Immune Thrombocytopenic Purpura (ITP) as an Uncommon Extraintestinal Complication of Crohn’s Disease: Case Vignette and Systematic Literature Review

Abstract

While the association of immune thrombocytopenic purpura (ITP) and inflammatory bowel disease (IBD) has been described in a few case reports, management of ITP as an extraintestinal manifestation of Crohn’s disease (CD) is less studied. There are approximately a dozen cases describing the management of patients dually diagnosed with CD/ITP. Previous reports postulated that the mechanism of ITP in CD was through the presence of circulating immune complexes in the serum and antigenic mimicry due to increased mucosal permeability in active colitis, versus increased mucosal production of TH1-type proinflammatory cytokines during CD flares, which may account for remission of ITP with surgery for CD. We present a case of a 27-year-old man who presented with medically refractory CD and ITP who responded to surgical management with colectomy and splenectomy, along with a systematic review of the literature. These cases suggest that colectomy should be considered in the treatment of medically refractory ITP among patients with concomitant CD.

Etiopathogenic and Clinical Aspects in Inflammatory Bowel Disease – Literature Review

“…The presence of immune thrombocytopenic purpura (ITP) in patients with IBD has been described in a limited number of cases. 28 Researchers support the theory of anti-genic mimicry between platelet surface and luminal antigens, thus creating a common immune-mediated pathway to mucosal inflammation and platelet destruction. Studies have shown increased levels of T-helper 1 and CD4 cells.…”

Resolved Hypereosinophilic Syndrome and Immune Thrombocytopenic Purpura in Ulcerative Colitis Patients Post Colectomy: A Case Series and Literature Review

“…In patients with UC, immune complexes escape from the gut to the circulating blood due to increased mucosal permeability during disease activity. 24 They cross-react with the platelet surface antigens, which carry similar peptides to bacterial glycoproteins in the gut and, subsequently, result in ITP. 24 Moreover, the autoreactive T-cells secrete eosinophilopoietic cytokines that promote eosinophils' survival, enhance their activity, and hinder their suppressive regulatory mechanisms.…”

“…24 They cross-react with the platelet surface antigens, which carry similar peptides to bacterial glycoproteins in the gut and, subsequently, result in ITP. 24 Moreover, the autoreactive T-cells secrete eosinophilopoietic cytokines that promote eosinophils' survival, enhance their activity, and hinder their suppressive regulatory mechanisms. 20,21 Colectomy, therefore, would stop these pathogenic mechanisms and subsequently prevent platelets destruction and hypereosinophilia.…”

“…However, evidence from the previous literature suggests molecular mimicry as a potential pathogenic mechanism for their co-occurrence. 24 In this article, we present a case of HES and a case of ITP occurring in the context of active ulcerative pancolitis that was refractory to medical treatment and only responded to surgical colectomy.…”

Immune Thrombocytopenic Purpura as an Extraintestinal Manifestation in a Patient with Ulcerative Colitis

“…Immune thrombocytopenic purpura (ITP) is a rare EIM of IBD, commonly associated with UC [3]. ITP is characterized by the presence of autoantibodies against platelet membrane antigens, leading to immune-mediated destruction of platelets [4]. The pathogenesis of the association between IBD and ITP is explained by the mimicry between the luminal mucosa and platelet membrane antigens, leading to immunogenic activation that favors platelet destruction in the spleen [5].…”