Relevance of lymphoproliferative disorders and of anti-C1 inhibitor autoantibodies in acquired angio-oedema

Cicardi, Marco; Beretta, Andrea; Colombo, Monica; Gioffré, Daniela; Cugno, Massimo; Agostoni, A

doi:10.1046/j.1365-2249.1996.d01-866.x

Cited by 98 publications

(61 citation statements)

References 25 publications

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“…A monoclonal autoantibody has been demonstrated in MGUS and NHL, but not in CLL. 120 Earlier reports of AAE in small lymphocytic lymphoma describe a B-cell CLPD but with an immunophenotype which would not now be considered CLL (FMC7 pos, sIg strong and CD5 negative). 125 So where CLL is related to AAE, it does not appear to be by an autoimmune mechanism, but rather by direct tumor consumption of C1-INH.…”

mentioning

confidence: 99%

Chronic lymphocytic leukemia and autoimmunity: a systematic review

Hodgson¹,

Ferrer²,

Montserrat³

et al. 2011

Haematologica

121

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mentioning

confidence: 99%

Chronic lymphocytic leukemia and autoimmunity: a systematic review

Hodgson¹,

Ferrer²,

Montserrat³

et al. 2011

Haematologica

121

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“…According to a previous report 5 MGUS and autoantibodies to C1-INH shared the same heavy and light chain isotypes in 9 patients.…”

mentioning

confidence: 84%

Lymphoproliferative disease and acquired C1 inhibitor deficiency

Castelli¹,

Deliliers²,

Zingale³

et al. 2007

Haematologica

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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. Haematologica 2007; 92:5: 716-718 Angioedema due to the acquired deficiency of the inhibitor of the first component of human complement (CI-INH) is a rare syndrome usually identified as acquired angioedema (AAE). One hundred and thirtysix cases are described literature.1-3 The clinical features of C1-INH deficiency, which can also be of genetic origin (hereditary angioedema, HAE), includes subcutaneous, non-pruritic swelling without accompanying urticaria, involvement of the upper respiratory tract, and partial obstruction of the gastrointestinal tract presenting as abdominal pain. Unlike HAE, AAE has no family history of angioedema, but is characterized by a This study aimed to clarify the relationship between the different forms of B cell proliferation present in AAE. Thirty-two patients were included, 23 of whom had been described elsewhere.3 Patients were followed for a median of 8 years (range: 1-24). C1-INH activity was measured with a chromogenic assay (Technochrome C1-INH, Technoclone GmbH, Vienna, Austria), antigenic measurements of C1-INH, C4 and C1q were performed by radial immunodiffusion (NOR-Partigen and [for C1q] LC-Partigen, Behring, Marburg, Germany), autoantibodies to C1-Inhibitor were detected by ELISA.4 Thirteen of 32 AAE patients (40%) fulfilled the diagnostic criteria for MGUS. According to a previous report 5 MGUS and autoantibodies to C1-INH shared the same heavy and light chain isotypes in 9 patients.Nine patients (28%) presented NHL. Based on WHO classification, 7 patients had indolent lymphoma (3 lymphoplasmacytoid lymphoma/Waldeström disease, 1 small lymphocytic lymphoma, 1 splenic marginal zone lymphoma, 1 nodal marginal zone B cells lymphoma) and 2 had high-grade malignant lymphoma (1 large B cell lymphoma and 1 mantle cell lymphoma with progression to large B cell lymphoma). All patients with indolent lymphoma had advanced stage with bone marrow infiltration. Follicular lymphoma, the most frequent istotype of indolent lymphoma, was only found in one patient. This agrees with other series of patients in which indolent lymphoma were described in the setting of autoimmune disorders. 6-7In 7 out of 9 patients, NHL was diagnosed at the onset of angioedema or developed thereafter after a minimum of 3 months to a maximum of 7 years. In 2 patients NHL was already present, but not treated, when angioedema appeared, after 6 years and 3 months respectively.Three patients received standard chemotherapy (CEOP: cyclophosphamide-vincristine and prednisone). One also received Rituximab and another received CEOP and subsequently fludarabine and cyclofosphamid...

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“…C1-INH production is normal or slightly increased. In many patients with type I, the paraproteinemia or M component actually behaves as an anti-C1-INH autoantibody, so some authors such as Cicardi suggest that the distinction between types I and II may be artiicial [37].…”

Section: Acquired Angioedemamentioning

confidence: 99%

Pathophysiology of Bradykinin-Mediated Angioedema: The Role of the Complement System

Palomo¹,

Caballero²

2017

A Comprehensive Review of Urticaria and Angioedema

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The "complement system" is one of the efector pathways of the immune system against microorganisms and tumor cells. The complement system can be activated through three major pathways: classical, lectin, and alternative. The sequential activation through the generation of complex enzymes from inactive zymogens produces a cascade in which a capable enzyme generates a large number of active downstream molecules.C1 inhibitor (C1-INH) is a serine protease inhibitor (serpin) that regulates the following closely interrelated proteolytic pathways: complement system, coagulation system, contact system, and ibrinolysis system. The absence or malfunction of C1-INH results in the presence of atacks of angioedema (AE) due to uncontrolled activation of the contact system, with the generation of bradykinin (BK), a vasoactive peptide released from high-molecular-weight kininogen (HMWK). Some drugs that inhibit the catabolism of BK have been implicated in the development of AE. These include angiotensin-converting enzyme inhibitors (ACEIs), dipeptidyl peptidase IV (DPP-IV) inhibitors, aminopeptidase P (APP) inhibitors, and neutral endopeptidase (NEP) inhibitors.We describe in this chapter the biochemistry pathways implicated in the pathophysiology of bradykininergic angioedema (BK-AE) and the role of the complement system in the prototype of BK-AE, in hereditary angioedema with C1-INH deiciency (C1-INH-HAE), and also in acquired angioedema with C1-INH deiciency (C1-INH-AAE).

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Relevance of lymphoproliferative disorders and of anti-C1 inhibitor autoantibodies in acquired angio-oedema

Cited by 98 publications

References 25 publications

Chronic lymphocytic leukemia and autoimmunity: a systematic review

Chronic lymphocytic leukemia and autoimmunity: a systematic review

Lymphoproliferative disease and acquired C1 inhibitor deficiency

Pathophysiology of Bradykinin-Mediated Angioedema: The Role of the Complement System

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