Journal of Hematopathology

, Volume 6, Issue 2, pp 71–82

Bone marrow histology in patients with a paroxysmal nocturnal hemoglobinuria clone correlated with clinical parameters

  • Sandra T. A. van Bijnen
  • Martine Ffrench
  • Nick Kruijt
  • Theo de Witte
  • Konnie M. Hebeda
  • Petra Muus
Original Article

DOI: 10.1007/s12308-013-0179-7

Cite this article as:
van Bijnen, S.T.A., Ffrench, M., Kruijt, N. et al. J Hematopathol (2013) 6: 71. doi:10.1007/s12308-013-0179-7
  • 190 Downloads

Abstract

Paroxysmal nocturnal hemoglobinuria (PNH) is a disease with variable presentation, including classical PNH (cPNH) and PNH with aplastic anemia (AA-PNH). Here, we describe bone marrow (BM) histology related to clinical findings in 67 patients with a PNH clone. Patients were divided in AA-PNH (n = 39) and cPNH (n = 28) based on clinical criteria and were compared to 17 AA patients without PNH clone. Median PNH clone size was higher in cPNH (75 %) than in AA-PNH (10 %) (p < 0.0001). BM cellularity was normal or increased in 65 % of cPNH, while it was decreased in AA-PNH and AA (95 % and 100 %) (p < 0.0001). Myelopoiesis and megakaryopoiesis were decreased in 85 % and 100 % of AA-PNH and 100 % of AA patients, but also in 86 % and 46 % of cPNH patients, even when peripheral blood values were normal. The percentage of CD59-deficient late-stage myeloid cells determined immunohistochemically correlated to PNH granulocyte clone size. Lymphoid nodules and increased mast cell numbers were present in all groups but more frequently in AA-PNH (38 % and 73 %) than in cPNH (20 % and 43 %). BM iron was decreased in 88 % of cPNH, while it was increased in AA-PNH (64 %) and AA (100 %) (p < 0.0001). Hemolysis was present in all cPNH but also in AA-PNH patients (67 %). In conclusion, cPNH patients had more cellular BM and more prominent erythropoiesis than AA-PNH patients. Nonetheless, cPNH patients also show AA features such as myeloid and megakaryocyte hypoplasia and inflammatory infiltrates, although more subtle than in AA-PNH. No significant differences were found between BM of AA patients with and without PNH clone.

Keywords

PNHAplastic anemiaBone marrow failureBone marrow histopathology

Supplementary material

12308_2013_179_MOESM1_ESM.tif (325 kb)
Supplementary Fig. 1Correlation between LDH levels and PNH granulocyte clone size. PNH granulocyte clone size (%) versus LDH levels (U/l) in the total group of AA-PNH and cPNH patients with available LDH levels at the time of first detection of the PNH clone (n = 64). Each dot represents a single patient. r2 and p value were calculated by linear regression analysis (TIFF 324 kb)

Copyright information

© Springer-Verlag Berlin Heidelberg 2013

Authors and Affiliations

  • Sandra T. A. van Bijnen
    • 1
  • Martine Ffrench
    • 2
  • Nick Kruijt
    • 3
  • Theo de Witte
    • 4
  • Konnie M. Hebeda
    • 3
  • Petra Muus
    • 1
  1. 1.Department of HematologyRadboud University Nijmegen Medical CentreNijmegenthe Netherlands
  2. 2.Laboratory of HematologyHospices Civils de LyonLyonFrance
  3. 3.Department of PathologyRadboud University Nijmegen Medical CentreNijmegenthe Netherlands
  4. 4.Department of Tumor ImmunologyNijmegen Centre for Molecular Life SciencesNijmegenthe Netherlands