@article{oai:ir.kagoshima-u.ac.jp:00011433, author = {MAEDA, Kunihiko and MATSUDA, Mikio and NAGASHIMA, Ryu-ichi and IMAI, Yutaka}, issue = {Suppl. 2}, journal = {鹿児島大学医学雑誌=Medical journal of Kagoshima University}, month = {2016-10-31}, note = {A Trial of Application of the Updated Kiel Classification to Extranodal Malignant Lymphomas with Advice of K. Lennert and AC Feller : Proceedings of the Second Seminar on Malignant Lymphoma in Kagoshima, Japan. April 13 - 15, 1994, We have encountered a case with unusual type of lymphoma possibly originating in spleen. The case was a 58 years old Japanese male. Clinically he exhibited marked hepato-splenomegaly, no systemic enlargement of lymph nodes (LNs) except for splenic hilar nodes, liver dysfunctions and appearance of a few atypical lymphoid cells in peripheral blood (PB). Splenectomy, lymphadenectomy of the splenic hilar nodes and the wedged-biopsy of liver were performed for histological diagnosis as well as microscopic examinations of bone marrow (BM) and PB. The enlarged spleen (1,200g) displayed diffuse proliferation of immunoblastic cells with prominent plasmacytic differentiation, especially in cords of red pulps and marginal zone of white pulps. These cells revealed immunoreactivity for DBB42, CD19 but minimal reactivity for CD20. Monotypic light chain of immunoglobulins (λ) and bitypic heavy chains (γ and μ) were also detected in their cytoplasms. These cells infiltrated exclusively in sinuses of splenic hilar LNs, in sinusoid of liver and scatteredly in BM. Many T-cells incuding some transformed cells also infiltrated into the hepatic sinusoid, BM and splenic white pulp. Atypical lymphocytes appeared in PB did not show villous projections as far as we examined. The patient keeps disease-free condition after splenectomy without any chemotherapy except for transient thrombocytopenia. The morphological and immuno-cytochemical features of the major proliferating cells indicated that they were corresponding to the terminal stage in B-cell differentiation. These cellular characteristics and the less aggressive chinical behavior suggested us a variant of lymphoplasmacytic lymphoma (polymorphic subtype) possibly derived from spleen as a possible diagnosis. There were, however, some discrepancies between this case and the previous reported cases especially on the distribution pattern of the lymphoma cells in spleen, liver, LNs and BM and on the accompanying T-cell proliferation. Large cell lymphoma arisiag from marginal zone and microvillous lymphoma should be considered for the differential diagnosis.}, pages = {147--154}, title = {A Case Report of Unusual Malignant Lymphoma Possibly Derived from Spleen}, volume = {47}, year = {} }