Hazem Abosheaishaa, MD, MSc1, Muhammad Haseeb ul Rasool, MD2, Mahmoud Nassar, MD, PhD2, Khaled Elfert, MD3, Islam Mohamed, MD4, Mohamed Eldesouki, MD5, Saphwat Eskaros, MD2 1Hazem Abosheaishaa, Bellerose, NY; 2Icahn School of Medicine at Mount Sinai, New York, NY; 3SBH Health System, Bronx, NY; 4University of Missouri-Kansas City, Kansas City, MO; 5Mayo Clinic, Rochester, MN
Introduction: Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening hematologic disorder characterized by microangiopathic hemolytic anemia, thrombocytopenia, and organ dysfunction due to disseminated microvascular platelet-rich thrombi. This case report highlights a rare presentation of small bowel ischemia and ischemic colitis secondary to TTP in a patient with a known history of SLE, emphasizing the importance of considering TTP as a potential cause of acute abdominal symptoms in SLE patients.
Case Description/Methods: A 35-year-old female patient with hypertension, SLE, and ESRD on regular hemodialysis presented with severe acute abdominal pain, diarrhea, vomiting, and bloody bowel movement with the ultimate diagnosis of small bowel ischemia and ischemic colitis after CT, EGD, and colonoscopy. Further investigation revealed TTP as the underlying cause, a rare complication in patients with SLE. Despite the challenges in differentiating TTP from other thrombotic microangiopathy syndromes, the diagnosis was confirmed through laboratory tests, including ADAMTS13 levels. The patient was treated with plasma exchange, steroids, and rituximab. Resection anastomosis surgery was done after the failure of the standard therapy and led to clinical improvement.
Discussion: This case emphasizes the importance of considering TTP in patients with SLE who present with acute abdominal symptoms. TTP can be idiopathic or secondary to a number of clinical conditions, including autoimmune disorders, infections, hematopoietic stem cell or solid organ transplants, neoplasia, drugs, and pregnancy. The pentad of fever, low platelet count, microangiopathic hemolytic anemia, neurological conditions, and renal dysfunction described the acute phase of TTP. Disseminated microvascular platelet-rich thrombi are associated with symptoms of organ ischemia in TTP with CNS and Kidneys are the most known organs to be affected by TTP. In 2013, See et al reported a case of acute ischemic colitis as a typical presentation of TTP and interestingly, Abeysundara et al described a case of jejunal stricture as a rare presentation of TTP in 2017. The standard therapy for TTP remains plasma exchange, steroids, and rituximab. Patients who do not respond to regular therapy have the option of surgical surgery, and early detection and intervention in these circumstances can be lifesaving.
Hazem Abosheaishaa indicated no relevant financial relationships.
Muhammad Haseeb ul Rasool indicated no relevant financial relationships.
Mahmoud Nassar indicated no relevant financial relationships.
Khaled Elfert indicated no relevant financial relationships.
Islam Mohamed indicated no relevant financial relationships.
Mohamed Eldesouki indicated no relevant financial relationships.
Saphwat Eskaros indicated no relevant financial relationships.
Hazem Abosheaishaa, MD, MSc1, Muhammad Haseeb ul Rasool, MD2, Mahmoud Nassar, MD, PhD2, Khaled Elfert, MD3, Islam Mohamed, MD4, Mohamed Eldesouki, MD5, Saphwat Eskaros, MD2. P2095 - Small Bowel Ischemia and Ischemic Colitis Secondary to Thrombotic Thrombocytopenic Purpura in a Patient with Systemic Lupus Erythematosus: A Rare Clinical Presentation, ACG 2023 Annual Scientific Meeting Abstracts. Vancouver, BC, Canada: American College of Gastroenterology.