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A 4-year-old female. Referred to the hematology department. SCENARIO C/C: easy bruising and "rash" for 3 days. HPI: She developed an acute onset of easy bruising and "rash" 3 days ago. She has not had epistaxis, oral bleeding, gross blood in urine or stools. No hemarthrosis. No joint pain. HPI: SCENARIO No fever. No appetite change, No weight loss. She had URTI symptoms approximately 2 weeks ago. Travel Hx.: No travel history.. Family Hx.: She has 2 older brothers, neither of whom have had bleeding symptoms. Family hx. is –ve for any bleeding tendency. No hx. of malignancy or autoimmune diseases. Past Hx.: SCENARIO No past hx of a similar problem. No past hx. of prolonged bleeding of wound or dental extraction. She had an URTI 2 weeks ago. Physical Examinations: Vital signs: Normal. Growth parameters: Normal. Physical Examinations: Head and Neck: No bleeding or bruises. Chest Examinations: SCENARIO Heart & Lung are normal. Abdominal Examinations: No tenderness. No organomegally. She has diffuse petechial rash is noted on her neck, trunk, extremities and groin. It is non-blanchable or varying ages. CNS Examination: Normal. Lab.: SCENARIO CBC: Hb: 12.8 g/dl. Hct: 38.5 %. WBC: 6,000 with normal differential. Platelets: 5,000 “low” PT: 12 seconds. PTT:32 seconds. Peripheral blood smear: Normal RBCs & WBCs. Platelets: reduced in number and large size. Dx.: SCENARIO ITP ► Bleeding Disorders Review Hemostasis Hemostasis It is the process to stop blood loss. Three Phases: Primary Hemostasis PRIMARY HEMOSTASIS PRIMARY HEMOSTASIS: Endothelium Injury Release of vasoconstrictors Exposure of Subendothelial Collagen Platelets Adhesion: vWF adhers Platelets to Subendothelial Collagen Via GPIb Platelets Aggrigation These Factors (ADP & TXA2) Stimulate Platelets to bind together via GPIIb & GPIIIa Platelets activated and release ADP & TXA2 PRIMARY HEMOSTASIS PRIMARY HEMOSTASIS: Primary Hemostasis Platelet GPIIb/GPIIIa vWF •Ends with formation of platelets plug. GPIIb/GPIIIa Platelet GPIb vWF •Assessment: •Platelets Counts: •150 – 450 X 103/ml. •Bleeding Time BT: •<8 mins. Secondary Hemostasis SECONDARY HEMOSTASIS SECONDARY HEMOSTASIS: Intrinsic Extrinsic PT/ INR aPTT Thrombin PT aPTT TT Depends on the activities of coagulation factors. Ends with formation of Fibrin clot. Secondary Hemostasis PT: Prothrombin Time: - Normal: 11 – 24 sec. aPTT: activated Partial Thromboplastin Time: - Normal: 22 – 35 sec. INR: International Normalization Ratio: - Normal: 0.9 – 1.2 Secondary Hemostasis PT: Play Tennis: - Tennis is played outside → Extrinsic Pathway. PTT: Play Table Tennis: - Table Tennis is played inside → Intrinsic Pathway. Resolution Secondary Hemostasis Resolution: Disorders of Primary Hemostasis Primary Hemostasis Platelet GPIIb/GPIIIa vWF GPIIb/GPIIIa Platelet GPIb vWF •Characterized by superficial bleeding. petechiae, ecchymoses Primary Hemostasis Disorders of Disorders of Primary Hemostasis Platelets Disorders Low Platelets Count Platelets Dysfunction “Thrombophillia” “Thrombocytopenia” ↓Production Aplastic anemia ↑Destruction ITP TTP HUS Sequestration Splenomegaly Heridatery •Bernard Soulier Syndrome: “GPIb Deficiency” •Glanzmans Syndrome: “GPIIb/IIIa Deficiency. Acquired •Drugs. •Uremia. Immune Thrombocytopenic Purpura ITP ITP: •Common cause of thrombocytopenia. •Peak age: 2 – 6 years. •M=F. ITP •Pathophysiology: •Antibodies bind to platelets membranes → destruction by spleen. C/P: •50% presents 1-3 weeks after viral illnesses ITP (URTIs). •Sudden onset of petechiae, purpura, echymosis, epistaxis,... •No hepatomegaly or splenomegaly. Lab. Findings: •CBC: •Platelets count: •Mild: < 100,000/mm3. ITP •Moderate: < 50,000/mm3. •Severe: < 20,000/mm3. •Bleeding Time BT: prolonged. •PT & aPTT are normal. Treatment: •Mild cases: •No treatment is required. •Severe cases: (Platelets count < 50,000/mm3 ): ITP •Prednisolon. •IVIG. •Dangerous bleeding (e.g., intracrainial): •Platelets. •High dose steroids. •IVIG. •Emergency splenectomy. Treatment: •Chronic ITP: •Cytotoxic drugs: e.g., cyclophosphamide. ITP •Splenectomy if not responding to medical treatment. Henoch-Schönlein purpura (HSP) Henoch-Schönlein purpura (HSP) Henoch-Schönlein purpura (HSP): •Also called: •Allergic Purpura, or •Anaphylactoid Purpura. HSP: •Etiology: •Unknown. ITP •Considered as hypersensitivity small vessels vasculitis. •In most of cases, there is a history of preceding URTI. •Age: 2 – 10 years. •M>F. HSP: • C/P: ITP •Skin rash: •Purpuric rash. •Distributed mainly on the lower limb and buttock. HSP: • C/P: •Joints: Arthritis. ITP •Abdomin: •Abdominal pain. •Bleeding: hematemesis or melena. •Intussusception. •Renal: •Edema, HTN, hemturea, oligurea. •Normal serum complements. HSP: • C/P: •CNS: ITP •RARE. •Convulsion. •Stroke. •Facial palsy,.. HSP: ITP • Lab Findings: •Platelets count: normal. •BT, PT & aPTT: Normal. •Serum complements: Normal. •↑ serum IgA. •Ocuult blood in stool. •US abdomen: intusseception. •Urine analysis & RFTs. HSP: •Renal functions should be followed up to 1 ITP year after recovery. •Treatment: •Analgesic: •to control abdominal pain and arthritis. •Prednisolon: HSP: •Prognosis: •Complete recovery is the rule unless ITP severe renal disease occurs. Von Willebrand’s Disease vWD: •Quantitative or qualitative defect in vWF. vWD •vWF has two major functions: •Needed for platelets adhesion. •Carries factor VIII. •Therefore, vWD affects both primary & secondary hemostasis. vWD: •Classification: vWD • Mild quantitative defect. • The most common. Type I Autosomal Dominant. • qualitative defect. • vWF dysfunction TypeII Autosomal Dominant. • severe total quantitative defect. • No vWF produced. Type III Autosomal Recessive. vWD: •C/P: •Mucosal and cutaneous bleeding. vWD • Easy bruising. •Epistaxis. •Menorrhagia. •In severe cases: •Soft tissue hematomas, •GI bleeding. •Hemarthroses. vWD: •Investigations: vWD •Platelets count: normal. •BT: increased. •PTT: prolonged. •VIII: decreased. vWD: •Treatment: vWD •Desmopressin (DDVP). •Factor VIII concentrate. Disorders of Secondary Hemostasis Disorders of Secondary Hemostasis Disorders of Secondary Hemostasis Congenital •Hemophilia Acquired •Liver diseases. •Vitamin K deficiency. •DIC. •Present with deep bleeding: Joint, muscles, GI tract, GU tract, •Excessive prolonged post-traumatic bleeding. Hemophilia Hemophilia: Hemophilia •X-linked Recessive. Hemophilia: Hemophilia C/P: •Deep bleeding: 5Hs: •Hemarthrosis. •Hematoma. •Hematochezia. •Hematuria. •Head hemorrhage. •Easily bruising. •Prolonged wound bleeding… Hemophilia Hemophilia: Types: • Factor VIII deficiency. • Factor IX deficiency. • Factor XI deficiency. • “Christmas disease” Hemophilia A Hemophilia B X-Linked Recessive Hemophilia C Autosomal Recessive Hemophilia Hemophilia: Investigations: •aPTT: prolonged. •PT (& INR): Normal. •Factor assay. Hemophilia Hemophilia: Treatment: •Hemophilia A: •Desmopressin (DDVP). •Recombinant Factor VIII. •Anti-fibrinolytic agents (e.g. tranexamic add) •Fresh frozen plasma??? Hemophilia Hemophilia: Treatment: •Hemophilia B: •Recombinant Factor IX. •Anti-fibrinolytic agents (e.g. tranexamic add) •Fresh frozen plasma. Hemophilia Hemophilia: Treatment: •Hemophilia C: •Recombinant Factor XI. •Anti-fibrinolytic agents (e.g. tranexamic add) •Fresh frozen plasma. Approach a Patient with Purpura Definition of Purpura: Purpura •Red, nonblanching maculopapular lesions. •Caused by intradermal capillary bleeding. Causes: Purpura •Caused by disorders affecting platelets or blood vessels. History: •Age ? History •Onset? Acute vs. Chronic. •Bleeding in other site: •Mucus membranes. •Joints. •Orifices. •Easily bruising. •Prolonged wound bleeding. •Associated symptoms: •Abdominal pain. •Bloody stool. •Hematemesis. •Hematurea. •Joints pain. History History: •Associated symptoms: •Fever. •Bone pain. •Polyurea? Oligurea? •Lethargy? •Headache? •Symptoms of anemia? •Appetite? •Weight loss? •Hx. of recent URTI “during last 3 – 4 weeks”. •Drug use •Family history •Maternal history •Social history Physical Examinations Physical Examinations: •Genaeral Look. •Vital signs. •Growth parameter. Physical Examinations Physical Examinations: •Characteristic of rach: •Distribution. •Color. •Size. •Palpable? •Blachable/Non Blanchable. •Bleeding in mucus membrane e.g., gum,.. •Nose? Epistaxis. •Pallor? Physical Examinations Physical Examinations: •Retinal hemorrhage? •Joint examination. •Lymph nodes? •Abdominal examination: •Tenderness? •Organomegally? •Rectal examination. Lab.: Investigations •CBC. •Coagulation profile. •PT, INR. •aPTT. •TT. •Factor assay? •Biopsy of rash: •IgA deposits “ Leukocytoclastic vasculitis” -- HSP. •BM: •If suspect leukemia; orgaanomegally,bone pain, lymphadenopathy, prolonged fever,…