Hepatitis C virus infection and locally advanced splenic marginal zone lymphoma
Splenic marginal zone lymphoma (SMZL) is a rare malignant B-cell neoplasm, usually with an indolent clinical course and favorable prognosis. Treatment options include chemotherapy, surgery, radiation and immunotherapy. In some recent studies an increased incidence of hepatitis C virus (HCV) infection in patients with SMZL was reported and its possible role in lymphomagenesis was emphasized. A 66-year-old woman with twelve-year history of HCV infection was admitted due to locally advanced abdominal tumor involving the spleen and the left part of the diaphragm. Transaminase serum levels were not elevated. Neither peripheral lymphadenopathy nor bone marrow pathology was found. Absolute blood lymphocyte, erythrocyte and platelet counts were normal. A splenectomy with partial diaphragm resection in one block was performed. Recovery was uneventful. Pathologic examination with immunohistochemistry revealed SMZL and confirmed a neoplastic infiltration of the resected diaphragm. Following surgery, chemotherapy (CHOP regimen) and immunotherapy (anti-CD20 antibody) were given. At the last follow-up 15 months after surgery, the patient was free of any symptoms of lymphoma. Surgical resection of even locally advanced SMZL with involvement of adjacent tissues can be performed as a diagnostic and therapeutic procedure. Splenectomy is especially indicated in symptomatic patients without other sites of the disease. HCV infection may result in increased risk of SMZL due to the induction of B-cell lymphoproliferation. Because of possible lymphoma regression following anti-viral therapy, a systematic screening for HCV in patients with SMZL seems to be valuable and helpful for treatment planning.
Key wordshepatitis C splenic neoplasm marginal zone lymphoma
Unable to display preview. Download preview PDF.
- 1.Arcaini L, Paulli M, Boveri E, Vallisa D, Bernuzzi P, Orlandi E, Incardona P, Brusamolino E, Passamonti F, Burcheri S, Schena C, Pascutto C, Cavanna L, Margini U, Lazzarino M: Splenic and nodal marginal zone lymphomas are indolent disorders at high hepatitis C virus seroprevalence with distinct presenting features but similar morphologic and phenotypic profiles. Cancer 100: 107–115, 2004PubMedCrossRefGoogle Scholar
- 2.Carbonari M, Caprini E, Tedesco T, Mazzetta F, Tocco V, Casato M, Russo G, Fiorilli M: Hepatitis C virus drives the unconstrained monoclonal expansion of VH1-69-expressing memory B cells in type II cryoglobulinemia: a model of infection-driven lymphomagenesis. J Immunol 174: 6532–6539, 2005PubMedGoogle Scholar
- 3.Hermine O, Lefrere F, Bronowicki JP, Mariette X, Jondeau K, Eclache-Sandreau V, Delmas B, Valensi F, Cacoub P, Brechot C, Varet B, Troussard X: Regression of splenic lymphoma with villous lymphocytes after treatment of hepatitis C virus infection. N Engl J Med 347: 89–94, 2002PubMedCrossRefGoogle Scholar
- 4.Iannitto E, Ambrosetti A, Ammatuna E, Colosio M., Florena AM, Tripodo C, Minardi V, Calvaruso G, Mitra ME, Pizzolo G, Menestrina F, Franco V: Splenic marginal zone lymphoma with or without villous lymphocytes. Hematologic findings and outcomes in a series of 57 patients. Cancer 101: 2050–2057, 2004PubMedCrossRefGoogle Scholar
- 6.Marasca R, Vaccari P, Luppi M, Zucchini P, Castelli I, Barozzi P, Cuoghi A, Torelli G: Immunoglobulin gene mutations and frequent use of VH1-69 and VH4-34 segments in hepatitis C virus-positive and hepatitis C virus-negative nodal marginal zone B-cell lymphoma. Am J Pathol 159: 253–261, 2001PubMedGoogle Scholar