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International Journal of Hematology
Published in: International Journal of Hematology 6/2012

01-12-2012 | Case Report

Two cases of pediatric essential thrombocythemia managed effectively with hydroxyurea

Authors: Vikas Dua, Satya Prakash Yadav, Vijay Kumar, Renu Saxena, Anupam Sachdeva

Published in: International Journal of Hematology | Issue 6/2012

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Abstract

Thrombocytosis is common in infancy and childhood. Essential thrombocythemia (ET), a myeloproliferative disorder, is a much less common cause of thrombocytosis in childhood. We report two cases of essential thrombocythemia in 5- and 10-year-old children, who presented with platelet counts of more than 1,000,000/mm3. Treatment is not recommended for ET in an asymptomatic patient in the absence of bleeding or thrombosis and a platelet count <1,500,000/mm3. Our first case had platelets >1,500,000/mm3, and a second child was symptomatic with recurrent headache. Both responded well to therapy with hydroxyurea (dose 15–30 mg/kg/day) and tolerated it well.
Literature
1.
2.
Denton A, Davis P. Extreme thrombocytosis in admissions to paediatric intensive care: no requirement for treatment. Arch Dis Child. 2007;92(6):515–6.PubMedCrossRef
3.
Yohannan MD, Higgy KE, al-Mashhadani SA, Santhosh-Kumar CR. Thrombocytosis. Etiologic analysis of 663 patients. Clin Pediatr (Phila).1994;33(6):340–3.
4.
Othman N, Isaacs D, Kesson A. Mycoplasma pneumoniae infections in Australian children. J Paediatr Child Health. 2005;41(12):671–6.PubMedCrossRef
5.
6.
7.
El-Harith el-HA, Roesl C, Ballmaier M, Germeshausen M, Frye-Boukhriss H, von Neuhoff N, et al. Familial thrombocytosis caused by the novel germ-line mutation p.Pro106Leu in the MPL gene. Br J Haematol. 2009;144(2):185–94.
8.
Spivak JL, Silver RT. The revised World Health Organization diagnostic criteria for polycythemia vera, essential thrombocytosis, and primary myelofibrosis: an alternative proposal. Blood. 2008;112(2):231–9.PubMedCrossRef
9.
Chen HL, Chiou SS, Sheen JM, Jang RC, Lu CC, Chang TT. Thrombocytosis in children at one medical center of southern Taiwan. Acta Paediatr Taiwan. 1999;40(5):309–13.PubMed
10.
11.
12.
Randi ML, Putti MC, Scapin M, Pacquola E, Tucci F, Micalizzi C, et al. Pediatric patients with essential thrombocythemia are mostly polyclonal and V617FJAK2 negative. Blood. 2006;108(10):3600–2.PubMedCrossRef
13.
Nakatani T, Imamura T, Ishida H, Wakaizumi K, Yamamoto T, Otabe O, et al. Frequency and clinical features of the JAK2 V617F mutation in pediatric patients with sporadic essential thrombocythemia. Pediatr Blood Cancer. 2008;51(6):802–5.PubMedCrossRef
14.
Gangat N, Tefferi A, Thanarajasingam G, Patnaik M, Schwager S, Ketterling R, et al. Cytogenetic abnormalities in essential thrombocythemia: prevalence and prognostic significance. Eur J Haematol. 2009;83(1):17–21.PubMedCrossRef
15.
Barbui T. What is the standard treatment in essential thrombocythemia. Int J Hematol. 2002;76(Suppl 2):311–7.PubMedCrossRef
16.
Randi ML, Putti MC. Essential thrombocythaemia in children: is a treatment needed? Expert Opin Pharmacother. 2004;5(5):1009–14.PubMedCrossRef
17.
Landolfi R, Di Gennaro L. Prevention of thrombosis in polycythemia vera and essential thrombocythemia. Haematologica. 2008;93(3):331–5.PubMedCrossRef
18.
Chintagumpala MM, Kennedy LL, Steuber CP. Treatment of essential thrombocythemia with anagrelide. J Pediatr. 1995;127(3):495–8.PubMedCrossRef
19.
Cortelazzo S, Finazzi G, Ruggeri M, Vestri O, Galli M, Rodeghiero F, et al. Hydroxyurea for patients with essential thrombocythemia and a high risk of thrombosis. N Engl J Med. 1995;332(17):1132–6.PubMedCrossRef
20.
Kinney TR, Helms RW, O’Branski EE, Ohene-Frempong K, Wang W, Daeschner C, Vichinsky E, Redding-Lallinger R, Gee B, Platt OS, Ware RE. Safety of hydroxyurea in children with sickle cell anemia: results of the HUG-KIDS study, a phase I/II trial Pediatric Hydroxyurea Group. Blood. 1999;94(5):1550–4.PubMed
21.
Heft VN, Fruchtman SR, Pickens CV, Rosse WF, Howard TA, Ware RE. Acquired DNA mutations associated with in vivo hydroxyurea exposure. Blood. 2000;95(11):3589–93.
22.
23.
Radaelli F, Onida F, Rossi FG, Zilioli VR, Colombi M, Usardi P, et al. Second malignancies in essential thrombocythemia (ET): a retrospective analysis of 331 patients with long-term follow-up from a single institution. Hematology. 2008;13(4):195–202.PubMedCrossRef
24.
Mavrogianni D, Viniou N, Michali E, Terpos E, Meletis J, Vaiopoulos G, et al. Leukemogenic risk of hydroxyurea therapy as a single agent in polycythemia vera and essential thrombocythemia: N- and K-ras mutations and microsatellite instability in chromosomes 5 and 7 in 69 patients. Int J Hematol. 2002;75(4):394–400.PubMedCrossRef
25.
Björkholm M, Derolf AR, Hultcrantz M, Kristinsson SY, Ekstrand C, Goldin LR, et al. Treatment-related risk factors for transformation to acute myeloid leukemia and myelodysplastic syndromes in myeloproliferative neoplasms. J Clin Oncol. 2011;29(17):2410–5.PubMedCrossRef
26.
Mizutani S, Kuroda J, Shimizu D, Horiike S, Taniwaki M. Emergence of chronic myelogenous leukemia during treatment for essential thrombocythemia. Int J Hematol. 2010;91(3):516–21.PubMedCrossRef
27.
Mialou V. Essential thrombocythemia in childhood: the French experience. Pediatr Blood cancer (SIOP abstract). 2005;45:PU005.
28.
El-Moneim AA, Kratz CP, Böll S, Rister M, Pahl HL, Niemeyer CM. Essential versus reactive thrombocythemia in children: retrospective analyses of 12 cases. Pediatr Blood Cancer. 2007;49(1):52–5.PubMedCrossRef
Metadata
Title
Two cases of pediatric essential thrombocythemia managed effectively with hydroxyurea
Authors
Vikas Dua
Satya Prakash Yadav
Vijay Kumar
Renu Saxena
Anupam Sachdeva
Publication date
01-12-2012
Publisher
Springer Japan
Published in
International Journal of Hematology / Issue 6/2012
Print ISSN: 0925-5710
Electronic ISSN: 1865-3774
DOI
https://doi.org/10.1007/s12185-012-1193-8

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