Def.: The disease belongs to the myeloproliferative syndromes. It is characterized by an increased number of platelets and increased proliferation of megakaryocytes in the marrow.
Epid .: Diseases occur mainly between 50 and 60 years of age.
Etiol .: Unknown.
Clin.: Blood clots of small and large vessels, which are manifested by paresthesia, visual disturbances, seizures. Mucosal bleeding appears as a result of platelet dysfunction. Moderate enlargement of the spleen may occur in 20-50% of patients.
Hist.: In aspiration biopsy – increased cellularity with proliferation of the megakaryopoietic lineage and proliferation of megakaryocytes.
DL: Increased number, abnormal shape and size of platelets.
Treatment: ASA is used in patients without clinical symptoms of thrombocythemia, and when the platelet count exceeds 1 / µl, cytoreductive drugs are administered. In patients with thromboembolic disorders, hydroxycarbamide is used. If no improvement is achieved, anagrelide or INF-α should be added to treatment.
Year.: The survival time of properly treated patients is similar to that of the healthy population.
Lit .: [1] Sanchez S., Ewton A.: Essentials Th rombocythemia: a review of diagnostic and pathologic features. Arch Pathol Lab Med 2006, 130; 1144-50. [2] Andersson B.S.: Essentials thrombocythemia: diagnosis and treatment, with special emphasis on the use of Anagrelide. Hematology 2002, 7; 173-77.
Source: A. Kaszuba, Z. Adamski: “Lexicon of dermatology”; XNUMXst edition, Czelej Publishing House