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SCENARIO A 4-year-old female. Referred to the hematology department. 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.

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SCENARIO HPI: 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.

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SCENARIO Past Hx.: 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.

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SCENARIO Physical Examinations: Head and Neck: No bleeding or bruises. Chest Examinations: 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.

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SCENARIO Lab.: 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.

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SCENARIO Dx.: ITP

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Bleeding Disorders

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Review Hemostasis

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Hemostasis It is the process to stop blood loss. Three Phases:

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Primary Hemostasis

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PRIMARY HEMOSTASIS PRIMARY HEMOSTASIS: Endothelium Injury Release of vaso- constrictors Exposure of Subendothelial Collagen Platelets activated and release ADP & TXA2 Platelets Aggrigation These Factors (ADP & TXA2) Stimulate Platelets to bind together via GPIIb & GPIIIa Platelets Aggrigation These Factors (ADP & TXA2) Stimulate Platelets to bind together via GPIIb & GPIIIa Platelets Adhesion: vWF adhers Platelets to Subendothelial Collagen Via GPIb

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PRIMARY HEMOSTASIS PRIMARY HEMOSTASIS:

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Primary Hemostasis Platelet vWF GPIb GPIIb/GPIIIa Assessment: Platelets Counts: 150 450 X 10 3 /ml. Bleeding Time BT:

ITP HSP: Etiology: Unknown. Considered as hypersensitivity small vessels vasculitis. In most of cases, there is a history of preceding URTI. Age: 2 10 years. M>F.

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ITP HSP: C/P: Skin rash: Purpuric rash. Distributed mainly on the lower limb and buttock.

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ITP HSP: C/P: Joints: Arthritis. Abdomin: Abdominal pain. Bleeding: hematemesis or melena. Intussusception. Renal: Edema, HTN, hemturea, oligurea. Normal serum complements.

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ITP HSP: C/P: CNS: RARE. Convulsion. Stroke. Facial palsy,..

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ITP HSP: Lab Findings: Platelets count: normal. BT, PT & aPTT: Normal. Serum complements: Normal. serum IgA. Ocuult blood in stool. US abdomen: intusseception. Urine analysis & RFTs.

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ITP HSP: Treatment: Analgesic: to control abdominal pain and arthritis. Prednisolon: Renal functions should be followed up to 1 year after recovery.

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ITP HSP: Prognosis: Complete recovery is the rule unless severe renal disease occurs.

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Von Willebrands Disease

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vWD vWD: Quantitative or qualitative defect in vWF. vWF has two major functions: Needed for platelets adhesion. Carries factor VIII. Therefore, vWD affects both primary & secondary hemostasis.

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vWD vWD: Classification: Mild quantitative defect. The most common. Type I qualitative defect. vWF dysfunction TypeII severe total quantitative defect. No vWF produced. Type III Autosomal Dominant. Autosomal Recessive.

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vWD vWD: C/P: Mucosal and cutaneous bleeding. Easy bruising. Epistaxis. Menorrhagia. In severe cases: Soft tissue hematomas, GI bleeding. Hemarthroses.

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vWD vWD: Investigations: Platelets count: normal. BT: increased. PTT: prolonged. VIII: decreased.

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vWD vWD: Treatment: Desmopressin (DDVP). Factor VIII concentrate.

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Disorders of Secondary Hemostasis

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Disorders of Secondary Hemostasis Congenital Acquired Hemophilia Liver diseases. Vitamin K deficiency. DIC. Present with deep bleeding: Joint, muscles, GI tract, GU tract, Excessive prolonged post-traumatic bleeding.

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Hemophilia

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Hemophilia: X-linked Recessive.

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Hemophilia Hemophilia: C/P: Deep bleeding: 5Hs : H emarthrosis. H ematoma. H ematochezia. H ematuria. H ead hemorrhage. Easily bruising. Prolonged wound bleeding

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Autosomal Recessive X-Linked Recessive Hemophilia Hemophilia: Types: Factor VIII deficiency. Hemophilia A Factor IX deficiency. Christmas disease Hemophilia B Factor XI deficiency. Hemophilia C

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Hemophilia Hemophilia: Investigations: aPTT: prolonged. PT (& INR): Normal. Factor assay.

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Hemophilia Hemophilia: Treatment: Hemophilia A: Desmopressin (DDVP). Recombinant Factor VIII. Anti-fibrinolytic agents (e.g. tranexamic add) Fresh frozen plasma???

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Hemophilia Hemophilia: Treatment: Hemophilia B: Recombinant Factor IX. Anti-fibrinolytic agents (e.g. tranexamic add) Fresh frozen plasma.

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Hemophilia Hemophilia: Treatment: Hemophilia C: Recombinant Factor XI. Anti-fibrinolytic agents (e.g. tranexamic add) Fresh frozen plasma.

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Approach a Patient with Purpura

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Purpura Definition of Purpura: Red, nonblanching maculopapular lesions. Caused by intradermal capillary bleeding.

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Purpura Causes: Caused by disorders affecting platelets or blood vessels.

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History History: Age ? 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.

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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

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Physical Examinations Physical Examinations: Genaeral Look. Vital signs. Growth parameter.

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Physical Examinations Physical Examinations: Characteristic of rach: Distribution. Color. Size. Palpable? Blachable/Non Blanchable. Bleeding in mucus membrane e.g., gum,.. Nose? Epistaxis. Pallor?

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Physical Examinations Physical Examinations: Retinal hemorrhage? Joint examination. Lymph nodes? Abdominal examination: Tenderness? Organomegally? Rectal examination.

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Investigations Lab.: 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,

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