ABSTRACT
Objective@#To describe the MICM (morphology, immunology, cytogenetics and molecular biology) characteristics of a case of acute myelomonocytic leukemia M 4C . @*Methods@#The medical history data of the case of M 4C admitted to our hospital was reviewed. The results of bone marrow cell morphology, cytochemical stains, bone marrow biopsy, immunophenotype, cytogenetics, molecular test and NGS (next-generation sequencing) of the case were analyzed. @*Results@#The bone marrow smear showed markedly active proliferation of bone marrow cells in which the myelomonocytic cells accounted for 85.6%. Cytochemical stains showed peroxidase (POX) stain partially and weakly positive; specific esterase AS-DCE partially positive; non-specific esterase α-NBE partially positive and smothered by sodium fluoride; non-specific esterase AS-DAE partially positive and smothered by sodium fluoride. Bone marrow biopsy showed hyperproliferative cells and diffused hyperplasia of blasts. Immunophenotype analysis showed that the abnormal cell population was positive for CD11B, CD64, CD56, cMPO, CD33, CD41, CD61, CD38 and CD58, but negative for CD13, CD34, CD117, CD7, CD123, HLA-DR, CD10, CD19, CD20, CD2, CD14, CD235, CD15, CD303, CD304, CD25, cCD79a, cCD3, cCD22, CD1a and TDT. Cytogenetic analysis showed 47, XY, t(9;11) (p22;q23),+mar. The molecular test for leukemia showed MLLT3/KMT2A gene rearrangement. NGS showed NRAS and TET2 mutation. The case was finally diagnosed as AML (acute myelomonocytic leukemia) M 4C with t(9;11)(p22;q23), MLLT3-KMT2A. @*Conclusion@#Leukemia M 4C may show the characteristics of both granulocytes and monocytes with complex morphological features. The combined examination of MICM should be necessary for the diagnosis of M 4C with great significance.
ABSTRACT
Objective To investigate the clinical efficacy and prognostic factors for M4/M5subtypes in chil-dren with acute myeloid leukemia(AML).Methods A retrospective analysis of the clinical data of M4/M5subtypes in Shanghai Children′s Hospital Affiliated to Shanghai Jiaotong University,from January 2009 to December 2014 was carried out.The long-term efficacy,prognosis and relapse factors were analyzed.Results The clinical data of 46 ca-ses were collected,among which 38 cases were treated with more than 2 courses,including 22 male,16 female,19 cases M4and 19 cases M5.The median age was 5 years.5-year overall survival(OS)rate and 5-year event-free survival (EFS)rate were(57.7 ± 9.3)% and(47.2 ± 8.9)%,and 5-year EFS of M4and M5were(52.4 ± 12.7)% and (45.4 ± 11. 9)%. Compared with the international risk stratification:5-year EFS rate of favorable-risk, intermediate-risk and poor-risk were(77.2 ± 12.4)%,(49.5 ± 14.9)% and(25.0 ± 19.8)%(χ2=6.305,P=0.043).Single factor analysis showed that extramedullary infiltration(χ2=4.828,P=0.028),Chromosome karyotype (χ2=10.178,P=0.017),the eighth day assessment(χ2=5.382,P=0.020)and course of treatment(χ2=4.771, P=0.029)were prognostic factors;multivariate analysis showed extramedullary infiltration(HR =5.323,95%CI:1.620-17.490,P=0.006)and less-than-6 courses of treatment(HR=6.186,95%CI:1.726-22.176,P=0.005)were the independent risk factors of affecting survival.Conclusions (1)Strengthening treatment and ade-quate courses of treatment are the critical to improve the overall curative effect in children with M4/M5subtypes.(2) Extramedullary infiltration was the risk factor for survival and recurrence in M4/M5subtypes.(3)It is suggested that the children who have the initial symptoms and molecular biology with poor prognostic factors choose hematopoietic stem cell transplantation as early as possible.
ABSTRACT
JUSTIFICATIVA E OBJETIVOS: O acidente vascular encefálico (AVE) hemorrágico em pacientes jovens é potencialmente grave. Dentre as causas hematológicas, destaca-se a leucemia mieloide aguda, representada na maioria dos casos pela leucemia promielocítica aguda (M3), sendo escassos os relatos de AVE hemorrágicos como apresentação inicial em pacientes com leucemiamielo monocítica aguda (M4). O objetivo deste estudo foi relatar um caso de AVE hemorrágico como apresentação inicial de leucemia mielomonocítica em paciente jovem e discutir seus aspectos clínicos, evolutivos e terapêuticos. RELATO DO CASO: Paciente do sexo masculino, 19 anos,que apresentou diagnóstico de leucemia mielomonocítica aguda (M4) com hemorragia intraparenquimatosa cerebral, embora os níveis plaquetários fossem de 56.000/mm3. Foi submetido à drenagem do hematoma intraparenquimatoso. Recebeu tratamento quimioterápico com citarabina e idarrubicina em doses convencionais, evoluindo com distúrbios metabólicos, hidroeletrolíticose óbito no quinto dia de internação. CONCLUSÃO: A leucemia mieloide aguda deve fazer parte dos diagnósticos diferenciais da provável causa de AVE hemorrágico em jovens, inclusive naqueles com níveis plaquetários considerados relativamente "seguros", uma vez que este não é o único fator causal de sangramento.
BACKGROUND AND OBJECTIVES: The hemorrhagic stroke in young patients is potentially serious. Among the hematological causes, we highlight the acute myeloid leukemia, represented in most cases by acute promyelocytic leukemia, being few reports of hemorrhagic stroke as initial presentation in patients with acute myelomonocytic leukemia (M4). The objective of this study was to report a case of hemorrhagic stroke as the initial presentation of myelomonocytic leukemia in a young patient and discuss its clinical, evaluative and therapeutic aspects. CASE REPORT : Male patient, 19 years-old who had as initial presentation of acute myelomonocytic leukemia an intraparenchymal cerebral hemorrhage, with platelet levels of 56.000/mm3. The patient underwent drainage of hematomas. He received chemotherapy with idarubicin and cytarabine in conventional doses, evolving with metabolic disorders, acute renal failure and death on the fifth day of hospitalization. CONCLUSION : Acute myeloid leukemia should be part of the differential diagnosis of the probable cause of hemorrhagic strokein young patients, including those with platelet levels considered relatively "safe", since this is not the only causative factor forbleeding.
Subject(s)
Humans , Male , Adult , Leukemia, Myeloid, Acute , Leukemia, Myelomonocytic, Acute , Stroke , ThrombocytopeniaABSTRACT
La coexistencia de enfermedades mieloproliferativas y linfoproliferativas en el mismo paciente no es común. La mayoría de los casos corresponden a pacientes que desarrollan leucemia aguda durante el curso evolutivo de una leucemia linfática crónica tratada con drogas quimioterápicas. Se presenta un caso de leucemia mielomonocítica aguda y leucemia linfática crónica B diagnosticadas simultáneamente en un paciente en el cual, el análisis por citometría de flujo utilizando un amplio panel de anticuerpos monoclonales, permitió identificar las diferentes poblaciones patológicas y determinar su inmunofenotipo característico. Una revisión de la bibliografía muestra solamente la descripción de casos aislados sin encontrar datos sobre la incidencia de esta asociación. Destacamos la utilidad de la técnica de citometría de flujo para identificar las células anormales que nos llevan al diagnóstico de estas dos enfermedades.
The coexistence of acute myeloid leukemia and chronic lymphocytic leukemia in the same patient is rare. The majority of the cases correspond to patients that developed acute leukemia during the evolutionary course of a chronic lymphatic leukemia following treatment with chemotherapy drugs. We report a case of acute myelomonocytic leukemia concurrent with untreated B-cell chronic lymphocytic leukemia in which the use of flow cytometry analysis with a large panel of monoclonal antibodies, allowed the demonstration of different pathological populations and determine immunophenotyping patterns. Published cases of simultaneous chronic lymphocytic leukemia and acute leukemia are reviewed. The use of multiparametric flow cytometry to differentiate the populations demonstrates the utility of this technology in the diagnosis of these hematological malignancies.
Subject(s)
Aged , Humans , Male , Antibodies, Monoclonal/analysis , B-Lymphocytes/pathology , Leukemia, Lymphocytic, Chronic, B-Cell/pathology , Leukemia, Myelomonocytic, Acute/pathology , Neoplasms, Multiple Primary/pathology , B-Lymphocytes/immunology , Clone Cells , Flow Cytometry , Immunophenotyping , Leukemia, Lymphocytic, Chronic, B-Cell/immunology , Leukemia, Myelomonocytic, Acute/immunology , Neoplasms, Multiple Primary/immunologyABSTRACT
Lineage switch from acute lymphoblastic leukemia (ALL) to acute myeloid leukemia (AML) is very rare. We report a case of a 9 yr-old ALL patient relapsed as acute myelomonocytic leukemia. At the initial diagnosis, the blast cell morphology and immunophenotype were consistent with the diagnosis of typical ALL (L1 subtype according to FAB classification). The BCR-ABL fusion gene was not found by reverse transcription-PCR. Complete remission (CR) was achieved after induction and consolidation chemotherapy (Children's Cancer Study Group 1891 protocol, CCG1891). Nine months, which is a very short time compared with other cases in the literatures, after the diagnosis of ALL, she relapsed with completely different blasts (typical AML, M4 according to FAB classification) in morphology, cytochemistry, and immunophenotyping. The karyotype has changed from 56,XY,+X,+Y,+Y,+4,+8,+10, +14,+17,-20,+21,+21,+21[6]/57,idem,+Y[19] to 46,XY,t(8;16)(p11.2;p13.1)[19]/46,XY[1], showing unrelated chromosomal abnormality to the karyotype at the initial diagnosis. Moreover, both findings were quite specific for each common cell ALL and acute myelomonocytic leukemia. These findings support that this case is completely different leukemic clones occurred at each leukemic expression. The treatment with AML 2000 protocol chemotherapy failed, and he underwent the chemotherapy with the combination of high dose cytarabine and mitoxantrone and has been in CR state for 21 months, until now.
Subject(s)
Child , Humans , Male , Cell Lineage , Cell Transformation, Neoplastic , Karyotyping , Leukemia, Myeloid, Acute/diagnosis , Precursor Cell Lymphoblastic Leukemia-Lymphoma/diagnosisABSTRACT
An 83-year-old man with evidence of multiple myelome was admitted to the hospital with a history of weakness. His serum protein immunoelectrophoresis demonstrated monoclonal IgA lambda and bone marrow aspiration revealed plasma cells 20%. Two months alter, hematological examinations showed erythrocytosis, luecocytosis and thrombocytosis. The bone marrow showed hypercellular with increased plasma cells and monoblasts. Serum vitamin B12 and vitamin B12 binding proteins confirmed the diagnosis of polycythemia vera. After treatment with Busulfan, Prednisolone and Endoxan for 6 months, his blood examination revealed increased monocytes, metamyelocytes and myelocytes. Bone marrow examination confirmed the diagnosis of acute myelomonocytic leukemia with plasma cells. This patient is an example of the simultaneous occurrence of lympoproliferative and myeoproliferative disorder.