Transmission and Scanning Electron Microscopy Study on Congenital Dyserythropoietic Anemia Type I

Conde, Eulogio; Мазо, Е Б; Baró, J; Lafarga, Miguel; Cuadrado, M A; Recio, M; Zubizarreta, A

doi:10.1159/000206735

Cited by 7 publications

(2 citation statements)

References 8 publications

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“…Many abnormalities (nuclear shape changes, multinuclearity, nuclear cytoplasmic asynchrony, maturation arrest, and internuclear chromatin bridging) resemble those of congenital dyserythropoietic anemia type I (CDA I) in humans. 4,11,12,16,17 Unsuccessful attempts to identify several secondary causes of dyserythropoiesis, including iron deficiency, B vitamin deficiency, and viral infection, suggest that it may be a primary congenital dyserythropoiesis. Few DNA ploidy and cell cycle distribution studies of CDA erythroblasts in humans have been done.…”

Section: Discussionmentioning

confidence: 99%

Congenital Dyserythropoiesis and Progressive Alopecia in Polled Hereford Calves: Hematologic, Biochemical, Bone Marrow Cytologic, Electrophoretic, and Flow Cytometric Findings

Steffen¹,

Elliott

Leipold

et al. 1992

J VET Diagn Invest

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Abstract. Congenital dyserythropoiesis with dyskeratosis is a slow, progressive, and often fatal disease in Polled Hereford calves. Affected calves have a macrocytic normochromic anemia with a mild reticulocytosis. Studies indicate that calves are hyperferremic with increased saturation of serum total iron binding capacity, which rules out iron deficiency as a cause. Other secondary causes of dyserythropoiesis, including cobalamin and folate deficiencies, are unlikely because serum cobalamin and folate levels of affected calves were normal. Virus isolation was negative, and failure to identify bovine retroviral antigens or antibodies from several calves suggested that viral agents were not involved. Bone marrow cytologic findings were similar to those in congenital hereditary dyserythropoiesis in humans and included occasional multinucleate cells, internuclear chromatin bridging between nuclei of partially divided cells, and, more frequently, irregular nuclear shapes and chromatin patterns. DNA content and cell cycle distribution of erythroid cells appeared normal, and no electrophoretic abnormalities were detected in erythrocyte membrane proteins. The Polled Hereford syndrome is similar in many ways to type I congenital dyserythropoiesis in humans and may be an appropriate biomedical model for studying erythroid proliferation during dyserythropoiesis.A congenital syndrome of anemia and alopecia in Polled Hereford calves characterized by nonregenerative anemia with ineffective erythropoiesis and dermatitis has been described recently.22 Cutaneous lesions are progressive, and hyperkeratotic dermatitis with dyskeratosis of individual stratum spinosum and follicula cells occurs r infundibuli.Within the Affected calves are unproductive and often die or must be disposed of prematurely. This paper further describes hematologic, biochemical, and bone marrow findings of affected calves. Materials and methodsTen Polled Hereford calves of similar ages, 5 normal and 5 previously diagnosed with congenital anemia and progressive alopecia, were compared. Calves were housed in barns or dry lots and fed alfalfa hay, grass hay, grain, and a protein supplement. Hemograms, including erythrocyte, leukocyte, reticulocyte, and differential blood counts, a were performed 3 times at monthly intervals.Serum sodium, potassium, chloride, glucose, urea nitrogen, creatinine, alkaline phosphatase, gamma glutamyl transferase, aspartate amino-transferase, total bilirubin, direct bilirubin, total protein, albumin, globulin, calcium, phosoFrom the Departments of Pathology (Steffen, Leipold, Smith) and Laboratory Medicine (Elliott), College of Veterinary Medicine, Kansas State University, Manhattan, KS 66506.Received for publication April 15, 1991. phorus b and Sorbitol dehydrogenase were determined. c Serum levels of cobalamin and folate were bioassayed.d The serum iron and total iron binding capacity were determined, and percent saturation of serum iron binding capacity was calculated. 21Thyroid assays were performed by a commercial laborato...

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Section: Discussionmentioning

confidence: 99%

Congenital Dyserythropoiesis and Progressive Alopecia in Polled Hereford Calves: Hematologic, Biochemical, Bone Marrow Cytologic, Electrophoretic, and Flow Cytometric Findings

Steffen¹,

Elliott

Leipold

et al. 1992

J VET Diagn Invest

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“…The contour of the nuclear membrane may be interrupted by invaginations, resulting in the presence of cytoplasm and even organelles within the nuclear region [26]. Internuclear bridges as long as 12 pm have been described [27].…”

Section: Specific Features Of Cda Imentioning

confidence: 99%

Congenital dyserythropoietic anemias

Marks

Mitus

1996

Am. J. Hematol.

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The congenital dyserythropoietic anemias (CDAs) are a group of relatively rare inherited anemias that share in common ineffective erythropoiesis and morphologic abnormalities of mature red blood cells and their precursors. Three major types of CDA and a number of variants have been described. The diagnosis and categorization of these disorders are facilitated by microscopic examination of the blood and bone marrow and by serologic testing. Management of patients currently consists of observation and supportive care. Because patients with CDAs may be at significant risk for secondary hemochromatosis, they require monitoring for this condition. Spienectomy may be of benefit in certain cases In which the anemia is particularly severe. Over the past few years advances have been made in understanding the pathogenesis of these disorders, and it now appears that CDA II results from enzymatic defects in the cellular glycosylation pathway. o 1-6 Wiley-Liss, Inc.

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Failure of Red Cell Production

Ball¹

2006

Pediatric Hematology

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Transmission and Scanning Electron Microscopy Study on Congenital Dyserythropoietic Anemia Type I

Cited by 7 publications

References 8 publications

Congenital Dyserythropoiesis and Progressive Alopecia in Polled Hereford Calves: Hematologic, Biochemical, Bone Marrow Cytologic, Electrophoretic, and Flow Cytometric Findings

Congenital Dyserythropoiesis and Progressive Alopecia in Polled Hereford Calves: Hematologic, Biochemical, Bone Marrow Cytologic, Electrophoretic, and Flow Cytometric Findings

Congenital dyserythropoietic anemias

Failure of Red Cell Production

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