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May-Hegglin anomoly

View larger version (72K): [in this window] [in a new window]   Figure 1. The patient is a 5-year-old male who was being evaluated for thrombocytopenia. A CBC included a hemoglobin of 12g/dl, a WBC of 10.3k/ul with a normal differential and a platelet count of 87,000/ul. The smear shows several large platelets, one of which approximates the size of the surrounding RBCs. Of equal note is the presence of blue Dohle body-like inclusions in the cytoplasm of the PMNs. A diagnosis of May-Hegglin anomaly was made. This is an autosomal dominant disorder resulting in thrombocytopenia with giant platelets, with Dohle body-like inclusions most evident in the neutrophils but also seen in monocytes and eosinophils. The defect results from mutation in the MHY9 gene on chromosome 22.

View larger version (68K): [in this window] [in a new window]   Figure 2. A higher magnification of the previous image, the intensely blue cytoplasmic inclusion is more evident. There is no evidence of toxic granulation present in the PMN as would be expected if this inclusion were a Dohle body.

View larger version (70K): [in this window] [in a new window]   Figure 3. A macrothrombocyte is present in this view. The PMNs have blue cytoplasmic inclusions bordering the cell surface membrane. These inclusions contain precipitated non-muscle myosin heavy chains characteristic of this group of congenital quantitative platelet disorders. Neutrophil function is this disorder is normal.

View larger version (85K): [in this window] [in a new window]   Figure 4. Typical dark blue cytoplasmic inclusion noted in May-Hegglin anomaly is illustrated.

View larger version (79K): [in this window] [in a new window]   Figure 5. Cytoplasmic inclusions similar to those noted in the neutrophils in the previous images can also be seen in eosinophils (illustrated in this image) and monocytes.

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This Article Figures Only Services Similar articles in this journal Alert me to new content in the Image Bank Download to citation manager Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Lazarchick, J. Articles by Post, G. R Search for Related Content PubMed Articles by Lazarchick, J. Articles by Post, G. R Related Collections Phagocytic disorders - Chediak-Higashi, Pelger-Huet, May-Hegglin Related ASH-SAP Chapter Related Image Bank Image Sets Related ASH Education Book Article Social Bookmarking                   What's this?