SummaryErythromelalgia, a characteristic aspirin-responsive microvascular thrombotic complication in essential thrombocythemia (ET), may develop despite oral anticoagulant treatment or treatment with heparin, suggesting that the generation of thrombin is not a prerequisite for its development. To study this, a cross-sectional comparison of the plasma levels of thrombomodulin (TM), platelet factor 4 (PF4), β-thrombo-globulin (β-TG), prothrombin fragment 1+2 (F1+2) and total degradation products of fibrin(ogen) (TDP) was made between 5 ET patients suffering from erythromelalgia, 16 asymptomatic ET patients and 20 control subjects, and after treatment with aspirin, respectively. Furthermore, 2 ET patients with a history of erythromelalgia were studied at regular time intervals after discontinuation of aspirin until erythromelalgia recurred. As compared with asymptomatic ET patients and control subjects erythromelalgia was characterized by significantly higher β-TG and TM levels but no significant differences were detected in either F1+2 or TDP levels. Treatment of erythromelalgia with aspirin resulted in disappearance of erythromelalgic signs and symptoms, which was paralleled by a significant decrease of β-TG and TM levels. Histopathologic and immunohistochemical analysis of biopsies derived from erythromelalgic skin areas of 2ET patients showed that erythromelalgic thrombi stained positively for von Wille-brand factor opposed to only a weak fibrin staining. Our data suggest that erythromelalgia is caused by the intravascular activation and aggregation of platelets with subsequent sludging or occlusion of the acral arterial microvasculature. The generation of thrombin appears not to be essential for the formation of these platelet thrombi, thereby giving a plausible explanantion for the inefficacy of coumadin derivatives and heparin in the prevention and treatment of erythromelalgia in essential thrombocythemia.
Summary. Patients with essential thrombocythaemia (ET)exhibit a decrease of large von Willebrand factor (VWF) multimers in plasma, which is inversely related to the platelet count. In the present study we investigated whether the decrease of large VWF multimers in plasma with increasing platelet counts is the consequence of increased turnover of large VWF multimers in vivo. To that end we measured the half-life times of endogenously released VWF:Ag and VWF:CBA (collagen binding activity) after intravenous administration of desmopressin (DDAVP) to nine ET patients and nine control subjects (N). In addition, the half-life times of VWF:Ag and VWF:CBA were also measured in four ET patients after cytoreduction of the increased platelet count to normal or nearly normal values. Estimated half-life times of VWF:Ag did not differ between ET patients and normals (11·0 Ϯ 4·0 h v 12·4 Ϯ 2·5 h, P > 0·05). Estimated half-life times of VWF:CBA were significantly lower in ET patients as compared with normal individuals (6·1 Ϯ 2·0 h v 8·4 Ϯ 2·5 h, P < 0·05). After cytoreduction of the increased platelet count to (nearly) normal values in all four ET patients the half-life time of VWF:CBA significantly (P ¼ 0·014) increased from 5·2 Ϯ 1·2 h to 8·7 Ϯ 2·0 h. Our data suggest that platelets may play a role in the homeostasis of circulating von Willebrand factor, which may compromise normal haemostasis at fairly increased platelet counts.
ObjectiveKetamine is a drug that can effectively treat neuropathic pain by blocking the N-methyl-D-aspartate receptor. It has been studied as a supplement to opioids for cancer pain, but its effectiveness for non-cancer pain is still limited. However, despite its usefulness in managing refractory pain, ketamine is not commonly used for home-based palliative care.MethodsA case report of a patient with severe central neuropathic pain who was treated with a subcutaneous continuous infusion of morphine and ketamine at home.ResultsThe introduction of ketamine in the patient’s treatment plan effectively controlled pain. Only one possible ketamine side effect was observed and easily treated with pharmacological and non-pharmacological measures.ConclusionsWe have found success in using subcutaneous continuous infusion of morphine and ketamine to alleviate severe neuropathic pain in a home setting. We also observed a positive impact on the patient’s family members' personal, emotional and relational well-being after ketamine was introduced.
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