title: An unusual indication for splenectomy in hairy cell leukaemia: a report of three cases with persistent splenomegaly after chemoimmunotherapy.
abstract: We describe three cases of relapsed hairy cell leukaemia (HCL) treated with pentostatin plus rituximab. All three achieved bone marrow complete remission but had persistent splenomegaly and hypersplenism. Because of the clinical uncertainty of its significance, they were all splenectomized. The spleen histology showed no evidence of HCL, but a five-fold thickening of the splenic capsule and areas of fibrosis in the red pulp. This process may have contributed to the lack of elasticity and caused the persistent splenomegaly. We discuss the clinical implications for future patient management. The three patients remain in remission at 1 + , 5 + and 9 + years.
authors.affiliation: Tel Aviv Sourasky Medical Centre, Tel Aviv, Israel.
authors.forename: Nadav
authors.initials: N
authors.lastname: Sarid
authors.affiliation: Queen's Hospital, Burton upon Trent, UK.
authors.forename: Humayun N
authors.initials: HN
authors.lastname: Ahmad
authors.affiliation: The Royal Marsden NHS Foundation Trust, London, UK.
authors.forename: Andrew
authors.initials: A
authors.lastname: Wotherspoon
authors.affiliation: The Royal Marsden NHS Foundation Trust, London, UK.
authors.forename: Claire E
authors.initials: CE
authors.lastname: Dearden
authors.affiliation: Division of Molecular Pathology, The Institute of Cancer Research, London, UK.
authors.forename: Monica
authors.initials: M
authors.lastname: Else
authors.affiliation: Division of Molecular Pathology, The Institute of Cancer Research, London, UK.
authors.forename: Daniel
authors.initials: D
authors.lastname: Catovsky
mesh_terms: D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002908:Chronic Disease; D004334:Drug Administration Schedule; D005260:Female; D006801:Humans; D007167:Immunotherapy / Q000009:adverse effects*; D007943:Leukemia, Hairy Cell / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D015649:Pentostatin / Q000008:administration & dosage; D012008:Recurrence; D012074:Remission Induction; D000069283:Rituximab / Q000008:administration & dosage; D013154:Spleen / Q000473:pathology; D013156:Splenectomy / Q000379:methods*; D013163:Splenomegaly / Q000209:etiology* / Q000473:pathology / Q000601:surgery
chemical_list: D015649:Pentostatin; D000069283:Rituximab
keywords: chemoimmunotherapy; hairy cell leukaemia; spleen histology; splenectomy; splenomegaly