Life-Threatening Thrombotic Thrombocytopenic Purpura Associated with Dental Foci
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J Clin Periodontol 2004; 31: 1019–1023 doi: 10.1111/j.1600-051X.2004.00617.x Copyright r Blackwell Munksgaard 2004 Printed in Denmark. All rights reserved Matthias Fenner1, Roland Frankenberger2, Katharina Life-threatening thrombotic 3 3 Pressmar , Stefan John , Friedrich Wilhelm Neukam1 and thrombocytopenic purpura Emeka Nkenke1 Departments of 1Oral & Maxillofacial Surgery, 2Operative Dentistry and associated with dental foci Periodontology and 3Medicine IV, University of Erlangen-Nuremberg, Germany Report of two cases Fenner M, Frankenberger R, Pressmar K, John S, Neukam FW, Nkenke E: Life- threatening thrombotic thrombocytopenic purpura associated with dental foci. Report of two cases. J Clin Periodontol 2004; 31: 1019–1023. doi: 10.1111/j.1600-051X. 2004.00617.x. r Blackwell Munksgaard, 2004. Abstract Background: Thrombotic thrombocytopenic purpura (TTP) is a rare haematological disease of unknown aetiology. This thrombotic microangiopathy is characterized by microvascular lesions with platelet aggregation. It is found in adults and can be associated with pregnancy, cancer, autoimmune diseases, bone marrow transplantation, drugs and bacterial as well as viral infections. The therapy requires a multi-disciplinary team approach involving dentistry. Even if TTP is immediately treated in an adequate manner, it still shows a mortality of up to 20%. Aim: To define a specific treatment concept for periodontal disease and decayed teeth in patients suffering from TTP based on the experiences gained from two cases. Conclusion: The two patient cases revealed a possible association of TTP with dental foci. Because of the severity and mortality of this disease, both prognosis evaluation Key words: dental foci; periodontal disease; and treatment standards of periodontologically compromised or decayed teeth have to systemic disease; thrombotic thrombocytopenic purpura be strictly followed in patients suffering from TTP. In order to avoid recurrence of TTP, it seems important to remove radically teeth of questionable prognosis. Accepted for publication 24 February 2004 Thrombotic thrombocytopenic purpura purpura, ecchymosis or spontaneous circulation and causes organ dysfunc- (TTP) is a life-threatening multi-system bleeding. Subsequently, haematuria tion like renal failure or hemiparesis by disease characterized by the classic pen- and renal failure occur. Neurological cerebral ischaemia. tad of thrombocytopenia, microangio- symptoms appear transiently and range The majority of TTP cases occur pathic haemolytic anaemia (MAHA), from headache and confusion to sei- without a known precipitating factor. neurological symptoms, progressive re- zures, hemiparesis and coma (Quasim & They are referred to as primary or nal failure and fever. Partridge 2001). idiopathic TTP. Secondary TTP shows The incidence of TTP is estimated to The aetiology and pathogenesis of an association with connective tissue be 3–7 cases per year per 1 million TTP are still not fully understood. Cur- disease, pregnancy, cancer and certain persons (Bell et al. 1991, Moake 1994). rently, it is assumed that an endothelial drugs like immunosuppressants. More- The reported mortality rate has drama- damage triggers processes leading to over, viral and bacterial infections have tically decreased during the past two microvascular lesions with platelet fi- been identified to be relevant aetiologi- decades. However, still up to 20% of the brin microthrombi occluding arterioles cal factors (Neild 1994, Creager et al. patients affected by this disease die and capillaries. Recent reports have 1998, Lara et al. 1999). A PUBMED despite adequate treatment (Quasim & shown that a deficiency regarding a query of the literature was performed to Partridge 2001). The clinical presenta- specific plasma protease (ADAMTS13) identify publications reporting an ex- tion may vary considerably as the being responsible for cleaving of the plicit association of TTP and dental classic pentad is seen only in less than von Willebrand factor plays a crucial infection (http://www.pubmed.de, date: half of the patients. A prodromal period role in microthrombi formation (Furlan 10–10–2003, keywords: TTP1foci/ resembling a viral, flue-like syndrome is et al. 1998, Tsai & Lian 1998, Remuzzi dental infection/oral infection/perio- common. Thrombocytopenia can lead to et al. 2002). The latter hinders micro- dontal). The recherche´ yielded no results. 1020 Fenner et al. Actually, none of the reports dealing The patient showed no history of Ultracain-DS 1:200,000, Aventis, Bad with TTP evaluates the role of dental bone marrow transplantation or intake Soden, Germany). A single-shot intra- foci in the occurrence and recurrence of of drugs like immunosuppressants caus- venous antibiotic prophylaxis with 10 the disease. Nevertheless, the detrimen- ing TTP. Different examinations were million IU penicillin G (Penicillin G tal effect of chronic dental infections on carried out to detect other factors that ‘‘Gru¨nenthal’’ 10 Mega, Gru¨nenthal, general health is well known. These are have been reported to be associated with Aachen, Germany) was administered considered to deteriorate the condition TTP. For detection of infectious foci perioperatively. At that time, platelet of medically compromised patients and cancerous lesions, a thorough phy- counts had normalized (285,000/ml). (Golder & Drinnan 1993, Meurman sical examination was carried out in- Surgical treatment involved removal of 1997). It has been shown that perio- cluding neurological and urological the five teeth showing mobility of grade dontal disease is a risk factor for status. Moreover, an ultrasound exam- II as well as scaling and root planing at atherosclerosis and other chronic dis- ination of the abdomen and a computed a single appointment. Excavation and eases (Seymour & Steele 1998, Garcia tomography of the chest were per- restoration of carious lesions were et al. 2001). As chronic diseases reveal formed. As no factors associated with performed at a second appointment. a slow progression over time, dental TTP could be detected, the patient was After the patient was discharged from treatment is focussed on preventive care scheduled for a dental examination by the hospital, he received further restora- to avoid dental infections. In contrast to the Department of Oral and Maxillofa- tive and prosthetic therapy and was chronic diseases, TTP exhibits a fulmi- cial Surgery of the University of Erlan- restored with a removable partial den- nant progression leading to death if it is gen-Nuremberg. Unfortunately, he ture. During a follow-up of 14 months, diagnosed or treated on delay (Rock et refused any further examination and there was no recurrence of TTP. al. 1991, Lau & Wun 1993). treatment. The patient was discharged Therefore, the aim of this presenta- from the hospital after a total of six tion is to highlight the differences plasma exchanges (platelet count in concepts of treatment of dental 285,000/ml) on day 8. At that time, all Case Report 2 foci between TTP and other systemic symptoms of TTP had disappeared. A 40-year-old non-smoking female was diseases. Ten days after discharge, the patient referred to the Department of Medicine again complained about increasing IV of the University of Erlangen- headache, nausea and arthralgies. On Nuremberg after a gynaecological ex- re-admission, the patient was disorien- amination because of massive vaginal Case Report 1 tated and showed a focal neurological bleeding. The patient reported a feeling A 51-year-old non-smoking male pre- deficit. Laboratory studies revealed a of lethargy and malaise for several days sented at the Department of Medicine platelet count of 4000/ml, as well as before the bleeding suddenly occurred. IV of the University of Erlangen- schistocytes in the peripheral blood The previous medical history was un- Nuremberg complaining of general ma- smear. The patient was diagnosed with eventful. laise and fever over a period of 1 week. a relapse of TTP and was transferred to On admission, the patient was dis- The patient reported two episodes of the ICU, where he received plasma orientated and developed cerebral palsy. thrombocytopenia that had occurred 1 exchange therapy for 10 days. At that Within a few hours, she became coma- and 2 years ago. There was no history of time, the patient was referred to the tose, and had to be put on a respirator. other general diseases. Department of Oral and Maxillofacial Physical examination revealed pete- On initial examination, the patient Surgery of the University of Erlangen- chiae and ecchymoses on the tibial was alert, oriented and did not show any Nuremberg. Intraoral examination re- regions as well as multiple carious le- signs of bleeding. Intraoral examination vealed poor oral hygiene, characterized sions, mobile teeth and desolate oral hy- showed multiple mobile and decayed by massive plaque deposits along the giene. Laboratory examinations showed teeth. Laboratory examinations revealed cervical margins of the dentition. In a platelet count of 4000/ml (reference a platelet count of 10,000/ml (reference both the mandible and maxilla, the range 140,000–400,000/ml), a haemo- range 140,000–400,000/ml) and a hae- gingiva displayed redness, swelling globin of 7.8 g/dl (reference range 12.0– moglobin value of 9.1 g/dl (reference and bleeding on gentle probing as well 15.0 g/dl) and schistocytes in the range 13.6–17.2 g/dl). Fragmented red as increased probing depth and attach- peripheral blood smear. TTP was diag- blood cells (schistocytes) were observed