Features of essential thrombocythaemia in childhood: a study of five children

Essential thrombocythaemia (ET) is usually considered a disease of the middle‐aged but, with the advent of automated platelet counting, ET is diagnosed with increasing frequency in young adults and, even more rarely, in children. We report five paediatric patients (four girls and one boy, mean age 8...

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Published in:British journal of haematology 2000-01, Vol.108 (1), p.86-89
Main Authors: RANDI, M. L, PUTTI, M. C, FABRIS, F, SAINATI, L, ZANESCO, L, GIROLAMI, A
Format: Article
Language:English
Subjects:
Biological and medical sciences
Bone Marrow Cells - pathology
Child
childhood
chronic myeloproliferative disorders
essential thrombocythaemia
Female
Headache - etiology
Hematologic and hematopoietic diseases
Hematology
Humans
Infant
Male
Medical sciences
Platelet Count
Platelet diseases and coagulopathies
Thrombocythemia, Essential - complications
Thrombocythemia, Essential - pathology
thrombocytosis
Thrombosis - etiology
Vena Cava, Inferior
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Summary:Essential thrombocythaemia (ET) is usually considered a disease of the middle‐aged but, with the advent of automated platelet counting, ET is diagnosed with increasing frequency in young adults and, even more rarely, in children. We report five paediatric patients (four girls and one boy, mean age 89 months) diagnosed with ET in agreement with Polycythaemia Vera Study Group criteria. The patients had a persistent thrombocytosis over 900 × 109/l and, at the time of diagnosis, their platelet count ranged between 1112 and 3178 × 109/l. A 9‐month‐old girl had thrombosis of the inferior cava vein, two children had headaches and two others remained asymptomatic throughout the follow‐up period. Megakaryocytes in the bone marrow were increased in number. The chromosomal analysis was normal, and bcr rearrangement was always negative. None of the patients had spontaneous BFU‐E or altered levels of serum erythropoietin and thrombopoietin. Two patients showed alteration of platelet aggregation, and all had decreased levels of intraplatelet serotonin. In spite of the diagnosis of ET in our patients, we are not sure that the cases reported here are really myeloproliferative disorders. The features could suggest that the cases observed may be affected by an ‘idiopathic thrombocytosis’ without myeloproliferation. Possible variants of ET are described in young adults, and the heterogeneous nature of ET is also suggested by our paediatric patients. Only careful long‐term follow‐up of patients such as these will clarify the natural history of these disorders and suggest therapeutic management.
ISSN:0007-1048
1365-2141
DOI:10.1046/j.1365-2141.2000.01817.x