This document discusses the case of a 23-year-old female diagnosed with essential thrombocythemia (ET). Her platelet count has been increasing over time and is now 2.6 million. She was previously evaluated for bone marrow biopsy 8 months ago which confirmed the diagnosis of ET. The document discusses further evaluating and managing her increasing platelet count.

1. Apichai Leelasiri, M.D. FACP Hematology Division Department of Medicine Phramongkutklao Hospital and College of Medicine How I Treat Polycythemia Vera & Essential Thrombocythemia

12. Provisional Dx Polycythemia vera

19. JAK2 Domains Levine RL and Gilliland DG. Blood 2008; 112: 2190-2198

20. JAK2: Wild-type & V617F Campbell PJ and Green AR. N Engl J Med 2006; 355: 2452-2466

21. Homozygosity of JAK2V617F Campbell PJ and Green AR. N Engl J Med 2006; 355: 2452-2466

24. PV: Major Minor Criteria Dx Swerdlow SH, et al, eds. Lyon, France. IARC Press; 2008: 18-65

35. Risk-Stratification in Polycythemia vera * HTN, DM, dyslipidemia, smoking, alteration of hemostatic factors ** Platelet > 1,500 x 10 9 /L Risk Category Age > 60 years or History of Thrombosis Generic Cardiovascular Risk Factors* Low No No Intermediate** No Yes High Yes Not applicable

36. Management of Polycythemia vera by Risk factors * Anagrelide or IFN alpha may be used with phlebotomy in selected cases Risk Category Risk Factors Management Low Age < 60 and no prior cardiovascular event Phlebotomy + low dose aspirin Intermediate* Generic cardiovascular risk factors High Age > 60 and/or prior cardiovascular events Myelosuppression + phlebotomy + low dose aspirin

62. ET: WHO 2008 Criteria Dx All 4 criteria must be met Swerdlow SH, et al, eds. Lyon, France. IARC Press; 2008: 18-65

69. Atypical Megakaryocytes

70. Atypical and Micromegakaryocytes

71. Risk-Stratification in Essential Thrombocythemia * HTN, DM, dyslipidemia, smoking, alteration of hemostatic factors ** Platelet > 1,500 x 10 9 /L Risk Category Age > 60 years or History of Thrombosis Generic Cardiovascular Risk Factors* Low No No Intermediate** No Yes High Yes Not applicable

72. Management of Essential Thrombocythemia by Risk factors * anagrelide, HU or IFN alpha may be used in stead of observation Risk Category Risk Factors Management Low Age < 60 and no prior cardio vascular event Nil or low-dose aspirin (no consensus) Intermediate* Generic cardiovascular risk factors Low-dose aspirin (no consensus) High Age > 60 and/or prior cardio vascular events Myelosuppression + low-dose aspirin

85. Role of HSCT in MPNs

87. Impact of P rimary D iagnosis on T ransplantation O utcome Kerbauy DM, et al. Biol Blood Marrow Transplant 2007: 13: 355-65

89. Thank you for Attention

110. Stem Cell Differentiation Campbell PJ and Green AR. N Engl J Med 2006; 355: 2452-2466

111. Pre-JAK2 Mutation Levine RL and Gilliland DG. Blood 2008; 112: 2190-2198

117. PMF: Major Minor Criteria Dx All 3 major + 2 minor Swerdlow SH, et al, eds. Lyon, France. IARC Press; 2008: 18-65

118. Levine RL and Gilliland DG. Blood 2008; 112: 2190-2198

119. Vannucchi AM, et al. CA Cancer J Clin 2009; 59: 171-191