OBJECTIVES: To investigate the clinical characteristics and prognosis of acute erythroleukemia (AEL) in children. METHODS: A retrospective analysis was conducted on the clinical data, treatment, and prognosis of 8 children with AEL treated at the First Affiliated Hospital of Zhengzhou University from January 2013 to December 2023. RESULTS: Among the 7 patients with complete bone marrow morphological analysis, 4 exhibited trilineage dysplasia, with a 100% incidence of erythroid dysplasia (7/7), a 71% incidence of myeloid dysplasia (5/7), and a 57% incidence of megakaryocytic dysplasia (4/7). Immunophenotyping revealed that myeloid antigens were primarily expressed as CD13, CD33, CD117, CD38, and CD123, with 4 cases expressing erythroid antigens CD71 and 2 cases expressing CD235a. Chromosomal analysis indicated that 2 cases presented with abnormal karyotypes, including +8 in one case and +4 accompanied by +6 in another; no complex karyotypes were observed. Genetic abnormalities were detected in 4 cases, with fusion genes including one case each of dup MLL positive and EVI1 positive, as well as mutations involving KRAS, NRAS, WT1, and UBTF. Seven patients received chemotherapy, with 6 achieving remission after one course of treatment; 2 underwent hematopoietic stem cell transplantation, and all had disease-free survival. Follow-up (median follow-up time of 6 months) showed that only 3 patients survived (2 cases after hematopoietic stem cell transplantation and 1 case during treatment). CONCLUSIONS Children with AEL have unique clinical and biological characteristics, exhibit poor treatment response, and have a poor prognosis; however, hematopoietic stem cell transplantation may improve overall survival rates.
Humans ; Male ; Female ; Prognosis ; Child, Preschool ; Retrospective Studies ; Child ; Leukemia, Erythroblastic, Acute/diagnosis* ; Infant ; Adolescent

OBJECTIVETo observe the biological characteristic and the prognoses in patients with acute erythroleukemia (AEL).

METHODSThe results of 167 patients with newly diagnosed AEL, from January 2003 and June 2013 in the First Affiliated Hospital of Soochow University, were reviewed by MICM.

RESULTSFlow cytometry analysis indicated that CD13(96.1%), CD33(95.1%), CD117(87.4%) and CD34 (79.4%) were highly expressed in AEL. 56 of 148 (37.8%) AEL patients had a variety of cytogenetic abnormalities, 27 of 148(18.2%) patients were complex karyotype (abnormalities involving 3 or more chromosomes), the abnormalities of chromosomes 3, 5, 7 and 8 were more frequently involved and the most common one was ＋8, accounting for 35.7% of all abnormal karyotype, followed by 5q- (17.9%). Mutation analysis showed CEBPA mutation ratio of AEL patients was 44.0% (11/25), that of NPM1 as 15.4% (4/26). Initial induced remission rate of AEL was 56.6% (30/53), compared by 33.3% (4/12) of MDSM6. Survival analysis showed that the overall survival in female was better than that in male (P=0.047). The overall survival time of transplantation group is significantly longer than chemotherapy group (P=0.000). The OS of 13-39 years old group was the best, 40-49 years old group took second place, >50 years old group appeared to be the worst.

CONCLUSIONAEL had its own unique biological features, and allogeneic hematopoietic stem cell transplantation could significantly improve its poor prognosis.

Adolescent ; Adult ; Female ; Humans ; Leukemia, Erythroblastic, Acute ; diagnosis ; Male ; Middle Aged ; Prognosis ; Remission Induction ; Young Adult

The aim of this study was to investigate the clinical characteristics and prognosis of acute erythroleukemia (AEL, AML-M6). The clinical features and results of morphologic, immunophenotypic, cytogenetic and molecular biologic detections were retrospectively analyzed in 13 cases of AEL from 305 acute leukemia patients hospitalized between October 2007 and October 2012. The results showed that the expression of erythroid and non-erythroid cells increased at the same time. The myeloid antigens mainly expressed CD13/CD33/CD117/CD34, while the erythroid antigens expressed Gly and CD71. The karyotypic detection indicated that there were 1 case with normal karyotype, 3 cases with simple karyotypic abnormality and 2 cases with complex karyotypic abnormality, the other cases were not detected. The molecular biological detection found that the poor prognosis gene existed in 5 cases [38.5% (5/13)], including 3 cases with MLL-MLL fusion gene, 1 case with MLL mutation, and 1 cases with NRAS gene mutation, the abnormal genes were not detected in remainder 8 cases. After chemotherapy with decitabine, the complete remission (CR) rate achieved 53.5% (7/13), partial remission (DR) rate achieved 15.4% (2/13). Finally, 8 patients received allo-HSCT, the median overall survival (OS) was 20.7 months, 3 year survival rate was 79%, 3 year disease-free survival rate was 78%. It is concluded that the acute erythroleukemia is a rare subtype of AML, which is transformed from MDS and has harmful genes and poor prognosis. Allo-HSCT and treatment with decitabine may enhance the survival rate of AEL.
Adolescent ; Adult ; Aged ; Child ; Child, Preschool ; Female ; Humans ; Karyotyping ; Leukemia, Erythroblastic, Acute ; diagnosis ; genetics ; Male ; Middle Aged ; Prognosis ; Retrospective Studies ; Survival Rate ; Young Adult

AML relapsing as ALL has rarely been reported. We describe the case of a 62-yr-old man who was diagnosed with erythroleukemia with a complex karyotype and achieved complete hematologic and cytogenetic remission after induction chemotherapy. However, 4 months after the initial diagnosis, he showed relapse with blasts showing a different morphology and immunophenotype and was diagnosed with precursor B-cell ALL. The relapsing precursor B-cell ALL presented with the same leukemic clones as the primary erythroleukemia. Cytogenetic analysis of his bone marrow (BM) at the time of the primary erythroleukemia showed complex karyotypic abnormalities, including monosomy 5 and monosomy 7. At relapse, his BM showed reemergence of these leukemic clones of complex karyotypic abnormalities with clonal switch. To our knowledge, this is the first case of a lineage switch from erythroleukemia to ALL.
Acute Disease ; Antimetabolites, Antineoplastic/therapeutic use ; Bone Marrow Cells/pathology ; Cell Lineage ; Cell Transformation, Neoplastic ; Chromosome Deletion ; Chromosomes, Human, Pair 5 ; Chromosomes, Human, Pair 7 ; Cytarabine/therapeutic use ; Drug Therapy, Combination ; Humans ; Immunophenotyping ; Karyotyping ; Leukemia, Erythroblastic, Acute/*diagnosis/drug therapy ; Male ; Middle Aged ; Monosomy ; Naphthacenes/therapeutic use ; Precursor B-Cell Lymphoblastic Leukemia-Lymphoma/*diagnosis/pathology ; Recurrence

In order to study whether erythroleukemia was really a subtype of acute leukemia, the clinical laboratory characteristics and development of disease in 21 cases of erythroleukemia were analyzed. The results indicated that the percentage of patients with leucocytopenia, anemia and thrombocytopenia were 42.9%, 81% and 81% respectively at the time of diagnosis. These were 85.7% of patients with myelocytes and premonocyte, 52.4% of patients with erythroblast in their blood smear respectively. All of the bone marrow showed active or significantly active proliferation. The median percentage of erythro-lineage, myeloblast of NEC and displasia were (58.3 +/- 8.0)%, (58.0 +/- 18.4)% and 66.7% respectively, that is different from typical AML. 52.4% of M(6) patients transferred to RAEB/RAEB-T and AML-M(2) subtype in the disease progression. 11/19 cases (57.4%) achieved remission (CR 10; PR 1) after chemotherapy. The median remission length were 6 months for CR patients and 2 months for PR patients, but most of CR patients displayed obvious displasia of bone marrow and cytopenia of blood in the period of CR. The median survival length of M(6) and MDS-->M(6) from time of diagnosis were 13.0 +/- 13.2 and 2.3 +/- 1.3 months respectively. It is concluded that there are differences between M(6) and typical AML. Most of M(6) patients would rather be classified MDS RAEB and RAEB-t with over-hyperplasia of erythron lineage than a subtype of AML.
Adult ; Aged ; Bone Marrow Examination ; Diagnosis, Differential ; Female ; Humans ; Leukemia, Erythroblastic, Acute ; blood ; diagnosis ; Male ; Middle Aged ; Myelodysplastic Syndromes ; classification ; diagnosis ; Retrospective Studies

Trichosporon beigelii is often resistant to the fungicidal effect of amphotericin B and can cause fatal disseminated infections in immunocompromised patients. We report a case of a disseminated T. beigelii infection with a favorable outcome in a patient with acute erythroleukemia and neutropenia. The patient presented a persistent fever, multiple erythematous skin lesions, and pulmonary infiltrates. T. beigelii was isolated from blood cultures in four days and also from cultures of abdominal skin lesion, sputum, and stool. The isolate was resistant to amphotericin B (MIC, 2 microgram/mL), and the respective fluconazole and itraconazole MICs were 4 and 1 microgram/mL. The patient was successfully treated with fluconazole plus amphotericin B in combination with granulocyte colony stimulating factor and leukocyte transfusion. This case shows the importance of early diagnosis and treatment with combination of amphotericin B and fluconazole as a prognostic factor of disseminated T. beigelii infections.
Amphotericin B* ; Colony-Stimulating Factors ; Early Diagnosis ; Fever ; Fluconazole* ; Granulocytes ; Humans ; Immunocompromised Host ; Itraconazole ; Leukemia, Erythroblastic, Acute ; Leukocyte Transfusion ; Neutropenia ; Skin ; Sputum ; Trichosporon*

We have performed analysis of the clinical and epidemic finding in 21 patients diagnosed acute erythroblastic leukemia (M6). M6/AML was 5.9%. The median age was 37.7618.33. Ratio male/female was 0.75 anemia. 4.8% of them had been exposed to possible radiation. 100% of them presented with anemia, 47.5% presented with fever and infection, 42.5% presented with hemorrhage, 38.1% presented symptoms with infiltration. We concluded that acute erythroblastic leukemia has more favorable than AML
Leukemia ; Leukemia, Erythroblastic, Acute ; diagnosis

An analysis of the morphologic characteristics was performed in 21 patients diagnosed acute erythroblastic leukemia (M6). The result showed that 95.2% of them had nucleated red cell and blast in the peripheral blood. The percentage of nucleated red cell in the peripheral blood was 17.571+/-4.04. Reticulocytes was 0.5+/-0.11% in peripheral blood. (proerythroblast + basophilic erythroblast) was 32+/-8.4%. (Polychromatic erythroblast + orthochromatic erythroblast) was 30.238+/-8.86%.
Leukemia, Erythroblastic, Acute ; Bone Marrow ; diagnosis