A 45 year old female patient presents with symptoms of easy bruisability and frequent headaches. Physical examination show a moderate splenomegaly. Blood counts shows a normal leucocyte count and a platelet count of 1000 x 103/cu mm. The leucocyte alkaline phosphatase score is normal. Which one of the following is the drug of choice for the treatment of this patient?
Answer is C (Anagrelide ) This is a case of essential thrombocytosis. Interferon a and Anagrelide are the recommended treatment modalities for reducing platelet count in this disorder. Previous editions of Harrisons and most other texts recommend hydroxyurea as the standard modality of treatment, but in view of the text mentioned in Harrisons 16th ,hydroxyurea cannot be picked as the answer. Essential thrombocytosis (essential thrombocythaemia; idiopathic thrombocvtosis; hemorrhagic thrombocythaemia) Essential thrombocytosis is a myeloproliferative disorder characterized by overproduction of platelets without a definable cause. Clinical features Laboratory Diagnostic features Symptoms * Elevated platelet count is hallmark (often >1000x103/ uL) * Haematocrit and RBC morphology normal * WBC count is mildly elevated/normal * Haemorrhagic tendencies : Easy bruising deg * Thrombotic tendencies : Microvascular occlusions - Erythromelalgia (mild neutrophillic leucocytosis) - Migraine (headache) * LAP is normal or elevated - Transient ischemic attacks * Philadelphia chromosome is absent Signs * Splenomegaly : usually mild/moderate massive splenomegaly is more characteristic ()1 other myeloproliferative disorders. Management in asymptomatic patient Management in symptomatic patient Elevated platelet count in asymptomatic patient Elevated platelet count in symptomatic patients * No therapy is recommended because agents used in treatment * Symptoms must be clearly identified to be a consequence of place the patient at risk of developing acute leukemia elevated platelet count Therapy with risk of acute leukemia Platelet reduction * Radioactive phosphorus * IFN a * Hydroxyuria * Anagrelide * Alkylating agents * Hydroxyurea: should be considered only if the above agents are not effective or tolerable .- Harrison
Hydroxyurea
Radioactive phosphorus
Anagrelide
None