title: Blastic transformation after splenectomy in a patient with nonvillous splenic marginal zone lymphoma with p53 overexpression: a case report.
abstract: A 61-year-old man with no subjective symptom was admitted to our hospital for further examination of the causes of anemia (hemoglobin, 9.5 g/dL) and thrombocytopenia (platelets, 9.2 x 10(4)/microL), which had been pointed out in a medical checkup half a year previously. A bone marrow examination showed 73% lymphoid cells. Immunophenotyping of these cells were CD19+CD20+CD3-CD5-CD10-CD23-, and light chain restriction (kappa) was positive by fluorescence-activated cell sorting analysis. A computed tomography scan showed mild splenomegaly. To confirm the diagnosis histologically, we performed a splenectomy. Finally, we diagnosed the patient's disease as nonvillous splenic marginal zone lymphoma (SMZL). A month after the splenectomy, the white blood cell count was remarkably increased to 7 x 10(4)/microL with the blastic transformation of lymphoid cells. We first treated the patient with fludarabine and then with the CHOP regimen (cyclophosphamide, hydroxydaunomycin, vincristine [Oncovin], and prednisone), but the disease was so refractory that the patient died of the disease 13 months after the splenectomy. Immunohistochemical staining and a molecular examination for p53 were carried out with specimens from the splenectomy. We found overexpression of the p53 protein in lymphoid cells and a point missense mutation in codon 280 at exon 8 that changed AGA (Arg) to AGT (Ser). This case may indicate the existence of a more aggressive subset of SMZL, suggesting a reconsideration of the roles of splenectomy and p53 overexpression in the diagnostic and therapeutic approaches to patients with SMZL.
authors.affiliation: Department of Internal Medicine, Asahikawa Kosei Hospital, 1-24, Asahikawa, 078-8211, Japan. yasukaki@seagreen.ocn.ne.jp
authors.forename: Yasutaka
authors.initials: Y
authors.lastname: Kakinoki
authors.forename: Hiroya
authors.initials: H
authors.lastname: Kubota
authors.forename: Hiroharu
authors.initials: H
authors.lastname: Sakurai
authors.forename: Teiko
authors.initials: T
authors.lastname: Sato
authors.forename: Yoshihiko
authors.initials: Y
authors.lastname: Tokusashi
mesh_terms: D004252:DNA Mutational Analysis; D017809:Fatal Outcome; D006801:Humans; D008213:Lymphocyte Activation; D018442:Lymphoma, B-Cell, Marginal Zone / Q000175:diagnosis* / Q000628:therapy; D008297:Male; D008875:Middle Aged; D020125:Mutation, Missense; D013156:Splenectomy; D013160:Splenic Neoplasms / Q000175:diagnosis* / Q000628:therapy; D016159:Tumor Suppressor Protein p53 / Q000235:genetics
chemical_list: D016159:Tumor Suppressor Protein p53