BACKGROUND: Distinguishing inherited thrombocytopenias from immune thrombocytopenia (ITP) can be difficult, and patients are therefore at risk of misdiagnosis and inappropriate treatments. Although it is known that the most common inherited forms of thrombocytopenia are characterized by increased platelet size, the diagnostic power of this feature has never been investigated. OBJECTIVES: The aim of this study was to test the hypothesis that platelet size can be used to differentiate ITP from inherited macrothrombocytopenias. PATIENTS/ METHODS: We measured mean platelet volume (MPV) and mean platelet diameter (MPD), within 2 h of blood sampling, in 35 patients with inherited macrothrombocytopenias [15 MYH9-related disease (MYH9-RD), three biallelic and 17 monoallelic Bernard-Soulier syndrome (BSS)], and 56 with ITP. Using receiving operating characteristic analysis, we searched for the best cut-off values to differentiate between these conditions. RESULTS: As expected, platelets were larger in inherited macrothrombocytopenias than in ITP. An MPD larger than 3.3 mum differentiated MYH9-RD and BSS from ITP with 0.89 sensitivity and 0.88 specificity, and an MPV larger than 12.4 fL had 0.83 sensitivity and 0.89 specificity. Combining MPD with MPV increased sensitivity and specificity to 0.97 and 0.89, respectively. CONCLUSION: Platelet size evaluation by both an appropriate cell counter and blood film examination is useful for differentiating inherited macrothrombocytopenias from ITP.
BACKGROUND: Distinguishing inherited thrombocytopenias from immune thrombocytopenia (ITP) can be difficult, and patients are therefore at risk of misdiagnosis and inappropriate treatments. Although it is known that the most common inherited forms of thrombocytopenia are characterized by increased platelet size, the diagnostic power of this feature has never been investigated. OBJECTIVES: The aim of this study was to test the hypothesis that platelet size can be used to differentiate ITP from inherited macrothrombocytopenias. PATIENTS/ METHODS: We measured mean platelet volume (MPV) and mean platelet diameter (MPD), within 2 h of blood sampling, in 35 patients with inherited macrothrombocytopenias [15 MYH9-related disease (MYH9-RD), three biallelic and 17 monoallelic Bernard-Soulier syndrome (BSS)], and 56 with ITP. Using receiving operating characteristic analysis, we searched for the best cut-off values to differentiate between these conditions. RESULTS: As expected, platelets were larger in inherited macrothrombocytopenias than in ITP. An MPD larger than 3.3 mum differentiated MYH9-RD and BSS from ITP with 0.89 sensitivity and 0.88 specificity, and an MPV larger than 12.4 fL had 0.83 sensitivity and 0.89 specificity. Combining MPD with MPV increased sensitivity and specificity to 0.97 and 0.89, respectively. CONCLUSION: Platelet size evaluation by both an appropriate cell counter and blood film examination is useful for differentiating inherited macrothrombocytopenias from ITP.
Authors: Roberta Bottega; Alessandro Pecci; Erica De Candia; Nuria Pujol-Moix; Paula G Heller; Patrizia Noris; Daniela De Rocco; Gian Marco Podda; Ana C Glembotsky; Marco Cattaneo; Carlo L Balduini; Anna Savoia Journal: Haematologica Date: 2012-10-25 Impact factor: 9.941
Authors: Patrizia Noris; Ginevra Biino; Alessandro Pecci; Elisa Civaschi; Anna Savoia; Marco Seri; Federica Melazzini; Giuseppe Loffredo; Giovanna Russo; Valeria Bozzi; Lucia Dora Notarangelo; Paolo Gresele; Paula G Heller; Nuria Pujol-Moix; Shinji Kunishima; Marco Cattaneo; James Bussel; Erica De Candia; Claudia Cagioni; Ugo Ramenghi; Serena Barozzi; Fabrizio Fabris; Carlo L Balduini Journal: Blood Date: 2014-07-02 Impact factor: 22.113