Erythromelalgic Thrombotic Thrombocythemia (ETT) and Hemorrhagic Trombocythemia (HT) in Patients with Essential Thrombocythaemia (ET) and Polycythaemia Vera (PV)

Abstract

Citation: Michiels JJ (2014) Erythromelalgic Thrombotic Thrombocythemia (ETT) and Hemorrhagic Trombocythemia (HT) in Patients with Essential Thrombocythaemia (ET) and Polycythaemia Vera (PV). J Hematol Thromb Dis 2: 169. AbstractWe analysed between 1908 and 1985 of two hundred cases of thrombocythemia from the literature between 1908 and 1985. Erythromelalgic, acrocyanotic ischemia and digital gangrene, cerebral or coronary ischemic events (erythromelalgic thrombotic thrombocyhemia: ETT) in 99 thrombocythemia patients (essential thrombocythemia: ET N=67 and polycythemia vera: PV N=32) occured at platelet counts in excess of 400 × 10 9 /L. Hemorrhagic complications at time of presentation of hemorrhagic thrombocythemia (HT) in 100 HT patients included melana and/or hematemesis, skin bleedings (bruises, subcutaneous hematomas, painfull hematomas after trauma, ecchymoses, but not petechiae, epistaxis and gum bleeding and secondary bleeding after trauma or surgery. ETT occurred at an early stage of thrombocythemia with a mean platelet count of 1110+477 × 10 9 /L, whereas HT occurred at significantly higher platelet counts in excess of 1000 × 10 9 /L with a mean platelet count of 2016+1070 × 10 9 /L. HT patients frequently had a previous history of ETT or paradoxical occurrences of both thrombosis and bleeding (ETT/PHT). The degree of thrombocythosis determined the sequential occurrence of ETT, HT in ET and PV patients. Hypersensitive thrombocythemic platelets (sticky platelets) are involved in the etiology of platelet-mediated thrombosis at platelet counts above 400 × 10 9 /L whereas bleedings at high platelet count is related to an acquired von Willebrand syndrome processes on top of a blood clot retraction disturbance with erythrocyte fall out, that causes painful subcutaneous hematomas with a central swelling (clot) after a blow, trauma and secondary bleeding after surgery in thrombocythemia patients. Jo u r n a l o f Hema to lo g y & Thro m b o e m bolic D is e a s es

Erythromelalgic Microvascular Disturbances, Major Thrombosis and Hemorrhagic Manifestations of Thrombocythemia in Patients with Essential Thrombocythemia and Polycythemia Vera: Therapeutic Implications

“…Correction of the platelet counts to normal is associated with no recurrences of microvascularevents and complete correction of the VWF-multimeric pattern and correction of all VWF-parameters to complete normal values. The study of Michiels et al [3][4][5][6][7][8][9][10][11][31][32][33][34] and Van Genderen et al [23][24][25][26][27] served as the scientific rationale for the ECLAP (European Collaboration on Low-dose Aspirin in PV) study design (1997) [33]. The ECLAP study nicely confirmed our concept of the effectiveness and safety of low dose aspirin in ET and PV in particular [38,39].…”

“…The bleeding manifestations in 100 HT cases ranged from gastrointestinal chronic occult blood loss, melena and hematemesis to mucocutaneous bruises, hematomas, ecchymoses, gum bleedings and secondary bleeding [33][34][35]. HT was usually associated with significant leukocytosis and splenomegaly and the platelet count at time of bleeding in 100 HT cases ranged from 800 to above 4000x10 9 [36,37] independent from the principal investigator (Michiels).…”

“…The risk stratification for thrombosis as low, intermediate and high thrombotic risk by Cortelazzo et al in 199040 has been derived from a historical cohort of 100 ET patients not treated with aspirin thereby overlooking the original and updated key references of Michiels et al [3,4,11,[31][32][33][34][35][36][37][38]41] on the demonstration of aspirin responsive plateletmediated arteriolar and arterial thrombotic tendency inherent to thrombocythemia in ET and PV patients.…”

“…In this prospective observational Rotterdam Total dose and treatment duration of busulphan courses and remission duration after discontinuation of busulphan in ET patients [31,32]. 250x10 9 /L in PV patients ( Figure 14) [33][34][35]. [36,37].…”

“…In 1980 [33][34][35] Michiels reviewed 100 case histories of primary hemorrhagic thrombocythemia (HT) and 99 cases of erythromelalgic thrombotic thrombocythemia (ETT, 67ET and 32 PV). The bleeding manifestations in 100 HT cases ranged from gastrointestinal chronic occult blood loss, melena and hematemesis to mucocutaneous bruises, hematomas, ecchymoses, gum bleedings and secondary bleeding [33][34][35].…”