Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0243026 (sepsis)
 52,417 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 63-year-old man was admitted for Campylobacter fetus sepsis and immunodeficiency syndrome with thymoma (Good's syndrome). Serological examination demonstrated hypoimmunoglobulinemia. Analysis of lymphocyte subsets in the peripheral blood and bone marrow showed marked decreases in the proportion of cells bearing B cell markers. However, there were no abnormalities of cellular immunity. This is a rare case of Good's syndrome in Japan in which the pathogenic mechanism involved a block in the early stage of B cell differentiation. Moreover, this is the first case ever reported of Campylobacter fetus sepsis associated with Good's syndrome.
 ...
PMID:Good's syndrome with a block in the early stage of B cell differentiation and complicated by Campylobacter fetus sepsis. 780 19

A 63-year-old man was admitted to our hospital for treatment of hypogammaglobulinemia with thymoma (Good's syndrome). Tests for immunological function showed an abnormality in humoral immunity with decreases in the proportion of cells bearing B-cell markers in the peripheral blood and bone marrow. The patient was found to have Campylobacter fetus sepsis caused by the hypogammaglobulinemia due to humoral immunodeficiency, and he was given gamma-globulin supplement. Thymectomy was performed due to enlargement of the thymoma after 4 years of follow-up and the pathological diagnosis was thymoma of the non-encapsulated, epithelial spindle cell type. Although there was no recurrence of thymoma, the hypogammaglobulinemia remained unchanged and the patient continued to suffer from repeated infections. Thus, we describe the case of a patient with Good's syndrome associated with independent humoral immunodeficiency in whom the effect of thymectomy for hypogammaglobulinemia was negative. In this respect, thymectomy was only beneficial when the thymoma proliferated and seemed to be more threatening than the hypogammaglobulinemia for the patient.
 ...
PMID:Thymoma associated with hypogammaglobulinemia (Good's syndrome): report of a case. 1199 14

This report presents the case of a 68-year-old female patient previously diagnosed with thymoma by her local doctor. She was referred to our hospital for surgery, and the thymoma was removed and diagnosed as a World Health Organization (WHO) classification type AB thymoma. After surgery, she experienced general malaise, a loss of appetite, and weight loss, so she visited our hospital in May 2019. A blood test showed hypogammaglobulinemia and low B lymphocytes. A bone marrow examination revealed no morphological abnormalities. Flow cytometric analysis indicated a marked decrease in both the B cell-related surface markers CD19 and CD20 and the T cell-related surface marker CD4, and the CD4/CD8 ratio was also low. She was diagnosed with Good's syndrome, and immunoglobulin replacement therapy was administered. She subsequently developed hemophagocytic lymphohistiocytosis (HLH) due to infection and was treated according to the HLH2004 protocol, but she finally succumbed to multiple organ damage as a result of sepsis. Given that Good's syndrome is associated with both humoral and cellular immune dysfunctions, affected patients tend to develop severe infections and have a poor prognosis. In such cases, early detection, regular immunoglobulin replacement therapy, and infection prevention therapies are important.
 ...
PMID:[Good's syndrome developing hemophagocytic lymphohistiocytosis following thymectomy]. 3222 89

Background: The role of thymus in the immune cascade of the body is still under active clinical scrutiny. Meanwhile, there is an unravelling of myriad presentations of thymoma with effects on various organ systems. These effects arise both due to autoimmunity or a paraneoplastic process secondary to thymoma. However, an occasional patient can be a conundrum and may give no clue regarding the pathogenesis and etiology of its clinical profile. Material and Methods: We present an enigmatic case of a 30-year-old male presenting with superficial fungal infections followed by multiaxial neurological involvement against a background of thymoma. Extensive workup for bacterial, viral, autoimmune and paraneoplastic aetiologies were negative. Thymectomy confirmed thymoma (Type AB) but failed to ameliorate the progression of symptoms. He developed recurrent episodes of severe sepsis which remitted with sensitive antibiotics only to reappear again. Immunological profile showed complete absence of peripheral circulating B-cells with reversal of CD4/CD8 ratio, findings compatible with Good's syndrome. Radiological findings showed only progressive brain atrophy without any hyperintensity. He was also treated with intravenous immunoglobulins but failed to respond with the same. Results: Autopsy revealed features of panencephalitis (neuronophagia, neuronal loss, perivascular lymphocytic cuffing, and microglial nodules) with virus particles detected ultrastructurally. While the distinction between seronegative autoimmune encephalitis versus a viral encephalomyelitis often blurs histologically, we speculated viral encephalomyelitis to have happened towards the latter part of his long illness. To our knowledge, this is a case of Good's syndrome presenting with seronegative autoimmune panencephalitis superimposed with a viral infection.
 ...
PMID:Seronegative panencephalitis complicated by viral encephalomyelitis in a case of Good's syndrome-a neuropathological report. 3328 Apr 65