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Recurrent Thrombotic Thrombocytopenic Purpura Associated With Helicobacter pylori: More Than a Gut Feeling
Esperance M Madera1, Keon J Sargon1, Domonick K Gordon1
1Internal Medicine, Mount Sinai Hospital, Chicago, USA.
Abstract:
Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening hematologic disorder characterized by severe ADAMTS13 deficiency, leading to uncontrolled platelet aggregation, microvascular thrombosis, and multi-organ dysfunction. Although the pathogenesis of immune-mediated TTP (iTTP) is primarily autoimmune, infectious agents, including Helicobacter pylori (H. pylori), have been implicated as potential triggers. However, the association between H. pylori and iTTP remains poorly defined. We describe a case of a 33-year-old male presenting with hematuria, thrombocytopenia, and laboratory evidence of microangiopathic hemolytic anemia. A markedly reduced ADAMTS13 activity and elevated inhibitor level confirmed the diagnosis of iTTP. The patient responded well to plasma exchange and rituximab but experienced relapse following therapy interruption. During the relapse, testing revealed a positive H. pylori stool antigen and eradication therapy was initiated. This raised the possibility of H. pylori contributing to disease recurrence. This case adds to emerging evidence suggesting a potential role of H. pylori in the pathogenesis or exacerbation of iTTP. Proposed mechanisms include molecular mimicry, platelet aggregation via von Willebrand factor interactions, P-selectin expression, and pylori urease, as well as through pro-inflammatory cytokine release. While observational data and isolated case reports have highlighted this association, direct causal links and the therapeutic impact of H. pylori eradication in iTTP require further investigation. The recurrence of iTTP in the setting of persistent H. pylori infection in this case underscores the need for further investigation into H. pylori as a modifiable risk factor in iTTP. Further research is needed to determine if targeted screening and eradication strategies may offer a novel adjunctive approach to reduce recurrence and improve long-term outcomes.
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