Molecular basis of RhD-positive/D-negative chimerism in two patients

East Mediterr Health J. 2004 Jan-Mar;10(1-2):228-41.

Abstract

This study investigated two patients with Rh chimerism: patient A, a healthy individual, and patient B with myelofibrosis. Flow cytometry studies showed two red blood cell populations of Rh phenotypes R1r and rr at percentages of about 25% and 75% respectively. Normal RhD transcript sequences were found following RT-PCR. Genomic DNA (gDNA) showed normal exon, intron, GATA regions and exon/intron boundary sequences except for a single base change in intron 7 (C --> A) of exon 7 in patient A. The major change found in both patients was the absence of RHD exon 9 DNA in gDNA isolated from peripheral blood. These findings suggest a somatic mutation, probably in a stem cell common to the myeloid lineage of both patients, and indicate that patient A may undergo malignant transformation in the future.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Aged
  • Base Sequence
  • Chimerism*
  • Exons / genetics
  • Female
  • Flow Cytometry
  • Gene Expression Profiling
  • Genome, Human
  • Genotype
  • Humans
  • Insulator Elements / genetics
  • Introns / genetics
  • Leukemia, Myelogenous, Chronic, BCR-ABL Positive / genetics*
  • Molecular Conformation
  • Molecular Sequence Data
  • Mosaicism
  • Mutation / genetics
  • Phenotype
  • Primary Myelofibrosis / genetics*
  • Reverse Transcriptase Polymerase Chain Reaction / methods
  • Rh-Hr Blood-Group System / genetics*

Substances

  • Rh-Hr Blood-Group System
  • Rho(D) antigen