Objective May-Hegglin anomaly, Sebastian syndromes, Fechtner syndromes and Epstein syndromes, are a group of autosomal dominant disorders caused by mutations of the MYH9 gene, which codes for nonmuscle myosin heavy chain IIA.Here we report two Chinese families with MYH9 disorders, and analyze the relationship between the clinical features and the E1841K mutation.Methods two MYH9 disorders families diagnosed as May Hegglin anomaly and Fechtner syndromes were included.Neutrophil NMMHCIIA and mutational analysis of MYH9 were analyzed by Immunofluorescence or an ABI 310 Genetic Analyzer.Result Most of the patients that come from the two families showed thrombocytopenia, giant platelets, and typical DShle body-like inclusion bodies in their neutrophils, which immunofluorescence were type Ⅰ pattern.An E1841K mutation was found in both two families.Conclusion The E1841K mutation was found in both two families though they expressed different clinical features.May be the nephritis, cataracts and deafness induced by an E1841K mutation was small portion of MYH9 disorders.More studys are needed to reveal the relationship between the clinical features and the E1841K mutation.