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Objective:To analyze the relationship between FAB morphological classification and World Health Organization (WHO) 2016 classification in children with acute erythroid leukemia(AEL), and to summarize the clinical features and prognosis.Methods:Clinical data of de nova childhood AEL patients from January 1, 2002 to December 31, 2019, in Pediatric Blood Disease Center, Institute of Hematology & Blood Disease Hospital were retrospectively analyzed.All of them were re-evaluated according to the WHO 2016 classification.Results:(1) A total of 20 patients were diagnosed as AEL by FAB classification.According to the criteria of WHO 2016, they were re-diagnosed as myelodysplastic syndromes (MDS)- refractory anemia with excess of blasts (11 cases), acute myeloid leukemia with MDS-related changes (3 cases), acute monocytic leukemia (1 case), and pure red leukemia (PEL, 5 cases). (2) Pathological hematopoiesis was frequently detected in bone marrow smears.Auer bodies were seen occasionally in some blasts.The most common antigen expressing were CD 117, CD 13, CD 33, CD 34, CD7, and CD 38.Karyotype analysis was performed in 18 cases successfully, involving 6 cases with abnormal karyotypes, including + 8, -7, 22p+ , t (3; 5: ? ), + 3q-, 15q-, and del (9)(q13). (3) Thirteen cases were treated by chemotherapy, and the one-course complete remission rate was 38.5%.By July 1, 2020, only 2 cases were alive without disease.The overall survival was 49 months and 11 months, respectively. Conclusions:Childhood AEL is susceptible to pathological hematopoiesis, poor response to early chemotherapy and poor prognosis.After re-evaluation according to WHO 2016 classification, most of them were diagnosed as MDS-related.Therefore, adjusting the suitable induction regimen with allogeneic hematopoietic stem cell transplantation may improve the prognosis.
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Objective To further understand the diagnosis,clinical features and prognosis of myeloid neoplasms with erythroblast more than 50％ of bone marrow(BM) nucleated cells in the WHO Classification(2016) by analyzing the clinical data,diagnosis and prognosis of 3 patients with myeloid leukemia.Methods The ages,medical histories,symptoms and signs,and laboratory examinations from 3 patients with myeloid neoplasms whose erythroblast cells were more than 50％ of BM nucleated cells when newly diagnosed were collected.Then,they were diagnosed with the WHO Classification criteria(2008) and the WHO Classification criteria(2016),respectively,and their prognosis was evaluated with the revised International Prognostic Scoring System(IPSS-R).Results According to the WHO Classification criteria(2008),all of 3 patients were diagnosed as acute erythroid leukemia(AEL).However,according to the WHO Classification criteria(2016),2 patients were diagnosed as myelodysplastic syndrome with excess blasts-2(MDS-EB-2),and 1 was diagnosed as acute myeloid leukemia(AML) with maturation.Moreover,their prognostic scores were also different.The former two patients were older men with significant dysplasia and complex genetic abnormalities,and had poor prognosis,while the latter was a middle-aged woman with no obvious dysplasia and genetic abnormalities,and had medium prognosis.Conclusion The WHO Classification(2016) is more reasonable than the WHO Classification(2008),which tends to focus more on the different biological characteristics of diseases,and may better distinguish two types of diseases with different clinical features and prognosis.
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Os aspectos epidemiológicos, clínicos, hematológicos e anatomopatológicos da leucemia eritroide aguda (LMA M6) foram estudados em 10 gatos que morreram em consequência dessa condição. Os resultados obtidos diferem daqueles previamente descritos na literatura nos seguintes aspectos: 1) a doença ocorreu na forma de um modelo bimodal relacionado à idade dos gatos afetados, em que 50 por cento tinham 1-3 anos de idade e 50 por cento tinham 10 anos de idade ou mais; 2) quase todos os gatos afetados (87,5 por cento) demonstravam policromasia, possivelmente decorrente de eritropoese extramedular; 3) em todos os casos havia múltiplos focos de células hematopoéticas, principalmente eritropoeticas, em múltiplos órgãos, que incluíam baço (85,7 por cento), linfonodos (71,4 por cento), fígado (57,1 por cento) e rim (28,6 por cento); 4) em alguns casos (28,6 por cento) esses focos podiam ser vistos macroscopicamente, na forma de metástases, mas sempre diferiam histologicamente da medula óssea quanto à proporção dos precursores eritroides envolvidos; 5) em pelo menos um caso ocorreu um continuum patologicum até outra forma de LMA (LMA M4), um fenômeno denominado "infidelidade de linhagem". Esse artigo discute essas diferenças e reforça os critérios fundamentais para se estabelecer o diagnóstico definitivo dessa que é a forma mais importante de leucemia em gatos na nossa região.
The epidemiological, clinical, hematological, and pathological aspects of acute erythroid leukemia (LMA M6) were studied in 10 cats that died from this condition. The results obtained in the present study differ from those previously described in the literature regarding the following aspects: 1) The disease occurred as a bimodal model related to the age of affected cats, in which 50 percent were1-3-years-old and 50 percent were 10 years-old or above; 2) almost all affected cats (87.5 percent) displayed polychromasia, possible resulting from extramedullar erythropoiesis; 3) in all cases there were multiple foci of hematopoietic cells, mainly erythropoietic, in multiple organs including spleen (85.7 percent), lymph nodes (71.4 percent), liver (57.1 percent), and kidney (28.6 percent); 4) in some cases (28.6 percent) these foci could be observed on gross examination as metastasis, but they always histologically differ from bone marrow in regard to the proportion of erythroid precursors involved; 5) at least in one case a continuum patologicum occurred with the condition evolving to another form of LMA (LMA M4), a phenomenon coined as "lineage infidelity". This paper discuss these differences and stresses the fundamental criteria to establish a definitive diagnosis of this condition, which is the most import form of leukemia in cats from our region.
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Acute erythroid leukemia in children is very. Here is a case of erythroleukemia in a child of a age 1.5 years, which was diagnosed on peripheral smear, bone marrow examination, cytochemistry but was confimed on immunophenotyping. CD45 versus side scatter demonstrated blast population (29%) expressing CD45 of variable intensity (dim to negative). The myeloid nature of blast population showed bright expression of CD13, CD33. These blasts also showed bright positivity of CD71 which showed erythroid nature of blasts. Flow cytometry can be comprehensive enough to completely subtype cases of leukemias/myelodysplastic syndromes, polycythemia rubra vera, non-neoplastic conditions like reactive erythroid hyperplasia following immunosuppressive therapy or viral infections or nutritional deficiencies, unlyzed RBCs or thrombocytosis which may mimic acute erythroid leukemia on flow cytometry.