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Lecture 32

Platelet Disorders

What is the life span of platelets? 7-10 days
What is contained in alpha granules of platelets? 1)von Willebrand Factor 2)Fibrinogen 3)Chemokines 4)Thrombospondin 5)P-selectin
What is contained in dense granules of platelets? 1)ADP/ATP 2)Calcium 3)Serotonin
A X-linked congenital thrombocytopenia syndrome characterized by the presence of small platelets, immunodeficiency, and eczema. Wiskott-Aldrich Syndrome (ACS)
A autosomal dominant congential thrombocytopenia syndrome characterized by large/pale platelets which lack alpha granules. Gray platelet syndrome
A congential thrombocytopenia syndrome characterized by neutrophil inclusions and giant platelets. Other findings include hearing loss, cataract, and renal defects. MYH9-related (May-Hegglin, Sebastian, Fechtner, Epstein)
What is pseudothrombocytopenia? A result of platelet clumping in EDTA tube. Also large and giant platlets are miscounted by automated instruments.
What is the criteria for diagnosis of Immune Thrombocytopenic Purpura (ITP)? It is a diagnosis of exlusion. 1)Plt count<150,000 2)Decreased plt survivial 3)Increased megakaryocytes in marrow 4)Normal WBC number and morphology 5)Normal spleen size
Age of onset of Acute vs. Chronic Immune Thrombocytopenia Purpura Acute ITP occurs in preadolescent population. Chronic ITP occurs in post-adolescent population.
Sex predilection of Acute vs. Chronic Immune Thrombocytopenic Purpura (ITP) Acute ITP: 1:1 (M:F) and Chronic ITP 1:3 (M:F)
Typical platelet count in Acute vs. Chronic Immune Thrombocytopenic Purpura (ITP) Acute ITP has platelet count <20,000. Chronic ITP has platelet counts between 20-80,000
(T or F) Acute Immune Thrombocytopenia Purpura typically resolves on its own. True.
(T or F) Chronic Immune Thrombocytopenic Purpura is self-limited. False. Chronic ITP does not resolve without treatment.
What lymphoproliferative disorder is associated with ITP? CLL
What autoimmune disorder is commonly associated with ITP? SLE
What infections are commonly associated with ITP? CMV, HIV
What is the treatment for ITP with platelet counts <20,000? Steroids, IVIg, anti-D
What is the treatment for ITP with platelet counts 20-30,000? If asymptomatic, treat with prednisone. If symptomatic, treat with steroids, IVIg, and anti-D
What is the treatment for asymtomatic ITP with platelet counts 30-100,000? No treatment is required
What is the treatment for ITP with platelet counts 30-100,000 and minor bleeding? Prednisone
What is the treatment for ITP with platelet counts 30-100,000 and major bleeding? Treat with Prednisone, IVIg, and anti-D
What are the available second line treatments for ITP? Cyclophosphamide, Vinca alkaloids, Danazol, Vit C, Protein A immunoadsorption/Plasma exchange, Interferon alpha, Azathioprine, Amicar
(T or F) Relapse of ITP can occur after splenectomy. True. Relapse typically occurs in first 6 months.
Describe the immune complex deposition of drug-induced thrombocytopenia The inciting drug and antibody attach to the platelet surface and is an "innocent bystander". The platelets are removed by the reticuloendothelial system.
What are common drugs that incite immune complex deposition drug-induced thrombocytopenia? Quinidine, NSAIDs, Gold, Sulfa, Dilantin
Describe the Hapten type drug-induced thrombocytopenia The inciting drug attaches to the platelet surface, forming a hapten. Antibodies are formed aganist the hapten and as a result the platelet/drug complex is removed by the recticuloendothelial system.
Pathogenesis of Heparin-Induced Thrombocytopenia Type II It is an immune-mediated condition. IgG directed aganist PF4-Hep complex on platelets. The IgG binds to platelet Fc(gamma)II receptors, promoting platlet activation. Platelet activation induces platelet clumping and fibrin formation.
What is the treatment of Heparin-Induced Thrombocytopenia Type II Discontinue heparin therapy
When is the typical onset of HITT? 1)5-10 days after heparin start 2)Within 24 hours if recent exposure to heparin (<3 months)
What is the typical change in platelet count in HITT? 50% decrease from baseline platelet count or platelet count <100,000
What are the clinical manifestations of Thrombotic Thrombocytopenic Purpura? 1)Low platelet count 2)Fever 3)Renal Failure 4)Neurologic findings 5)Microangiopathic hemolytic anemia
Pathogenesis of TTP Either congential lack of ADAMSTS13 enzyme or acquired antibodies to ADAMSTS13 enzyme
What is the treatment for TTP? Plasma exchange
An obstetric complication characterized by low platelets, hypertension, and proteinuria. Preeclampsia
An obstetric complication characterized by elevated liver enzymes, low platelets, and hemolytic anemia. HELLP (H)emolytic anemia, (E)levated liver enzymes, (L)ow (P)latelet count
A common cause of renal failure in children associated with E. coli. Clinical manifestations include low platelet counts, fever, and microangiopathic hemolytic anemia. Hemolytic Uremic Syndrome
What is the inheritance pattern of Bernard Soulier Disease? Autosomal recessive
Pathogensis of Bernard Soulier Disease It is a disorder of platelet adhesion due to the absence of gycoprotein Ib receptor on the platelet
Pathogenesis of Glanzmann Thrombasthenia An intrinsic platelet defect associated with defect in Glanzmann IIbIIIa receptors on platelets resulting in defect in platelet aggregation.
(T or F) Each unit of platelet concentrate is from an individual donor. True.
How long are platelets viable at room temperature on a rocker? 5 days
Definition of Thrombocytosis Platelet count>450,000/uL
Created by: UVAPATH4