Idiopathic thrombocytopenic purpura: pathophysiology and management.
Our understanding of the pathophysiology of ITP owes to pioneering work of W J Harrington in 1951, delineating the immunologic nature of platelet destruction. In ITP, antibody-coated platelets are destroyed by macrophages of RES. However, other mechanisms are also implicated: C-mediated platelet lysis and newly described C-independent peroxide injury. Both induce platelet fragmentation and lysis, generating procoagulant platelet microparticles (PMP). A third mechanism of platelet consumption in the microvasculature is proposed, based on overlapping syndromes of ITP and TTP in some patients. In assessing hemostasis in ITP, platelet counts alone is not sufficient. Evaluation of platelet clumping, giant platelets, and platelet activation, marked by increased PMP is useful. Patients with platelet activation or giant platelets bleed less and detection of clumping prevents unwarranted therapy. Thrombotic complications may develop in ITP. A syndrome, characterized by recurrent TIA-like symptoms, progressive memory loss due to ischemic small vessel disease is described. The management of ITP should include the search for and elimination of underlying causes and careful evaluation of hemostasis. Therapy is divided into definitive vs symptomatic measures. The former including splenectomy, danazol, chemotherapy offers lasting remission after therapy was stopped, while the later including glucocorticoids, gammaglobuin, antiD antibodies and others increases platelet counts but seldom sustains remission upon withdrawal. Danazol therapy is up-dated since it is an effective and safe definite measure in ITP.
Ahn
Yeon S
YS
University of Miami, Dept. of Medicine, USA.
Horstman
Lawrence L
LL
D003613:Danazol / Q000627:therapeutic use; D019468:Disease Management; D049673:History, 20th Century; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000209:etiology / Q000266:history / Q000503:physiopathology / Q000628:therapy
D007166:Immunosuppressive Agents; D003613:Danazol