Child with bleeding problems edited
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The patient, a 10-year-old boy, presented with pain, swelling and reduced movement of both elbows and bruises on his knees, and has a history of hemophilia A diagnosed at age 1. Examination found swollen and warm elbows with restricted range of motion, and bruises on the knees. He was treated with factor VIII replacement therapy over 5 days which improved the right elbow but the left elbow still had some restriction.
Child with bleeding problems edited
- 1. Dr Neoh Hui Pheng Hospital Seberang Jaya
- 2. Def : consequence of tightly regulated processes that maintain blood in a fluid, clot-free state in normal vessels while introducing the rapid formation of a localized hemostatic plug at the site of vascular injury. Mechanism vasocontriction formation of platelet plug coagulation cascade fibrinolysis
- 8. Comparing coagulation factor and platelet defects Coagulation factor defects Platelet disorders and von Willebrand's disease Bruising on trunk and limbs Large bruises Small bruises Bleeding from cuts Relatively slight Profuse Nosebleeds Uncommon Common, frequently profuse and of long duration Gastrointestinal bleeding Uncommon Common Haematuria Common Rare Haemarthrosis In severe haemophilia Very rare Bleeding after surgery or dental extraction Up to a day's delay before bleeding occurs Immediate bleeding
- 10. Defect in clotting mechanism HemophiliaA : Deficiency of FactorVIII ( 85%) Hemophilia B : Deficiency of Factor IX ( 15%) 70 % X linked recessive. 30 % spontaneous mutation with no family hx.
- 11. Rarely,bleeding symptoms may present from birth ( Factor 8 and 9 do not cross placenta) - Intracranial hemorrhage /prolonged oozing from heel stick/venepuncture sites. - Most children present with easy bruising when crawling and walking ( ard 9-12 months) - Hemarthrosis – characteristic. - large joints ( ankle, knee, elbow ) – swollen and painful ( ankle joint : often the earliest joint involved. )
- 12. Other bleeding tendencies: Epitaxis, gum bleeding , hematuria. Bleeding can be spontaneously, after trauma , operation or dental procedures. Lungs/ CVS / PA : unremarkable
- 13. FBC Coagulation screen : PT/ APTT RP / LFT Specific factor assay : Factor 8 or factor 9 Von willebrand screen even ifAPTT normal . Others : - Infective screen ( at diagnosis and yearly) : Hep B/ Hep C /HIV - Platelet aggregation if highly suspicious of platelet defect.
- 14. Classification Clinical manifestation Severe (<1% of normal) •Manifest in infancy when child reaches toddler stage • Spontaneous bleeding – in muscles or joints (haemarthroses) • Excessive bleeding after minor trauma, postoperatively, or after intramuscular childhood vaccinations Moderate ( 1-5% of normal) •Manifest after 2 years of life • Moderate trauma causes bleeding episodes • Occasionally spontaneous bleeding occurs Mild ( >5 % - < 40% of normal) •Often diagnosed in teenagers and adults • Significant trauma/surgery or dental procedures to induce bleeding • No spontaneous bleeding
- 15. First Aid ( PRICE) : Pressure, Rest, Ice, Elevation FactorVIII /IX replacement Infuse factor 8 by slow IV push at the rate not exceeding 100U/ min in young children. FFP and cryoprecipitate should not be used as there is higher risk of viral transmission.
- 16. Type of bleed FactorVIII dose Factor IX dose Hemarthrosis 20U/kg 40 U /kg Soft tissue or muscle bleeds 30-40U/kg 60-80U /kg Intracranial hemorrhage or Surgery 50U /kg 100 U /kg
- 17. Calculate the required dose: - FactorVIII : ( % rise reqd) x ( wt in kg) x 0.5 - Factor IX : (% rise reqd) x ( wt in kg) x 1.4 Type of bleed % rise required duration Haemarthroses 30-40% 2-3 days Soft tissue /Muscle bleed ( Risk of compression/comp artment syndrome) 40-50% 4-5 days Intracranial bleed /operation 100% 7-10days
- 18. Analgesic often not required ( as there is rapid pain relief after missing factor concentrate is infused) - AVOID I.M injection - Don’t use Aspirin/NSAIDs – affect platelet fx - Acetaminophen with or without opioids can provide adequate pain control
- 19. Dental care is required as dental caries are a regular source of bleeding. In severe cases, dental clearance with factor replacement will be required. Medic alert bracelet Register with hemophilia society.
- 20. 1) Joint destruction : Recurrent hemarthrosis into the same joint osteoarthritis and joint deformity. Preventable by prompt and adequate factor 8 replacement. 2) Infection : Hep B/Hep C and HIV - All hemophiliacs must be immunised with Hep B
- 21. 3) Inhibitors: Antibodies directed against the exogeneous FactorVIII and IX neutralising the clotting activity. Can develop at ANY age, but usually 10-20 exposure days. Suspected when there is lack of response to replacement therapy instead of higher doses. 2 agents – “bypassing” the deficient clotting factor : i) Recombinant activated FactorVII ( rfVII or Novoseven) ii) FEIBA ( Factor Eight Inhibitor Bypass Activity) Immune tolerance induction Refer to hematologist in specialised centres.
- 24. neonatal alloimmune thrombocytopenia - Thrombocytopenia in < 6 months Sepsis and infections ( eg HIV ) Drug induced thrombocytopenia Hematological Malignancy -eg : Acute leukemia Congenital marrow failure syndromes -eg : Fanconi anemia , thrombocytopenia with absent radius Autoimmune disorders - Eg : SLE , Evan syndrome Primary immunodeficiency syndromes -Wiskott-aldrich syndrome Immune thrombocytopenic purpura
- 25. Isolated thrombocytopenia with NORMAL blood counts in a patient, with no clinically apparent alternative cause of thrombocytopenia . In children, ITP is an acute but self limiting that resolves spontaneously. Autoantibodies bind to platelet membrane antigen Increased platelet destruction Subtypes : 1) Acute ITP 2) Chronic ITP
- 26. Usually acute onset. Majority will have h/o viral infection in the preceding 2-4 weeks. Can be present as mild cutaneous bleed like petechiae , to mucosal bleeds like gum bleeding or epitaxis , to life threathening bleeds like Intracranial hemorrhage.
- 27. Based on history, examination and investigation. Physical examination : Absence of hepatosplenomegaly or lymphadenopathy. FBC : Isolated low platelet, normal Hb andTWBC FBP : Normal, apart from reduced larger platelet, no abnormal cells. Coagulation profile : prolonged BT, normal PT and APTT.
- 28. Usually not require BMA, unless child present with Atypical features ( eg : Organomegaly, significant lymphadenopathy, abnormal blood counts or suspicious FBP. ) Before starting steroid therapy ( to avoid partially inducing an undiagnosed acute leukemia) If there is failure to respond to Immunoglobulin therapy When there is persistent thrombocytopenia more than 6 months. Thrombocytopenia recurs after initial response to treatment.
- 29. Antinuclear factor and DNA antibodies Coomb’s test CMV serology ( < 1 yr old ) Coagulation profile ( suspected NAI and inherited bleeding disorder) HIV testing for those at risk ( eg parents RVD + or IVDU ) Immunoglobulin factor for those with recurrent infection
- 30. Most children remit spontaneously - 70% achieve platelet count > 50x 109 /L by the end of 3rd week. Careful observation with monitoring of platelet count , without specific treatment is appropriate for patient with : - Platelet count > 20x 109 /L without bleeding - Platelet count > 30x 109 /L with only cutaneous purpura - repeat FBC within 7-10 days to ensure there is no evidence of serious evolving marrow condition. Advise precautions with physical activities , avoidance of contact sports and seeking immediate medical attention if bleeding occurs should be advised.
- 31. Hospitalise the child if - Severe life threathening bleding eg ICH regardless of platelet count - Platelet count < 20x 109 /L with evidence of bleeding - Platelet count < 20x 109 /L without bleeding but inaccessible to health care. - Parents request due to lack of confidence in home care.
- 32. Treatment indicated if - Life threathening bleeding like ICH - Platelet count < 20x 109 /L with mucosal bleeding - Platelet count < 10x 109 /L with any bleeding Choices of treatment: i) Oral prednisolone 2mg/kg/day for 14 days then taper off ii) Oral prednisolone 4mg/kg/day for 4 days iii) IVIG 0.8mg/kg/dose for a single dose.
- 33. Note that the above mentioned regimes do not help to reduce bleeding complications or mortality or influence progression to chronic ITP. S/E of IVIG ( 15-75%) : - fever, flushing, headache, nausea, aseptic meningitis, transmission of Hep C ( older preparation ) Steroid should not be continued if there is no response or if there is a rapid relapse after withdrawal. Treatment should not be directed at increasing the platelet count above a preset level but rather on the clinical status of the patient.
- 34. Persistent thrombocytopenia after 6 months of onset ( in 20% ) Wide spectrum of manifestation: - Mild asymptomatic low platelet count -> intermittent relapsing symptomatic thrombocytopenia -> rare stubborn and persistent symptomatic and hemorrhagic disease.
- 35. Feature Acute ITP Chronic ITP Peak age Children (2-6 yrs) Adults (20-40 yrs) Female:male 1:1 3:1 Antecedent Infection Common Rare Onset of symptoms Abrupt Insidious Platelet count at presentation <20 000 <50 000 Duration 2-6 weeks Long term Spontaneous remission Common Uncommon
- 36. Try to give enough time for the disease to remit spontaneously. EXCLUDE other causes of thrombocytopenia. Asymptomatic child – Observe and conservative + precaution in physical activity Symptomatic- short course of treatment like acute ITP Counselling to parents- natural history of disease and detecting symptoms and complications. Parents should be confident in taking care of child with persistent low platelet count at home. Must know when and how to seek early medical attention.
- 37. For child with persistent bleeding. MUST d/w paediatric hematologist before initiating. Pulses of steroid : - Oral dexamethasone 1mg/kg given on 4 consecutive days every 4 weeks for 4 months. Intermittent anti-RhD Immunoglobulin treatment for Rh +ve : 45-50mcg/kg – May cause drop in Hb level. Splenectomy is rarely indicated.
- 38. Platelet count PT APTT Bleedin g time Thrombin time Additional test Hemophilia A N N Prolonged N Factor 8 low Hemophilia B N N Prolonged N Factor 9 low Von Willebrand’ s disease N N Prolonged or Normal N VWF / Factor 8 low - Impaired ristocetin impaired platelet aggregation Liver disease Low Prolonged prolonged N (Rarely prolonged ) DIC Low Prolonged prolonged Grossly prolonged Oral N Grossly Prolonged N
- 39. April 2013 10 yrs old , Chinese , boy, BW : 30kg p/w : 1) pain, swelling and reduced movement for both elbows X 2/7 2) Bruises over bilateral knee Denies history of recent contact sports. Denies history of trauma Denies history of fall. No other bleeding tendency like epitaxis, gum bleeding, hemetemesis, hemoptysis, hematuria, malena, petechiae or bruises at other body part.
- 40. He has had multiple previous admissions since young for joint swellings or soft tissue injury like calf swelling and bruises. Child was previously followed up in Hospital SultanahAminah since 1 yr old of age. Blood investigation during 1 yr old: - PT 11.9 - APTT 106.6 - FactorVIII <1.0% - VonWillebrand factor 103.1%
- 42. He was diagnosed with Hemophilia A since 1 yr old. In 2012, he was referred to HSJ for follow up due to logistic reason . May 2012 : right thigh swelling after a fall - FactorVIII 750U ( 30U/kg ) x 3 doses August 2012:right elbow swelling and bruises after falling down in basketball court. - FactorVIII 750U x 15 doses September 2012 : bilateral knee bruises after playing with a friend. - FactorVIII 750 U x 7 doses November 2012: Swelling over elbow . Admitted to HSAH in SP. FactorVIII given, child was not admitted.
- 43. Development history: standard 4 student with average performance. ( frequent absence from school due to frequent admissions) Family & Social history: Father is single parent. Mother is cambodian, was told to be carrier of hemophilia. Parents already divorced. He is the only son.
- 44. o/e : Alert, pink , active and comfortable. Not dyspneic, not tachypneic. Good perfusion , good pulse volume, CRT < 2 secs. No petechiae over body. No gum bleeding/nose bleeding. Lungs : A/E equal, clear CVS : DRNM PA : soft, not distended, no liver / spleen palpable. Local findings :both elbows slightly swollen and warm on touch. Restricted ROM : 75-135 degrees bilaterally. Bruises over both knees. However, bilateral knee has full ROM.
- 45. Ix : - FBC : Hb 12.8 ,TWC 8.4 , Plt 414 - PT 12.6, APTT 85.5 , INR 1.0 Management: - Given FactorVIII 750 U BD ( 25mg/kg/dose) for 2 days, followed by 750U OD for 3 days. A total of 7 doses given. - Subsequently, child had full ROM in right elbow, however, his left elbow still slightly restricted to 15-145 degrees.
- 46. TCA stat if hemarthrosis/ soft tissue bleeding / bleeding tendency. Advise child not to take part in contact sports.