ABSTRACT
No abstract available.
Subject(s)
Adult , Humans , Male , Middle Aged , Bone Marrow/pathology , DNA Mutational Analysis , Leukemia, Myelomonocytic, Chronic/diagnosis , Leukemia, Neutrophilic, Chronic/diagnosis , Leukocyte Disorders/diagnosis , Mutation , Prognosis , Receptors, Colony-Stimulating Factor/genetics , Recurrence , Stem Cell Transplantation , Transplantation, HomologousABSTRACT
La leucemia mielomonocítica crónica es un desorden clonal de las células progenitoras hematopoyéticas clasificado como neoplasia mielodisplásica mieloproliferativa. Se caracteriza por la presencia de monocitosis absoluta y persistente en la sangre periférica (> 1.0 x 10 9/L) con la presencia de displasia celular y mieloproliferación en la médula ósea. Los pacientes presentan síntomas relacionados a las citopenias y al estado de hipercatabolismo y al examen físico se encuentra esplenomegalia. El diagnóstico se establece con la combinación del examen de la lámina de sangre periférica, el aspirado medular y la biopsia de médula ósea, el inmunofenotipaje y el estudio molecular de las anomalías que se presentan. El pronóstico de la enfermedad es malo. El tratamiento de elección es el trasplante alogénico de progenitores hematopoyéticos con régimen de acondicionamiento de toxicidad reducida. En alrededor del 30 por ciento de los pacientes la enfermedad se transforma en una leucemia mieloide aguda...
Chronic myelomonocytic leukemia is a clonal hematopoietic stem cell disorder classified as a myelodysplastic/myeloproliferative neoplasm. It is characterized by absolute monocytosis (>1.0 x 10(9)/ L) in the peripheral blood that persists for at least 3 months, with dysplastic and myeloproliferation in the bone marrow. Patients may show symptoms related to cytopenias and hypercatabolic state with splenomegaly. The diagnosis is established by combination of complete blood count, peripheral blood smear, bone marrow aspirate, bone marrow biopsy, immunophenotypic profile and study of molecular abnormalities. The prognosis is bad. The treatment of election is the hematopoietic allogeneic stem cell transplantation with regimen of reduced toxicity. In around 30 percent of these patients the disease transforms to acute myeloid leukemia...
Subject(s)
Humans , Leukemia, Myelomonocytic, Chronic/diagnosis , Leukemia, Myelomonocytic, Chronic/physiopathologyABSTRACT
La pielosis es una rara entidad, de teología poco clara: tóxica, infecciosa relacionada a inmunodepresión (enfermedades consuntivas) inflamatoria o vascular, caracterizada por la presencia de espacios quísticos múltiples, con secuestro hemático, que hasta puede llegar a producir trastornos de la hemostasia, induciendo una extrema fragilidad y por ende la ruptuta facial o espontánea del órgano, a veces sin aumentar el volúmen del mismo. Mucho más rara es su asociación con la leucemia mioelomonocítica crónica (LMMC) sindrome mieloproliferativo crónico de diagnóstico controvertido. Habiendo estudiado en detalle de médula ósea, el baso y la citogenética, concluimos que se trata de una verdadera mieloproloiferación. Sugerimos además un estricto control clínico-hematológico y por imágenes para evaluar adecuadamente a aquellas patologías que se acompañan de grandes esplenomegalias en algún momento de su evolución
Subject(s)
Humans , Splenic Rupture , Leukemia, Myelomonocytic, Chronic/diagnosis , Leukemia, Myelomonocytic, Chronic/therapyABSTRACT
El objetivo del presente reporte es la descripción de un paciente de la Clínica Pediátrica "B" del hospital "Pereira Rossell", portador de la asociación: Neurofibromatosis de Von Recklinghausen (NF); Xantogranulomatosis Cutánea Juvenil (XCJ) y Leucemia Mielomonocítica Crónica Juvenil (LMMCJ)
Subject(s)
Humans , Male , Child, Preschool , Leukemia, Myelomonocytic, Chronic , Neurofibromatoses , Xanthogranuloma, Juvenile , Leukemia, Myelogenous, Chronic, BCR-ABL Positive/drug therapy , Leukemia, Myelomonocytic, Chronic/complications , Leukemia, Myelomonocytic, Chronic/diagnosis , Neurofibromatoses/complications , Neurofibromatoses/diagnosis , Neurofibromatoses/genetics , Podophyllotoxin/therapeutic use , Prednisone/therapeutic use , Splenectomy , Xanthogranuloma, Juvenile/complications , Xanthogranuloma, Juvenile/pathologyABSTRACT
Fourteen consecutive cases of chronic myelomonocytic leukaemia aged 6 to 73 (mean 40.5) years were reviewed to define the natural history of the disease and the risk of acute transformation. The common presenting features included anaemia, fever, purpura, and bleeding tendencies. Abnormal karyotypes were seen in 4 of 6 patients subjected to cytogenetic analysis. Low dose cytosine arabinoside achieved complete remission in two and partial remission in one, of the four patients treated with this modality. The mean survival was 5.6 (range 2-12) months and two patients) evolved to acute myeloid leukaemia. The long term survival with the present form of therapy in chronic myelomonocytic leukaemia is poor.
Subject(s)
Adolescent , Adult , Aged , Antineoplastic Agents/therapeutic use , Biopsy, Needle , Blood Cell Count , Bone Marrow/pathology , Child , Female , Fetal Hemoglobin/analysis , Humans , India , Leukemia, Myelomonocytic, Chronic/diagnosis , Male , Middle Aged , Survival RateABSTRACT
Myelodysplasia, characterized by varied reductions of peripheral blood elements with normal or hypercellular bone marrow, is reltively frequent among older patients and may evolve to acute leukemia. We reviewed findings in 35 patients whon, according to the FAB classification were distributed as follows: simple refractory anemia (RA) 34%, sideroblastic refractory anemia (SRA) 14%, refractory anemia with excess blast forms (RAEB) 31%, chromic myelomonocytic leukemia (CMML) 12% and refractory anemia eith excess blast forms in transformation (RAEBT 9%). Cytogenetic studies performed in 16 patients were abnormal in 5(31%), al among patients with poor prognosis forms of the disorder. All patients had anemia; thrombopenia and neutropenia were more frequent in subtypes RAEB, CMML and RAEBT). Mean survival rate was 30 months, significantly greater in RA and SRA comapred to the other groups. Infections and development of acute leukemia were the causes of death