Marked Elliptocytosis and Schistocytosis in Hematopoietic Dysplasia

Hartz, John W.; Buss, David H.; White, Dougľas R.; Bond, M. Gene; Scharyj, M.

doi:10.1093/ajcp/82.3.354

Cited by 21 publications

(17 citation statements)

References 22 publications

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“…Hartz et al [6] reported elliptocytosis and schistocytosis in a 78-year-old male patient present ing with a refractory anaemia that meets the diagnos tic criteria for MDS. The patient died 3 years later.…”

Section: Discussionmentioning

confidence: 99%

“…Some subgroups and closely related disorders of hereditary elliptocytosis, such as severe haemolytic elliptocytosis and hereditary pyropoikilocytosis, are characterized by striking poikilocytosis, microelliptocytosis and fragmentation, while there are only a few if any typi cal elliptocytes [4], Recently, elliptocytosis and schistocytosis were documented in haematopoietic dysplasia [6] and polycythaemia vera [7], In this report we describe marked elliptocytosis and schistocytosis as an acquired mani festation of myelodysplasia in two patients; one de veloped an acute myeloblastic leukaemia, the other is on follow-up and is treated for a refractory anaemia.…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Elliptocytosis and Schistocytosis in Myelodysplasia: Report of Two Cases

Rummens¹,

Verfaillie²,

Criel³

et al. 1986

Acta Haematol

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Marked elliptocytosis and schistocytosis are described as unusual manifestations of haematopoietic dysplasia in two patients. The first patient, whose history was negative for inherited haemolytic anaemias, presented these prominent features on his first admission; 22 months later he developed an acute myeloblastic leukaemia. In the second patient, followed since 4 years for an autoimmune thrombocytopenic purpura, elliptocytosis and schistocytosis appeared 17 months before a pancytopenia established. The patient is now on follow-up and is treated for a refractory anaemia. In both cases bone marrow examinations revealed the typical criteria for myelodysplasia and this diagnosis was confirmed by cytogenetic analysis.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Elliptocytosis and Schistocytosis in Myelodysplasia: Report of Two Cases

Rummens¹,

Verfaillie²,

Criel³

et al. 1986

Acta Haematol

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“…A few case reports presenting an association between MDS and elliptocytosis have already been presented [5][6][7][8][9][10]. In two cases, elliptocytosis was associated with a qualitative and/ or quantitative alteration in the protein 4.1R [7,10].…”

Section: Introductionmentioning

confidence: 91%

Association between myeloid malignancies and acquired deficit in protein 4.1R: A retrospective analysis of six patients

et al. 2007

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Constitutional deficit in the erythroid protein 4.1 (4.1R), a structural component of the erythrocyte membrane, is implicated in hereditary elliptocytosis. Acquired deficit in protein 4.1R have been rarely described in myelodysplastic syndromes. Here, we report a series of six patients presenting a myelodysplastic or a myeloproliferative disease in association with an elliptocytosis curve on osmotic gradient ektacytometry and a significant decrease in protein 4.1R level. We confirm that deficit in protein 4.1R is recurrent in myeloid malignancies and should be particularly investigated when deletion del (20 q) is present, since we found this chromosomal abnormality in four out of six patients.

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“…Table 1. Cases of myelodysplastic syndrome with marked elliptocytosis Hartz et al, 1984Rummens et al, 1986Ideguchi et al, 1993Boavida et al, 1997Ishida et al, 1999 In conclusion, this is the seventh reported case of MDS with marked elliptocytosis. It is likely that the characteristic elliptocytosis is derived from acquired clones with abnormal development of the erythroid lineage, and is disproportionately associated with reduced protein 4.1 and deletion of chromosome 20q.…”

Section: Discussionmentioning

confidence: 77%

Protein 4.1 deficiency and deletion of chromosome 20q are associated with acquired elliptocytosis in myelodysplastic syndrome

et al. 2004

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We report a case of myelodysplastic syndrome (MDS), associated with prominent elliptocytosis. A 66-year-old male presented with peripheral pancytopenia, and was diagnosed with MDS [refractory anaemia (RA)]. Apart from marked elliptocytosis, dyshaematopoietic features were not evident in his peripheral blood or hypercellular bone marrow. After 18 months, he had progressed to RA with excess blasts in transformation. Analysis of red blood cell membrane proteins by sodium dodecyl sulphate-polyacrylamide gel electrophoresis (SDS-PAGE) showed a reduced quantity of protein 4.1 (30% of control). Deletion of chromosome 20q was identified by conventional cytogenetic analysis and fluorescence in situ hybridization. Marked elliptocytosis, persistent for more than 17 months, decreased strikingly after chemotherapy with idarubicin and Ara-C. These findings suggest that acquired elliptocytosis occurred as an unusual morphological feature of MDS, associated with abnormalities of protein 4.1 and chromosome 20q.

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Marked Elliptocytosis and Schistocytosis in Hematopoietic Dysplasia

Cited by 21 publications

References 22 publications

Elliptocytosis and Schistocytosis in Myelodysplasia: Report of Two Cases

Elliptocytosis and Schistocytosis in Myelodysplasia: Report of Two Cases

Association between myeloid malignancies and acquired deficit in protein 4.1R: A retrospective analysis of six patients

Protein 4.1 deficiency and deletion of chromosome 20q are associated with acquired elliptocytosis in myelodysplastic syndrome

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