Pathophysiology/etiology

Santhosh-Kumar CR, Yohannan MD, Higgy KE, al-Mashhadani SA.

Thrombocytosis in adults: analysis of 777 patients.
J Intern Med. 1991;229:493-5.
Description: Prospective case-series of 777 patients with thrombocytosis. Provides prospective data on the most frequent causes of thrombocytosis.
PubMed citation number: 2045755

Fialkow PJ, Faguet GB, Jacobson RJ, et al.

Evidence that essential thrombocythemia is a clonal disorder with origin in a multipotent stem cell.
Blood. 1981;58:916-9.
Description: This paper exploited the X-linked G6PD locus in female patients with essential thrombocythemia to demonstrate the clonal nature of the disorder.
PubMed citation number: 7296002

Diagnosis

Murphy S, Peterson P, Iland H, Laszlo J.

Experience of the Polycythemia Vera Study Group with essential thrombocythemia: a final report on diagnostic criteria, survival, and leukemic transition by treatment.
Semin Hematol. 1997;34:29-39.
Description: A review of the original and up-dated diagnostic criteria for ET.
PubMed citation number: 9025160

Treatment

Cortelazzo S, Finazzi G, Ruggeri M, et al.

Hydroxyurea for patients with essential thrombocythemia and a high risk of thrombosis.
New Engl J Med. 1995;332:1132-6.
Description: RCT of hydroxyurea versus no hydroxurea to maintain platelet count of < 600,000/mm3 in high-risk patients with ET. Hydroxyurea significantly decreased the incidence of thromboembolic complications.
PubMed citation number: 7700286

Lengfelder E, Griesshammer M, Hehlmann R.

Interferon-alpha in the treatment of essential thrombocythaemia.
Leuk Lymphoma. 1996;22:135-142.
Description: Review of the published literature on IFN and ET.
PubMed citation number: 8951784

Ruggeri M, Finazzi G, Tosetto A, et al.

No treatment for low-risk thrombocythaemia: results from a prospective study.
Br J Haematol. 1998;103:772-7.
Description: Prospective cohort study of low-risk patients with essential thrombocythaemia (ET) treated expectantly. Demonstrated that in this population conservative management is a safe strategy.
PubMed citation number: 9858229

Finazzi G, Ruggeri M, Rodeghiero F, et al.

Second malignancies in patients with essential thrombocythaemia treated with busulphan and hydroxyurea: long-term follow-up of a randomized clinical trial.
Br J Haematol. 2000;110:577-83.
Description: Long-term follow-up of the RCT with hydroxyurea. At a median f/u of 73 months hydroxyurea versus control continued to very significantly decrease the risk of a thromboembolic event. However, 3.9% of the patients treated with hydroxyurea alone and 33% of patients treated with busulfan followed by hydroxyurea developed neoplasia. None of the patients who never received chemotherapy developed neoplasia.
PubMed citation number: 10997967

Storen EC, Tefferi A.

Long-term use of anagrelide in young patients with essential thrombocythemia.
Blood. 2001;97:863-866
Description: A retrospective case series of 35 young patients with ET treated with anagrelide for 11 years. With anagrelide patients had excellent control of the platelet count (sustained normalization in 54% of patients). 20% of the patients had thrombotic complications, 20% had hemorrhagic complications. Complications tended to occur at a platelet count of > 400. Progressive anemia was noted with anagrelide.
PubMed citation number: 11159509

Wright CA, Tefferi A.

A single institutional experience with 43 pregnancies in essential thrombocythemia.
Eur J Haematol. 2001;66:152-9.
Description: A case series that reports that 1/3 of pregnancies in patients with ET end in spontaneous abortion and that this incidence is not decreased by treating the ET.
PubMed citation number: 11350483

General reviews

Harrison CN, Green AR.

Essential thrombocythemia.
Hematol Oncol Clin N Am. 2003;17:1175-90.
Description: Review of the pathobiology, clinical presentation, diagnosis and management of ET.
PubMed citation number: 14560781

Schafer, AI. Thrombocytosis.

New Engl J Med. 2004;350:1211-9.
Description: Concise review paper of the approach to an elevated platelet count and the management of ET.
PubMed citation number: 15028825

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