Approach to the child with unexplained thrombocytopenia
- Donald L Yee, MD
Donald L Yee, MD
- Associate Professor of Pediatrics
- Baylor College of Medicine
- Section Editors
- Donald H Mahoney, Jr, MD
Donald H Mahoney, Jr, MD
- Section Editor — Pediatric Hematology
- Professor of Pediatrics
- Baylor College of Medicine
- Lawrence LK Leung, MD
Lawrence LK Leung, MD
- Editor-in-Chief — Hematology
- Section Editor — Disorders of Hemostasis and Coagulation
- Professor of Medicine
- Stanford University School of Medicine
Thrombocytopenia, defined as a platelet count <150,000/microL, is clinically suspected when a child develops characteristic clinical symptoms, which include a petechial rash, easy bruising or bleeding, or mucosal hemorrhage. Thrombocytopenia is often asymptomatic and may present as an incidental finding during routine evaluation or during laboratory investigations performed for other reasons. A common cause of thrombocytopenia in children is immune thrombocytopenia (ITP, previously known as idiopathic thrombocytopenic purpura), for which the consensus definition is more restrictive, requiring a platelet count <100,000/microL .
Our approach to the evaluation of thrombocytopenia in children will be reviewed here. Other topic reviews with information relevant to the child with thrombocytopenia include the following:
- 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; 113:2386.
- Lusher JM. Clinical and laboratory approach to the patient with bleeding. In: Nathan and Oski's Hematology of Infancy and Childhood, 6th ed, Nathan, DG, Orkin, SH, Ginsburg, D, and Look, AT (Eds), Saunders, Philadelphia, 2003. p. 1515.
- Vesely S, Buchanan GR, Cohen A, et al. Self-reported diagnostic and management strategies in childhood idiopathic thrombocytopenic purpura: results of a survey of practicing pediatric hematology/oncology specialists. J Pediatr Hematol Oncol 2000; 22:55.
- Crosby WH. Editorial: Wet purpura, dry purpura. JAMA 1975; 232:744.
- Kumar R, Kahr WH. Congenital thrombocytopenia: clinical manifestations, laboratory abnormalities, and molecular defects of a heterogeneous group of conditions. Hematol Oncol Clin North Am 2013; 27:465.
- Balduini CL, Savoia A, Seri M. Inherited thrombocytopenias frequently diagnosed in adults. J Thromb Haemost 2013; 11:1006.
- Drachman JG. Inherited thrombocytopenia: when a low platelet count does not mean ITP. Blood 2004; 103:390.
- Alter BP. Diagnosis, genetics, and management of inherited bone marrow failure syndromes. Hematology Am Soc Hematol Educ Program 2007; :29.
- Israels SJ, Kahr WH, Blanchette VS, et al. Platelet disorders in children: A diagnostic approach. Pediatr Blood Cancer 2011; 56:975.
- Moake JL. Thrombotic microangiopathies. N Engl J Med 2002; 347:589.
- Mathew P, Chen G, Wang W. Evans syndrome: results of a national survey. J Pediatr Hematol Oncol 1997; 19:433.
- Miller BA, Schultz Beardsley D. Autoimmune pancytopenia of childhood associated with multisystem disease manifestations. J Pediatr 1983; 103:877.
- Savoia A, De Rocco D, Panza E, et al. Heavy chain myosin 9-related disease (MYH9 -RD): neutrophil inclusions of myosin-9 as a pathognomonic sign of the disorder. Thromb Haemost 2010; 103:826.
- Balduini CL, Pecci A, Savoia A. Recent advances in the understanding and management of MYH9-related inherited thrombocytopenias. Br J Haematol 2011; 154:161.
- Monteagudo M, Amengual MJ, Muñoz L, et al. Reticulated platelets as a screening test to identify thrombocytopenia aetiology. QJM 2008; 101:549.
- PLATELET COUNT AND BLEEDING RISK
- BLEEDING SYMPTOMS
- Cutaneous bleeding
- Mucosal bleeding
- Intracranial hemorrhage
- DIAGNOSTIC EVALUATION
- Verification of thrombocytopenia
- Physical examination
- Laboratory evaluation
- - Complete blood count
- - Peripheral blood smear
- - Bone marrow examination
- - Other tests
- - Reticulated platelets
- SUMMARY AND RECOMMENDATIONS