Isolated thrombocytopenia in childhood: what if it is not immune thrombocytopenia?

Singapore Med J. 2018 Jul;59(7):390-393. doi: 10.11622/smedj.2018089.

Abstract

Introduction: Childhood immune thrombocytopenia (ITP) remains a diagnosis of exclusion when isolated thrombocytopenia is not part of another disease process. In practice, the diagnosis of ITP can only be confirmed when thrombocytopenia resolves or is excluded after the recognition of a primary cause.

Methods: The records of 87 consecutive children with isolated thrombocytopenia seen over a nine-year period in a private paediatric haematology practice were reviewed retrospectively. Children in whom a primary cause was eventually found were the subjects of a further descriptive study.

Results: 9 (10%) children with isolated thrombocytopenia were not diagnosed with ITP because a primary disease was found. Of these nine cases, four had thrombocytopenia recognised during the neonatal period, consisting of perinatal cytomegalovirus infection (n = 2), meconium aspiration pneumonia (n = 1) and transient abnormal myelopoiesis associated with Down syndrome (n = 1). The remaining five children were each found to have familial thrombocytopenia, portal hypertension, cutaneous mastocytosis, May-Hegglin anomaly and systemic lupus erythematosus. Two of them had a history of failure of response to corticosteroid therapy.

Conclusion: Secondary thrombocytopenia is not uncommon in a tertiary paediatric specialty practice with adequate evaluation. Thrombocytopenia occurring during the newborn period and failure of steroid therapy are predictive of secondary cases.

Keywords: differential diagnosis; idiopathic thrombocytopenic purpura; immune thrombocytopenia; neonatal thrombocytopenia; secondary thrombocytopenia.

MeSH terms

  • Adolescent
  • Child
  • Child, Preschool
  • Cytomegalovirus Infections / complications
  • Cytomegalovirus Infections / diagnosis
  • Diagnosis, Differential
  • Down Syndrome / complications
  • Down Syndrome / diagnosis
  • Hearing Loss, Sensorineural / complications
  • Hearing Loss, Sensorineural / diagnosis
  • Hematology
  • Humans
  • Hypertension, Portal / complications
  • Hypertension, Portal / diagnosis
  • Infant
  • Infant, Newborn
  • Leukemoid Reaction / complications
  • Leukemoid Reaction / diagnosis
  • Lupus Erythematosus, Systemic / complications
  • Lupus Erythematosus, Systemic / diagnosis
  • Mastocytosis, Cutaneous / complications
  • Mastocytosis, Cutaneous / diagnosis
  • Meconium Aspiration Syndrome / complications
  • Meconium Aspiration Syndrome / diagnosis
  • Pneumonia / complications
  • Pneumonia / diagnosis
  • Purpura, Thrombocytopenic, Idiopathic / diagnosis*
  • Purpura, Thrombocytopenic, Idiopathic / etiology
  • Retrospective Studies
  • Thrombocytopenia / complications
  • Thrombocytopenia / congenital
  • Thrombocytopenia / diagnosis*
  • Thrombocytopenia / etiology

Supplementary concepts

  • MYH9-Related Disorders
  • Myeloproliferative Syndrome, Transient