RESUMO
BACKGROUND: Multiple symmetric lipomatosis (MSL) is a relatively uncommon entity of unknown etiology characterized by symmetrically subcutaneous accumulation of nonencapsulated adipose tissue. Approximately 200 to 300 cases have been published. OBJECTIVES: The aims of this article are to report the case of a 58-year-old Brazilian patient with MSL and provide a comprehensive overview of the current concepts concerning this disease. METHODS: Our search yielded 28 articles on MSL, including case reports and reviews of the literature. RESULTS: MSL predominantly affects Mediterranean males with a history of chronic alcohol abuse. It is usually asymptomatic and may be associated with diabetes mellitus, hyperlipidemia, hyperuricemia, macrocytic anemia, and oral cancer. Surgical resection is the best treatment option. CONCLUSION: The case reported is a classic presentation of MSL; however, it is particularly uncommon owing to the association with immune thrombocytopenic purpura. This association has been described only once in the medical literature.
Assuntos
Lipomatose Simétrica Múltipla/diagnóstico , Humanos , Lipomatose Simétrica Múltipla/complicações , Lipomatose Simétrica Múltipla/imunologia , Masculino , Pessoa de Meia-Idade , Púrpura Trombocitopênica/complicações , Púrpura Trombocitopênica/imunologia , Fatores de Risco , Tomografia Computadorizada por Raios XRESUMO
Evaluar la eficacia y tolerancia de la Inmunoglobulina G Intravenosa (IgG IV) en niños con púrpura Trombocitópenica Inmune (PTI). Estudio prospectivo, longitudinal y descriptivo. Se trataron 25 niños diagnosticados con P.T.I. en la consulta de Hematología del Hospital general San Carlos Dr. Egor Nucete, San Carlos estado Cojedes (Enero 2.000-Diciembre 2.003) con inmunoglubina endovenosa. Al ingreso, el recuento plaquetario fue menos de 20 x 109/L. Veinte pacientes (80%) alcanzaron más de 50 x 109/L. Plaquetas en las primeras 72 horas de terapia. A los 21 días, 18 pacientes (72%) manifestaron una respuesta excelente (mayor de 150 x 109/L. plaquetas). Seis pacientes (24%) evolucionaron hacia la cronicidad. Ningún paciente presentó hemorragia intracraneana. No se observaron reacciones adversas al medicamento. Se demuestra una buena eficacia y tolerancia con la administración de la Ig G IV a la dosis de 0.4 g/ Kg/ día, por 3 días.
Assuntos
Imunoglobulinas Intravenosas/administração & dosagem , Imunoglobulinas Intravenosas , Imunoglobulinas Intravenosas/uso terapêutico , Plaquetas/imunologia , Púrpura Trombocitopênica/imunologia , Púrpura Trombocitopênica/terapia , Resultado do Tratamento , Alergia e Imunologia , VenezuelaRESUMO
CONTEXTO: Púrpura trombocitopênica neonatal aloimune (PTNA) é uma doença neonatal caracterizada por aloimunização materna contra as plaquetas fetais, que apresentam antígenos herdados do pai. Podem ocorrer hemorragias cerebrais, levando à morte ou a anomalias neurológicas permanentes. RELATO DE CASO: Mulher saudável, de 30 anos, deu à luz, por parto cesariano na 36ª semana de gestação, seu primeiro filho. Com 10 horas de vida, o recém-nascido apresentou petéquias e contagem de 8 x 103 plaquetas/µl no sangue periférico; foi medicado com imunoglobulina e recebeu alta após 18 dias de internação, com 100 x 103 plaquetas/µl. A causa da trombocitopenia não foi elucidada na época. Um ano depois, a criança morreu de neuroblastoma. Como os pais desejavam outro filho, foram encaminhados para investigação da trombocitopenia. Genotipagem plaquetária e pesquisa de anticorpos antiplaquetários foram realizadas, mostrando total falta de concordância entre os sistemas HPA-1 do pai (HPA-1a1a) e da mãe (HPA-1b1b) e anticorpos anti-HPA-1a no soro da mãe. Concluímos que o primeiro bebê nasceu com PTNA. Por isso, na segunda gravidez, a mãe foi tratada com diversas infusões de imunoglobulina intravenosa. Foi realizado cuidadoso monitoramento por ultra-som, com resultados normais para mãe e feto durante a gravidez. O segundo bebê nasceu por cesárea às 39 semanas, apresentando 92 x 103 plaquetas/µl seis horas após o nascimento. As plaquetas do recém-nascido foram genotipadas como HPA-1a1b e o soro da mãe novamente mostrou anticorpos anti-HPA-1a. Não houve hemorragia. A terapia de infusão de imunoglobulina foi efetiva na prevenção da PTNA no segundo filho.
Assuntos
Humanos , Masculino , Feminino , Gravidez , Recém-Nascido , Adulto , Antígenos de Plaquetas Humanas/genética , Imunoglobulinas Intravenosas/uso terapêutico , Doenças do Recém-Nascido/imunologia , Complicações Hematológicas na Gravidez/imunologia , Púrpura Trombocitopênica/congênito , Testes Genéticos , Antígenos de Plaquetas Humanas/imunologia , Evolução Fatal , Genótipo , Doenças do Recém-Nascido/prevenção & controle , Isoanticorpos/análise , Isoanticorpos/imunologia , Neuroblastoma/etiologia , Contagem de Plaquetas , Complicações Hematológicas na Gravidez/prevenção & controle , Púrpura Trombocitopênica/imunologia , Púrpura Trombocitopênica/prevenção & controleRESUMO
CONTEXT: Neonatal alloimmune thrombocytopenic purpura (NAITP) is a neonatal disorder characterized by maternal alloimmunization against fetal platelet antigens inherited from the father. Intracranial hemorrhage leading to death or permanent neurological disability may occur in the fetus. CASE REPORT: A healthy 30-year-old woman gave birth to her first baby by cesarean after an uneventful 36-week pregnancy. Ten hours after birth, the infant presented severe petechiae, with platelet count of 8 x 10(3)/microl. The mother's platelet count was normal (180 x 10(3)/microl). The infant re ceived intravenous immunoglobulin and was discharged 18 days later, with platelet count of 100 x 10(3)/microl. The cause of thrombocytopenia was not elucidated at that time. One year later, the infant died of neuroblastoma. Since the parents wanted another child, they were referred for investigation of this thrombocytopenia. Platelet genotyping and platelet antibody screening were performed, showing total HPA-1 system mismatch between mother (HPA-1b1b) and father (HPA-1a1a), with anti-HPA-1a antibodies in the mother's serum. We concluded that the first baby was born with NAITP. Thus, in the second pregnancy, the mother was treated with several infusions of intravenous immunoglobulin. Careful ultrasound monitoring was performed, with normal results for mother and fetus throughout the pregnancy. The second baby was born by cesarean at 39 weeks, presenting 92 x 10(3) platelets/microl six hours after birth. The baby's platelets were genotyped as HPA-1a1b and the mother's serum again showed anti-HPA-1a antibodies. No clinical bleeding was observed. Intravenous immunoglobulin therapy was an effective treatment for preventing NAITP in the second baby.
Assuntos
Antígenos de Plaquetas Humanas/genética , Imunoglobulinas Intravenosas/uso terapêutico , Doenças do Recém-Nascido/prevenção & controle , Púrpura Trombocitopênica/congênito , Adulto , Antígenos de Plaquetas Humanas/imunologia , Evolução Fatal , Feminino , Testes Genéticos , Genótipo , Humanos , Recém-Nascido , Doenças do Recém-Nascido/diagnóstico , Doenças do Recém-Nascido/imunologia , Integrina beta3 , Isoanticorpos/análise , Isoanticorpos/imunologia , Masculino , Neuroblastoma/etiologia , Contagem de Plaquetas , Gravidez , Púrpura Trombocitopênica/imunologia , Púrpura Trombocitopênica/prevenção & controleRESUMO
BACKGROUND: This is an evaluation of the treatment of 63 patients with chronic immune thrombocytopenic purpura (54 splenectomized and nine nonsplenectomized) with weekly doses of anti-D (IgG)-coated red blood cells (RBCs). METHODS: All patients were given one 5-15 microg/kg/dose of intravenous (i.v.) anti-D (IgG)-coated RBCs per week (average of 300 microg/dose/week) for a median 3-month period (3-6 months). Treatment modality was evaluated on a weekly basis by platelet counts, measuring of hemoglobin levels, and performance of Coombs tests. RESULTS: All patients presented a clinical response. Fifty-two patients (82.5%) increased their platelet count (PC) and 45 (69.8%) increased their PC >50 x 10(9)/L. In 34 cases, response was sustained. Six of nine nonsplenectomized patients (67%) increased PC, thus avoiding splenectomy; four patients attained a stable complete response (CR). Similar platelet responses were observed in homozygous and heterozygous Rh (D)-positive patients (Rh/Hr phenotypes). Currently, after >10 years, 43 patients present a now permanent complete response with platelet count >50 x 10(9)/L. Ten patients subsequently decreased their platelet count, although they were able to attain CR after receiving six doses of anti-D (IgG)-coated RBCs. CONCLUSIONS: Based on our study of Fc receptor blockade treatment with anti-D (IgG)-coated RBCs with the most difficult cases of ITP, which resulted in a 69.8% successful response rate, we concluded that weekly prescription of anti-D (IgG)-coated RBCs is an effective approach to treating chronic refractory ITP.
Assuntos
Transfusão de Eritrócitos , Eritrócitos/imunologia , Imunoglobulina G/imunologia , Púrpura Trombocitopênica/imunologia , Púrpura Trombocitopênica/terapia , Receptores Fc/imunologia , Imunoglobulina rho(D)/imunologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Pré-Escolar , Feminino , Humanos , Imunoglobulina G/química , Masculino , Pessoa de Meia-Idade , Púrpura Trombocitopênica/sangue , Imunoglobulina rho(D)/química , Baço/fisiologia , Fatores de TempoAssuntos
Humanos , Animais , Cães , Coelhos , Ratos , Ensaios Clínicos como Assunto , Hemorragia/mortalidade , Hemorragia/prevenção & controle , Hemostáticos/administração & dosagem , Contagem de Plaquetas , Substitutos Sanguíneos/administração & dosagem , Substitutos Sanguíneos/história , Trombocitopenia/complicações , Trombocitopenia/terapia , Transfusão de Plaquetas/normas , Animais de Laboratório , Aspirina , Biotina/administração & dosagem , Tempo de Sangramento , Púrpura Trombocitopênica/imunologia , Púrpura Trombocitopênica/terapia , Trombastenia/terapia , TromboseAssuntos
Humanos , Animais , Cães , Coelhos , Ratos , Transfusão de Plaquetas/normas , Trombocitopenia/terapia , Trombocitopenia/complicações , Hemorragia/prevenção & controle , Hemorragia/mortalidade , Contagem de Plaquetas/métodos , Hemostáticos/administração & dosagem , Ensaios Clínicos como Assunto/métodos , Substitutos Sanguíneos/história , Substitutos Sanguíneos/administração & dosagem , Tempo de Sangramento , Aspirina , Biotina/administração & dosagem , Trombose , Púrpura Trombocitopênica/imunologia , Púrpura Trombocitopênica/terapia , Trombastenia/terapia , Animais de LaboratórioRESUMO
El objetivo del presente trabajò fuè evaluar la presencia de anticuerpos antiplaquetarios de tipo IgG e IgM en pacientes con enfermedades trombocitopenicas. Fueron estudiados 31 pacientes con el diagnòstico clìnico de purpura trombocitopenica, 23 niños y 8 adultos, provenientes del hospital de clìnicas. Se determinò la presencia de anticuerpos antiplaquetarios de tipo IgG e IGM por el mètodo de Inmunofluorescencia indirecta. Del total de pacientes estudiados, 87 por ciento de los pacientes presentaron anticuerpos antiplaquetarios. De los cuales 36 por ciento fueron anticuerpos de tipo IgG positivo, 1,6 por ciento anticuerpos de tipo IgM positivo y 35 por ciento presentaron ambos anticuerpos, IgG e IgM. este estudio demuestra que el test de Inmunofluorescencia es un mètodo ùtil para la detecciòn de anticuerpos antiplaquetarios que se deberìa utilizar de rutina para el diagnòstico de las trombocitopenias.
Assuntos
Anticorpos/efeitos da radiação , Anticorpos/imunologia , Púrpura Trombocitopênica/diagnóstico , Púrpura Trombocitopênica/enfermagem , Púrpura Trombocitopênica/imunologia , Testes Imunológicos , Técnica Direta de Fluorescência para Anticorpo/enfermagemRESUMO
OBJECTIVE: To examine the effectiveness of cyclic oral high-dose (HD) dexamethasone therapy in pediatric patients with chronic immune thrombocytopenic purpura (ITP), which has been reported to cause complete remission in adults with chronic ITP. STUDY DESIGN: Eleven children with primary chronic ITP, with a median disease duration of 28 months (range, 6 to 120 months), were treated with cycles of HD dexamethasone therapy. RESULTS: Excellent short-term responses (initial platelet counts < or = 50 x 10(9)/L, increasing to > 100 x 10(9)/L within 72 hours of completion of an HD dexamethasone cycle) were observed in 78% of 41 cycles. Long-term effects include one complete response (platelet count > or = 150 x 10(9)/L) and three partial responses (platelet count > or = 50 and < 150 x 10(9)/L) in 11 children followed for 6 or more months after completing cyclic HD dexamethasone therapy. Because side effects were substantial, three children did not complete their sixth treatment cycle. At day 6 of treatment, B lymphocytes were significantly increased (p = 0.005). CONCLUSIONS: Dexamethasone, given orally in high doses, is an effective drug in achieving short-term platelet responses, but it induced long-term remissions in fewer than half of the children with well-established chronic ITP. Its effect on B lymphocytes requires further elucidation. A prospective, controlled study will be needed to establish whether cyclic HD dexamethasone therapy can alter the natural history of children with early chronic ITP and thus avoid splenectomy.
Assuntos
Dexametasona/administração & dosagem , Glucocorticoides/administração & dosagem , Púrpura Trombocitopênica/tratamento farmacológico , Administração Oral , Adolescente , Plaquetas/imunologia , Criança , Pré-Escolar , Doença Crônica , Dexametasona/efeitos adversos , Esquema de Medicação , Feminino , Glucocorticoides/efeitos adversos , Humanos , Imunoglobulinas/sangue , Contagem de Leucócitos/efeitos dos fármacos , Masculino , Contagem de Plaquetas/efeitos dos fármacos , Púrpura Trombocitopênica/sangue , Púrpura Trombocitopênica/imunologia , Indução de RemissãoRESUMO
BACKGROUND: Neonatal alloimmune thrombocytopenia (NAIT) is a result of fetomaternal incompatibility. Platelet destruction is caused by a maternal antibody directed against a fetal platelet antigen inherited from the father and lacking on the mother's platelets. The incidence and features of transplacental alloimmunization depend on the frequency of expression of platelet specific antigens; which are highly variable among different populations. AIM: To determine the prevalence and characteristics of transplacental alloimmunization in a large group of pregnant women in Chile. MATERIAL AND METHODS: We studied 3,041 samples obtained during the third trimester of gestation. In all samples, anti platelet antibodies were screened by ELISA with platelet membranes fixed to a microtiter plate. Positive samples were further studied for antigenic specificity with the monoclonal antibody specific immobilization of platelet antigens (MAIPA) test. RESULTS: Anti platelet antibodies were found in 261 samples (8.5%). The MAIPA test identified 6 samples with antibodies directed against major platelet membrane glycoproteins, 2 anti GPIb, 2 anti GPIIb/IIIa and 2 anti GPIa/IIa. In four cases, anti HLA antibodies coexisted. Two cases corresponded to well defined platelet antigen systems: one anti HPA-1a and one anti HPA-5b. No clinical evidence of thrombocytopenia of the newborn was detected in all these cases with anti GP antibodies. CONCLUSIONS: A prevalence of platelet specific antibodies of 0.2% with only one anti HPA-1a was detected. These findings are in contrast with those of other populations but in accordance with the low frequency of the HPA-1 b/b phenotype in the Chilean population. The very low incidence of platelet specific antibodies and the lack of association with clinical thrombocytopenia in the newborn, do not support the recommendation of routine antenatal screening to all women in Chile.