title: Pure red cell aplasia complicating the course of long-standing mantle cell lymphoma.
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.
authors.affiliation: Department of Haematology, School of Medicine, Laikon General Hospital, National and Kapodistrian University of Athens, 11527, Athens, Greece.
authors.forename: Alexander
authors.initials: A
authors.lastname: Kanellopoulos
authors.forename: Katrina
authors.initials: K
authors.lastname: Koutsi
authors.forename: George
authors.initials: G
authors.lastname: Georgiou
authors.forename: Themis
authors.initials: T
authors.lastname: Ntalagiorgos
authors.forename: Kyriaki
authors.initials: K
authors.lastname: Petevi
authors.forename: George
authors.initials: G
authors.lastname: Boutsikas
authors.forename: Loula
authors.initials: L
authors.lastname: Papageorgiou
authors.forename: Gabriela
authors.initials: G
authors.lastname: Gainaru
authors.forename: Pagona
authors.initials: P
authors.lastname: Flevari
authors.forename: Maria K
authors.initials: MK
authors.lastname: Angelopoulou
authors.forename: John
authors.initials: J
authors.lastname: Meletis
authors.forename: Theodoros P
authors.initials: TP
authors.lastname: Vassilakopoulos
mesh_terms: D000369:Aged, 80 and over; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D000970:Antineoplastic Agents / Q000627:therapeutic use; D001803:Blood Transfusion; D001853:Bone Marrow / Q000473:pathology; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D016130:Immunophenotyping; D020522:Lymphoma, Mantle-Cell / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D012010:Red-Cell Aplasia, Pure / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D000069283:Rituximab; D013163:Splenomegaly; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D000970:Antineoplastic Agents; D000069283:Rituximab