Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UNIPROT:Q06643 (non-Hodgkin's lymphoma)
 11,307 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

This paper describes a middle-aged patient who developed repeated episodes of swelling of the orofacial tissues after dental treatment. On investigation, C1 inhibitor, C1q, C2, and C4 levels were all markedly reduced, and a diagnosis of acquired C1 inhibitor deficiency was made. The patient had been diagnosed with non-Hodgkin's lymphoma (NHL) 2 years previously and had undergone a successful course of chemotherapy. The development of her episodes of angioedema prompted thorough reinvestigation and a recurrence of NHL was identified. Therefore, acquired C1 inhibitor deficiency heralded a recurrence, although this had not been a manifestation when NHL was first diagnosed. The patient underwent a further course of chemotherapy and remains well, although C1 inhibitor, C1q, C2, and C4 levels remain reduced.
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PMID:Acquired angioedema in non-Hodgkin's lymphoma. 1731 33

Angioedema due to acquired deficiency of the C1-inhibitor is a bridging condition between autoimmunity and lymphoproliferation. We report 32 patients with acquired C1 inhibitor deficiency: 23 have anti C1-inhibitor autoantibodies; 13 have monoclonal gammopathies of unknown significance and 9 have non-Hodgkin's lymphoma. Our series suggest that different forms of B cell disorders coexist and/or evolve into each other in acquired angioedema.
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PMID:Lymphoproliferative disease and acquired C1 inhibitor deficiency. 1748 6

Angioedema due to an acquired deficiency in the inhibitor of the first component of human complement (CI-INH) is a rare syndrome that is usually identified as acquired angioedema (AAE). The clinical features of C1-INH deficiency, which may also be of genetic origin (hereditary angioedema, HAE), include subcutaneous, non-pruritic swelling, involvement of the upper respiratory tract, and abdominal pain due to partial obstruction of the gastrointestinal tract. Unlike those with HAE, AAE patients have no family history of angioedema and are characterised by the late onset of symptoms and various responses to treatment due to the hypercatabolism of C1-INH. The reduction in C1-INH function leads to activation of the classical complement pathway and complement consumption, as well as activation of the contact system leading to the generation of the vasoactive peptide bradykinin, increased vascular permeability, and angioedema. AAE is frequently associated with lymphoproliferative diseases ranging from monoclonal gammopathies of uncertain significance (MGUS) to non-Hodgkin's lymphoma (NHL) and/or anti-C1-INH inactivating autoantibodies. The coexistence of true B cell malignancy, non-malignant B cell proliferation and pathogenic autoimmune responses suggests that AAE patients are all affected by altered B cell proliferation control although their clinical evolution may vary.
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PMID:Angioedema due to acquired C1-inhibitor deficiency: a bridging condition between autoimmunity and lymphoproliferation. 1901 72

Isolated angioedema may be the presenting symptom of acquired C1 inhibitor (C1-INH) deficiency. C1-INH deficiency is associated with lymphoproliferative disorders. Treatment of the underlying disease can result in a complete reversal of clinical and complement abnormalities. We describe a 41-year-old woman who was referred to our emergency department with recurrent episodes of isolated angioedema. Initially, her angioedema was linked to the use of angiotensin receptor blockers. However, after discontinuation of this drug angioedema recurred. Additional investigations revealed the presence of acquired C1-INH deficiency caused by an indolent non-Hodgkin's lymphoma. Treatment with rituximab resulted in complete clinical and biochemical remission of the acquired angioedema.
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PMID:[Recurrent isolated angioedema as a result of acquired C1 inhibitor deficiency]. 2466 32