Case Report

International Journal of Hematology

, Volume 99, Issue 6, pp 777-781

First online:

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

  • Alexander KanellopoulosAffiliated withDepartment of Haematology, School of Medicine, Laikon General Hospital, National and Kapodistrian University of Athens
  • , Katrina KoutsiAffiliated withDepartment of Haematology, School of Medicine, Laikon General Hospital, National and Kapodistrian University of Athens
  • , George GeorgiouAffiliated withDepartment of Haematology, School of Medicine, Laikon General Hospital, National and Kapodistrian University of Athens
  • , Themis NtalagiorgosAffiliated withDepartment of Haematology, School of Medicine, Laikon General Hospital, National and Kapodistrian University of Athens
  • , Kyriaki PeteviAffiliated withDepartment of Haematology, School of Medicine, Laikon General Hospital, National and Kapodistrian University of Athens
  • , George BoutsikasAffiliated withDepartment of Haematology, School of Medicine, Laikon General Hospital, National and Kapodistrian University of Athens
  • , Loula PapageorgiouAffiliated withDepartment of Haematology, School of Medicine, Laikon General Hospital, National and Kapodistrian University of Athens
  • , Gabriela GainaruAffiliated withDepartment of Haematology, School of Medicine, Laikon General Hospital, National and Kapodistrian University of Athens
  • , Pagona FlevariAffiliated withDepartment of Haematology, School of Medicine, Laikon General Hospital, National and Kapodistrian University of Athens
    • , Maria K. AngelopoulouAffiliated withDepartment of Haematology, School of Medicine, Laikon General Hospital, National and Kapodistrian University of Athens
    • , John MeletisAffiliated withDepartment of Haematology, School of Medicine, Laikon General Hospital, National and Kapodistrian University of Athens
    • , Theodoros P. VassilakopoulosAffiliated withDepartment of Haematology, School of Medicine, Laikon General Hospital, National and Kapodistrian University of Athens Email author 

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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 aplasia Mantle cell lymphoma Rituximab Cyclosporine-A