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USMLE

2016-12-13 07:30:52   0  举报

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Hemostasis

USMLE

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Primary

Process

Vasoconstriction

Platelet Adhesion

PLT Degranulation

PLT Aggregation

Disorders

Abnormalities in PLT

Quantitative

Immune Thrombocytopenic Purpura(ITP)

Type

Acute

Children

Weeks after viral infection

Self-limited, resolving within weeks

Chronic

Adults

Women of childbearing age

May secondary of SLE

IgG produced by plasma cells in the spleen

Treatment

Corticosteroid

Children respond well, relapse in adults

IVIG

Raise PLT count, short-lived

Splenectomy

Microangiopathic Hemolytic Anemia

Disease

Thrombotic Thrombocytopenic Purpura(TTP)

Decreased ADAMTS13

Hemolytic Uremic Syndrome(HUS)

E coli O157:H7 dysentery

Clinical Findings

Skin and Mucosal Bleeding

Fever

Renal Insufficiency(HUS)

Thrombi involve vessels of kidney

CNS Abnormalities(TTP)

Thrombi involve vessels of CNS

Lab Findings

Prolonged bleeding time

Normal PT/PTT

Anemia with schistocytes

Increased Megakaryocytes on bone marrow biopsy

Treatment

Plasmapheresis

Corticosteroids(TTP esp)

Qualitative

Bernard-Soulier Syndrome

Genetic GPIb deficiency

Thrombocytopenia with enlarged PLT

PLT adhesion impaired

Glanzmann Thrombasthenia

Genetic GPIIb/IIIa deficiency

Secondary

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