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Table of Contents
Vol. 118, No. 2, 2007
Issue release date: September 2007
Section title: Review
Acta Haematol 2007;118:88–98
(DOI:10.1159/000105676)

Hematological Manifestations of Primary Mitochondrial Disorders

Finsterer J.
Krankenanstalt Rudolfstiftung, Vienna, Austria

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Article / Publication Details

First-Page Preview
Abstract of Review

Received: February 14, 2007
Accepted: May 08, 2007
Published online: July 18, 2007
Issue release date: September 2007

Number of Print Pages: 11
Number of Figures: 0
Number of Tables: 7

ISSN: 0001-5792 (Print)
eISSN: 1421-9662 (Online)

For additional information: http://www.karger.com/AHA

Abstract

At onset mitochondrial disorders (MID) frequently manifest as a mono-organic problem but turn into multisystem disease during the disease course in most of the cases. Organs/tissues most frequently affected in MID are the cerebrum, peripheral nerves, and the skeletal muscle. Additionally, most of the inner organs may be affected alone or in combination. Hematological manifestations of MID include aplastic, megaloblastic, or sideroblastic anemia, leukopenia, neutropenia, thrombocytopenia, or pancytopenia. In single cases either permanent or recurrent eosinophilia has been observed. Hematological abnormalities may occur together with syndromic or nonsyndromic MIDs. Syndromic MIDs, in which hematological manifestations predominate, are the Pearson syndrome (pancytopenia), Kearns-Sayre syndrome (anemia), Barth syndrome (neutropenia), and the autosomal recessive mitochondrial myopathy, lactic acidosis and sideroblastic anemia syndrome. In single cases with Leigh’s syndrome, MERRF (myoclonic epilepsy and ragged-red fiber) syndrome, Leber’s hereditary optic neuropathy, and Friedreich’s ataxia anemia has been described. Anemia, leukopenia, thrombocytopenia, eosinophilia, or pancytopenia can frequently also be found in nonsyndromic MIDs with or without involvement of other tissues. Therapy of blood cell involvement in MID comprises application of antioxidants, vitamins, iron, bone marrow-stimulating factors, or substitution of cells.

© 2007 S. Karger AG, Basel


Article / Publication Details

First-Page Preview
Abstract of Review

Received: February 14, 2007
Accepted: May 08, 2007
Published online: July 18, 2007
Issue release date: September 2007

Number of Print Pages: 11
Number of Figures: 0
Number of Tables: 7

ISSN: 0001-5792 (Print)
eISSN: 1421-9662 (Online)

For additional information: http://www.karger.com/AHA


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Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
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