512 Case report

Acute thrombotic thrombocytopenic purpura after pneumococcal vaccination Yuki Kojimaa, Haruhiko Ohashia, Tomonobu Nakamurab, Hiroyuki Nakamuraa, Hideyuki Yamamotoa, Yasuhiko Miyataa, Hiroatsu Iidaa and Hirokazu Nagaia We report the case of a 68-year-old woman with acute thrombotic thrombocytopenic purpura (TTP) that developed after pneumococcal vaccination. She was found in a confusional state with high fever 15 days after vaccination. Laboratory data showed hemolytic anemia and thrombocytopenia, and blood smear showed fragmented erythrocytes. TTP was diagnosed based on the clinical presentation, and was subsequently confirmed by the absence of ADAMTS13 activity and the presence of inhibitor against ADAMTS13 in serum. She was successfully treated using plasma exchange and corticosteroids, and no recurrence has been identified. This appears to represent the first report of TTP following pneumococcal vaccination. Blood Coagul Fibrinolysis

Introduction Thrombotic thrombocytopenic purpura (TTP) is a rare, but life-threatening disease involving microangiopathic hemolytic anemia, thrombocytopenia, with or without neurological or renal abnormalities [1]. Cases of acute TTP are generally idiopathic, but different triggering factors may exist, such as bacterial or viral infections, collagen diseases, malignancies, use of drugs such as quinine, ticlopidine, clopidogrel, and pregnancy [2]. Most cases are caused by acquisition of inhibitor against ADAMTS13 (an acronym for a disintegrin and metalloprotease with thrombospondin1-like domains), a von Willebrand factor (VWF)-cleaving protease. Vaccination can be associated with autoimmune pathology, for example, Guilain–Barre syndrome and acute disseminated enchephalomyelitis. A small number of patients with acute TTP after influenza vaccination have been reported [3–6]. Herein, we report a case in which acute TTP developed after pneumococcal vaccination.

Case report A 68-year-old woman without significant medical or family history or medication was admitted to our hospital in a confusional state with high fever. She was icteric and displayed petechiae on the lower extremities. Fifteen days prior to admission, she had received vaccination against pneumococcus (23-valent pneumococcal polysaccharide vaccine, PPV23). 0957-5235 ß 2014 Wolters Kluwer Health | Lippincott Williams & Wilkins

25:512–514 ß 2014 Wolters Kluwer Health | Lippincott Williams & Wilkins. Blood Coagulation and Fibrinolysis 2014, 25:512–514 Keywords: autoimmune, pneumococcal vaccines, thrombotic thrombocytopenic purpura a

Department of Hematology and bDepartment of Nephrology, National Hospital Organization Nagoya Medical Center, Nagoya, Japan Correspondence to Yuki Kojima, Department of Hematology, National Hospital Organization Nagoya Medical Center, 4-1-1 Sannomaru, Naka-ku, Nagoya 4600001, Japan Tel: +81 52 951 1111; fax: +81 52 951 0664; e-mail: [email protected] Received 18 July 2013 Revised 5 October 2013 Accepted 4 December 2013

Laboratory results were as follows: platelet count, 11  10 9 per litre; red blood cells, 225  10 9 per litre; hemoglobin, 68 g/l; reticulocytes, 11.7%; total bilirubin, 4.05 mg/dl; lactate dehydrogenase (LDH), 1.971 IU/l; serum creatinine, 0.059 mmol/l; blood urea nitrogen, 30 mg/dl; prothrombin time, 16.2 s (s); activated partial thromboplastin time, 32.0 s; fibrinogen, 379 ml/dl; and fibrin degradation products, 31 mg/ml. A peripheral blood smear showed fragmented erythrocytes. Direct and indirect antiglobulin (Coombs) tests yielded negative results. Chest and abdominal computed tomography showed no abnormalities, and both blood and urine cultures remained sterile. Based on these findings, acute TTP was diagnosed. Figure 1 shows details of the clinical course. The patient received plasma exchange in combination with intravenous methylprednisolone administration (500 mg per day) on day 1. On day 2, the platelet count increased to 52  10 9 per litre and total bilirubin decreased to 2.06 IU/l, so plasma exchange was not performed again, although administration of fresh frozen plasma (10 ml/kg) was continued. Five days after initiating treatment, the patient showed normal mental status, and a tendency toward improved laboratory findings (platelet count, 214  10 9 per litre; total bilirubin, 1.26 mg/dl; LDH, 358 IU/l). ADAMTS13 activity measured by ELISA was less than 0.5% (normal, 70.0–120.0%) and inhibitor against ADAMTS13 by Bethesda method was detected from DOI:10.1097/MBC.0000000000000058

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TTP after pneumococcal vaccination Kojima et al. 513

Fig. 1

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Acute thrombotic thrombocytopenic purpura after pneumococcal vaccination.

We report the case of a 68-year-old woman with acute thrombotic thrombocytopenic purpura (TTP) that developed after pneumococcal vaccination. She was ...
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