Bleeding and clotting disorders in children
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Transcript of Bleeding and clotting disorders in children
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11/04/2023 1
Bleeding and coagulation disorders in
childrenDr.K.V.Giridhar
Associate Prof. of Pediatrics
GMC. Ananthapuramu, A.P.,India.
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Definition:• Spontaneous bleeding.• Excessive & Prollanged bleeding following trauma.
• Petechiae, purpura, echymosis, bruises, hematoma, hemarthrosis, IC bleeds, occult GI bleeds, melena, epistaxis.
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Bleeding disorders
Coagulation
disorders
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Hemostasis
Tissue phasePlatelet phaseClotting PhaseUnstable clot PhaseClot stabilization PhaseClot retraction PhaseClot resolving Phase
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THE CLOTTING MECHANISM
INTRINSIC EXTRINSIC
PROTHROMBIN(II) THROMBIN(III)
FIBRINOGEN
FIBRIN
(I)V
X
Tissue ThromboplastinCollagen
VIIXII
XI
IXVIII
VII
PTPTT Vit.K, Liver
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Etiology
• VESSEL WALL ABNORMALITIES:
• PLATELETS DISORDER:
• COAGULATION DISORDER:
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Vessel Abnormalities• increased vascular fragility• manifest by petechial hemorrhages of
skin/mucous membranes• not life threatening bleeding1. 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
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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.
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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.
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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
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PLATELETS DISORDER:
• QUANTITATIVE PLATELETS DISORDERs
• QUALITATIVE PLATELET DYSFUNCTION :
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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.
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(Thrombocytopenia) Causes:
Marrow DisorderAplastic anemiaHem. malignancyMyelodysplastic
disorderB12 deff.
Non Marrow DisorderImmune disordersITP, Drug inducedSec: ALL, SLEPost transfusionDIC, TTPHU syndrome, HyperspleenismHeamangiomasSepsisViral infection
Management:
Rx Underlying cause
Platelet transfusion
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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.
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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) Peak incidence 20-50 years of age.
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IDIOPATHIC THROMBOCYTOPENIC
PURPURA. Δ LAB:platelets below 10,000 /ml.Bone marrow will appear normal.RxPREDNISONONE: 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.
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EVANS SYNDROME:
• ITP + Autoimmune hemolytic anemia.
• These pts shows spherocytosis, reticulocytosis + anemia.
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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
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QUALITATIVE PLATELET DISORDERBERNARD 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
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QUALITATIVE PLATELET DISORDERGLANZMANN’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 bleedingLAB:Platelets no’s and morphology are normal B.T is prolonged Rx:Platelet transfusion
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QUALITATIVE PLATELET DISORDERVON-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.
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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
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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.
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• HEAMOPHILIA – A (CLASSIC TRUE HAEMOPHILIA)
• HAEMOPHILLIA – B (CHRISTMAS DISEASE).
• X-linked recessive Inheritance.
COAGULATION DISORDER:CONGENITAL BLEEDING DISORDER:
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HEAMOPHILIA – A (CLASSIC TRUE HAEMOPHILIA)• X-linked disorder • Due to deff. of factor VIIIC/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
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Hemophilia A
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HAEMOPHILLIA – B (CHRISTMAS DISEASE)
• Due to deff: of factor IX S/S:• Same in type ARx• Factor IX infusionLONG TERM COMPLICATIONCOMPLICATION 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
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COAGULATION DISORDERACQUIRED BLEEDING DISORDER
• DIC• LIVER DISEASE • RENAL DISEASE
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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.
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DIC:
CAUSESInfectious:• E Coli• Nessieria
meningitis• Strep pneumonia• MalariaCancer • 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
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DIC
LAB:• Thrombocytopenia• Prolong PT• APTT may be normal/increased• Low fibrinogen• Increased level FDP/D-dimmer
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Treatment of DIC
Rx. Underlying cause.General Measures:• Correction of dehydration• Renal failure• Acidosis and • ShockReplacement:• 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.
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Approach to a child with bleeding disorder
Bleeding Not sick sic
kSuperficial bleeds
Deep Bleeds
CBC, BT Factor assay, Gene analysis
Bone marrow
Blood culture
CBC, Bonemarrow
LFT
RFT
FDP
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