scholarly journals Refractory Abdominal Pain in a Patient with Chronic Lymphocytic Leukemia: Be Wary of Acquired Angioedema due to C1 Esterase Inhibitor Deficiency

2018 ◽  
Vol 2018 ◽  
pp. 1-5
Author(s):  
Abdullateef Abdulkareem ◽  
Ryan S. D’Souza ◽  
Joshua Mundorff ◽  
Pragya Shrestha ◽  
Oluwaseun Shogbesan ◽  
...  
Keyword(s):  
Abdominal Pain ◽  
Chronic Lymphocytic Leukemia ◽  
Upper Airway ◽  
Recurrent Abdominal Pain ◽  
Final Diagnosis ◽  
Lymphocytic Leukemia ◽  
C1 Inhibitor Deficiency ◽  
Acquired Angioedema ◽  
C1 Esterase Inhibitor ◽  
Esterase Inhibitor

Acquired angioedema due to C1 inhibitor deficiency (C1INH-AAE) is a rare and potentially fatal syndrome of bradykinin-mediated angioedema characterized by episodes of angioedema without urticaria. It typically manifests with nonpitting edema of the skin and edema in the gastrointestinal (GI) tract mucosa or upper airway. Edema of the upper airway and tongue may lead to life-threatening asphyxiation. C1INH-AAE is typically under-diagnosed because of its rarity and its propensity to mimic more common abdominal conditions and allergic reactions. In this article, we present the case of a 62-year-old male with a history of recently diagnosed chronic lymphocytic leukemia (CLL) who presented to our hospital with recurrent abdominal pain, initially suspected to haveClostridium difficilecolitis and diverticulitis. He received a final diagnosis of acquired angioedema due to C1 esterase inhibitor deficiency due to concomitant symptoms of lip swelling, cutaneous nonpitting edema of his lower extremities, and complement level deficiencies. He received acute treatment with C1 esterase replacement and icatibant and was maintained on C1 esterase infusions. He also underwent chemotherapy for his underlying CLL and did not experience further recurrence of his angioedema.

scholarly journals Intestinal Angioedema: Case Report and Literature Review

2018 ◽  
Vol 2 (2) ◽  
Keyword(s):  
Abdominal Pain ◽  
Recurrent Abdominal Pain ◽  
High Morbidity ◽  
Factor Xii ◽  
Adequate Treatment ◽  
C1 Esterase Inhibitor ◽  
History Of ◽  
Biochemical Testing ◽  
Medication Allergy ◽  
Esterase Inhibitor

Hereditary angioedema (HAE) is the deficiency or dysfunction of C1 esterase inhibitor (C1-INH). However, it may also occur due to either increased activity of factor XII / estrogen levels or through an unidentified cause. It manifests the attacks of swelling involving the skin and / or the mucosa / sub mucosa of different organs. The attacks may be the result of a specific trigger or occur spontaneously. The intestinal angioedema is clinically presented with moderate or severe abdominal pain, associated with nausea, vomiting, diarrhea and / or ascites, and interpreted as an “acute abdomen”. The treatment is into three distinct phases: treatment for acute attacks, short-term prevention, and long-term prophylaxis. The 26-year-old woman with food and medication allergy presents with thirteen-year history of recurrent abdominal pain diffuse and associated with diarrhea, nausea and hands, lips and eyelids swelling. During this period, she did several exams and six laparoscopies that only revealed a small amount of free intraperitoneal fluid. Biochemical testing performed at that time revealed the C1 esterase inhibitor, decreased protein level, and C4 level, and she was, then, diagnosed in adults with intestinal involvement with HAE. After the adequate treatment and prophylaxis, she evolved with reduction of the number of attacks. The late diagnosis is associated with high morbidity. Therefore, it is extremely important the recognition and investigation of HAE with involvement in intestinal patients with recurrent attacks of unexplained abdominal pain.

scholarly journals Angioedema of the Tongue Due to Acquired C1 Esterase Inhibitor Deficiency

2003 ◽  
Vol 31 (1) ◽  
pp. 99-102 ◽  
Author(s):  
G. Dobson ◽  
D. Edgar ◽  
J. Trinder
Keyword(s):  
Intensive Care Unit ◽  
Airway Obstruction ◽  
Haematological Malignancy ◽  
Ventilatory Support ◽  
Upper Airway ◽  
Upper Airway Obstruction ◽  
Treatment Modalities ◽  
Acquired Angioedema ◽  
C1 Esterase Inhibitor ◽  
Esterase Inhibitor

We describe the management of an 83-year-old woman who presented with upper airway obstruction due to angioedema of the tongue. Following definitive airway management, investigation showed a diagnosis of acquired C1 esterase inhibitor deficiency (acquired angioedema) that was considered to be subsequent to haematological malignancy. Resolution of the macroglossia followed treatment with C1 esterase inhibitor concentrate, but the patient failed to wean from ventilatory support and died in the Intensive Care Unit. This case report highlights the potential for acquired angioedema to cause upper airway obstruction. The various treatment modalities for acquired C1 esterase inhibitor deficiency are summarized.

scholarly journals Acquired complement C1 esterase inhibitor deficiency in a patient with a rare SERPING1 variant with unknown significance

2019 ◽  
Vol 12 (9) ◽  
pp. e231122
Author(s):  
Eva Rye Rasmussen ◽  
Kasper Aanæs ◽  
Marianne Antonius Jakobsen ◽  
Anette Bygum
Keyword(s):  
Abdominal Pain ◽  
Upper Airway ◽  
Allergic Reactions ◽  
C1 Inhibitor ◽  
C1 Esterase Inhibitor ◽  
Wide Range ◽  
Unknown Significance ◽  
Life Threatening ◽  
Vasoactive Mediator ◽  
Esterase Inhibitor

Angioedema (AE) is caused by a wide range of diseases and pharmaceuticals; it can become life-threatening when located to the airways. Patients with deficiency or malfunction of complement C1 esterase inhibitor (hereditary or acquired) experience recurrent AE due to an accumulation of the vasoactive mediator bradykinin (BK). Complement C1 inhibitor normally decreases BK production, so a reduced function hereof causes increased levels. The diagnosis of hereditary or acquired AE can be difficult due to similarities to allergic reactions (swelling, abdominal pain, rash). We describe a 35-year-old man presenting with upper-airway AE progressing rapidly and promptly required cricothyroidotomy. Complement and autoantibody screening together with sequencing of SERPING1 were performed and gave the diagnosis of acquired complement C1 esterase inhibitor deficiency. The patient is unusual to have this disease before the age of 40 years. No associated comorbidities were found. It is important to know that antiallergic medication is not effective in BK-mediated AE.

scholarly journals When abdominal pain knocks the door: an unusual presentation of chronic lymphocytic leukemia

2019 ◽  
Vol 2019 (5) ◽  
Author(s):  
Digdem Ozer Etik ◽  
Nuretdin Suna ◽  
Pelin Borcek ◽  
Fatih Hilmioglu
Keyword(s):  
Abdominal Pain ◽  
Chronic Lymphocytic Leukemia ◽  
Lymphocytic Leukemia ◽  
Unusual Presentation

scholarly journals A case of hereditary angioedema due to C1-inhibitor deficiency with recurrent abdominal pain diagnosed 40 years after the occurrence of the initial symptom

2021 ◽  
Author(s):  
Daisuke Honda ◽  
Isao Ohsawa ◽  
Keiichi Iwanami ◽  
Hisaki Rinno ◽  
Yasuhiko Tomino ◽  
...  
Keyword(s):  
Abdominal Pain ◽  
Hereditary Angioedema ◽  
Recurrent Abdominal Pain ◽  
Acute Attack ◽  
Severe Abdominal Pain ◽  
Initial Symptom ◽  
C1 Inhibitor ◽  
Self Administration ◽  
C1 Inhibitor Deficiency ◽  
Appropriate Treatment

AbstractHereditary angioedema due to C1-inhibitor deficiency (HAE-C1-INH) is a rare disease, which induces an acute attack of angioedema mediated by bradykinin. HAE-C1-INH can cause serious abdominal pain when severe edema develops in the gastrointestinal tract. However, because it takes a long time, 13.8 years on average in Japan, from the occurrence of the initial symptom to the diagnosis due to low awareness of the disease, undiagnosed HAE-C1-INH patients sometimes undergo unnecessary surgical procedures for severe abdominal pain. We herein present a 56-year-old patient with HAE-C1-INH, who underwent numerous abdominal operations. He frequently needed hospitalization with the administration of opioid due to severe abdominal pain. However, after he was accurately diagnosed with HAE-C1-INH at 55 years of age, he could start self-administration for an acute attack with icatibant, a selective bradykinin B2 receptor antagonist. Consequently, he did not need hospitalizing for ten months after the beginning of the treatment. A series of an accurate diagnosis and appropriate treatment for HAE-C1-INH improved his quality of life. Thus, HAE-C1-INH should be considered, when we meet patients with unidentified recurrent abdominal pain. This case highlights significance of an early diagnosis and appropriate treatment for HAE-C1-INH.

Etiology and predictors of cluster attacks of hereditary angioedema that recur despite pharmaceutical treatment

2021 ◽  
Vol 42 (4) ◽  
pp. 317-324
Author(s):  
Felix. A. Johnson ◽  
Magdalena Wirth ◽  
Zhaojun Zhu ◽  
Janina Hahn ◽  
Jens Greve ◽  
...  
Keyword(s):  
Hereditary Angioedema ◽  
Upper Airway ◽  
Poor Quality ◽  
Prophylactic Therapy ◽  
On Demand ◽  
C1 Esterase Inhibitor ◽  
Physical Stimuli ◽  
Inhibitor Treatment ◽  
Esterase Inhibitor

Introduction: Hereditary angioedema (HAE) is a disease that leads to recurrent swelling of the skin and mucous membranes, including the upper airway tract. Apart from being deadly, these attacks can be debilitating, which leads to a poor quality of life in patients. Clinicians are occasionally confronted with patients who have recurrent attacks despite treatment with C1 esterase inhibitor concentrate or β2-receptor antagonists. The goal of this study was to investigate repeated attacks that occur 48 hours to 7 days (“cluster attacks”) after treatment, to determine why they occur and the factors that may be associated with them, and thus to prevent their occurrence. Methods: We conducted a multicenter mixed retrospective-prospective study with data acquired from all documented attacks in our patients with collective (n = 132) between 2015 and 2018. Results: Eighty-five percent (n = 132) of our total patient collective (N = 156) agreed to participate in the study. Nine percent of these patients (n = 12) had cluster attacks, with a total of 48 cluster attacks. The data procured from the patients were mixed retrospective‐prospective. Approximately 72% of all the cluster attacks were caused by exogenous stimuli (41% due to psychological stress, 29% due to physical stimuli, and 2% due to menstruation). Cluster attacks occurred in 7% of the patients who received prophylactic therapy in comparison with 12.5% of patients who received on-demand therapy. Cluster attacks comprised 48.4% of all the attacks that patients with cluster-attacks (n= 9) experienced. In addition, the patients who were underdosing their C1 esterase inhibitor treatment had cluster attacks more often. A lower “time to repeated attack” was seen in the patients who received on-demand therapy compared with those who received prophylactic therapy. Discussion: The percentage of the patients who had attacks as a result of exogenous triggers was higher in the cluster-attack group (70.5%) compared with the general HAE population (30‐42%). Repeated attacks, therefore, were strongly associated with external triggers. The patients who received prophylactic treatment and who experienced cluster attacks were highly likely to have been underdosing, which may explain the repeated attacks despite treatment. In the patients prone to cluster attacks, prophylaxis should be considered.

scholarly journals Chronic Lymphocytic Leukemia as an Unusual Cause of Rapid Airway Compromise

2017 ◽  
Vol 2017 ◽  
pp. 1-4
Author(s):  
Adrian R. Bersabe ◽  
Joshua T. Romain ◽  
Erin E. Ezzell ◽  
John S. Renshaw
Keyword(s):  
Chronic Lymphocytic Leukemia ◽  
Airway Obstruction ◽  
Head And Neck ◽  
Cervical Lymphadenopathy ◽  
Upper Airway ◽  
Lymphocytic Leukemia ◽  
Upper Airway Obstruction ◽  
Airway Compromise ◽  
History Of ◽  
Prevalent Form

Chronic Lymphocytic Leukemia (CLL) is the most prevalent form of non-Hodgkin’s lymphoma (NHL) in Western countries predominantly affecting adults over the age of 65. CLL is commonly indolent in nature but can present locally and aggressively at extranodal sites. Although CLL may commonly present with cervical lymphadenopathy, manifestation in nonlymphoid regions of the head and neck is not well described. CLL causing upper airway obstruction is even more uncommon. We describe a case of a patient with known history of CLL and stable lymphocytosis that developed an enlarging lymphoid base of tongue (BOT) mass resulting in rapid airway compromise.

Pharmacokinetics, Clinical Efficacy and Safety of Plasma-Derived Nanofiltered C1 Inhibitor Concentrate for Treatment of Hereditary and Acquired Angioedema.

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 1988-1988
Author(s):  
J. J. Hofstra ◽  
E. van Twuyver ◽  
I. Kleine-Budde ◽  
C. W. Choi ◽  
M. Levi ◽  
...  
Keyword(s):  
Phase Ii Study ◽  
Phase Iii ◽  
Pharmacokinetic Parameters ◽  
Efficacy And Safety ◽  
C1 Inhibitor ◽  
Acquired Angioedema ◽  
C1 Esterase Inhibitor ◽  
Phase Iii Study ◽  
Mean Time ◽  
Esterase Inhibitor

Abstract RATIONALE: From the early 70’s, C1 inhibitor concentrate manufactured from pooled human plasma has been available to patients with hereditary and acquired angioedema (HAE and AAE) and in 1997 a highly purified C1 inhibitor (Cetor®) was introduced. Many precautions have been taken to minimize the potential risk of viral transmission (e.g. rigorously controlled whole blood collection systems, extensive screening of each individual donation for a variety of blood-borne viruses, pasteurisation). To further minimize the potential risk of viral transmission, a 15 nm filtration was implemented in the manufacturing process giving rise to C1-inhibitor-N (anofiltered). DESIGN: A randomised, double-blind, controlled cross-over phase II study was conducted in which our primary objective was to compare the pharmacokinetics of the newly developed concentrate with conventional C1-inhibitor concentrate in HAE patients without signs of an attack of angioedema. Secondly, an open-label phase III study in patients with an HAE attack was performed to investigate whether the introduction of the virus reducing 15nm filtration step in the manufacturing process of the concentrate did not affect the efficacy and safety of the product. RESULTS: Thirteen patients were enrolled in the phase II study. No differences between conventional C1-inhibitor concentrate and nanofiltered C1-inhibitor concentrate were detected with regard to the primary pharmacokinetic parameters clearance, volume of distribution, and the fraction of C1-inhibitor-N detected by the antigen assay relative to the functional assay. Therefore incremental recovery, mean residence time, half-life and the area under curve were equivalent for both products. In the phase III study, 8 HAE patients were enrolled. In these 8 patients, 14 attacks qualified as acute angioedema attack. The mean time-to-relief for attacks treated with new C1-inhibitor concentrate was 3.0 (SD 2.5) hours. Historical data showed that treatment with conventional C1-inhibitor concentrate resulted in time-to-relief of 3.9 (SD 6.2) hours, whereas for untreated attacks this was 24.7 (SD 19.9) hours. The attacks treated with new C1-esterase inhibitor concentrate had a mean time-to-resolve of 18.6 hours (SD 13.1) whereas medical history showed an time-to-resolve of 17.8 hours (SD 17.2) for conventional C1-inhibitor. Untreated attacks showed a mean time-to-resolve of 63.6 hours (SD 31.0). CONCLUSION: The newly developed nanofiltered C1-esterase inhibitor has equal pharmacokinetic properties compared to the conventional concentrate. The viral reduction step (15 nm filtration) in the production process of Cetor did not induce changes in the efficacy and safety in the treatment of acute angioedema attacks and in the pharmacokinetic parameters.

Acquired Angioedema as Unusual Cause of Recurrent Abdominal Pain

2018 ◽  
Vol 113 (Supplement) ◽  
pp. S1390
Author(s):  
Sumant Arora ◽  
Barakat Aburajab Altamimi ◽  
Avraham Levin
Keyword(s):  
Abdominal Pain ◽  
Recurrent Abdominal Pain ◽  
Acquired Angioedema
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