A rare combination: chylous polyserositis and autoimmune myelofibrosis as a presentation of systemic lupus erythematosus.

Chylous polyserositis and autoimmune myelofibrosis occurring concomitantly inn a case of SLE are a rare phenomenon. We here report a case of a 38-year-old woman who was admitted with a history of cough and shortness of breath for 1½ months along with fever and abdominal distension for 1 month. She also had arthralgias, weight loss and pancytopenia. She was diagnosed as a case of SLE with Chylous polyserositis and autoimmune myelofibrosis. She was started on steroids and immunosuppressive therapy, to which she responded. To summarize, this is the first case report where chylous polyserositis and pancytopenia due to autoimmune myelofibrosis occurred which was responsive to steroids and immunosuppressive therapy.

Gastrointestinal carcinoid tumor and chylous ascites, a rare association with a poor prognosis. A case report.

Gastrointestinal carcinoid is a rare tumor. The association of this tumor with chylous ascites is uncommon. A review of the English-language literature carried out in 2002 identified only 15 cases. We report a case of chylous ascites, gastrointestinal carcinoid tumor and elevated blood levels of CA-125 in a patient who did not respond to chemotherapy.

The lymphatics in the pathophysiology of thoracic and abdominal surgical pathology: immunological consequences and the unexpected role of microsurgery.

The authors report their experience in the diagnosis and treatment of lymphatic and chylous disorders in the thoracic and abdominal areas. Sixteen patients (10 adults, 6 children) affected by primary chylous ascites with associated syndromes and consequent immunological incompetence were studied. Diagnostic investigations included abdominal sonography scans, lymphoscintigraphy, and lymphography combined with computed tomography and laparoscopy. Surgical treatment included laparoscopy, drainage of ascites and/or the chylothorax, treatment of abdominal and retroperitoneal chylous leaks, exeresis of lymphodysplastic tissues, ligation of incompetent lymph vessels also by CO(2) LASER, and chylo-venous and lympho-venous microsurgical shunts. Eleven patients did not have a relapse of the ascites and four patients had a persistence of a small quantity of ascites with no protein imbalance. All patients had an improvement of their immunocompetence. Median follow-up was 5 years. We demonstrated that the use of microsurgery is remarkably advantageous for performing a causal treatment of the dysfunction.

Primary chylopericardium with pulmonary shadow and large granular lymphocytosis: a case report.

A 79-year-old man with primary chylopericardium associated with large granular lymphocytosis was followed for more than 26 years. Except for development of dyspnea on exertion during the past four years and more recently cough with sputum production, he has remained largely asymptomatic over this interval. Based on detailed examinations of cellular and humoral immunity, we speculate that increased natural killer cell activity and an increased number of large granular lymphocytes circulating in his peripheral blood represent a reactive response to production of various cytokines secondary to persistent loss (? chyloptysis) and sequestration of central lymph.

Lymphocyte subpopulations in children with abnormal lymphatic circulation.

Peripheral blood lymphocyte subsets were enumerated in five children with abnormal lymphatic circulation (three with lymphangiectasia, one with chylothorax, and one child with chyloperitoneum). All patients were lymphopenic. The percentage and absolute number of blood T-lymphocytes (CD3) were low in two children and normal in the other children. The percentage and absolute number of helper/inducer lymphocytes (CD4) were markedly reduced in all patients. The percentage of suppressor/cytotoxic lymphocytes (CD8) was normal or elevated in all children, and the absolute number of CD8 cells was normal in three patients. The CD4/CD8 ratio was reversed in all patients. In the two patients tested, the proliferative response of peripheral blood mononuclear cells to mitogens was reduced. T-lymphocyte subsets were measured in the pleural or peritoneal fluid of three patients, and the CD4/CD8 ratio was normal or increased. In each child, the CD4/CD8 ratio in the lymphatic fluids was markedly higher than the CD4/CD8 ratio of the blood (4.0/0.45, 1.75/0.95, and 1.3/0.85). The reversed CD4/CD8 ratio in the blood in cases of chronic loss of chyle may be due to either selective transport of CD4 lymphocytes into the lymphatic fluids or a shorter half-life of CD8 compared to CD4 lymphocytes. This finding may in part explain the abnormal cellular immunity previously observed in patients with lymphangiectasia.

[A case of Nonne-Milroy syndrome associated with chyloabdomen. Immunologic study]. / Un caso de síndrome de None Milroy asociado a quiloabdomen. Estudio inmunológico

A case of None Milroy syndrome with chiloabdomen in a 7 year old girl is reported. The most important features are the immunological alterations (depressed delayed immunity in the affected extremity and decreased level of IgA). A review of the literature is made.