Bleeding and
coagulation disorders
in children
Dr.K.V.Giridhar
Associate Prof. of Pediatrics
GMC. Ananthapuramu, A.P.,
Ind...
Definition:
• Spontaneous bleeding.
• Excessive & Prollanged
bleeding following trauma.
• Petechiae, purpura, echymosis, b...
Bleeding
disorders
Coagulation
disorders
Hemostasis
Tissue phase
Platelet phase
Clotting Phase
Unstable clot Phase
Clot stabilization Phase
Clot retraction P...
THE CLOTTING MECHANISM
INTRINSIC EXTRINSIC
PROTHROMBIN(II) THROMBIN(III)
FIBRINOGEN
FIBRIN
(I)
V
X
Tissue ThromboplastinCo...
Etiology
• VESSEL WALL ABNORMALITIES:
• PLATELETS DISORDER:
• COAGULATION DISORDER:
Vessel Abnormalities
• increased vascular fragility
• manifest by petechial hemorrhages of skin/mucous
membranes
• not lif...
VESSEL WALL ABNORMALITIES:
EHLERS DANLOS DISEASE:
• Congenital disorder of collagen
synthesis
• in which capillaries are p...
VESSEL WALL ABNORMALITIES:
HERIDITARY HEMORHAGIC TELANGECTSIASIS
• Dominant inherited condition.
• Telengectias, are small...
Henoch-Schonlein purpura
(nonthrombocytopenic)
• generalized hypersensitivity vasculitis
• uncertain cause(immune mediated...
PLATELETS DISORDER:
• QUANTITATIVE PLATELETS
DISORDERs
• QUALITATIVE PLATELET
DYSFUNCTION :
QUANTITATIVE PLATELETS DISORDER
(Thrombocytopenia)
Mechanisms:
1 Failure of megakaryocytic maturation.
2 Excessive platele...
(Thrombocytopenia) Causes:
Marrow Disorder
Aplastic anemia
Hem. malignancy
Myelodysplastic
disorder
B12 deff.
Non Marrow D...
IDIOPATHIC THROMBOCYTOPENIC
PURPURA(ITP)
• An autoimmune antibody IgG is
formed against unknown antigen of
platelets membr...
IDIOPATHIC THROMBOCYTOPENIC
PURPURA. (Clinical Features)
In Children(acute):
Often precipitated by viral infection and
us...
IDIOPATHIC
THROMBOCYTOPENIC PURPURA.
Δ LAB:
platelets below 10,000 /ml.
Bone marrow will appear normal.
Rx
PREDNISONONE: 1...
EVANS SYNDROME:
• ITP + Autoimmune hemolytic
anemia.
• These pts shows
spherocytosis, reticulocytosis +
anemia.
QUALITATIVE PLATELET DISORDER
CONGENITAL:
Glanzmann‟s
thrombosthenia
Bernard souliar
syndrome
Von Willibrand‟s
disease
ACQ...
QUALITATIVE PLATELET DISORDER
BERNARD SOULIER SYNDROME:
Autosomal recessive intrinsic platelets disorder.
Due to lack of g...
QUALITATIVE PLATELET DISORDER
GLANZMANN’s THROMBASTHENIA:
Autosomal recessive disorder.
Lack of receptors (glycoprotein Ib...
QUALITATIVE PLATELET DISORDER
VON-WILLIBRAND’S DISEASE:
• Autosomal dominant(gene for vWF is
located on chromosome 12.)
• ...
VON-WILLIBRAND’S DISEASE:
Clinical Features:
• Mucosal bleeding (mild-massive)
LAB:
• Reduced level of vWF which often acc...
COAGULATION DISORDER:
Coagulation factor disorder can
either due to single factor def., i.e.
a “congenitaldeficiency”, eg ...
• HEAMOPHILIA – A (CLASSIC TRUE
HAEMOPHILIA)
• HAEMOPHILLIA – B (CHRISTMAS
DISEASE).
• X-linked recessive Inheritance.
COA...
HEAMOPHILIA – A (CLASSIC TRUE
HAEMOPHILIA)
• X-linked disorder
• Due to deff. of factor VIII
C/F:
• Bleeding occurs as bru...
Hemophilia A
HAEMOPHILLIA – B (CHRISTMAS
DISEASE)
• Due to deff: of factor IX
S/S:
• Same in type A
Rx
• Factor IX infusion
LONG TERM C...
COAGULATION DISORDER
ACQUIRED BLEEDING DISORDER
• DIC
• LIVER DISEASE
• RENAL DISEASE
DISSAMINATED INTRAVASCULAR
COAGULATION
• DIC is condition characterized by thrombosis
within circulation.
• DIC can be ind...
DIC:
CAUSES
Infectious:
• E Coli
• Nessieria meningitis
• Strep pneumonia
• Malaria
Cancer
• Lung,Pancreas,
• Prostate
CLI...
DIC
LAB:
• Thrombocytopenia
• Prolong PT
• APTT may be normal/increased
• Low fibrinogen
• Increased level FDP/D-dimmer
Treatment of DIC
Rx. Underlying cause.
General Measures:
• Correction of dehydration
• Renal failure
• Acidosis and
• Shoc...
Approach to a child with bleeding disorder
Bleeding
Not sick sick
Superficial bleeds Deep Bleeds
CBC, BT Factor
assay, Gen...
THANKYOU
Bleeding and clotting disorders in children
Bleeding and clotting disorders in children
Bleeding and clotting disorders in children
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Bleeding and clotting disorders in children

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Bleeding and clotting disorders in children

  1. 1. Bleeding and coagulation disorders in children Dr.K.V.Giridhar Associate Prof. of Pediatrics GMC. Ananthapuramu, A.P., India. 4/16/2014 1
  2. 2. Definition: • Spontaneous bleeding. • Excessive & Prollanged bleeding following trauma. • Petechiae, purpura, echymosis, bruises, hematoma, hemarthrosis, IC bleeds, occult GI bleeds, melena, epistaxis.
  3. 3. Bleeding disorders Coagulation disorders
  4. 4. Hemostasis Tissue phase Platelet phase Clotting Phase Unstable clot Phase Clot stabilization Phase Clot retraction Phase Clot resolving Phase
  5. 5. THE CLOTTING MECHANISM INTRINSIC EXTRINSIC PROTHROMBIN(II) THROMBIN(III) FIBRINOGEN FIBRIN (I) V X Tissue ThromboplastinCollagen VIIXII XI IX VIII VII PT PTT Vit.K, Live r
  6. 6. Etiology • VESSEL WALL ABNORMALITIES: • PLATELETS DISORDER: • COAGULATION DISORDER:
  7. 7. Vessel Abnormalities • increased vascular fragility • manifest by petechial hemorrhages of skin/mucous membranes • not life threatening bleeding 1. congenital: a. Ehlers-Danlos syndrome (AD) b. hereditary hemorrhagic telangiectasia (AD) 2. acquired: a. hypersensitive vasculitis (i) drug reaction : immune complex deposit in vessel walls(Thaizide diuetics) (ii) Henoch-Schonlein purpura b. scurvy (vit C deficiency) Lab: BT, Plt count, PT, PTT will be normal
  8. 8. VESSEL WALL ABNORMALITIES: EHLERS DANLOS DISEASE: • Congenital disorder of collagen synthesis • in which capillaries are poorly supported by s/c collagen • ecchymosis are commonly observed.
  9. 9. VESSEL WALL ABNORMALITIES: HERIDITARY HEMORHAGIC TELANGECTSIASIS • Dominant inherited condition. • Telengectias, are small aneurysms found on finger tips, face, nasal passages, tongue and GIT. • few people develop pulmonary A/V malformation. • Pt. develops recurrent bleeding/epistaxis/ occult GIT bleeding, leads to Iron def. anemia Rx. • Iron therapy for blood loss. • Local cautery/laser therapy for single lesion • Estrogens may be tried.
  10. 10. Henoch-Schonlein purpura (nonthrombocytopenic) • generalized hypersensitivity vasculitis • uncertain cause(immune mediated) clinical Sx: • Purpura(palpable) • colicky abdominal pain • polyarthralgia • acute glomerulonephritis Rx: Prednisolone for2-4 weeks
  11. 11. PLATELETS DISORDER: • QUANTITATIVE PLATELETS DISORDERs • QUALITATIVE PLATELET DYSFUNCTION :
  12. 12. QUANTITATIVE PLATELETS DISORDER (Thrombocytopenia) Mechanisms: 1 Failure of megakaryocytic maturation. 2 Excessive platelets consumption after their release into circulation i.e ITP, DIC etc. 3 Platelets sequestration in enlarged spleen i.e HYPERSPLEENISM. S/S: · Petechial cutaneous bleeding, intracranial bleeding and oozing from mucus membrane & skin surface. · Lab: decreased platelets count and prolong bleeding time.
  13. 13. (Thrombocytopenia) Causes: Marrow Disorder Aplastic anemia Hem. malignancy Myelodysplastic disorder B12 deff. Non Marrow Disorder Immune disorders ITP, Drug induced Sec: ALL, SLE Post transfusion DIC, TTP HU syndrome, Hyperspleenism Heamangiomas Sepsis Viral infection Management: Rx Underlying cause Platelet transfusion
  14. 14. IDIOPATHIC THROMBOCYTOPENIC PURPURA(ITP) • An autoimmune antibody IgG is formed against unknown antigen of platelets membrane/surface. • Antipletelet antibody binds to complement, but platelets are not destroyed by direct lysis. • Destruction takes place in spleen, where spleenic macrophages destroyes antibody coated platelets.
  15. 15. IDIOPATHIC THROMBOCYTOPENIC PURPURA. (Clinical Features) In Children(acute): Often precipitated by viral infection and usually self limited  Asymptomatic not febrile.  Present with mucosal/skin bleeding, mennorrhagia, purpura, petechiae . Adults(chronic):  Commonly affects female.  Ratio 2:1 (male/female ratio)
  16. 16. IDIOPATHIC THROMBOCYTOPENIC PURPURA. Δ LAB: platelets below 10,000 /ml. Bone marrow will appear normal. Rx PREDNISONONE: 1-2 mg/kg/day. Immunoglobulin: 1g/kg/day 2-3 days. DANAZOLE: 600mg/day response rate is 50% IMMUNOSUPPERESSIVE DRUGS: i-e vincristine, vinblastine, azathioprine, cyclosprin, cyclophosphomide. SPLEENECTOMY: Prognosis: The prognosis will be good, if disease is initially controlled with prednisolone, spleenectomy is definite Rx.
  17. 17. EVANS SYNDROME: • ITP + Autoimmune hemolytic anemia. • These pts shows spherocytosis, reticulocytosis + anemia.
  18. 18. QUALITATIVE PLATELET DISORDER CONGENITAL: Glanzmann‟s thrombosthenia Bernard souliar syndrome Von Willibrand‟s disease ACQUIRED Myeloproliferative disorder. Uremia Drugs i-e NSAIDS Aspirin Autoantibody Fibrin degradation products
  19. 19. QUALITATIVE PLATELET DISORDER BERNARD SOULIER SYNDROME: Autosomal recessive intrinsic platelets disorder. Due to lack of glycoprotein (Gp1b), receptor for vonWillibrand‟s factor. Clinical Features: Presents with mucosal bleeding and post operative oozes. LAB: Thrombocytopenia may be present, and Plt.s are abnormally large in size. BT is prolonged Von Willibrand’s factor Normal Rx: Platelet transfusion
  20. 20. QUALITATIVE PLATELET DISORDER GLANZMANN’s THROMBASTHENIA: Autosomal recessive disorder. Lack of receptors (glycoprotein Ib & IIIa) for fibrinogen on platelets. Platelets fails to aggregate in respons to ADP, collagen, thrombin. Clinical Features: Mucosal bleeding LAB: Platelets no‟s and morphology are normal B.T is prolonged Rx: Platelet transfusion
  21. 21. QUALITATIVE PLATELET DISORDER VON-WILLIBRAND’S DISEASE: • Autosomal dominant(gene for vWF is located on chromosome 12.) • vWF is synthesized by endothelial cells and megakaryocytes • It acts as carrier protein for factor VIII by non-covalent bond. A defect therefore leads to decreased plasma factor VIII level. • It also forms bridges b/w platelets and sub endothelium. • There fore defect of vWF leads to prolonged bleeding.
  22. 22. VON-WILLIBRAND’S DISEASE: Clinical Features: • Mucosal bleeding (mild-massive) LAB: • Reduced level of vWF which often accomplished by sec: reduction in factor VIII and prolonged bleeding time (B.T) Rx: • MILD HAEMORRHAGES: Desmopressin 0.3 μg/kg, after which vWF levels usually raise 3 in 30-90 minutes • MASSIVE HAEMORRHAGES: Factor VIII
  23. 23. COAGULATION DISORDER: Coagulation factor disorder can either due to single factor def., i.e. a “congenitaldeficiency”, eg factor VIII resulting in HAEMOPHILIA-A or due to multiple factor def., which is an „‟acquired” eg Sec: to liver disease or warfarin therapy.
  24. 24. • HEAMOPHILIA – A (CLASSIC TRUE HAEMOPHILIA) • HAEMOPHILLIA – B (CHRISTMAS DISEASE). • X-linked recessive Inheritance. COAGULATION DISORDER: CONGENITAL BLEEDING DISORDER:
  25. 25. HEAMOPHILIA – A (CLASSIC TRUE HAEMOPHILIA) • X-linked disorder • Due to deff. of factor VIII C/F: • Bleeding occurs as bruising at the age of 6 months. • Trauma results in excessively bleeding. • Recurrent bleeding /hemorrhage in knee, elbow, ankle, and hip. (Hemarthrosis) • Mucus membrane /internal bleeding of mouth, lips, gums, brain and kidney also occur • Muscle haematoma esp. calf and Psoas muscle Rx • Factor VIII infusion
  26. 26. Hemophilia A
  27. 27. HAEMOPHILLIA – B (CHRISTMAS DISEASE) • Due to deff: of factor IX S/S: • Same in type A Rx • Factor IX infusion LONG TERM COMPLICATION COMPLICATION due to repeated hemorrhage: • Arthropathy of large joints eg knee, elbow • Muscle atrophy due to haematoma • Mononeuropathy due to pressure of haematoma. COMPLICATION due to therapy • Antifactor VIII antibody develops • Virus transmission Hepatitis A-B-C-D + HIV
  28. 28. COAGULATION DISORDER ACQUIRED BLEEDING DISORDER • DIC • LIVER DISEASE • RENAL DISEASE
  29. 29. DISSAMINATED INTRAVASCULAR COAGULATION • DIC is condition characterized by thrombosis within circulation. • DIC can be induced by different mechanisms. • Due to Endothelial cell damage by endotoxins in G –ve septicemia results in tissue factor release which in turn leads to coagulation cascade activation through extrinsic pathway. • The presence thromboplastin from damaged tissue, placenta & fat embolus (following brain injury & Fractures) may activate coagulation • This results in excessive consumption of platelets and coagulation factors, with secondary activation of fibrinolysis leading to bleeding tendency.
  30. 30. DIC: CAUSES Infectious: • E Coli • Nessieria meningitis • Strep pneumonia • Malaria Cancer • Lung,Pancreas, • Prostate CLINICAL FEATURES: Bleeding & thrombosis, bleeding is more than thrombosis. Subacute DIC: Occurs primarily in cancerous pts results in superficial + deep venous thrombosis. Other Manifestation: High incidence of cardio respiratory failure
  31. 31. DIC LAB: • Thrombocytopenia • Prolong PT • APTT may be normal/increased • Low fibrinogen • Increased level FDP/D-dimmer
  32. 32. Treatment of DIC Rx. Underlying cause. General Measures: • Correction of dehydration • Renal failure • Acidosis and • Shock Replacement: • Platelets transfusion if platelets counts below 10,000/l • cryoprecipitate to maintain plasma fibrinogen level above 150 mg/dl • FFP • Heparin, if there is DVT, Pulmonarythrombosis.
  33. 33. Approach to a child with bleeding disorder Bleeding Not sick sick Superficial bleeds Deep Bleeds CBC, BT Factor assay, Gene analysis Bone marrow Blood culture CBC, Bonemarrow LFT RFT FDP
  34. 34. THANKYOU
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