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    Thrombocytopenia: DR Chamilka Jayasinghe

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    Thrombocytopenia is a low platelet count defined as less than 150,000/mm3. Platelets form plugs that stop bleeding in three phases: adhesion, release of chemical signals, and aggregation. Causes include decreased production, sequestration in the spleen, increased destruction, or dilution. Immune thrombocytopenic purpura is the most common cause, where antibodies destroy platelets in the spleen. It typically presents with bruising and bleeding and is diagnosed through blood tests and bone marrow biopsy. Treatment involves steroids, IVIG, or splenectomy in severe cases.

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    Thrombocytopenia: DR Chamilka Jayasinghe

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    rikarz
    135 views31 pages
    Thrombocytopenia is a low platelet count defined as less than 150,000/mm3. Platelets form plugs that stop bleeding in three phases: adhesion, release of chemical signals, and aggregation. Causes include decreased production, sequestration in the spleen, increased destruction, or dilution. Immune thrombocytopenic purpura is the most common cause, where antibodies destroy platelets in the spleen. It typically presents with bruising and bleeding and is diagnosed through blood tests and bone marrow biopsy. Treatment involves steroids, IVIG, or splenectomy in severe cases.

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    Thrombocytopenia

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    Thrombocytopenia is a low platelet count defined as less than 150,000/mm3. Platelets form plugs that stop bleeding in three phases: adhesion, release of chemical signals, and aggregation. Causes include decreased production, sequestration in the spleen, increased destruction, or dilution. Immune thrombocytopenic purpura is the most common cause, where antibodies destroy platelets in the spleen. It typically presents with bruising and bleeding and is diagnosed through blood tests and bone marrow biopsy. Treatment involves steroids, IVIG, or splenectomy in severe cases.

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    135 views31 pages

    Thrombocytopenia: DR Chamilka Jayasinghe

    Uploaded by

    rikarz
    Thrombocytopenia is a low platelet count defined as less than 150,000/mm3. Platelets form plugs that stop bleeding in three phases: adhesion, release of chemical signals, and aggregation. Causes include decreased production, sequestration in the spleen, increased destruction, or dilution. Immune thrombocytopenic purpura is the most common cause, where antibodies destroy platelets in the spleen. It typically presents with bruising and bleeding and is diagnosed through blood tests and bone marrow biopsy. Treatment involves steroids, IVIG, or splenectomy in severe cases.

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    Thrombocytopenia

    Dr Chamilka Jayasinghe

    Introduction & definition Haemopoeisis Mechanism of platelet plug formation Pathophysiology Causes of thrombocytopenia History & examination Laboratory investigations Immune thrombocytopenia

    Introduction & definition


    Thrombocytopenia is defined as a platelet count less than 150,000/mm3 Platelets circulate in vivo with a life span of 10-14 days. Thrombopoeitin is the major hormone that controls its production. Platelets are non nucleated cellular fragments produced by megakaryocytes within the bone marrow & other tissues. The normal platelet count is 150-400 *109/L

    Haemostasis
    Occurs in 3 phases 1. Vascular spasm 2. Platelet plug formation
    primary haemostasis

    3. Coagulation (2ry haemostasis)

    MECHANISM OF PLATELET PLUG FORMATION


    OCCURS IN THREE PHASES 1. Platelet adhesion- The first phase begins when platelets detect damage to a blood vessel and begin to adhere to the exposed surfaces. 2. Platelet release reaction- once stuck to a site of damage the platelets begin to change. Firstly they create extensions so that they can contact each other & then they release their contents. There are 2 types of chemical packages(granules) held within the cytoplasm of platelets; alpha granules that contain clotting factors, growth factors & fibroblasts ; & dense granules that contain ADP,ATP,Calcium ions & Serotonin.Other components are also present within the platelet that aids its function.Nearby platelets are stimulated into action by the release of ADP and Thromboxane A2(A prostoglandin found within platelets) Thromboxane and Serotonin act to cause vasoconstriction.

    3. Platelet aggregation:
    The ADP acts to make the nearby platelets sticky & adhere to the other recruited platelets,& when the collection is large enough it creates a platelet plug stopping the loss of blood through holes in small vessels.

    Platelet defects lead to signs & symptoms of primary haemostasis. 1ry haemostatic disorders are characterized by prolonged bleeding time And the characteristic physical examination findings are petechiae & purpura.

    Causes of thrombocytopenia

    1. Decreased productioncongenital/acquired 2. Sequestration of platelets within an enlarged spleen or other organ 3. Increased destructionimmune/nonimmune 4. Spurious 5. Dilutional

    1. 2.

    Immune thrombocytopenic purpura Drug induced thrombocytopenia-immune process/megakaryocyte injury Na valproate,phenytoin,sulfonamides,cotrimoxazole 3.Non immune platelet destruction- DIC/HUS/TTP Kassabach Merrit 4.Sequestration-massive splenomegaly infections,Infiltrative,neoplastic,obstructive,haemolytic conditions 5.Congenital Thrombocytopenic syndrome TAR Wiskott Aldrich syndrome 6. Neonatal alloimmune thrombocytopenic purpura 7.Acquired disorders causing decreased production Infiltrative disorders-Malignancies-ALL,Lymphomas Aplastic processes acquired/constitutional Platelet function disorders Congenital-Von Willebrand disease,Bernard Soulier,Glanzman Acquired liver ,renal disease ,eosinophilia

    History & examination


    Purpose is to differentiate between 1ry & 2ry haemostatic disorders & whether an inherited or acquired disorder. Epistaxis,bleeding gums,bleeding from tooth extractions Haemoptysis,haematemesis,haematuria,haematochezia,mele na rarely the initial symptoms Menstrual history Bleeding after childbirth Details of previous surgery Bleeding following circumcision Delayed bleeding from umbilical stump/defective wound healing Drug history Haemarthrosis *

    Examination
    Petechiae ,purpura,ecchymoses
    Splenomegaly,hepatomegaly* Lymphadenopathy* Haemarthrosis,deep muscle haematomas* Pallor* Features of collagen vascular disorders-SLE* Dysmorphism* * atypical features

    Laboratory investigations
    FBC Blood picture- platelet clumps,Giant platelets, RBC fragmentation Prolonged bleeding time Platelet function tests Bone marrow examination

    Normal bone marrow -Megakaryocytes

    Immune thrombocytopenic purpura


    1. One of the most common autoimmune disorders It occurs in 2 distinct clinical types An acute self limiting form observed almost exclusively in children 5 per 100,000 persons 2. A chronic form observed mostly in adults 3-5 per 100,000 & rarely in children. It is caused by autoantibodies to platelets.The antigenic target appears to be the glycoprotein 11b/111a complex The complex is trapped in the spleen and removed by splenic macrophages.The mechanism of origin of Abs is not known. Occurs commonly in healthy individuals & is only rarely the initial manifestation of lupus, HIV other auto immune disorders

    Clinical features-ACUTE ITP


    Onset is usually acute In>50% cases follows a viral infection,1-4 weeks later Cutaneous bleeding Mucosal bleeding-palatal petechiae,epistaxis,haematuria,G.I bleeding,menorrhagia Intracranial haemorrhage No hepatosplenomegaly OR lymphadenopathy

    Natural history
    In 70-80% of children with acute ITP spontaneous resolution will occur within 6 months

    10-20% of children will go on to develop chronic ITP

    Investigations
    FBC Blood picture to exclude other causes of thrombocytopenia

    Indications for bone marrow aspiration

    1.

    Presence of atypical features e.g organomegaly,significant lymphadenopathy,abnormal blood counts,suspicious blood picture 2. Before starting steroid therapy 3. Not responding to IV Ig therapy 4. Persistent thrombocytopenia beyond 6 months 5.Thrombocytopenia that recurs after initial response to treatment

    If atypical presentation
    ANA Coombs Ultrasound abdomen HIV testing

    Indications for hospitalization


    1. Severe life threatening bleeding (e.g ICH) regardless of platelet count 2. Plt count <20,000 with evidence of bleeding 3. Plt count <20,000 without bleeding but inaccessible to health care 4. Parents request admission *most childhood ITP remit spontaneously

    Indications for treatment


    1. Life threatening bleeding episode (e.g ICH) regardless of platelet count 2. Plt count <20,000with mucosal bleeding 3. Plt count <10,000 with any bleeding

    Treatment modalities
    1. Oral prednisolone 1-4 mg/kg for 2-3 wks or till plt count>20,000/mm3 then taper rapidly 2. IV methylprednisolone 3. IV Immunoglobulin 0.8-1g/kg/day *1-2 days Gives a rapid rise in platelets in 95% of patients 4. IV Anti Rh(D) immunoglobulin in Rhesus positive patients *all are effective in raising platelet count IVIG is the quickest No evidence that any of these treatments reduce complications or mortality from ITP. No influence on progression to chronic ITP. **platelet transfusions are CONTAINDICATED in acute ITP unless in the management of ICH.

    Intracranial Haemorrhage
    The most feared complication of ITP with a 50% mortality rate Cumulative risk of ICH in newly diagnosed ITP child within 1st year is <1% Risk is highest with platelet count <20,000,history of head trauma,aspirin use &presence of cerebral AV malformation. 50% of all ICH occurs after 1 month of presentation,30% after 6 months

    Emergency treatment of ITP with ICH


    1. IVIG 1g/kg/day for 2 days 2. IV anti Rh(D) 50-75 micrograms/kg 3. High dose IV Methylprednisolone 30mg/kg/day for 3 days 4. Platelet transfusion 5. Neurosurgical intervention if indicated 6. Splenectomy if other modalities fail & craniotomy required

    Chronic ITP

    Persistent thrombocytopenia after 6 months of onset (20%) Wide spectrum of manifestations -mild symptomless low platelet count -intermittent relapsing symptomatic thrombocytopenia Persistent symptomatic,haemorrhagic disease

    Management of chronic ITP



    1. 2.

    Majority will remit with time Exclude other causes of thrombocytopenia (immunodeficiency,lymphoproliferative disorders,collagen disorders &HIV infection) Symptomless children can be left without therapy and followed up in clinic Symptomatic children may require short courses of therapy for relapses Intermittent pulses of IVIg Intermittent anti-Rh(D) antibody treatment 3.Intermittent pulses of steroid

    Indications for splenectomy


    1. Persistence of disease after 12 months with 2. Bleeding symptoms and 3. platelet count <10,000/mm3 4. No response or only transient success with intermittent IVIg and Anti D or pulse steroids 5. No contraindications to surgery * restriction of lifestyle

    Over 70% remission following splenectomy


    Pre-splenectomy immunization against pneumococci,haemophilus influenzae type b and menenigococci ,mandatory 2 weeks before surgery Post splenectomy penicillin prophylaxis Preferable to avoid till child is at least over 5 years of age.

    Post splenectomy relapse


    Causes unknown in most cases - accessory spleen/s Treatment options: 1. Danazol 6. Cyclosporine 2. Vincristine 7.Dapsone 3. Azathioprine 4. Cyclophosphamide 5. Alpha interferon

    END

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