AJCP / REVIEW ARTICLE 176 Am J Clin Pathol 2000;113:176-190 © American Society of Clinical Pathologists Inherited Giant Platelet Disorders Classification and Literature Review

A b s t r a c t Inherited giant platelet disorders are extremely rare. The aim of this article is to review the clinical and laboratory features of this heterogeneous group and to arrive at a working classification. We conducted our literature search using the National Library of Medicine database. A total of 12 clinical entities were described. We classified them into 4 groups depending on the clinical and structural abnormalities. The pathophysiology of these disorders is largely unknown, and more research is needed, particularly in the light of recent advances in laboratory medicine. This review may provide a valuable reference for clinicians and may form a basis for future classification and research. Inherited giant platelet disorders (IGPDs) are a group of rare disorders characterized by thrombocytopenia, large platelets, and variable bleeding symptoms. The purposes of this study were to summarize the incidence, frequency, clinical manifestations, laboratory findings, and pathogenesis in each of these disorders and to clas-sify the IGPDs based on clinical and laboratory findings and pathogenesis