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  • • Fever

    • Thrombocytopenic purpura

    • Hemolytic anemia

    • Neurologic abnormalities

    • Renal failure

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Epidemiology

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  • • Cause is unknown: Believed to be autoimmune response to endothelial cell antigen in small vessels

    • It is most common between ages 20 and 50 years

    • The thrombocytopenia is probably due to a shortened platelet lifespan

    • The microangiopathic hemolytic anemia is produced by passage of RBCs over damaged small blood vessels containing fibrin strands

    • The anemia is often severe, and it may be aggravated by hemorrhage secondary to thrombocytopenia

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Symptoms and Signs

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  • • Fever

    • Purpura and ecchymosis

    • Neurologic changes

    • -Headache

      -Confusion

      -Aphasia

      -Lethargy

      -Hemiparesis

      -Seizures

      -Coma

    • Hepatomegaly

    • Splenomegaly

    • Bleeding

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Laboratory Findings

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  • • Thrombocytopenia

    • Anemia

    • Increased lactate dehydrogenase (LDH)

    • Normal prothrombin time (PT), partial thromboplastin time (PTT), international normalized ratio (INR)

    • Reticulocytosis

    • Increased bilirubin

    • Decreased haptoglobin

    • Negative Coombs test

    • Fragmented RBCs on peripheral smear

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  • • End-organ damage (renal failure, neurologic changes) due to occlusion of arteries and capillaries by hyaline membranes composed of platelets and fibrinogen

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Rule Out

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  • • Other causes of thrombocytopenia and anemia

    • -Disseminated intravascular coagulation

      -Evan syndrome

      -Endocarditis

      -Vasculitis

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  • • CBC

    • PT, PTT, INR

    • LDH

    • Reticulocyte count

    • Serum bilirubin

    • Serum haptoglobin

    • Coombs test

    • Peripheral blood smear

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When to Admit

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  • • Severe thrombocytopenia

    • Bleeding

    • Neurologic changes

    • Acute renal failure

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When to Refer

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  • • All patients should be managed in consultation with a hematologist

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  • • Plasmapheresis with plasma exchange, corticosteroids, antiplatelet agents

    • Splenectomy can be used as an adjunct to medical treatment in order to reduce platelet and RBC loss

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Surgery

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Indications

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  • • As an adjunct to medical treatment

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Medications

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  • • Corticosteroids

    • Antiplatelet drugs (aspirin and dipyridamole)

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Treatment Monitoring

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  • • Platelet count

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Complications

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  • • Renal failure

    • Neurologic changes

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Prognosis

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  • • 65% remission with splenectomy alone

    • With combined therapy, prolonged remission can be achieved in most patients

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References

Allford SL et al. Current understanding of the pathophysiology of thrombotic thrombocytopenic purpura. J Clin Path. 2000;53:497.  [PubMed: 10961171]
Rock GA. Management of thrombotic thrombocytopenic purpura. Br J Hematol. 2000;109:496.  [PubMed: 10886194]

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