Hemorrhagic Events in Adult Celiac Disease Patients. Case Report and Review of the Literature

Abstract

Background & Aims: Celiac disease (CD) presents with a wide spectrum of extra-digestive symptoms, including hemorrhagic manifestations. The aim of this review was to conduct an extensive analysis of the hemorrhagic events reported in adult CD patients.Methods: Case report and review of the literature. Pubmed (MEDLINE) database search from January 1970 onwards was performed using the medical subject headings [MeSH] terms “celiac disease” AND “blood coagulation disorders”, “hemorrhage”, “hematoma”, “hematuria”, “hemoptysis”, “epistaxis”, “hemosiderosis”.Only case reports were identified on the search theme. Information on patients’ characteristics, diagnostic features, coagulation parameters, symptomatology duration, and evolution under treatment were systematically collected and summarized.Results: We present the case of a 40-year-old man hospitalized for spontaneous muscular hematomas, in whom CD was diagnosed. We performed a review of the literature and summarized the published case reports of 46 CD patients, aged between 19 and 74 years, 64% of male gender. In 25% of cases, the symptomatology was present for more than 5 years prior to CD diagnosis. The clinical hemorrhagic events were as follows: 15 patients had gastrointestinal bleeding, 9 hemoptysis, 4 epistaxis, 6 hematuria, 8 cutaneous hematoma, petechia or ecchymoses, and only in 1 case hemarthrosis, hemorrhagic vesicular dermatitis, subcortical hemorrhage, or adrenal hemorrhage. Sixty percent of the patients had digestive symptoms, while the rest had only extradigestive CD involvement. The Lane Hamilton syndrome was defined in 15 patients. The evolution under a gluten-free diet was favorable in most cases.Conclusion: This review of case reports aims to increase awareness to hemorrhagic events, rare but possible life-threatening conditions, as part of the CD clinical spectrum. To the best of our knowledge, this is the first review of all types of hemorrhagic events in adult CD patients.

Celiac crisis, a rare occurrence in adult celiac disease: A systematic review

“…In some cases, the electrolyte imbalances led to neurologic or cardiologic manifestations, which could be the main presenting feature in CC; a high index of suspicion is needed among physicians with regard to these atypical and very rare presentations, as they were previously shown to have poor awareness in regard to CD non-intestinal features[19]. In addition, CC can also present with bleeding diathesis, which was the case for 4/42 of the reviewed cases; as previously reported, hemorrhagic events in CD patients encompass a wide range of clinical manifestations, such as gastrointestinal bleeding, hemoptysis, epistaxis, hematuria, hematomas or ecchymosis[20].…”

Hematologic Manifestations in Celiac Disease—A Practical Review

“…Hemorrhagic events can be the presenting feature of CD, including cases of celiac crisis with profound malabsorption and coagulation deficits [44]. A recent review of the literature has found only case reports of hemorrhagic events, comprising otorhinolaryngology, digestive, urology, muscular and alveolar bleeding (the latter defining the Lane Hamilton syndrome) [45]. The mechanism behind hemorrhagic diathesis in CD is mainly represented by vitamin K deficiency, while some studies have also theorized mimicry between factor XIII and tissue transglutaminase [45,46].…”

“…A recent review of the literature has found only case reports of hemorrhagic events, comprising otorhinolaryngology, digestive, urology, muscular and alveolar bleeding (the latter defining the Lane Hamilton syndrome) [45]. The mechanism behind hemorrhagic diathesis in CD is mainly represented by vitamin K deficiency, while some studies have also theorized mimicry between factor XIII and tissue transglutaminase [45,46]. Management of hemorrhage consists of intravenous vitamin K and GFD, along with specific measures according to bleeding site.…”

Lane-Hamilton Syndrome: An Illustration of Extraintestinal Manifestations of Celiac Disease

“…Of children with IPH, estimates from available literature suggest that up to 30% have CD, with 75% of LHS patients presenting in childhood. 19…”