Malformation of the Erythrocytes in a Case of Atypical Retinitis Pigmentosa

Bassen, Frank A.; Kornzweig, Abraham L.

doi:10.1182/blood.v5.4.381.381

Cited by 338 publications

(38 citation statements)

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“…The distorted erythrocytes seen in our experimental animals strikingly resembled the 'acanthocytes' of a rare hereditary human disease whch is associated with an abnormal lipid metabolism (Bassen and Kornzweig, 1950). In this disease all plasma lipid levels are reduced and P-lipoprotein is virtually absent (Ways, Read and Hanahan, 1963), findings which are the direct antithesis of those in the plasma of cholesterol-fed rabbits (Hirsch and Nailor, 1956;Fishberg, Friedfeld, Hoffmann, Stoller and Fishberg, 1950).…”

Section: Discussionmentioning

confidence: 58%

Haemolytic Anaemia in Cholesterol‐Fed Rabbits

Silver¹,

McMillan²,

Silver³

1964

Br J Haematol

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Section: Discussionmentioning

confidence: 58%

Haemolytic Anaemia in Cholesterol‐Fed Rabbits

Silver¹,

McMillan²,

Silver³

1964

Br J Haematol

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“…In the 35years since the recognition of abetalipoproteinaemia (Bassen and Kornzweig, 1950) over 50 cases have been reported (Herbert et al, 1983). The typical clinical course consists of presentation in infancy with steatorrhoea and failure to thrive.…”

Section: Discussionmentioning

confidence: 99%

Abetalipoproteinaemia in adults: Role of vitamin therapy

MacGilchrist

Mills

Noble

et al. 1987

J of Inher Metab Disea

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The retinal and neurological complications of abetalipoproteinaemia may be preventable by replacing vitamins A and E from an early age, but their role in adult presentations is less clear. Two adult females with abetalipoproteinaemia have received 8 and 10 years respectively of replacement therapy with vitamins A, E and linoleic acid. In Case 1, visual function improved objectively on commencing therapy but has subsequently deteriorated and her neuropathy has slowly progressed. The rate of progression of neurological impairment in Case 2 was slowed but not halted by therapy, and her severe visual disturbance was unaffected. Replacement by fat soluble vitamins has only a limited role in the management of abetalipoproteinaemia once irreversible neurological/retinal damage has occurred.

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“…The existence of a syndrome known as acanthocytosis [3], accompanied by inhibition of red cell sedimentation and a tendency to haemolysis must here be mentioned first. This rare disease is recognized by the abnormal shape of the red cells, which are spherical and crenated (acanthocytes = spiny red cells), by a coeliac syndrome, pigmentary degeneration of the retina and a neurological syndrome of the ataxic type.…”

Section: Haemolytic Anaemias Caused By a Dejiciency Of Inhibitors Of mentioning

confidence: 99%

The Role of Lysophosphatides and Fatty Acids in Haemolysis

Jeannet

Hässig

1964

Vox Sanguinis

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Summary The part played in the mechanism of haemolysis by the lysophosphatides and the fatty acids is reviewed. The following hypothesis is put forward: the haemolysis might be due under certain physiological and pathological circumstances to an increase of the lysophosphatide/phosphatide ratio within the erythrocyte membrane. Three main mechanisms may produce haemolysis: 1. an alteration of the erythrocyte metabolism, 2. the activation of the plasmatic phosphatidase A and 3. the activation of complement at the cell surface. Several arguments in favour of the role of the above mechanisms in haemolytic conditions are presented. Résumé Le rôle joué par les lysophosphatides et les acides gras dans les mécanismes de I'hémolyse est étudié à la lumière des données de la littérature. L'hypothèse suivante est proposée et discutée: dans diverses conditions physiologiques et pathologiques, I'hémolyse est conditionnée par une augmentation du rapport lysophosphatides‐phosphatides dans la membrane érythrocytaire; celle‐ci peut être le résultat de trois mécanismes principaux: 1. une altération intrinsèque du métabolisme érythrocytaire, 2. une activation de la phosphatidase A plasmatique et 3. une activation du complément à la surface érythrocytaire. Les arguments plaidant pour l'intervention de ces mécanismes dans les états hémolytiques sont passés en revue. Zusammenfassung An Hand der Literatur wird die Bedeutung der Lysophosphatide und der Fettsäuren bei der Hämolyse untersucht. Folgende Hypothese wird zur Diskussion gestellt: Wesentlich für das Zustandekommen der Hämolyse unter physiologischen und pathologischen Bedingungen ist eine Zunahme des Lysophosphatid Phosphatidverhältnisses in der Erythrozytenmembran. Die Verschiebung dieses Verhält‐nisses ist die Folge von drei Mechanismen: 1. einer Veränderung des Erythrozytenstoffwechsels, 2. einer Aktivierung der plasmatischen Phosphatidase A und 3. einer Komplementaktivierung an der Erythrozytenoberfläche. Die Argumente, die für und wider diese Hypothese sprechen werden eingehend dargelegt.

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Malformation of the Erythrocytes in a Case of Atypical Retinitis Pigmentosa

Cited by 338 publications

References 0 publications

Haemolytic Anaemia in Cholesterol‐Fed Rabbits

Haemolytic Anaemia in Cholesterol‐Fed Rabbits

Abetalipoproteinaemia in adults: Role of vitamin therapy

The Role of Lysophosphatides and Fatty Acids in Haemolysis

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