Platelet Disorders. By : Saja Al-Oran

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1 Platelet Disorders By : Saja Al-Oran

2 Introduction The platelet arise from the fragmentation of the cytoplasm of megakaryocyte in the bone marrow. circulate in the blood as disc-shaped anucleate particles for 8-10 days. Platelets are responsible for primary hemostatic function Normal count is 150, ,000/mL.

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5 Quantity Disorders : 1-Thrombocytopenia : platelet count <150,000 causes : 1. Decreased production 2. increased destruction : (most common cause ) a.immune (ITP, HIT,SLE) b.non-immune (DIC,TTP) 3. Sequestration from splenomegaly 4. Dilutional after transfusions or hemorrhage

6 Causes :

7 CONT. Spontaneous bleeding occur when the platelet count falls below 20 10^9/L. Thrombocytopenia (< 10 10^9/L) may result in retinal haemorrhage and potentially fatal intracranial bleeding, but this is rare.

8 Clinical feature : Cutaneous bleeding ; petechiae (<2mm), purpura (2-10mm), ecchymosis (>10 mm) Splenomegaly. Mucosal bleeding: epistaxis, menorrhagia, hemoptysis, bleeding in GI, genitourinary. Excessive bleeding after procedures or surgery. Gingival bleeding.

9 Investigation : CBC ( decrease platelet count ) Bleeding time, PT, PTT Peripheral smear

10 Treatment : 1. Treat the underlying cause. 2. Platelet transfusion use depending on the cause and severity of thrombocytopenia. 3. Discontinue NSAIDs, other antiplatelet agents, and anticoagulants.

11 Idiopathic Thrombocytopenic Purpura (ITP) : (ITP) : is mediated by autoantibodies, most often directed against the platelet membrane glycoprotein IIb/IIIa, resulting in premature removal of platelet from the circulation by cells of the reticulo-endothelial system. The presentation depends on the degree of thrombocytopenia. (Spontaneous bleeding <20 10^9/L ). In adults, ITP more commonly affects females. Patients aged over 65 years should have a bone marrow examination to look for an accompanying B cell malignancy. IN ITP, peripheral blood film normal, greatly reduced platelet count, increase in megakaryocytes

12 Management of ITP : Many patients with stable compensated ITP and a platelet count of more than /L do not require treatment to raise the platelet count, except at times of increased bleeding risk, such as surgery. First-line therapy for patients with spontaneous bleeding with prednisolone 1 mg/kg daily. Second-line therapy : thrombopoietin receptor agonist (TPO-RA) eltrombag and romilpoistim,and splenectomy.

13 Other causes of thrombocytopenia : Thrombotic thrombocytopenia purpura (TTP) : widespread clots, renal malfunction, sheared RBC s on smear. Heparin induced thrombocytopenia (HIT) : recent history of commencement of heparin, widespread thrombosis possible. Disseminated intravascular coagulation (DIC) :fulminant infection signs, pregnant,always increase PT/PTT.

14 Thrombocytosis : 2-Thrombocytosis : (high platelet count) The most common reason it is reactive to another process such as infection, connective tissue disease, malignancy, iron deficiency,acute haemolysis or gastrointestinal bleeding. The presenting clinical features are usually those of the underlying disorder and haemostasis is rarely affected. Reactive thrombocytosis is distinguished from the myeloproliferative disorders by the presence of uniform small platelets, lack of splenomegaly, and the presence of an associated disorder.

15 Cont

16 Quality Disorders : 1-Von Willebrand s Disease (vwd) : autosomal dominant condition of deficient vonwillebrand s factor, sometime associated with FVIII deficiency. Clinical feature : ( epistaxis,bruising, gingival bleeding,petechiae,etc) Labs : bleeding time elevated, PT & PTT (normal ), platelet count normal. Ristocetin platelet test. Treatment : supportive ( iron replacement), DDAVP (desomopressin ), platelet transfusion if necessary.

17 2-Bernard-soulier syndrome : autosomal recessive condition of abnormal Gp1b expression. Clinical feature : ( epistaxis,bruising, gingival bleeding,petechiae,etc) Labs : bleeding time elevated, PT & PTT (normal ), platelet count low**. Ristocetin platelet test. Treatment : supportive (avoid antiplatelet), platelet transfusion if necessary.

18 3-Glanzmann s thrombasthenia : these conditions are mild disorders, with bleeding typically occurring after trauma or surgery but rarely spontaneously, it is autosomal recessive condition of abnormal GpIIb/IIIa. Clinical feature : (menorrhagia,bruising, gingival bleeding, epistaxis,etc) Labs : bleeding time elevated, PT & PTT (normal ), platelet count normal. Ristocetin platelet test. Treatment : supportive (avoid antiplatelet), platelet transfusion if necessary.

19 Thank you O

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