Subjects:
BloodWork, Free Research Articles, Lymphoid Neoplasia
Topics:
prolymphocytes, splenic marginal zone b-cell lymphoma
A 68-year-old woman with a history of splenic marginal zone lymphoma (SMZL) diagnosed 6 years earlier presented with nasal congestion and was found to have normocytic anemia, marked lymphocytosis (white blood cell count, 118 900/µL; 81% lymphocytes), normal platelet count, and elevated serum lactate dehydrogenase (451 U/L) and uric acid (14.4 mg/dL). She had been treated with rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP), as well as idelalisib, ibrutinib, and splenectomy. Computed tomography scans revealed para-aortic lymphadenopathy. A peripheral blood smear showed 57% prolymphocytes (panel A: Wright-Giemsa stain; 100× objective, original magnification ×1000). Bone marrow biopsy revealed an infiltration of medium-size lymphoma cells with many lymphoma cells containing prominent nucleoli (panel B: hematoxylin and eosin stain; 4× objective, original magnification ×40; panel C: 40× objective, original magnification ×400). By using peripheral blood flow cytometry, we detected a kappa-restricted B-cell population showing low to moderate side scatter (SS) and bright CD45 characteristics without coexpression of CD5 or CD10 (panel D). Immunohistochemistry showed that the lymphoma cells were positive for PAX-5 and negative for CD3, CD5, cyclin D1, and SOX-11 (inset) (panel E: 40× objective, original magnification ×400). Compared with the karyotype shown at initial diagnosis (46,XX,t(2;14)(p11.2;q32),del(7)(q31q36),t(7;10)(q36;q22),i(8)(q10),t(9;14)(p12;q32.3)[9]/46,XX[11]), 2 sidelines with additional abnormalities were demonstrated: (46,XX,t(2;14)(p11.2;q32),del(7)(q31q36),t(7;10)(q36;q22),i(8)(q10),t(9;14)(p12;q32.3)[2]/46,sl,del(1)(q42),dup(6)(p12p23)[14]/47,sdl,+del(7)[2]/46,XX[2]). This indicates clonal evolution and disease progression. The findings support prolymphocytic transformation of SMZL.
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A 68-year-old woman with a history of splenic marginal zone lymphoma (SMZL) diagnosed 6 years earlier presented with nasal congestion and was found to have normocytic anemia, marked lymphocytosis (white blood cell count, 118 900/µL; 81% lymphocytes), normal platelet count, and elevated serum lactate dehydrogenase (451 U/L) and uric acid (14.4 mg/dL). She had been treated with rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP), as well as idelalisib, ibrutinib, and splenectomy. Computed tomography scans revealed para-aortic lymphadenopathy. A peripheral blood smear showed 57% prolymphocytes (panel A: Wright-Giemsa stain; 100× objective, original magnification ×1000). Bone marrow biopsy revealed an infiltration of medium-size lymphoma cells with many lymphoma cells containing prominent nucleoli (panel B: hematoxylin and eosin stain; 4× objective, original magnification ×40; panel C: 40× objective, original magnification ×400). By using peripheral blood flow cytometry, we detected a kappa-restricted B-cell population showing low to moderate side scatter (SS) and bright CD45 characteristics without coexpression of CD5 or CD10 (panel D). Immunohistochemistry showed that the lymphoma cells were positive for PAX-5 and negative for CD3, CD5, cyclin D1, and SOX-11 (inset) (panel E: 40× objective, original magnification ×400). Compared with the karyotype shown at initial diagnosis (46,XX,t(2;14)(p11.2;q32),del(7)(q31q36),t(7;10)(q36;q22),i(8)(q10),t(9;14)(p12;q32.3)[9]/46,XX[11]), 2 sidelines with additional abnormalities were demonstrated: (46,XX,t(2;14)(p11.2;q32),del(7)(q31q36),t(7;10)(q36;q22),i(8)(q10),t(9;14)(p12;q32.3)[2]/46,sl,del(1)(q42),dup(6)(p12p23)[14]/47,sdl,+del(7)[2]/46,XX[2]). This indicates clonal evolution and disease progression. The findings support prolymphocytic transformation of SMZL.

Prolymphocytic transformation is typically described in chronic lymphocytic leukemia. Increased prolymphocytes have also been reported in mantle cell lymphoma with leukemic expression. Cases of SMZL with increased prolymphocytes are extremely rare.

A 68-year-old woman with a history of splenic marginal zone lymphoma (SMZL) diagnosed 6 years earlier presented with nasal congestion and was found to have normocytic anemia, marked lymphocytosis (white blood cell count, 118 900/µL; 81% lymphocytes), normal platelet count, and elevated serum lactate dehydrogenase (451 U/L) and uric acid (14.4 mg/dL). She had been treated with rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP), as well as idelalisib, ibrutinib, and splenectomy. Computed tomography scans revealed para-aortic lymphadenopathy. A peripheral blood smear showed 57% prolymphocytes (panel A: Wright-Giemsa stain; 100× objective, original magnification ×1000). Bone marrow biopsy revealed an infiltration of medium-size lymphoma cells with many lymphoma cells containing prominent nucleoli (panel B: hematoxylin and eosin stain; 4× objective, original magnification ×40; panel C: 40× objective, original magnification ×400). By using peripheral blood flow cytometry, we detected a kappa-restricted B-cell population showing low to moderate side scatter (SS) and bright CD45 characteristics without coexpression of CD5 or CD10 (panel D). Immunohistochemistry showed that the lymphoma cells were positive for PAX-5 and negative for CD3, CD5, cyclin D1, and SOX-11 (inset) (panel E: 40× objective, original magnification ×400). Compared with the karyotype shown at initial diagnosis (46,XX,t(2;14)(p11.2;q32),del(7)(q31q36),t(7;10)(q36;q22),i(8)(q10),t(9;14)(p12;q32.3)[9]/46,XX[11]), 2 sidelines with additional abnormalities were demonstrated: (46,XX,t(2;14)(p11.2;q32),del(7)(q31q36),t(7;10)(q36;q22),i(8)(q10),t(9;14)(p12;q32.3)[2]/46,sl,del(1)(q42),dup(6)(p12p23)[14]/47,sdl,+del(7)[2]/46,XX[2]). This indicates clonal evolution and disease progression. The findings support prolymphocytic transformation of SMZL.
View largeDownload PPT

A 68-year-old woman with a history of splenic marginal zone lymphoma (SMZL) diagnosed 6 years earlier presented with nasal congestion and was found to have normocytic anemia, marked lymphocytosis (white blood cell count, 118 900/µL; 81% lymphocytes), normal platelet count, and elevated serum lactate dehydrogenase (451 U/L) and uric acid (14.4 mg/dL). She had been treated with rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP), as well as idelalisib, ibrutinib, and splenectomy. Computed tomography scans revealed para-aortic lymphadenopathy. A peripheral blood smear showed 57% prolymphocytes (panel A: Wright-Giemsa stain; 100× objective, original magnification ×1000). Bone marrow biopsy revealed an infiltration of medium-size lymphoma cells with many lymphoma cells containing prominent nucleoli (panel B: hematoxylin and eosin stain; 4× objective, original magnification ×40; panel C: 40× objective, original magnification ×400). By using peripheral blood flow cytometry, we detected a kappa-restricted B-cell population showing low to moderate side scatter (SS) and bright CD45 characteristics without coexpression of CD5 or CD10 (panel D). Immunohistochemistry showed that the lymphoma cells were positive for PAX-5 and negative for CD3, CD5, cyclin D1, and SOX-11 (inset) (panel E: 40× objective, original magnification ×400). Compared with the karyotype shown at initial diagnosis (46,XX,t(2;14)(p11.2;q32),del(7)(q31q36),t(7;10)(q36;q22),i(8)(q10),t(9;14)(p12;q32.3)[9]/46,XX[11]), 2 sidelines with additional abnormalities were demonstrated: (46,XX,t(2;14)(p11.2;q32),del(7)(q31q36),t(7;10)(q36;q22),i(8)(q10),t(9;14)(p12;q32.3)[2]/46,sl,del(1)(q42),dup(6)(p12p23)[14]/47,sdl,+del(7)[2]/46,XX[2]). This indicates clonal evolution and disease progression. The findings support prolymphocytic transformation of SMZL.

Prolymphocytic transformation is typically described in chronic lymphocytic leukemia. Increased prolymphocytes have also been reported in mantle cell lymphoma with leukemic expression. Cases of SMZL with increased prolymphocytes are extremely rare.

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2021
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