Aspirin cures erythromelalgia and cerebrovascular disturbances in JAK2-thrombocythemia through platelet-cycloxygenase inhibition

Figure 16 Longterm observations in a 72-year-old woman with a three years history of burning painful red or blue toes and forefoot sole of the right foot (erythromelalgia) presented in December 1978 with spontaneous hemorrhages of large ecchymoses on the chest and subcutaneous hematotomas. Paradoxical occurrences of platelet mediated thrombosis and bleeding in this case with thrombocythemia (ET with features of PV in the bone marrow: Prodromal PV) 1978 peak 1 in the figure. The first episode of erythromelalgia for 3 years followed by simultaneous occurrence of thrombotic thrombocythemia and hemorrhagic thrombocythemia at platelet counts around 1100 × 10⁹/L in 1982. Aspirin was effective at peak 1 for the relief of erythromelalgia, which was associated with a further increase of platelet count to about 1500 × 10⁹/L. During periods of thrombocythemia at peak 1, 2, 3 and 4 busulphan induced complete remissions of thrombocythemia resulted in normal platelet counts below 400 × 10⁹/L, which was associated with no recurrence of erythromelalgia when not on aspirin. At peak 2 and 3 in the figure, recurrence of a second and third episode of thrombotic thrombocythemia occurred at platelet counts between 600 and 800 × 10⁹/L. In 1992 at peak 4 the patient suffered from an episode of thrombotic and hemorrhagic thrombocythemia at platelet counts of 1040 × 10⁹/L. Again aspirin relieved the erythromelalgia, which was associated with a further increase of platelet count to to around 1500 × 10⁹/L but the hemorrhagic manifestation persisted which associated with acquired von Willebrand factor deficiency type 2A (acquired von Willebrand syndrome: AVWS and disappeared after correction of platelet count to normal) (Figure 15).