RESUMO
Renal lymphangiectasia is a rare disorder characterized by dilatation of peripelvic, renal and perirenal lymphatic ducts. The exact etiology is not known. Congenital forms and ac-quired forms have been described. The latter has been attributed to obstruction of draining retro-peritoneal lymphatic ducts caused by either infection, inflammation or any other cause. We des-cribe the rare association of renal lymphangiectasia with chronic myeloid leukemia, which is probably not yet reported in the medical literature.
Assuntos
Nefropatias/etiologia , Leucemia Mielogênica Crônica BCR-ABL Positiva/complicações , Linfangiectasia/etiologia , Contagem de Células Sanguíneas , Humanos , Rim/fisiopatologia , Nefropatias/sangue , Linfangiectasia/sangue , Anormalidades Linfáticas/sangue , Sistema Linfático/fisiopatologia , Masculino , Pessoa de Meia-IdadeRESUMO
Lymphangiectasia is a congenital or acquired disorder characterized by abnormal, dilated lymphatics with a variable age of presentation. We describe a case of lymphangiectasia with intestinal and pulmonary involvement in an adolescent female, who presented with many of the classic features including chylous pleural effusions, lymphopenia, hypogammaglobinemia, and a protein-losing enteropathy. She also presented with recurrent lower gastrointestinal bleeding, which is infrequently described. The patient did not improve with bowel rest and a low-fat medium-chain triglyceride diet and had little improvement with octreotide acetate therapy. However, she had a clinical response to antiplasmin therapy, trans-4-aminothylcyclohexamine carboxylic acid (tranexamic acid) in terms of serum albumin and gastrointestinal bleeding. She continues to have exacerbations of her condition, as well as persistent lymphopenia and chronic pleural effusions.
Assuntos
Antifibrinolíticos/uso terapêutico , Linfangiectasia Intestinal/tratamento farmacológico , Linfangiectasia/tratamento farmacológico , Doenças Torácicas/tratamento farmacológico , alfa 2-Antiplasmina/uso terapêutico , Adolescente , Feminino , Hemorragia Gastrointestinal/tratamento farmacológico , Humanos , Linfangiectasia/sangue , Linfangiectasia/diagnóstico por imagem , Linfangiectasia Intestinal/sangue , Linfangiectasia Intestinal/diagnóstico por imagem , Radiografia Torácica , Albumina Sérica/análise , Doenças Torácicas/sangue , Doenças Torácicas/diagnóstico por imagem , Ácido Tranexâmico/uso terapêutico , Resultado do TratamentoRESUMO
A thrombocytopenic patient with pulmonary hemangiolymphangiectasia proven by biopsy, was studied for platelet sequestration with In-111-labeled autologous platelets. The 24-hour scan showed diffuse increased uptake throughout both lung fields, indicating platelet sequestration by the diffuse pulmonary hemangiolymphangiectasia.
