ABSTRACT
Neste estudo é relatado o quadro clínico de cinco crianças com linfoma não-Hodgkin secundário a imunodeficiência ou imunossupressão: três portadoras do vírus da imunodeficiência humana, uma com imunodeficiência primária e uma após transplante hepático. De acordo com a classificação atual, os tipos histológicos foram: linfoma linfoblástico de células B precursoras (2), linfoma cutâneo de grandes células anaplásico (1), linfoma de células B periféricas, sugestivo de Burkitt (1), e linfoma linfoblástico de células T precursoras (1). Todos os pacientes foram submetidos a quimioterapia, sendo que dois estão em remissão clínica, dois morreram e um continua em tratamento. Após a introdução da terapia anti-retroviral combinada e o aumento dos transplantes de órgãos sólidos ocorre maior risco de neoplasia nesses pacientes. Desse modo, é importante o seguimento desses pacientes para determinar os fatores de risco para o desenvolvimento de neoplasias e definir adequada estratégia de tratamento.
The outcomes of five children with non-Hodgkin's lymphomas associated with immunodeficiency or immunosuppression is reported: three children with HIV, one with primary immunodeficiency and one after liver transplantation. According to the REAL classification, two patients had precursor B-lymphoblastic lymphomas, one had an anaplastic large cell lymphoma, one had a peripheral B-cell neoplasm suggestive of Burkitt's lymphoma, and one had precursor T-lymphoblastic lymphoma. All patients received chemotherapy. Two are in complete remission, two died and one remains under treatment. There has been an increasing awareness of the risk of non-Hodgkin's lymphoma and lymphoproliferative disorders after active antiretroviral therapy and with the expansion of solid organ transplant programs in the pediatric setting. Thus, for these patients it is important to establish risk factors for hematological disorders and determine the optimal and safest treatment.
Subject(s)
Lymphoma, Non-Hodgkin , Therapeutics , B-Lymphocytes , T-Lymphocytes , Child , Lymphoma, B-Cell , Burkitt Lymphoma , Acquired Immunodeficiency Syndrome , HIV , Immunosuppression Therapy , Health Strategies , Lymphoma, Large-Cell, Anaplastic , Drug Therapy , Precursor Cell Lymphoblastic Leukemia-Lymphoma , Lymphoma , Lymphoproliferative Disorders , NeoplasmsABSTRACT
OBJECTIVE: Autoimmune hemolytic anemia is characterized by the production of autoantibodies against erythrocyte membrane antigens. This study was carried out to identify the clinical, immunological and outcome characteristics of autoimmune hemolytic anemia patients treated at the (HC-UFMG) Pediatric Hematology Unit and the Hemocentro de Belo Horizonte. METHODS: We evaluated 17 patients younger than 15 years old admitted from 1988 to 2003 were evaluated. Autoimmune hemolytic anemia diagnosis was based on the presence of acquired hemolysis and confirmed by positive direct Coombs polyspecific test results. Clinical, laboratory, and outcome data were obtained from patient records. RESULTS: The median age at diagnosis was 10.5 months. The direct Coombs polyspecific test was positive in 13 and negative in four patients. Monospecific testing was performed for 14 patients. The most frequent red cell autoantibody was IgG (five patients), followed by IgM in two. Thirteen patients had severe anemia and needed blood transfusions. Underlying diseases were identified in four patients: systemic lupus erythematosus, Hodgkin's lymphoma, autoimmune hepatitis and Langerhans cell histiocytosis. The remaining patients were classified as having primary disease. The median follow-up period was 11 months (5 to 23 months). Three children died, two after splenectomy and one with complications of the underlying disease. CONCLUSION: Autoimmune hemolytic anemia is rare in children and adolescents. Although patients usually respond to corticosteroids and/or immunoglobulin, fatal cases can occur. Prognosis is worse in patients with chronic underlying diseases.
Subject(s)
Humans , Male , Female , Infant , Child, Preschool , Child , Adolescent , Anemia, Hemolytic, Autoimmune/diagnosis , Anemia, Hemolytic, Autoimmune/complications , Anemia, Hemolytic, Autoimmune/therapy , Coombs Test , Follow-Up Studies , Hemolysis , Retrospective Studies , Severity of Illness Index , Splenectomy , TherapeuticsABSTRACT
Objetivo: Este estudo foi realizado com o objetivo de avaliar causas de baixa estatura monossintomática na infância, enfatizando causas de má-absorçäo intestinal, especialmente doença celíaca. Métodos: Foram avaliadas, em um desenho transversal, crianças com estatura abaixodo terceiro percentil ou taxa de crescimento inferior a 5cm/ano. A seguinte propedêutica foi realizada: avaliaçäo hematológica, bioquímica, endocrinológica, idade óssea, pesquisa sorológica de anticorpos antigliadina, gordura fecal, dosagem de cloretos no suor e biópsia jejunal. Resultados: Um total de 51 crianças foi estutado, sendo que a maioria foi incluída no grupo dos variantes da normalidade. Näo foram encontradas portadoras de doença celíaca, mas identificaram-se 4 crianças com provável fibrose cística, assintomáticas em relaçäo ao trato respiratório e gastrintestinal. Conclusäo: Além da doença celíaca, a fibrose cística deve ser incluída no diagnóstico diferencial de baixa estatura na infância...