para>Acute onset of diffuse subcutaneous bleeding with bruises of different ages may indicate severe thrombocytopenia.
Generally, children will not bruise or develop petechiae spontaneously until the platelet count is <20,000/mm3.
Idiopathic thrombocytopenic purpura, leukemia, aplastic anemia, and so forth, can cause this bleeding.
A hematologist should be consulted because of the risk of potentially life-threatening bleeding.
Chronic history of recurrent bleeding may indicate an inherited coagulation defect such as von Willebrand disease or hemophilia. Familial history may be positive, although von Willebrand disease often goes undiagnosed into adulthood if there has been no challenge such as surgery.
Various bleeding scores available but not fully validated in pediatrics; may be helpful to direct history taking
Table 1How to estimate the age of bruisesView LargeTable 1How to estimate the age of bruises1. NewPurple, dark red2. 1-4 daysDark blue to brown3. 5-7 daysGreenish to yellow4. >7 daysYellow
Phase 2: Perform screening tests for bleeding disorders to categorize the abnormality.- Platelet count to assess level of thrombocytopenia
- PT/PTT: Prolongation of either one or both of these may aid in diagnosis of von Willebrand disease, coagulation factor deficiencies, liver disease, and vitamin K deficiency.
- Testing for von Willebrand disease and platelet aggregation disorders generally need to be done by a hematologist for accuracy and interpretation.
- Bleeding time and PFA-100: Both tests are controversial and rarely used in pediatrics.
History
- Question: Significant bruising in the neonatal period?
- Significance: May indicate neonatal thrombocytopenia, congenital infections, and sepsis with disseminated intravascular coagulation
- Question: Bleeding in the neonatal period?
- Significance: Hemophilia. Other inherited disorders of coagulation may not be diagnosed until a child is older; tend to be mild; may be uncovered with preoperative testing or postoperative bleeding complications. Idiopathic thrombocytopenic purpura may occur at any age.
- Question: Pattern of bruising?
- Significance: In a younger child, may indicate normal toddler activity, child abuse, or religious practices such as coining
- Question: Use of aspirin, ibuprofen, cough syrups with guaifenesin, and/or antihistamines?
- Significance: Platelet dysfunction; use of these drugs may also unmask an otherwise mild inherited bleeding disorder.
- Question: Ecchymosis or petechiae?
- Significance: Infections such as meningococcemia or viruses and collagen vascular diseases may present with these.
- Question: Familial history?
- Significance: Positive familial history of inherited disorders of coagulation factors or platelet aggregation may aid in directing the workup. Negative familial history does not rule out any of these disorders.
Physical Exam
- Finding: Good appearance, with a history of an antecedent viral illness?
- Significance: Those with idiopathic thrombocytopenic purpura often appear well, although often with a history of an antecedent viral illness.
- Finding: Ill appearance?
- Significance: It should raise concerns about malignancy, infection (especially meningococcemia), or other acquired coagulation factor deficiencies such as those seen with liver failure.
- Finding: Bruising in unusual locations (back, genitalia, thorax)?
- Significance: Should raise suspicions of child abuse, especially if lesions are in different stages of healing or suggest the pattern of a hand or belt
- Finding: Purpura confined mostly to the legs?
- Significance: Typical of Henoch-Sch ¶nlein purpura
- Finding: Multiple ecchymoses in the pretibial regions?
- Significance: This is typical of normal toddler activity.
- Finding: Petechiae entirely above the nipple line?
- Significance: Consistent with Valsalva maneuver, severe cough, and viral infections
- Finding: Deeper bleeding in muscles and joints?
- Significance: Hemophilia
- Finding: Bleeding in mucous membranes?
- Significance: Severe thrombocytopenia, streptococcal pharyngitis, varicella, measles, and other viral infections can cause this.
- Finding: Gingival and/or mucous membrane bleeding?
- Significance: Von Willebrand disease can present with this. Severe thrombocytopenia and ITP may also present with oral bleeding.
- Finding: Involvement of the reticuloendothelial system?
- Significance: Can be found with malignancies such as leukemia or with viral or bacterial infections, indicated by hepatosplenomegaly or lymphadenopathy
- Finding: Upper extremity limb malformations and bruising?
- Significance: May present with syndromes such as Fanconi anemia and thrombocytopenia absent radii (TAR)
Alert
Factors that make this an emergency include the following:
- Severe thrombocytopenia below 10,000-20,000/mm3 carries a higher risk of spontaneous internal bleeding including intracranial bleeding.
- Bleeding or bruising accompanied by evidence of leukemia or other malignancy
- Evidence of sepsis (disseminated intravascular coagulation) or meningococcemia
Alert
- The amount of bruising may not correlate with the amount of internal bleeding that has occurred.
- Hemophiliacs can significantly drop their hemoglobin during a thigh or psoas bleed without having much in the way of ecchymosis.
- A child presenting with idiopathic thrombocytopenic purpura may have bruises and petechiae from head to toe without changing the hemoglobin much at all.
Diagnostic Tests & Interpretation
- Test: CBC
- Significance: Platelet count is the most important; abnormalities of WBC or Hgb may aid in diagnosis of bone marrow infiltration or failure.
- Test: PT
- Significance: Elevation may indicate warfarin ingestion or factor VII and/or vitamin K deficiencies.
- Test: aPTT
- Significance: Prolongation is seen with hemophilia and may be seen in von Willebrand disease.
- Test: Both PT and PTT
- Significance: Both are prolonged in disseminated intravascular coagulation, liver failure, and severe vitamin K deficiency.
- Test: Bleeding time
- Significance: Lengthened in platelet aggregation disorders and with drug effects
- Test: Fibrinogen
- Significance: Decreased in liver failure, disseminated intravascular coagulation
- Test: Urinalysis
- Significance: Hematuria and/or proteinuria may indicate Henoch-Sch ¶nlein purpura, nephrotic syndrome, or other vasculitis.
Treatment
General Measures
- Thrombocytopenia precautions for platelets <20,000-50,000-toddlers may need a helmet until platelet count recovers; patients with hemophilia may need restricted activity, generally not needed for patients with von Willebrand disease. Depends on underlying cause.
- Factor replacement for hemophilia
- Platelet transfusion for thrombocytopenia due to decreased production
- IVIG, steroids, Rh immune globulin, thrombopoietin (TPO) mimetics for ITP
Issues for Referral
- Outpatient evaluation for bruising without significant thrombocytopenia, family history of bleeding disorder
- Suspected child abuse
Admission Criteria
Severe thrombocytopenia, suspected child abuse, significant bleeding, significant head trauma
Ongoing Care
Follow-up Recommendations
Patient Monitoring
Recurrent and chronic ITP possible
Complications
Significant bleeding with a bleeding disorder, thrombocytopenia
Additional Reading
- Berntorp E. Progress in haemophilic care: ethical issues. Haemophilia. 2002;8(3):435-438. [View Abstract]
- Buchanan GR. Bleeding signs in children with idiopathic thrombocytopenic purpura. J Pediatr Hematol Oncol. 2003;25(Suppl 1):S42-S46. [View Abstract]
- Geddis AE, Balduini CL. Diagnosis of immune thrombocytopenic purpura in children. Curr Opin Hematol. 2007;14(5):520-525. [View Abstract]
- Horton TM, Stone JD, Yee D, et al. Case series of thrombotic thrombocytopenic purpura in children and adolescents. J Pediatr Hematol Oncol. 2003;25(4):336-339. [View Abstract]
- Khair K, Liesner R. Bruising and bleeding in infants and children-a practical approach. Br J Haematol. 2006;133(3):221-231. [View Abstract]
- Kos L, Shwayder T. Cutaneous manifestations of child abuse. Pediatr Dermatol. 2006;23(4):311-320. [View Abstract]
- O'Brien SH. An update on pediatric bleeding disorders: bleeding scores, benign joint hypermobility, and platelet function testing in the evaluation of the child with bleeding symptoms. Am J Hematol. 2012;87(Suppl 1):S40-S44. doi:10.1002/ajh.23157. [View Abstract]
- Wight J, Paisley S. The epidemiology of inhibitors in haemophilia A: a systematic review. Haemophilia. 2003;9(4):418-435. [View Abstract]
Codes
ICD09
- 924.9 Contusion of unspecified site
- 782.7 Spontaneous ecchymoses
- 286.9 Other and unspecified coagulation defects
- 287.5 Thrombocytopenia, unspecified
- 459.89 Other specified disorders of circulatory system
ICD10
- T14.8 Other injury of unspecified body region
- R23.3 Spontaneous ecchymoses
- D68.9 Coagulation defect, unspecified
- D69.6 Thrombocytopenia, unspecified
- R58 Hemorrhage, not elsewhere classified
SNOMED
- 125667009 contusion (disorder)
- 271813007 Petechiae (disorder)
- 16922007 Hereditary coagulation factor deficiency (disorder)
- 302215000 Thrombocytopenic disorder (disorder)
FAQ
- Q: Is hemophilia always diagnosed in the newborn period?
- A: No. A familial history may provide clues, but a significant number of patients represent a spontaneous mutation. Additionally, not all boys with hemophilia will bleed with circumcision, and the diagnosis may not be made until the infants become more active.
- Q: What is a common cause of bruising among girls?
- A: Girls may first come to attention at menarche and be diagnosed at that time with von Willebrand disease. Rarely, girls whose fathers have hemophilia may be unfavorably lyonized and, therefore, have decreased factor levels consistent with mild hemophilia.
- Q: Can boys with a family history of von Willebrand disease be circumcised?
- A: Yes, but only within the first few days of life and testing does not need to be completed prior to procedure. If procedure is not completed in the neonatal period, testing may need to be delayed until 6 months of life or later.