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Two different positions emerged from the discussion within the ICSH Working Group: (i) the inclusion under the definition of schistocytes of any type of small fragment of RBC cytoplasm; (ii) the requirement of specific morphology for RBC fragments to be included in the definition of schistocyte. I have read and accept the Wiley Online Library Terms and Conditions of Use Shareable Link Use the link below to share a full-text version of this article with your friends and colleagues. Learn more. Copy URL. The detection of schistocytes is an important morphological clue to the diagnosis of thrombotic microangiopathic anemia (TMA). Reporting criteria between different laboratories, however, are not uniform, owing to variability of shape and nature of fragments, as well as subjectivity and heterogeneity in their morphological assessment. Lack of standardization may lead to inconsistency or misdiagnosis, thereby affecting treatment and clinical outcome. The Schistocyte Working Group of the International Council for Standardization in Haematology (ICSH) has prepared specific recommendations to standardize schistocyte identification, enumeration, and reporting. They deal with the type of smear, method of counting, morphological description based on positive criteria (helmet cells, small, irregular triangular, or crescentshaped cells, pointed projections, and lack of central pallor). A schistocyte count has a definite clinical value for the diagnosis of TMA in the absence of additional severe red cell shape abnormalities, with a confident threshold value of 1. Automated counting of RBC fragments is also recommended by the ICSH Working Group as a useful complement to the microscope, according to the high predictive value of negative results, but worthy of further research and with limits in quantitation. They are usually absent or very rare in blood films of healthy individuals. The finding of schistocytes in the peripheral blood, especially in the absence of additional severe morphological RBC abnormalities, should lead to a prompt investigation for the presence of a thrombotic microangiopathy ( Moake, 2002 ). Thrombotic microangiopathic anemia (TMA) includes two major syndromes: thrombotic thrombocytopenic purpura (TTP) and hemolyticuremic syndrome. The prognosis for TTP, in particular, is poor without specific and immediate treatment. In recent years, the efficacy and lifesaving potential of early plasma exchange for TTP have increased the diagnostic importance of a schistocyte count even before the development of overt symptoms ( Rock, Porta BobbioPallavicini, 2000 ). A new developing area for schistocyte counting is monitoring of patients after transplantation of hematopoietic stem cells, in which TMA is a frequent and severe complication ( Zomas et al., 1998; Martinez et al., 2005; Ruutu et al., 2007; Lesesve et al., 2011 ). RBC fragments similar to schistocytes can be found in nonTMArelated genetic or acquired RBC disorders (e.g. RBC membrane defects, thalassemia, megaloblastic anemia, primary myelofibrosis, and thermal injuries). ![]() Precise morphological criteria for the recognition and enumeration of schistocytes still remain poorly defined, and there is variability in the morphological interpretation and reporting between laboratories and observers ( Lesesve et al., 2005 ). Lack of standardization may lead to inconsistent diagnostic information, thereby affecting treatment and clinical outcome. Although identification of RBC fragments apparently seems straightforward, there is no published consensus definition about what a schistocyte actually is ( Lesesve, Salignac Lecompte, 2001 ). Observer bias because of the specific request for schistocyte count has also been described ( Burns, Lou Pathak, 2004 ). Besides inconsistency of identification criteria, this variability may also depend on differences in the counting method (i.e. RBCs counted, the use of counting aids such as the Miller disk, the heterogeneity of reporting to physicians (presentabsent, qualitative or quantitative assessment). Only criteria on which there was full consensus have been included in the recommendation. Schistocytes are always smaller than intact red cells and can have the shape of fragments with sharp angles and straight borders, small crescents, helmet cells, keratocytes, or microspherocytes. A microscope check is needed for positive and macrocytic samples. Schistocytes are described in the literature with an infinite variability in shape, which encompasses all irregularly distorted RBCs, bizarre poikilocytes, burrshaped erythrocytes, atypical hyperchromic spherocytes, RBC ghosts, etc. Dacie Lewis, 1968; Glassy, 1998 ).
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