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, ×10 hematoxylin and eosin; Fig 3B, ×40 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, ×40 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.

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The author(s) indicated no potential conflicts of interest.

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