Causes of Thrombocytopenia in pediatrics other than ITPPresentation Transcript
Causes ofThrombocytopeniain pediatrics otherthan ITPPrepared by:-Asmaa Rabie Abdel MonemA 5th grade med studentAlexandria University Faculty of Medicine
Causes of thrombocytopeniaincreased destruction Immune mechanism Iodopathic (ITP) Secodary to infection, drugs, SLE Non-immune mechanism platelet consumption DIC ,HUS microangiopathic hemolyticanemia platelet destruction Hypersplenism , drugs Prothetic heart valve sequesteration Large spleenDecreased production Bone marrow depression Hereditary Fanconi anemia TAR syndrome Acquired Drugs , chemotherapy Inefction, hepatitis ,HIV,EBV Bone marrow infiltration Leukemia ,neuroblastoma Storage disease
Secondary immune mechanismDrug-Induced Thrombocytopenia•A number of drugs are associatedwith immune thrombocytopenia asthe result of either an immuneprocess or a megakaryocyte injury.
Heparin-induced hrombocytopenia (and rarely thrombosis) is seldom seen inpediatrics but occurs when, after exposure to heparin, the patient developsan antibody directed against the heparin/ platelet factor IV complex.
Non immune Platelet Destructionincrease platelet consumptionDIChemolytic-uremic syndromethromboticthrombocytopenic purpuraThe syndromes of DIC, hemolytic-uremic syndrome, and thromboticthrombocytopenic purpura share the hematologic picture ofThe microangiopathic hemolytic anemia is characterized by thepresence of RBC fragments, including helmetcells, schistocytes, spherocytes, and burr cells.
Hemolytic-Uremic Syndrome (HUS)Abnormal red cell morphologyNormal numbers ofmegakaryocytes.
Combined Platelet and Fibrinogen Consumption SyndromesKasabach-Merritt SyndromeHemangiomaplatelet trapping and activation ofcoagulation with fibrinogenconsumption and generation offibrin(ogen)
Non immune Platelet DestructionSequestration•Individuals with massive splenomegalydevelop thrombocytopenia, since thespleen acts as a sponge for plateletsand sequesters large numbers.•Most such patients will also have mildleukopenia and anemia on the CBC.Individuals who havethrombocytopenia caused by splenicsequestration should undergo a work-up to diagnose the etiology ofsplenomegaly, includinginfectious, infiltrative, neoplastic, obstructive, and hemolytic causes.
Thrombocytopenia–absent radius(TAR)The thrombocytopenia of TARsyndrome frequently remitsover the first few years of life.
Fanconi anemiaAplastic anemia not presentat birth, develops about 6yr of age; fatal withoutbone marrow transplant;chromosomal breakagechallenge test available forearly diagnosis.
Wiskott-Aldrich syndrome (WAS)MicrothrombocytopeniaEczemaRecurrent infectionsX-linkedNormal number ofmegakaryocytesSuccessful bonemarrowtransplantation curesWAS.Microthrombocytes
Refrences1. Nelson textbook of pediatrics 19th edition,Chapter 476.2. Dan L. Longo, HARRISON’S Hematology andOncology;3. Childhood idiopathic thrombocytopenic purpura(itp): over 40 year of experiences; MedicalJournal of Islamic World Academy of Sciences19:4, 151-160, 2011.-http://www.uptodate.com/contents/clinical.4-of-evaluation-and-manifestationschildren-in-thrombocytopenia