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Clinical features that help to distinguish primary immune thrombocytopenia (ITP) from other causes of thrombocytopenia in children

Clinical features that help to distinguish primary immune thrombocytopenia (ITP) from other causes of thrombocytopenia in children
Findings that support the diagnosis of ITP Findings that suggest an alternative diagnosis
Finding Potential alternative diagnoses
History and clinical presentation

Previously healthy child with abrupt onset of petechiae and easy bruising; otherwise well-appearing

Antecedent viral illness or vaccination (noted in approximately 60% of cases)

No systemic signs or symptoms other than mucocutaneous bleeding

Systemic symptoms (eg, fever, anorexia, weight loss, bone or joint pain, nonpetechial rashes, gastrointestinal symptoms, headaches)
  • Malignancy
  • Immunodeficiency (eg, CVID, WAS, DiGeorge syndrome)
  • Infection (eg, EBV, CMV, HIV)
  • Autoimmune disorder (eg, SLE, ALPS)
  • Acquired or inherited bone marrow failure (aplastic anemia)
History of recurrent infections and/or growth failure
  • Immunodeficiency (eg, CVID, WAS, 22q11.2 deletion syndrome)
Family history of thrombocytopenia or bleeding problems
  • Inherited platelet disorder
  • Inherited bone marrow failure syndrome
Examination
Normal examination other than mucocutaneous bleeding (petechiae, bruises) Lymphadenopathy or hepatosplenomegaly
  • Malignancy
  • ALPS
  • Infection (eg, EBV, CMV, HIV)
Dysmorphic features, skeletal abnormalities (eg, short stature, thumb and forearm abnormalities), abnormal skin pigmentation, deafness, or cataract
  • Inherited platelet disorder
  • Inherited bone marrow failure syndrome
Laboratory features
Isolated thrombocytopenia (no RBC or WBC abnormalities on CBC or peripheral blood smear)* Concomitant RBC and/or WBC abnormalities on CBC and/or peripheral blood smear*
  • Leukemia
  • Bone marrow failure syndrome
  • Concomitant AIHA (Evans syndrome)
  • ALPS
  • Thrombotic microangiopathy (eg, TTP, HUS)
Platelet count at presentation is usually <30,000/microL (often <20,000/microL) Mild thrombocytopenia (ie, platelet count >30,000/microL)
  • Inherited platelet disorder
  • Immunodeficiency (eg, CVID, WAS, 22q11.2 deletion syndrome)
  • Infection (eg, EBV, CMV, HIV)
Normal platelet size on peripheral blood smear; normal to mildly elevated MPV Giant or small platelets
  • Inherited platelet disorder
Previous normal platelet count Low platelet count noted on previous CBCs without ever having a documented normal platelet count
  • Inherited platelet disorder
  • Inherited bone marrow failure syndrome
This table summarizes clinical features that are consistent with childhood ITP and features that suggest an alternative cause of thrombocytopenia. ITP is largely a clinical diagnosis made in well-appearing patients with isolated thrombocytopenia and without other systemic signs or symptoms. ITP is a diagnosis of exclusion, so other causes of thrombocytopenia must be excluded. For additional details, refer to UpToDate topic on ITP in children.

AIHA: autoimmune hemolytic anemia; ALPS: autoimmune lymphoproliferative syndrome; CBC: complete blood count; CMV: cytomegalovirus; CVID: common variable immunodeficiency; EBV: Epstein-Barr virus; HIV: human immunodeficiency virus; HUS: hemolytic uremic syndrome; MPV: mean platelet volume; RBC: red blood cell; SLE: systemic lupus erythematosus; TTP: thrombotic thrombocytopenic purpura; WAS: Wiskott-Aldrich syndrome; WBC: white blood cell.

* Anemia may be seen in children with considerable ITP-related bleeding (eg, epistaxis or heavy menstrual bleeding), and the WBC count may be modestly elevated or decreased in children with antecedent or ongoing viral infections. Anemia out of proportion to blood loss, marked leukocytosis or leukopenia, or pancytopenia should prompt consideration of alternative diagnoses. Findings on peripheral smear that are inconsistent with ITP include early WBC forms (blasts), schistocytes, spherocytes, and giant platelets.

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