Thrombocytopenia History: PMHx: Drug, EtOH, cancer, hypothyroidism and autoimmune diseaseCauses:Marrow Hypoplasia Idiopathic Investigations:disorders Drug-induced: cytotoxics, FBC antimetabolites, thiazides PBF CT disease – a/w HypoT4 – do TFT LFT Infiltration Leukaemia U/S HBS – for splenomegaly & liver cirrhosis Myeloma Bone marrow biopsy - ↑ or normal megakaryocytes in ITP Carcinoma Hepatitis B serology Myelofibrosis ANA, dsDNA Osteopetrosis B12 / Folate deficiency – a/w alcoholism Rx:↑ pltlet DIVC – usually secondary to an infection Treat underlying conditionconsumption ITP Pltlet transfusion if <10 X 109/L, persistent bleeding or life threatening TTP bleeds (eg GI hemorrhage) Viral infections eg EBV, HIV ± splenectomy for hyperspenism—vaccinate pt first Bacterial infections: eg Gram – septicaemiaHypersplenism Lymphomas Liver disease – commonly caused by alcohol or Hep B. Causes portal hypertension resulting in hypersplenism, and ↓ production of thrombopoeitin which ↓ pltlet production in turn* exclude medications as a cause of bruising – aspirin, antipltlets, warfarin,NSAIDs, sulphur drugs, anti-malariaFeatures: Spontaneous bleeding – when platelet < 30 X 109/L Purpura / bruising / petechiae Oral (gums) / nasal (epistaxis) / GI / GU bleeding (menorrhagia) Optic fundal hemorrhage – in severe thrombocytopenia; may lead to fatal intracranial bleed**need to ddx from clotting factor d/o: haemarthrosis, previous h/obleeding tendencies and probs during op (is it new acquired prob orcongenital?) Digitally signed by DR WANA HLA SHWE DN: cn=DR WANA HLA SHWE, c=MY, o=UCSI University, School of Medicine, KT-Campus, Terengganu, ou=Internal Medicine Group, email@example.com Reason: This document is for UCSI University, School of Medicine students. Date: 2009.03.08 09:33:27 +0800
Idiopathic Thrombocytopenic Purpura (ITP) Thrombotic Thrombocytopenic Purpura (TTP)Pathophysiology: Pathophysiology: AutoAb vs pltlet membrane glycoprotein IIb-IIIa causing premature Defective metopoproteinen resulting in difficulties breaking down fibrin, removal of pltlets by monocytes/macrophages which causes thrombosis and thrombocytopenia. Presents in 2 settings: in children, and in adults Dx by exclusion Clinical features: similar to HUS except HUS afx K more (commonest cause of ARF in children) and brain less cf TTP brain more, K lessClinical features: Pentad of -1. In Children Onset 2-3 wks post viral illness 1. Fever Sudden onset purpura, oral and nasal bleeding 2. Thrombocytopenia No palpable splenomegaly 3. Microangiopathic haemolytic anaemia (MAHA) Normal PBF other than thrombocytopenia 4. Renal dysfunction / impairment Bone marrow: increased megakaryocytes 5. Neurological symptoms – fits, weakness, confusion, loss of vision Check that there is no DIVC or other systemic Purpura illnesses GI & intracerebral bleeds Haematuria2. In Adults More common in females Proteinuria Insidious onset Raised bilirubin and LDH; decreased haptoglobulin Chronic with remissions and relapses a/w CA stomach – check for this * Thrombosis leads to renal dysfunction, anaemia and neurologicalManagement: symptoms1. Children Usually self-limiting within a few wks - Withhold treatment if only mild symptoms Causes : similar causes to HUS Prednisolone 2mg/kg/day – for moderate or severe • Infection—E. coli O157, Shigella, pneumococcus, Coxsackievirus purpura, bruising or epistaxis, or pltlet <10 X 109/L • Drugs—OCP, cyclosporine, 5FU, ticlopidine Pltlet transfusion – for persistent epistaxis, GI bleed, • Tumours retinal hemorrhages suggestive of intracranial bleed • Pregnancy IVIg – for persistent bleed while on steroids • HIV2. Adults Less responsive to treatment. Relapses common • SLE Prednisolone 1mg/kg/day Inx: Pltlet transfusion for persistent / potentially life- • FBC—low Hb and platelets, raised neutrophils threatening bleeds • PBF—fragmented RBC IVIg (1g/kg) for severe hemorrhage or life-threatening • U/E/Cr—low Na, high urea/Cr/uric acid bleeds to raise pltlets temporarily. • Decreased haptoglobulin, Increased bili, LDH (haemolysis) Splenectomy + vaccination (pneumococcus, Management: meningococcus & H. influenzae) – if 2 relapses occur. Replaces metopoproteinen by plasmaphoresis or FFP 2/3 response rate. Platelet infusion for severe bleeding Life-long steroids if relapses are frequent Steroids Immunosuppressants (eg vincristine, Vincristine and splenectomy for relapses cyclophosphamide) – if unresponsive to splenectomy
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