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Molecular genetic haplotype segregation studies in three families with X-linked lymphoproliferative disease

  • Immunology/Allergology
  • Published:
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Abstract

Three families with X-linked lymphoproliferative disease were studied. Affected males clinically presented with severe or fatal infectious mononucleosis, acquired hypogammaglobulinaemia, hypergammaglobulinaemia M, and malignant lymphoma including Hodgkin disease. Haplotype analysis using various DNA markers from Xq25-q27 allowed the prediction of the carrier status in females and identification of the XLP status in asymptomatic males.

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Abbreviations

EA :

early antigen

EBNA :

Epstein-Barr nuclear antigen

EBV :

Epstein-Barr virus

PCR :

polymerase chain reaction

RFLP :

restriction fragment length polymorphism

theta :

recombination fraction

VCA :

viral capsid antigen

HIGMI :

X-linked hyper-IgM syndrome

XLP :

X-linked lymphoproliferative disease

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Authors and Affiliations

  1. Department of Paediatrics, University of Würzburg, Josef-Schneider-Strasse 2, D-97080, Würzburg, Germany

    V. Schuster, S. Seidenspinner & H. W. Kreth

  2. Department of Human Genetics, University of Würzburg, Josel-Schneider-Strasse 2, D-97074, Würzburg, Germany

    T. Grimm & W. Kreß

  3. Department of Paediatrics, University of Frankfurt, D-60596, Frankfurt, Germany

    S. Zielen

  4. Children's Hospital, D-58505, Lüdenscheid, Germany

    M. Bock

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  1. V. Schuster
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Schuster, V., Seidenspinner, S., Grimm, T. et al. Molecular genetic haplotype segregation studies in three families with X-linked lymphoproliferative disease. Eur J Pediatr 153, 432–437 (1994). https://doi.org/10.1007/BF01983408

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  • DOI: https://doi.org/10.1007/BF01983408

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