Autoantibodies and Lymphoproliferative Diseases in Acquired C1-Inhibitor Deficiencies

Cicardi, Marco; Zingale, Lorenza C.; Pappalardo, Emanuela; Folcioni, Anna; Agostoni, A

doi:10.1097/01.md.0000085055.63483.09

Cited by 122 publications

(88 citation statements)

References 71 publications

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“…6 Clinical symptoms are similar to those observed in HAE. An edema develops in adults with a negative family history.…”

Section: Characteristic Features Of Bradykinin-mediatedsupporting

confidence: 64%

“…An edema develops in adults with a negative family history. 6,16,54 There are 2 subtypes of AAE. 6,16 AAE type I is connected with a decrease in C1-INH concentrations in the course of lymphoproliferative diseases usually connected with B lymphocytes and an increase in C1-INH wear-off.…”

Section: Characteristic Features Of Bradykinin-mediatedmentioning

confidence: 99%

“…In contrast to HAE, in AAE, usually the C1q level is low. 6,54 Bradykinin-mediated angioedema by renin-angiotensin-aldosterone system blockers The best known and most common RAE is an edema of the tongue after the use of ACEIs in patients treated for hypertension (Supplementary material online, Figure S6). 1,4,15,54 It can also be induced by AT1-receptor blockers.…”

Section: Characteristic Features Of Bradykinin-mediatedmentioning

confidence: 99%

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Bradykinin-mediated angioedema

Obtułowicz¹

2016

Polish Archives of Internal Medicine

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“…6 Clinical symptoms are similar to those observed in HAE. An edema develops in adults with a negative family history.…”

Section: Characteristic Features Of Bradykinin-mediatedsupporting

confidence: 64%

Section: Characteristic Features Of Bradykinin-mediatedmentioning

confidence: 99%

Section: Characteristic Features Of Bradykinin-mediatedmentioning

confidence: 99%

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Bradykinin-mediated angioedema

Obtułowicz¹

2016

Polish Archives of Internal Medicine

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“…AAE associated with hematologic malignancy was found by Cicardi et al in 35% of the patients presenting acquired angioedema while association with MGUS was found in 32% of the patients [9]. B-cell non-Hodgkin lymphoma (NHL) is the most frequent lymphoproliferative disorder responsible for recurrent angioedema.…”

Section: Discussionmentioning

confidence: 99%

Acquired Angioedema Revealing a B cell Non Hodgkin Lymphoma in A Tunisian Man

Boussetta¹,

Ghedira²,

Ms³

et al. 2017

Intern Med

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Angioedema is a condition described as a transient, non-pruritic, non-pitting and self-limiting local swelling of cutaneous and mucosal tissues that completely resolve in 1 to 5 days. Acquired C1-inhibitor deficiency or acquired angioedema (AAE) may be associated with a lymph proliferative disease. We report the case of a 52 year-old man, without medical history, presented recurrent edema affecting the face and the tongue which regressed spontaneously within 8 to 10 days. Serum compliment levels were as follows; CH50: 15 UI/mL (32-58), C4=0.008 g/L (0.162-0.503), C3: 0,846 g/L (0.743-1.62), C1q=84 mg/L (100-250).C1 esterase inhibitor protein level was 190 mg/l (150-350) with a functional rate at 30% (70-130%). Blood count showed leucocytis with lymphocytic predominance. Bone marrow biopsy showed a CD20+B cell non hodgkin lymphoma. The diagnosis of (AAE) revealing a B cell non Hodgkin lymphoma was retained and the patient had been treated by chemotherapy with good clinical course.

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“…Type 2 AAE is associated with autoantibodies against C1-INH. 9,11,12 Deficiency or dysfunction of C1-INH can cause a deregulation of the fibrinolytic system, of the complement pathway, of the contact system, and of the coagulation cascade, resulting in release of vasoactive substances, an increase in vessel permeability, and development of diffuse edemas. 4,9,13 Hereditary and acquired forms of angioedema are clinically indistinguishable.…”

mentioning

confidence: 99%