Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0079731 (B-cell lymphoma)
 16,671 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Job's or hyper immunoglobulin E recurrent infection syndrome (Hyper-IgE syndrome) is a rare, often inherited multisystem disorder, characterized by cutaneous abscesses, pneumonia, elevated IgE levels and skeletal defects. We report a case of a 22-year-old man with Job's syndrome who presented with back pain. He was found to have diffuse large B-cell lymphoma involving his second lumbar vertebrae and spleen. Treatment with dose-adjusted EPOCH-rituximab (DA-EPOCH-R) chemotherapy achieved a complete remission after 4 cycles. A review of reported cases of lymphoma in Job's syndrome indicates an increase in relative risk of 259 (95% confidence interval 102, 416). The cause of the increased risk has yet to be defined but has similarities to a pathogenetic model of AIDS related lymphoma. In previous reports of lymphoma in Job's syndrome, patients presented with extranodal disease and had poor outcomes. With appropriate chemotherapy and hematological support, lymphoma associated with Job's syndrome can achieve complete remission.
 ...
PMID:Non-Hodgkin's lymphoma in Job's syndrome: a case report and literature review. 1562 72

Job's syndrome or hyperimmunoglobulin E syndrome (HIES) is a rare primary immunodeficiency. Characterized by recurrent pulmonary and skin infections with elevated IgE serum level, this syndrome has been considered a risk factor for lymphoma. Although the majority of cases were diagnosed in childhood, there are still a few cases that were diagnosed in adulthood. It is necessary to recognize these two conditions since the treatment for lymphoma patients with HIES should be adjusted and needs a thorough care. Here we present a case of diffuse large B cell lymphoma in a patient with symptoms of HIES diagnosed during chemotherapy.
 ...
PMID:Diffuse Large B-Cell Lymphoma and Job's Syndrome: A Case Report. 3295 95