Erythroid-predominant myelodysplastic syndromes: enumeration of blasts from nonerythroid rather than total marrow cells provides superior risk stratification

Wang, Sa A.; Tang, Guilin; Fadare, Oluwole; Hao, Suyang; Raza, Azra; Woda, Bruce A.; Hasserjian, Robert P.

doi:10.1038/modpathol.2008.142

Cited by 40 publications

(30 citation statements)

References 27 publications

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“…We acknowledge that the strict WHO definition for PEL helps avoid confusion with other conditions of erythroid hyperproliferation with a left-shifted maturation seen in both non-neoplastic and neoplastic conditions. 4,18 However, if the immature erythroblasts represent a monotonous, overtly neoplastic proliferation, we question the rationale for the requirement of Z80% erythroblasts to qualify a case for PEL as other types of AML (including megakaryocytic) require only Z20% blasts.…”

Section: Discussionmentioning

confidence: 99%

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

Liu

Hasserjian

et al. 2011

Modern Pathology

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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.

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Section: Discussionmentioning

confidence: 99%

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

Liu

Hasserjian

et al. 2011

Modern Pathology

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“…After rapid clinical deterioration, his goals of care were limited to palliation, and he expired 2 days later. PEL is a rare type of acute myeloid leukemia, which often arises from preceding myelodysplastic syndromes and may be therapy related in some cases [1][2][3][4]. Here, we present a highly unique and, to our knowledge, the first reported case of a PEL evolving out of a myeloid-neoplasm thought to be secondary to treatment for CLL.…”

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confidence: 94%

Pure erythroid leukemia evolving from a therapy‐related myelodysplastic syndrome secondary to treatment for chronic lymphocytic leukemia

Sadrzadeh

Fathi

2012

American J Hematol

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A 75-year-old male carried a diagnosis of chronic lymphocytic leukemia (CLL) for many years for which he received several courses of chemotherapy, in succession cladribine, cyclophosphamide, vincristine, and prednisone, and fludarabine and cyclophosphamide. Approximately 14 years after his CLL diagnosis, he developed progressive and refractory thrombocytopenia which did not respond to anti-CLL therapy. A bone marrow biopsy was performed and was markedly hypercellular and left shifted. There were several lymphoid aggregates consistent with CLL, but comprising only about 20% of the marrow cellularity. The hypercellular areas of hematopoiesis contained increased small, dysplastic megakaryocytes, and increased blasts (Image 1A), confirmed by CD34 immunohistochemistry (Image 1B). Cytogenetics revealed a t(7;14)(p11.2;q32) abnormality in five of 20 metaphases. Overall, these pathologic features were consistent with a therapy-related myelodysplastic syndrome. He was treated with 5-azacitidine, and a repeat bone marrow biopsy after two cycles of therapy revealed a decrease in the number of CD341 blasts, indicating a modest response. Five months after his diagnosis of therapy-related myeloid neoplasm, due to worsening pancytopenia and persistent fevers, he underwent a bone marrow biopsy, which revealed evolution to pure erythroid leukemia (PEL) (Image 1C,D), with primitive, glycophorin positive erythroid blasts present in cohesive sheets (Image 1E) that were CD34-negative and comprised 80% of the cellularity. Also, present were aggregates of persistent PAX5-positive CLL cells

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“…In a different study, the same authors analyzed a cohort of patients with MDS and expanded erythropoiesis and found that cytogenetic risk-category and blast numbers counted as a percentage of non-erythroid bone marrow cells (<5% versus ≥5%) had a strong prognostic significance. 5 The same authors also separately described a group of 18 patients with primary erythroid leukemia in which complex karyotypes were noted in all 16 patients with available cytogenetic data. 14 Kasyan et al 8 reviewed a cohort of 90 patients with hematologic malignancies with expanded erythropoiesis, dividing them according to the WHO 2008 classification.…”

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“…1 Several groups have recently discussed the diagnosis, definition and prognosis of AEL and MDS with expanded erythropoiesis (>50% erythropoietic precursors in bone marrow smears). [4][5][6] The common findings were high incidence of unfavorable karyotypes, low frequency of NPM1, FLT3 and RAS mutations, and poor prognosis. 4,[6][7][8][9][10] In this issue of Haematologica, Bacher et al 4 have compared some genetic and clinical aspects of AML and MDS with expanded erythropoiesis.…”

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Acute myeloid leukemia with expanded erythropoiesis

Porwit¹,

Vardiman²

2011

Haematologica

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Depending on previous clinical history, number of blasts in the bone marrow, and results of cytogenetic analysis, hematologic malignancies with expanded erythropoiesis (more than 50% of bone marrow erythropoietic cells) may fall under various categories of the 2008 edition of the World Health Organization (WHO) classification of tumors of hematopoietic and lymphoid tissues (Table 1). 1The general threshold of blast percentage for the diagnosis of acute myeloid leukemia (AML) is 20% of total peripheral blood or bone marrow cells. However, cases with fewer than 20% blasts and more than 50% of erythroid precursors in the bone marrow are classified as acute erythroid leukemia (AEL, erythroid/myeloid) if blasts account for 20% or more of the non-erythroid cells. If blasts constitute less than 20% of nonerythroid cells, the cases are classified into various categories of myelodysplastic syndromes (MDS). Patients with 80% or more of bone marrow cells representing immature cells committed exclusively to the erythropoietic lineage are classified as having pure erythroid leukemia.The WHO 2008 classification has also defined the category of AML with myelodysplasia-related changes (AML-MRC) as acute leukemia with 20% or more peripheral blood or bone marrow blasts with morphological features of myelodysplasia or a prior history of MDS or MDS/myeloproliferative neoplasm or MDS-related genetic abnormalities (Table 2), and absence of prior cytotoxic therapy and the specific genetic abnormalities that would classify the case as AML with recurrent genetic abnormalities. Several studies confirmed the negative prognostic significance of the MDSrelated genetic abnormalities.2,3 Concerning cases with more than 50% erythroid precursors, the authors of the WHO 2008 classification stated that cases with 20% or more blasts should be classified as AML-MRC if MDS-related cytogenetic abnormalities or multilineage dysplasia in more than 50% of cells in two or more lineages are found. It has also been pointed out that patients with AEL often have complex karyotypes but the possibility of including AEL cases with complex karyotypes and less than 20% bone marrow blasts in the AML-MRC category has not been addressed. 1Several groups have recently discussed the diagnosis, definition and prognosis of AEL and MDS with expanded erythropoiesis (>50% erythropoietic precursors in bone marrow smears).4-6 The common findings were high incidence of unfavorable karyotypes, low frequency of NPM1, FLT3 and RAS mutations, and poor prognosis. 4,[6][7][8][9][10] In this issue of Haematologica, Bacher et al. 4 have compared some genetic and clinical aspects of AML and MDS with expanded erythropoiesis. On the basis of the observed better 2-year overall survival rate of MDS patients (n=104) in comparison to those classified as having AML (combined cohort of 77 AEL and 24 AML-MRC), the authors propose continuing separating MDS with expanded erythropoiesis from AML. It should be noted that the patients in this study classified as having MDS and carrying an unfavorab...

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Erythroid-predominant myelodysplastic syndromes: enumeration of blasts from nonerythroid rather than total marrow cells provides superior risk stratification

Cited by 40 publications

References 27 publications

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

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

Pure erythroid leukemia evolving from a therapy‐related myelodysplastic syndrome secondary to treatment for chronic lymphocytic leukemia

Acute myeloid leukemia with expanded erythropoiesis

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