Do infections provoke exacerbations and relapses of thrombotic thrombocytopenic purpura?

• Published in: Journal of clinical apheresis 2007-02, Vol.22 (1), p.21-25
• Main Authors: Cserti, Christine M., Landaw, Stephen, Uhl, Lynne
• Format: Article
• Language: English
• Subjects: Adult; exacerbations; Female; Humans; infection; Infection - complications; Infection - etiology; Infection - microbiology; Plasma Exchange; Purpura, Thrombotic Thrombocytopenic - etiology; Purpura, Thrombotic Thrombocytopenic - microbiology; Purpura, Thrombotic Thrombocytopenic - therapy; Recurrence; relapse; Splenectomy - adverse effects; thrombotic thrombocytopenic purpura (TTP)
• ISSN: 0733-2459; 1098-1101
• DOI: 10.1002/jca.20114

Several case reports have suggested an association between infections and thrombotic thrombocytopenic purpura (TTP). In Case 1, a 37‐year‐old female presented with TTP 6 times over 7 years, requiring 242 therapeutic plasma exchanges (TPE), for a per‐course range of 4–57 TPE (median 48), and treatment durations of 4–241 days (median 71 days), largely on account of multiple exacerbations (range 0–3, median 3). Twelve of 17 (71%) of her presentations or exacerbations were associated with suspected infections, with confirmation in 9 episodes. These included pulmonary TB, CMV pneumonitis, mucocutaneous HSV, ventilator‐associated or urinary tract‐associated gram‐negative sepsis, central line‐associated staphylococcal bacteremia, and cellulitis. Except for TB, all infections occurred after splenectomy, which had been performed on day 33 of presentation 1. In Case 2, a 24‐year‐old female presented with TTP 3 times over 15 months. Her courses were managed with brief courses of TPE (5–11 treatments per course, median 5). Suppressed ADAMTS13 levels due to inhibitors were confirmed twice. Presentation 1 was antedated by atypical community acquired pneumonia. Presentation 3 (and possibly 2) followed prolonged, progressive, antibiotic‐refractory periodontal infections ultimately requiring exodontic surgery. Our cases add to a literature that suggests that infection may be associated with exacerbations or relapses of TTP in some patients. Our patients demonstrated repeated TTP exacerbations in association with different infectious agents. A better understanding of the possible relationship between infection and clinical expression of TTP might lead to improved treatment decisions for patients with this complex illness. J. Clin. Apheresis. 22:, 2007 © 2007 Wiley‐Liss, Inc.