International Journal of Hematology

, Volume 78, Issue 4, pp 344–348 | Cite as

Unusual Association between Increased Bone Resorption and Presence of Paroxysmal Nocturnal Hemoglobinuria Phenotype in Multiple Myeloma

  • Evangelos Terpos
  • Michalis Samarkos
  • Christos Meletis
  • Effie Apostolidou
  • Maria Tsironi
  • Konstantinos Korovesis
  • Despina Mavrogianni
  • Nora Viniou
  • John Meletis
Case Report


Paroxysmal nocturnal hemoglobinuria (PNH) clones deficient in glycosylphosphatidylinositol-anchored molecules, including CD55 and CD59, have been previously described in patients with multiple myeloma (MM). The aim of this study was to investigate the possible association between existence of the PNH phenotype and myeloma bone disease. Forty-three patients with newly diagnosed MM were the subjects of the study. Radiographic evaluation of the skeleton was performed in all patients at diagnosis. The following biochemical markers were measured: bone resorption markers (tartrate-resistant acid phosphatase isoform 5b [TRACP-5b]and N-terminal cross-linking telopeptide of type-I collagen [NTX]), bone formation markers (bone alkaline phosphatase [bALP] and osteocalcin [OC]), osteoprotegerin (OPG), soluble receptor activator of nuclear factor κB ligand (sRANKL), and interleukin 6 (IL-6). Detection of CD55- and/or CD59-deficient red cell populations was performed after diagnosis. Patients with MM had elevated mean baseline NTX, TRACP-5b, sRANKL, and IL-6 levels compared with controls, whereas the mean values of bALP, OC, and OPG were significantly decreased. Four patients had no osteolytic lesions, whereas 8 patients had 1 to 3 lytic lesions, and 31 patients had more than 3 lytic lesions and/or pathologic fractures in the skeletal survey. CD55- and/or CD59-deficient red cell populations were observed in 56% of patients with MM. There was a strong correlation between the presence of PNH-like erythrocytes and increased bone resorption, as measured by NTX,TRACP-5b, and sRANKL/OPG ratio (P < .03,P < .02, andP < .02, respectively). There was also a significant correlation between PNH phenotype and severe bone disease (P < .02). These results suggest that there is a possible link between PNH phenotype and increased osteoclastic activity in MM owing to a potential effect of myeloma microenvironment on a preexisting PNH clone. Further studies are required for clarifying this phenomenon and investigating possible mechanisms of this unusual association.

Key words

Multiple myeloma Paroxysmal nocturnal hemoglobinuria Tartrate-resistant acid phosphatase isoform 5b (TRACP-5b) N-terminal cross-linking telopeptide of type I collagen (NTX) Soluble receptor activator of nuclear factor κB ligand (sRANKL) 


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Copyright information

© The Japanese Society of Hematology 2003

Authors and Affiliations

  • Evangelos Terpos
    • 1
    • 2
  • Michalis Samarkos
    • 2
  • Christos Meletis
    • 2
  • Effie Apostolidou
    • 2
  • Maria Tsironi
    • 2
  • Konstantinos Korovesis
    • 2
  • Despina Mavrogianni
    • 2
  • Nora Viniou
    • 2
  • John Meletis
    • 2
  1. 1.Department of Hematology251 General Air Force HospitalAthensGreece
  2. 2.First Department of Internal MedicineUniversity of Athens School of Medicine, Laikon HospitalAthensGreece

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