Dr-Majid Qanavat Ped. Hematologist oncologist Isfahan university -1396 Bleeding Disorders Dr-Majid Qanavat Ped. Hematologist oncologist Isfahan university -1396

Approach to bleeding tendency in infants and children

VASCULAR PHASE PLATELET PHASE PLASMA PHASE Coagulation system VASCULAR PHASE PLATELET PHASE PLASMA PHASE

Coagulation system Blood vessel injury Vasoconstriction Simplistic View of Vascular Injury

Practical Approach to a Child With Bleeding HISTORY: AGE OF ONSET SEX, Family History LOCATION I TYPE OF BLEEDING DURATION OF BLEEDING MEDICATIONS ASSOCIAED SYMPOMS REVIEW OF SYSTEMS

Practical Approach to a Child With Bleeding (cont Practical Approach to a Child With Bleeding (cont.) PHYSICAL EXAMINATION : PETECHIAE ECHYMOSES JOINT BLEED & DEEPSEATED HEMATOMAS HEPATOSPLENOMEGALY SIGNIFICANT LYMPHADENOPATHY ACTIVE AND PLAYFUL VS. ILL LOOKING DYSMORPHIC FEATURE CAFE-AU-LAIT SPOTS HEMANGIOMAS

Approach to a bleeding patient (cont.)

Approach to a bleeding patient (cont.)

Approach to a bleeding patient Primary homeostasis Vascular causes : "Meningococcemia"

Henoch-Schonlein Purpura

Hemangiomas Kassalback-Merritt syndrome  

Coagulation test The results are as good as the corrected samples collection: Standards: Time from sample to test: - PT 24 hours , - pTT 4 hours. Blood/citrate ratio:1/9.

Laboratory approach to 2° H. defect

Laboratory approach to 2° H. defect

Haemostatic disorders which may present with normal coagulation screen and full blood count Mild van Willebrand disease Mild haemophilia A or B Mild factor XI or other single factor deficiency Factor XIII deficiency Glanzmann thrombasthenia Platelet storage pool disease Platelet release defect Vitamin C deficiency

Platelets Disorders -Acquired-Aspirin; Uremia -Glanzmann's Functional disorders -Acquired-Aspirin; Uremia Inherited -Glanzmann's -Bernard-Soulier -Gray platelet syndrome

Thrombocytopenia Absent Radius(TAR)

ITP •Most have benign &limited course Treatment options: 1.Conservative - wait & watch 2. Aggressive- - Steroids - IVIG - Rhogam - Rituximab - splenectomy

Von Willebrand Disease The most common inherited bleeding disorder Occurs in 1% of the population   Type 1 : mild deficiency of VWF Type2 : multimer dysfunction Type3: severe deficiency of VWF

Von Willebrand Disease(cont) DIAGNOSIS: Prolong PTT & BT SYMPTOMS: Epistaxis or gingival bleeding Menorrhaqra Post-partum hemorrhage Post-surgical bleeding Bleeding post-dental extraction

Von Willebrand Disease(cont)

Hemophilia Caused by an absence or decreased amount of a procoagulant VIII -Hemophilia A affects - 1 :5000 males XI -Hemophilia B affects - 1 :30000 males XI -Hemophilia C - Rare /Ethnicity

Extensive bruising of the left forearm and hand in a patient with hemophilia.

Hemophilia Arthropathy and Muscle Bleeding

Clinical Severity of Bleeding Disorders

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