pure erythroid leukemia (PEL) is an extremely rare disorder characterized by neoplastic proliferation of immature erythroblasts. A 66-year-old man, who had received chemoradiotherapy for hypopharyngeal cancer, was admitted because of pancytopenia. Bone marrow was infiltrated with 81% proerythroblasts positive for CD71 and CD235a. An increased number of macrophages with active hemophagocytosis was also present. Chromosome analysis showed hypodiploid complex abnormalities. The patient died of progressive disease despite induction chemotherapy. Erythroblastic infiltration into the liver and hemophagocytosis in the spleen were found at autopsy. Therapy-related PEL with hemophagocytic syndrome and hepatic infiltration of PEL has never been reported.

show abstract

Acute erythremic myelosis (true erythroleukaemia): a variant of AML FAB-M6

Cited by 40 publications

References 16 publications

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

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

Pure erythroid leukemia: a reassessment of the entity using the 2008 World Health Organization classification

Therapy-Related Pure Erythroid Leukemia with Hepatic Infiltration and Hemophagocytic Syndrome

Contact Info

Product

Resources

About