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Journal of Clinical Oncology, Vol 26, No 9 (March 20), 2008: pp. 1553-1555 © 2008 American Society of Clinical Oncology. DOI: 10.1200/JCO.2007.14.5847
Intravascular Large B-Cell Lymphoma With Acute Abdomen As a Presenting Symptom in a Patient With Systemic Lupus ErythematosusDivision of Clinical Immunology, Advanced Biomedical Applications, Graduate School of Comprehensive Human Sciences, University of Tsukuba, Tsukuba, Japan; Department of Internal Medicine (Neurology and Rheumatology), Shinshu University School of Medicine, Matsumoto, Japan
Division of Clinical Immunology, Advanced Biomedical Applications, Graduate School of Comprehensive Human Sciences, University of Tsukuba, Tsukuba, Japan
Division of Clinical and Experimental Hematology, Advanced Biomedical Applications, Graduate School of Comprehensive Human Sciences, University of Tsukuba, Tsukuba, Japan
Division of Diagnostic Pathology, Biomolecular and Integrated Medical Sciences, Graduate School of Comprehensive Human Sciences, University of Tsukuba, Tsukuba, Japan A 63-year-old woman with a 14-year history of systemic lupus erythematosus (SLE) presented with severe lower abdominal pain and hematochezia on the morning of May 4, 2006, and she was admitted to our hospital that night. She had been receiving treatment with prednisolone at 11 mg/d. On physical examination, lymphadenopathy, skin rash, and arthritis were not noted. The abdomen had hypogastric tenderness to palpation without peritoneal signs. WBC count, C-reactive protein, and lactate dehydrogenase (LDH) were elevated at 12,200/µL (normal: 4,000 to 9,000/µL), 2.35 mg/mL (normal: 0 to 0.2 mg/dL), and 555 U/L (normal: 124-232 U/L), respectively. Other laboratory values were within normal limits. Abdominal x-ray revealed gas in the small intestine. The patient had a nasogastric tube put in place and was forbidden to eat. Because she showed peritoneal signs on the morning of May 5, abdominal computed tomography (CT) was done immediately and revealed enlargement of the small intestine (Fig 1A, arrow) and superior mesenteric vein thrombosis (Fig 1B, arrow).
Segmentectomy of the small intestine and colostomy were performed at once. A resection specimen showed ischemic necrosis of the small intestine due to mesenteric vein thrombosis (Fig 2). Serum C3, C4, CH50, anti-dsDNA antibody, anti–β2-glycoprotein 1 cardiolipin antibody, and diluted Russell viper venom test ratio were 107 mg/dL (normal: 65 to 135 mg/dL), 21 mg/dL (normal: 13 to 35 mg/dL), 41.4 U/mL (normal: 25 to 51 U/mL), less than 2.0 U/mL (normal: < 6 U/mL), less than 1.2 U/mL (normal: <3.5 U/mL), and 1.1 (normal: < 1.3), respectively, on May 8. Histopathological analysis of a resection specimen showed that the lumina of the mesenteric veins contained clusters of large, abnormal lymphoid cells (Fig 3A, x10 hematoxylin and eosin; Fig 3B, x40 hematoxylin and eosin).
Immunohistochemical analysis showed that the abnormal cells were positive for CD20 and CD79a and negative for CD3, CD5, and CD10 (Fig 3C, x40 CD20). Hence, intravascular large B-cell lymphoma (IVLBCL) was diagnosed. Bone marrow showed normal cellularity without either lymphoma cells or hemophagocytic macrophages. CT of the whole body and brain magnetic resonance imaging failed to show the focus of the lymphoma. Soluble interleukin-2 receptor (sIL-2R) was elevated at 718 U/mL (normal: 190 to 650 U/mL). We thought that the lymphoma was aggravated because LDH was elevated at 988 U/L on May 25, and we started chemotherapy on May 27. The patient was treated with six cycles of CHP (cyclophosphamide, doxorubicin, and prednisolone) and rituximab. LDH and sIL-2R were normalized after chemotherapy. She has remained well with no recurrence for 15 months after the diagnosis of lymphoma. IVLBCL is a rare and systemic disease characterized by the presence of lymphoma cells only in the lumina of small vessels, particularly capillaries.1 Since the first case of IVLBCL in 1959,2 more than 300 cases have been reported to date.3 These reports revealed highly variable symptoms resulting from occlusion of small vessels by neoplastic cells in a variety of organs.1-7 It displays some differences in clinical presentation among diverse geographic areas, more frequently with CNS and cutaneous symptoms in Western countries and with hemophagocytic syndrome in Asian countries.1,3-7 In the literature, we found only one case of IVLBCL with acute abdomen due to occlusion of mesenteric veins.8 In general, this is an extremely aggressive lymphoma that responds poorly to chemotherapy, and death occurs in most cases within a short time of presentation.1,3-7 Murase et al reported that 62 patients in Japan were treated with anthracycline-based chemotherapies, with 39 of 58 (67%) cases evaluated achieving an objective response; median survival overall for the 62 cases was 13 months.3 On the other hand, anthracycline-based chemotherapies are associated with a nearly 60% response rate and a 3-year overall survival rate higher than 30% in Western countries.5,7 More than 30 cases of rituximab-combined chemotherapy in IVLBCL patients have been reported in the literature, and in more than half of them, sustainable remission was described, though the follow-up periods were less than 3 years in most cases.3,9-11 We treated our patient with six cycles of CHP and rituximab after operation; we did not administer vincristine because she had surgery on the small intestine and vincristine may possibly produce intestinal obstruction as a side effect. Abdominal pain is found in 8% to 37% of patients with SLE.12 Such pain is caused by various diseases including duodenal or gastric ulcer, pancreatitis, serositis, intra-abdominal abscess, ischemic bowel disease, and intra-abdominal vasculitis.12,13 In our case, abdominal CT showed superior mesenteric vein thrombosis. Therefore, we considered that the cause of the abdominal pain was superior mesenteric vasculitis or thrombosis due to SLE until we diagnosed the illness as IVLBCL by histopathology of a resection specimen. Medina et al reported that patients with intra-abdominal vasculitis or thrombosis had higher SLE Disease Activity Index (SLEDAI) scores than patients without intra-abdominal vasculitis or thrombosis among SLE patients with acute abdomen.13 In our case, her SLEDAI score was 0. In the literature, we found only one case of IVLBCL in a patient with SLE,14 though the association between autoimmune diseases and lymphoma is well known.15-18 The largest studies by far showed an overall three- to four-fold increased risk of non-Hodgkin lymphoma in association with SLE.15-18 The highly variable symptoms resulting from occlusion of small vessels in IVLBCL patients mimic any of the symptoms of the SLE itself or even of an associated antiphospholipid syndrome. SLEDAI score allowed us to rule out an SLE flare in our case. We reported this case of IVLBCL with acute abdomen due to occlusion of mesenteric veins in a patient with SLE because of its rarity. Our case suggests that combination therapy with surgical resection, CHP and rituximab may be effective in patients with IVLBCL of mesenteric veins. AUTHORS’ DISCLOSURES OF POTENTIAL CONFLICTS OF INTEREST The author(s) indicated no potential conflicts of interest.
REFERENCES 1. Gatter KC, Warnke RA: Intravascular large B-cell lymphoma, in Jaffe ES, Harris NL, Stein H, Vardiman JW (eds): World Health Organization: Pathology and Genetics of Tumors of Heamatopoietic and Lymphoid Tissues. Lyon, France, IARC Press, 2001, pp 177-178 2. Pfleger VL, Tappeiner J: On the recognition of systematized endotheliomatosis of the cutaneous blood vessels (reticuloendotheliosis?) [in German]. Der Hautarzt 10:359-363, 1959 3. Murase T, Yamaguchi M, Suzuki R, et al: Intravascular large B-cell lymphoma (IVLBCL): A clinicopathologic study of 96 cases with special reference to the immunophenotypic heterogeneity of CD5. Blood 109:478-485, 2007 4. DiGiuseppe JA, Nelson WG, Seifter EJ, et al: Intravascular lymphomatosis: A clinicopathologic study of 10 cases and assessment of response to chemotherapy. J Clin Oncol 12:2573-2579, 1994 5. Ferreri AJM, Campo E, Seymour JF, et al: Intravascular lymphoma: Clinical presentation, natural history, management and prognostic factors in a series of 38 cases, with special emphasis on the "cutaneous variant." Br J Haematol 127:173-183, 2004[CrossRef][Medline] 6. Murase T, Nakamura S, Kawauchi K, et al: An Asian variant of intravascular large B-cell lymphoma: Clinical, pathological and cytogenetic approaches to diffuse large B-cell lymphoma associated with haemophagocytic syndrome. Blood 109:478-485, 2004 7. Ponzoni M, Ferreri AJM, Campo E, et al: Definition, diagnosis, and management of intravascular large B-cell lymphoma: Proposals and perspectives from an international consensus meeting. J Clin Oncol 25:3168-3173, 2007 8. Kröber SM: Intravascular lymphoma as cause of acute abdomen [in German]. Pathologe 28:51-54, 2007[CrossRef][Medline] 9. Weichert G, Martinka M, Rivers JK: Intravascular lymphoma presenting as telangectasias: Response to rituximab and combination chemotherapy. J Cutan Med Surg 7:460-463, 2003[Medline] 10. Bouzani M, Karmiris T, Rontogianni D, et al: Disseminated intravascular B-cell lymphoma: Clinicopathological features and outcome of three cases treated with anthracycline-based immunochemotherapy. Oncologist 11:923-928, 2006 11. Wu SJ, Chou WC, Ko BS, et al: Severe pulmonary complications after initial treatment with rituximab for the Asian-variant of intravascular lymphoma. Haematologica 92:141-142, 2007 12. Hallegua DS, Wallace DJ: Gastrointestinal and hepatic manifestations, in Wallace DJ, Hahn BH (eds): Dubois’ lupus erythematosus (ed 6) Philadelphia, PA, Lippincott Williams & Wilkins, 2002, pp 843-861 13. Medina F, Ayala A, Jara LJ, et al: Acute abdomen in systemic lupus erythematosus: The importance of early laparotomy. Am J Med 102:100-105, 1997 14. Sanchez-Cano D, Callejas-Rubio JL, Vilanova-Mateu A, et al: Intravascular lymphoma in a patient with systemic lupus erythematosus: A case report. Lupus 16:525-528, 2007 15. Björnadal L, Löfström B, Yin L, et al: Increased cancer incidence in a Swedish cohort of patients with systemic lupus erythematosus. Scand J Rheumatol 31:66-71, 2002[CrossRef][Medline] 16. Bernatsky S, Boivin JF, Joseph L, et al: An international cohort study of cancer in systemic lupus erythematosus. Arthritis Rheum 52:1481-1490, 2005[CrossRef][Medline] 17. Zintzaras E, Voulgarelis M, Moutsopoulos HM: The risk of lymphoma development in autoimmune diseases. Arch Intern Med 165:2337-2344, 2005 18. Smedby KE, Baecklund E, Askling J: Malignant lymphomas in autoimmunity and inflammation: A review of risks, risk factors, and lymphoma characteristics. Cancer Epidemiol Biomarkers Prev 15:2069-2077, 2006
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Copyright © 2008 by the American Society of Clinical Oncology, Online ISSN: 1527-7755. Print ISSN: 0732-183X
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