May-Hegglinsche Anomalie

Lechner, K; Breddin, K; Moser, Karen; Stockinger, L; Wenzel, Elke

doi:10.1159/000208814

Cited by 14 publications

(5 citation statements)

References 17 publications

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“…The "disc to sphere" transformation, which normally precedes all of the many platelet functions (adhesion/aggregation, release, clot retraction [Born, 1970]), has not been quantitatively studied to date. The relationship between platelet shape and disease has only been quantitatively reported for May-Hegglin thrombocytopathy using transmission electron microscopy (Lechner et al, 1969), but support for continuing this approach comes from the few known studies of platelet size in disease (Bigel et al, 1967a,b;Enticknap et al 1969;Garg et al, 1971;Kraytman, 1973). Finally, these geometric measurements promise to become clinically practical with the use of image processing systems developed for similar studies of erythrocyte geometry (Eden, 1972;Evans and Leblond, 1973).…”

Section: Discussionmentioning

confidence: 99%

“…Some studies have been made on the change in size of human erythrocytes (Child et al, 1970) and of platelets (Bigel et al, 1967a,b;Entick-nap et al, 1969;Garg et al, 1971;Kraytman, 1973) with known disease states using light microscopy. Such studies relating cell shape, rather than size, have only been made with electron microscopy (Bessis, 1973;Lechner et al, 1969).…”

Section: Introductionmentioning

confidence: 99%

“…), with both brlghtfield (Canham and Burton, 1968;Jay, 1975) and interference-holographic microscopy (Eden, 1972;Evans and Fung, 1972;Evans and Leblond, 1973;Tsang, 1974). Such studies have not been reported to date for blood platelets, which have been generally qualitatively analyzed for size and shape with phase-contrast microscopy (Bessis, 1973;Bigel et al, 1967a,b;Garg et al, 1971;Zucker and Borrelli, 1954) and with electron microscopy (Barnhart et al, 1972;Hovig, 1968;Lechner et al, 1969;Shimamoto et al, 1973). These studies of individual platelets have been almost exclusively limited to platelets settled or spread onto supporting surfaces (Bessis, 1973).…”

Section: Introductionmentioning

confidence: 99%

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Geometry of normal mammalian platelets by quantitative microscopic studies

Frojmovic¹,

Panjwani²

1976

Biophysical Journal

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The shape distributions of normal and hardened human and rabbit erythrocytes and platelets were obtained for edge-on orientations of a few hundred freely rotating cells from analyses of microphotographs obtained similarly as by Ponder(1930, Q. J. Exp. Physiol. 20:29) by phase-contrast microscopy at 800 X magnification. Major average diameters (d) and thicknesses (t) were estimated for both normal and hardened cells, and were used to calculate an average geometric axis ratio, rp = t/d, which increases to unity as cells become more spherical. Our fixation procedure did not alter these shape parameters: rp was unchanged for erythrocytes, with d and t values similar to those reported by Ponder (1930); platelets had d X t = 3.6 +/- 0.7 mum X 0.9 +/- 0.3 mum and 3.1 +/- 0.4 mum X 0.6 +/- 0.3 mum, respectively, for human and rabbit cells, with rp = 0.26 and 0.20, respectively. Agreement in rp was found with data obtained by a novel rheo-optical method which allows for a direct statistical averaging for large populations (greater than 100 X 10(3) cells). Histograms and linear correlation studies were made of the above three parameters (d,t,rp), as well as volume (V), total surface area are (S), and sphericity index (S.I.) calculated for both "prolate ellipsoid" and "disc with rounded edges" models. Results indicate very high linear correlations between rp - t, rp - S. I., and d -S, with high correlations for t - V,d -V and S. Data are in agreement with the few reports in the literature determined by other methods, with the best model for platelets appearing to be an oblate spheroid.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Geometry of normal mammalian platelets by quantitative microscopic studies

Frojmovic¹,

Panjwani²

1976

Biophysical Journal

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“…Its classical features are thrombocytopenia, giant platelets and basophilic inclusions (Dohle bodies) in a variable proportion of the leu cocytes. Hegglin et al (51) consider the M-H -A a disease , but the vast majority of subjects with this " disease" are relatives of propositi in whom the anomaly is found by chance (51)(52)(53)(54)(55)(56)(57)(58). Wassmu t h and his associates (52) were so convin ced t hat their thirteen family members with the "classical findings" were perfectly healthy t hat the platelets were not even counted.…”

Section: Mode Of Platelet Platelet Platelet Discrimi-descriptive Clasmentioning

confidence: 99%

Evidence of Phylogenetic Canalisation of the Circulating Platelet Mass in Man

Behrens¹

1972

Thromb Haemost

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SummaryPhylogenetically mammalian platelets are an apocrine secretion from nucleated thrombocytes, and biologically the number of circulating fragments is irrelevant compared with their total mass and biochemical function. The literature on human genetic derangements affecting the circulating platelet mass is reviewed. The product of platelet count and platelet volume is shown to be “canalised” since many congenital forms of thrombocytopenia are associated with a reciprocal increase in the mean platelet volume. Considerations of platelet size are essential since macrothrombocytopenia is a racial characteristic of many Mediterranean peoples and probably also occurs frequently in others races. The location of platelet disorders in relation to the phylogenetic canal provides an objective classification which has diagnostic, therapeutic and prognostic significance.

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“…The May-Hegglin [1] anomaly is a rare hereditary condition with an autosomal dominant trait, charac terized by giant platelets and Dohle inclusion bodies in the granulocytes [2][3][4]. May first described the anomaly in 1909 [2], and in 1945, Hegglin described the condition in a man and his two sons [3].…”

Section: Introductionmentioning

confidence: 99%

Successful Splenectomy in May-Hegglin Anomaly: Report of a Case with Platelet Kinetic Studies

Raveh

Krausz²,

Eldor³

1989

Acta Haematol

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Bleeding diathesis is not necessarily one of the conspicuous and serious characteristics of the raac-rothrombocytopenic syndromes, which include the May-Hegglin anomaly. Nevertheless, occasionally prolonged bleeding time may endanger organs or even life. We report a case of a young girl with severe thrombocytopenia due to May-Hegglin anomaly, who was facing blindness because of intraocular haemorrhages. Being unable to intervene in the pathogenesis of the disease, we performed splenectomy after proving splenic sequestration of platelets. This procedure allowed the performance of a successful bilateral vitrectomy.

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May-Hegglinsche Anomalie

Cited by 14 publications

References 17 publications

Geometry of normal mammalian platelets by quantitative microscopic studies

Geometry of normal mammalian platelets by quantitative microscopic studies

Evidence of Phylogenetic Canalisation of the Circulating Platelet Mass in Man

Successful Splenectomy in May-Hegglin Anomaly: Report of a Case with Platelet Kinetic Studies

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