Bleeding Syndrome in Children

1. General Introduction:

Bleeding syndrome refers to an abnormal condition of bleeding caused by factors related to blood clotting, platelets, and blood vessel walls. In children, the most common bleeding syndromes are idiopathic thrombocytopenic purpura (ITP), hemophilia, and Schönlein-Henoch purpura (SH).

2. Pathogenesis:

• Idiopathic Thrombocytopenic Purpura (ITP):

• Platelet adhesion:

• GPIb – blood vessel wall

• GPIb-IIIA – fibronectin/fibrinogen

• Platelet dysfunction: Platelet count is good, but they don’t stick together.

• Causes of bleeding:

• Blood vessel wall: Lack of oxygen, malnutrition.

• Weak blood vessel wall: Vitamin C deficiency, PP deficiency.

• Allergic vasculitis: Schönlein-Henoch.

• Vascular malformations: Hereditary hemorrhagic telangiectasia (HHT), von Willebrand.

• Glanzmann Thrombasthenia: Deficiency of platelet adhesion factor 2b3a, reducing platelet adhesion ability.

• Bernard-Soulier Syndrome: Deficiency of factor 1b, reducing the ability to adhere to the blood vessel wall.

• Hemophilia:

• Hemophilia A: Deficiency of clotting factor VIII.

• Hemophilia B: Deficiency of clotting factor IX.

• Factor 11, 12 deficiency:

• Schönlein-Henoch Purpura: Allergic vasculitis due to an immune response to an antigen.

3. Diagnosis:

• Laboratory tests:

• Prolonged clotting time (CT) + normal platelet count (PC): Decreased platelet count, platelet dysfunction.

• Normal CT + prolonged PC: Due to plasma factors.

• Both CT and PC prolonged: Severe thrombocytopenia.

• Both CT and PC normal: Due to injury, mechanical causes.

• Prolonged APTT, normal PT: Hemophilia A, B, or factor 11, 12 deficiency.

• Normal APTT, prolonged PT: Factor VII deficiency.

• Both APTT and PT prolonged: Factor 10, 5, 2, 1 deficiency or due to antithrombinase-type anticoagulant drugs.

• MAIPA test (+): Check for autoantibodies against platelet glycoproteins.

• Diagnostic criteria for idiopathic thrombocytopenic purpura (ITP):

• Bleeding in multiple forms.

• Platelet count <100,000/mm3.

• No abnormalities in hematologic lines, white blood cells.

• No other diseases causing thrombocytopenia.

• Normal blood count, no direct Coombs test (+).

• Liver, spleen, lymph nodes not enlarged, no signs of systemic disease.

• Diagnosis of Hemophilia:

• Only APTT is prolonged, other tests are normal.

• Decreased levels of factor VIII, IX.

4. Epidemiology:

• Idiopathic Thrombocytopenic Purpura (ITP): Most common in ITP in children, accounting for 12% of blood and hematopoietic disorders in children. Usually occurs at the age of 5-7, female > male.

• Hemophilia: Inherited as a recessive gene on the X chromosome, only manifested in boys.

• Schönlein-Henoch Purpura: Common in children aged 3-15, incidence of 10-70/100,000 population, male: female = 1.2-1.8/100.

5. Clinical Manifestations:

• Idiopathic Thrombocytopenic Purpura (ITP):

• Appears 1-4 weeks after viral infection.

• Multiple forms, multiple locations, multiple ages.

• Bleeding from gums, nose, mouth, internal organs.

• Spleen not enlarged.

• Positive tourniquet test.

• Anemia equivalent to the level of bleeding.

• Hemophilia:

• Hematoma, subcutaneous hematomas, hematomas in muscles.

• Schönlein-Henoch Purpura:

• Arthritis, usually large joints of lower extremities > upper extremities, transient, migrating, few joints.

• Swelling, pain, no effusion or heat.

• Significant pain, limited mobility.

6. Severity of Disease:

• Idiopathic Thrombocytopenic Purpura (ITP):

• Mild: Skin.

• Moderate: Whole body in young children, bleeding from gums, nose, throat.

• Severe: Bleeding in multiple locations, internal bleeding.

• Very severe: Life-threatening.

• Hemophilia:

• Mild: 5%-<40%: Rarely spontaneous bleeding, bleeding after severe trauma or surgery.

• Moderate: 1-5%: Occasional spontaneous bleeding, bleeding after minor trauma and surgery.

• Severe: <1%: Spontaneous bleeding.

7. Treatment:

• Idiopathic Thrombocytopenic Purpura (ITP):

• Mild: Observation.

• Moderate: Prednisolone.

• Severe: Methylprednisolone or IVIG.

• Step 2: TPO-RA > rituximab > splenectomy.

• Hemophilia:

• Hemophilia A: Clotting factor VIII 1IU/kg, increase factor VIII level to 2IU/kg.

• Hemophilia B: Clotting factor IX 1IU/kg, increase factor IX level to 1IU/kg.

• Supportive medications:

• Desmopressin: For mild/moderate hemophilia.

• Tranexamic acid: Antifibrinolytic drug.

• EACA: Antiemetic drug.

8. Complications:

• Hemophilia:

• Musculoskeletal disorders.

• HIV, HBV, HCV infection.

• Inhibitors: IgG4 against factor 8,9.

9. Prenatal Diagnosis:

• Hemophilia:

• Placental biopsy at 9-14 weeks, higher miscarriage rate than 1.3%.

• Amniocentesis at 15-17 weeks.

10. Notes:

• Thrombocytopenic bleeding is common in infants under 3 months old.

• Hemophilia is usually detected in the toddler stage.

• Characteristics of bleeding in ITP:

• Sudden onset after infection or no history of illness.

• Spontaneous bleeding or after minor trauma.

• Bleeding in multiple forms: dots, spots, large and small hematomas.

• Liver, lymph nodes, spleen not enlarged.

• Anemia equivalent to the level of bleeding.

• Positive tourniquet test.

11. Conclusion:

Bleeding syndrome in children is a concerning health issue. Early diagnosis and appropriate treatment help to minimize complications and improve the quality of life for children.