Thromkid – a Competence Network for Functional Platelet Abnormalities

Abstract

Hereditary disorders of platelet function are often complex. Bleeding due to defective platelet function may be intermittent and unpredictable. Only few guidelines concerning testing for platelet function and treatment of affected patients have been developed so far. These experiences prompted the authors to initiate a collaborative patterns- of-practice survey and to establish a competence network in order to improve diagnosis and treatment of affected patients in German speaking countries (Thromkid quality project). Materials and Methods: Data were obtained by using standardized survey protocols between May 2005 and August 2006. Results: A total of 50 centers were identified. 88% agreed to participate in Thromkid. The laboratories offered a median of 4 tests. All laboratories offered platelet aggregometry. 28 laboratories used the PFA-100® closure time. Further widely used tests were the in vivo bleeding time and flow cytometry. 215 affected patients were identified 123 of whom fulfilled the authors’ set of criteria, 43% were considered poorly characterized. Morbus Glanzmann, aspirin-like defects, and receptor defects were the most common diagnoses. Discussion: Future activities include i) to establish a web-based register of all centers, ii) to establish a patient registry, and iii) to prepare the basis for well-designed clinical studies resulting in rational and clinically useful guidelines for diagnosis and treatment of affected patients.

Strategies in Clinical and Laboratory Diagnosis of Inherited Platelet Function Disorders in Children

“…Light transmission aggregometry (LTA) is poorly standardised and requires relatively large volumes of freshly drawn blood, but is still considered the 'gold standard' for the evaluation of platelet function; this was confirmed by the THROM-KID Quality Project [6,24]. Recently, the Clinical and Laboratory Standards Institute (CLSI), the Platelet Physiology Subcommittee of the SSC of the International Society on Thrombosis and Haemostasis (ISTH), and the North American Specialised Coagulation Laboratory Association (NAS-COLA) published extensive surveys and guidelines on the standardisation of LTA [25][26][27].…”

“…Presented information includes data from the THROMKID Quality Project of the Permanent Paediatric Group of the German Thrombosis and Haemostasis Research Society (GTH). The main part of this study was a collaborative patterns-of-practice survey intended to overview diagnosis and treatment of patients with functional platelet abnormalities in Austria, Germany, and Switzerland [6,7].…”

Inherited Disorders of Platelet Function in Pediatric Clinical Practice: A Diagnostic Challenge

“…Diagnosis of inherited disorders of platelet function in clinical settings is still cumbersome: 1) most analyses are restricted to freshly drawn and large volumes of blood samples; 2) platelet testing is time-consuming and in general not readily available and often poorly standardized in routine laboratories; and 3) many new techniques are reserved to a very limited number of highly specialized centres. Only recently, task forces of the ISTH, GTH and other scientifi c societies have made a huge eff ort to develop guidelines and meaningful recommendations for the diagnosis of patients with inherited platelet disorders [2,8,9,27,29] . It is uncertain whether any of these guidelines or recommendations have been adopted into clinical practice.…”

Cellular Haemostaseology – Diagnosis and Therapeutic Monitoring of Inherited and Acquired Functional Platelet Disorders

“…The principle of action is described in detail, allowing a sound estimation of the currently available and evolving methods to diagnose platelet function disorders. Fi-nally, Werner Streif together with Martin Olivieri, Stefan Weickardt, Wolfgang Eberl, Ralf Knoefler [5] give a very informative review on the results of the Thromkid competence network for functional platelet abnormalities of the Paediatric Group of the German Thrombosis and Haemostasis Research Society (GTH). The survey is based on the German-speaking population and allows for the first time a thorough insight in the commonly used methods and diagnostic strategies applied for the detection of functional platelet disorders.…”