International Journal of Hematology

, Volume 99, Issue 6, pp 777–781

Pure red cell aplasia complicating the course of long-standing mantle cell lymphoma

Authors

  • Alexander Kanellopoulos
    • Department of Haematology, School of Medicine, Laikon General HospitalNational and Kapodistrian University of Athens
  • Katrina Koutsi
    • Department of Haematology, School of Medicine, Laikon General HospitalNational and Kapodistrian University of Athens
  • George Georgiou
    • Department of Haematology, School of Medicine, Laikon General HospitalNational and Kapodistrian University of Athens
  • Themis Ntalagiorgos
    • Department of Haematology, School of Medicine, Laikon General HospitalNational and Kapodistrian University of Athens
  • Kyriaki Petevi
    • Department of Haematology, School of Medicine, Laikon General HospitalNational and Kapodistrian University of Athens
  • George Boutsikas
    • Department of Haematology, School of Medicine, Laikon General HospitalNational and Kapodistrian University of Athens
  • Loula Papageorgiou
    • Department of Haematology, School of Medicine, Laikon General HospitalNational and Kapodistrian University of Athens
  • Gabriela Gainaru
    • Department of Haematology, School of Medicine, Laikon General HospitalNational and Kapodistrian University of Athens
  • Pagona Flevari
    • Department of Haematology, School of Medicine, Laikon General HospitalNational and Kapodistrian University of Athens
  • Maria K. Angelopoulou
    • Department of Haematology, School of Medicine, Laikon General HospitalNational and Kapodistrian University of Athens
  • John Meletis
    • Department of Haematology, School of Medicine, Laikon General HospitalNational and Kapodistrian University of Athens
    • Department of Haematology, School of Medicine, Laikon General HospitalNational and Kapodistrian University of Athens
Case Report

DOI: 10.1007/s12185-014-1563-5

Cite this article as:
Kanellopoulos, A., Koutsi, K., Georgiou, G. et al. Int J Hematol (2014) 99: 777. doi:10.1007/s12185-014-1563-5

Abstract

Pure red cell aplasia (PRCA) is a rare cause of severe hypoplastic anemia characterized by profound depletion of erythroid precursors. Although PRCA may be associated with lymphoproliferative diseases, it has never been described in mantle cell lymphoma (MCL). We report what to our knowledge is the first case of a patient with indolent, non-nodal MCL complicated by PRCA. The patient presented with severe hypoproliferative anemia in the setting of a long-standing diagnosis of B-cell chronic lymphocytic leukemia. Bone marrow studies revealed the complete absence of erythroid progenitors. Cyclin D1 positivity on immunohistochemistry, confirmed by a positive FISH for t(11;14) (q13;q32), established the final diagnosis of MCL in conjunction with PRCA. Rituximab monotherapy led to rapid remission of splenomegaly and the leukemic picture, but the patient achieved transfusion independency only with subsequent administration of cyclosporine-A, and remained so during the subsequent 15 months despite the gradual disease recurrence.

Keywords

Pure red cell aplasiaMantle cell lymphomaRituximabCyclosporine-A

Copyright information

© The Japanese Society of Hematology 2014