Percorsi clinici
Piastrinopenia immunomediata in età adolescenziale ed evoluzione in lupus eritematoso sistemico
Immune thrombocytopenia in adolescents and evolution to systemic lupus erythematosus: clinical reasoning through the description of three cases
Giorgio Costagliola, Rita Consolini
UO di Pediatria, Servizio di Immunologia Clinica e di Laboratorio, Università di Pisa
Febbraio 2018 - pagg. 97 -100
Abstract
Thrombocytopenia, defined by a platelet count lower than 100,000 mm3, in children and
adolescents is more frequently ascribable to an immune pathogenesis (Immune Thrombocytopenia,
or ITP). ITP is defined “persistent” if it is still present after three months from diagnosis,
and “chronic” if remission is not reached after 12 months from the first finding. ITP in
childhood usually follows infections of the upper respiratory tract, and presents a self-limiting
clinical course, while in adolescents chronic forms are more frequent and require a complex
clinical management. The present paper focuses on chronic ITP in adolescents, introducing
three cases in which the finding of thrombocytopenia preceded by several years the diagnosis
of Systemic Lupus Erythematosus. From the analysis of the presented cases and literature
data appears that, even if it is not possible to predict the progression to SLE, patients not responsive
to conventional therapies typically developed this condition. Furthermore, the paper
suggests an approach that is useful for the confirmation of clinical suspect of ITP and for the
surveillance of patients with a high risk of developing the chronic form, in order to identify
among them those that will evolve to systemic autoimmune diseases.
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Bibliografia
1. Yu C, Gershwin ME, Chang C. Diagnostic
criteria for systemic lupus erythematosus: a
critical review. J Autoimmun 2014;48-49:10-3.
2. Bryant N, Watts R. Thrombocytopenic syndromes
masquerading as childhood immune
thrombocytopenic purpura. Clin Pediatr (Phila)
2011;50(3):225-30.
3. Patuzzo G, Barbieri A, Tinazzi E, et al. Autoimmunity
and infection in common variable
immunodeficiency (CVID). Autoimmun Rev
2016;15(9):877-82.
4. Thomas AS, Mehta AB, Hughes DA. Diagnosing
Gaucher disease: an on-going need
for increased awareness amongst haematologists.
Blood Cells Mol Dis 2013;50(3):212-7.
5. Consolini R, Calleri A, Macchia P. La conta
delle piastrine reticolate: metodologia e applicazioni
cliniche. Riv Ital Pediatr 1999;25:287-93.
6. Stasi R, Willis F, Shannon MS, Gordon-
Smith EC. Infectious causes of chronic immune
thrombocytopenia. Hematol Oncol Clin
North Am 2009;23(6):1275-97.
7. Provan D, Stasi R, Newland AC, et al. International
consensus report on the investigation
and management of primary immune thrombocytopenia.
Blood 2010;115(2):168-86.
8. Cooper N. A review of the management of
childhood immune thrombocytopenia: how
can we provide an evidence-based approach?
Br J Haematol 2014;165(6):756-67.
9. Rodeghiero F, Stasi R, Gernsheimer T, et
al. Standardization of terminology, definitions
and outcome criteria in immune thrombocytopenic
purpura of adults and children: report
from an international working group. Blood
2009;113(11):2386-93.
10. Lowe EJ, Buchanan GR. Idiopathic thrombocytopenic
purpura diagnosed during
the second decade of life. J Pediatr 2002;141
(2):253-8.
11. Consolini R, Legitimo A, Caparello MC.
The centenary of immune thrombocytopenia
- Part 1: revising nomenclature and pathogenesis.
Front Pediatr 2016;4:102.
12. Heitink-Polle KM, Nijsten J, Boonacker
CW, de Haas M, Bruin MC. Clinical and laboratory
predictors of chronic immune thrombocytopenia
in children: a systematic review
and meta-analysis. Blood 2014;124(22):
3295-307.
13. Altintas A, Ozel A, Okur N, et al. Prevalence
and clinical significance of elevated antinuclear
antibody test in children and adult
patients with idiopathic thrombocytopenic
purpura. J Thromb Thrombolysis 2007;24
(2):163-8.
14. Panzer S, Penner E, Graninger W, Schulz
E, Smolen JS. Antinuclear antibodies in patients
with chronic idiopathic autoimmune thrombocytopenia
followed 2-30 years. Am J Hematol
1989;32(2):100-3.
15. Zimmerman SA, Ware RE. Clinical significance
of the antinuclear antibody test in selected
children with idiopathic thrombocytopenic
purpura. J Pediatr Hematol Oncol 1997;19(4):
297-303.
16. Al-Sayes FM, Hindawi SI, Damanhouri
GA, Attallah SM, Azaher FA, Akbar DH. Autoimmune
thrombocytopenia. Is it a different
disease or different aspects of a single
disease? Saudi Medical Journal 2012;33(2):
182-5.
Corrispondenza: giorgio.costagliola@hotmail.com