Leukocytosis and high hematocrit levels during abdominal attacks of hereditary angioedema

Ohsawa, Isao; Nagamachi, Seiji; Suzuki, Hiyori; Honda, Daisuke; Sato, Nobuyuki; Ohi, Hiroyuki; Horikoshi, Satoshi; Tomino, Yasuhiko

doi:10.1186/1471-230x-13-123

Cited by 26 publications

(24 citation statements)

References 15 publications

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“…Ohsawa and colleagues measured CRP levels in 17 HAE patients with abdominal attacks. White blood cell counts were significantly higher compared to attacks located elsewhere, and consistent with our observation there was no further rise in CRP within the first hours after onset of symptoms [29].…”

Section: Discussionsupporting

confidence: 91%

“…Furthermore, we did not use absolute levels of CRP for the analyses, but rather focused on the CRP variations in individual patients over time and on levels relative to those of regional normal healthy controls. Moreover, our data of a low-grade inflammation in asymptomatic HAE patients are confirmed by a recent study on leucocytosis in HAE patients [29]. In that study, seven of 13 HAE patients had CRP levels just above the normal level at inclusion, when they were without symptoms.…”

Section: Discussionsupporting

confidence: 87%

“…Our findings reported here leave room for a role of B1R, as CRP increases in HAE patients point to increased exposure to inflammatory cytokines which can up-regulate B1R on the vessel endothelium. This hypothesis of B1R involvement during HAE attacks is strengthened by the study mentioned above, showing that cytokine levels increase during an HAE attack [29]. Interestingly, patients with multiple locations of attacks had higher CRP levels (Fig.…”

Section: Discussionmentioning

confidence: 54%

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C-reactive protein levels in hereditary angioedema

Hofman

Relan

Hack

2014

Clinical and Experimental Immunology

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SummaryHereditary angioedema (HAE) patients experience recurrent episodes of angioedema attacks that can be painful, disfiguring and even life-threatening. The disorder results from a mutation in the gene that controls the synthesis of C1-inhibitor (C1INH). C1INH is a major regulator of activation of the contact system. It is often assumed that attacks results from uncontrolled local activation of the contact system with subsequent formation of bradykinin. To evaluate the involvement of inflammatory reactions in HAE, we analysed C-reactive protein (CRP) levels. HAE patients included in a clinical database of recombinant human C1-inhibitor (rhC1INH) studies were evaluated. For the current study we analysed CRP levels when patients were asymptomatic, during a clinical attack and in a follow-up period, and correlated these with the clinical manifestations of the attack. Data from 68 HAE patients were analysed and included CRP levels on 273 occasions. While asymptomatic, 20% of the patients analysed had increased CRP. At the onset of the attack (P = 0·049) and during the next 24 h CRP rose significantly (P = 0·002) in patients with an abdominal location, and post-attack levels were significantly higher in these patients than in patients with attacks at other locations (P = 0·034). In conclusion, CRP levels are elevated in a substantial proportion of asymptomatic HAE patients. Levels of CRP increase significantly during an abdominal attack. These data suggest low-grade systemic inflammatory reactions in HAE patients as well as a triggering event for attacks that starts prior to symptom onset.

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Section: Discussionsupporting

confidence: 91%

Section: Discussionsupporting

confidence: 87%

Section: Discussionmentioning

confidence: 54%

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C-reactive protein levels in hereditary angioedema

Hofman

Relan

Hack

2014

Clinical and Experimental Immunology

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“…Sometimes abdominal symptoms of AE, such as crampy or extreme pain with or without nausea/vomiting or diarrhea, can be misdiagnosed with several abdominal conditions. This can result in unnecessary surgical interventions, such as appendicectomy [62,63].…”

Section: The Importance Of a Correct Diagnosis To Use The Right Therapymentioning

confidence: 99%

Hereditary and Acquired Angioedema: Heterogeneity of Pathogenesis and Clinical Phenotypes

Bova

Feo

Parente

et al. 2018

Int Arch Allergy Immunol

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Recurrent angioedema (AE) without wheals is increasingly recognized as a clinical entity and a frequent cause of admission to the emergency room. The Hereditary Angioedema Working Group (HAWK) classification allowed the scientific community to go beyond the semantic confusion that dominated this topic for decades. This classification distinguishes hereditary and acquired forms of AE, either related or unrelated to C1 inhibitor deficiency. Recently, additional mechanisms have been involved in the AE pathogenesis, including the uncontrolled activation of factor XII, generation of vasoactive mediators that induce dysregulation of endothelial functions, and bidirectional interactions between mast cell-derived mediators and the plasma contact system. Thus, recurrent AE can be determined by multiple and concurrent mechanisms that may generate distinct clinical phenotypes of the disease. Frequency, severity, and the location of attacks are quite different from patient to patient and, even in the same patient, they may change throughout the course of life. The severity of the clinical phenotype strongly influences the burden of the disease and patients’ quality of life. Despite major advances in our understanding of recurrent AE, many unsolved questions remain, leaving several unmet needs for patients and caregivers. This review is focused on a description of different AE phenotypes and the concurrent mechanisms leading to their pathogenesis. A better definition of cellular and molecular pathways responsible for the distinct AE phenotypes may help to improve diagnosis and may lead to a personalized approach to prophylaxis and treatment of the disease.

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“…In another case study, a 34-year-old woman who presented to the emergency department with severe abdominal cramping on several occasions was treated for presumed gastroenteritis; this patient, too, was later found to have HAE 32. In a recent retrospective study, significant leukocytosis with neutrophilia and high hematocrit levels and no elevations in C-reactive protein levels were present in all patients with HAE during an acute gastrointestinal attack,33 suggesting that such a laboratory profile may be a consideration in the differential diagnosis of HAE.…”

Section: Clinical Presentation Of Type I and Type Ii Haementioning

confidence: 99%

Hereditary angioedema: what the gastroenterologist needs to know

Ali¹,

Borum²

2014

CEG

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Up to 93% of patients with hereditary angioedema (HAE) experience recurrent abdominal pain. Many of these patients, who often present to emergency departments, primary care physicians, general surgeons, or gastroenterologists, are misdiagnosed for years and undergo unnecessary testing and surgical procedures. Making the diagnosis of HAE can be challenging because symptoms and attack locations are often inconsistent from one episode to the next. Abdominal attacks are common and can occur without other attack locations. An early, accurate diagnosis is central to managing HAE. Unexplained abdominal pain, particularly when accompanied by swelling of the face and extremities, suggests the diagnosis of HAE. A family history and radiologic imaging demonstrating edematous bowel also support an HAE diagnosis. Once HAE is suspected, C4 and C1 esterase inhibitor (C1-INH) laboratory studies are usually diagnostic. Patients with HAE may benefit from recently approved specific treatments, including plasma-derived C1-INH or recombinant C1-INH, a bradykinin B2-receptor antagonist, or a kallikrein inhibitor as first-line therapy and solvent/detergent-treated or fresh frozen plasma as second-line therapy for acute episodes. Short-term or long-term prophylaxis with nanofiltered C1-INH or attenuated androgens will prevent or reduce the frequency and severity of episodes. Gastroenterologists can play a critical role in identifying and treating patients with HAE, and should have a high index of suspicion when encountering patients with recurrent, unexplained bouts of abdominal pain. Given the high rate of abdominal attacks in HAE, it is important for gastroenterologists to appropriately diagnose and promptly recognize and treat HAE, or refer patients with HAE to an allergist.

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Leukocytosis and high hematocrit levels during abdominal attacks of hereditary angioedema

Cited by 26 publications

References 15 publications

C-reactive protein levels in hereditary angioedema

C-reactive protein levels in hereditary angioedema

Hereditary and Acquired Angioedema: Heterogeneity of Pathogenesis and Clinical Phenotypes

Hereditary angioedema: what the gastroenterologist needs to know

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