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An inguinal lymph node biopsy of a woman with a one-month history of a progressive fever, fatigue, dyspnea, skin rash, and lymphadenopathy revealed a well-preserved basic structure, hyperplastic germinal centers, and an interfollicular region containing polyclonal plasma cell sheets, suggesting plasma cell-type multicentric Castleman disease (MCD). We initiated prednisolone and anti-interleukin (IL)-6 antibody (tocilizumab), without success. A biopsy specimen re-evaluation detected CD20-positive atypical large B cells infiltrating the small vessels within and around the lymph node and its capsule. We diagnosed her with intravascular large B-cell lymphoma (IVLBCL). Lymphoma cells were weakly positive for IL-6 by immunohistochemical staining. IL-6 from lymphoma cells may have caused the MCD-like presentation as a paraneoplastic etiology. Malignant lymphoma should be excluded before diagnosing MCD.

BackgroundThe gastrointestinal tract is a relatively common involvement site in lymphoma and, in such cases, intestinal perforation is a concern before and during chemotherapy. The prediction of intestinal perforation prior to chemotherapy is difficult, and there is no standard strategy to minimize the frequency of severely adverse gastrointestinal events in lymphoma cases.Case presentationThe 61-year-old female patient had a history of primary central nervous system lymphoma (PCNSL), diagnosed histologically as diffuse large B cell lymphoma (DLBCL). We administered six courses of intensive chemotherapy consisting of high-dose methotrexate and sequential whole-brain irradiation (40.5 Gy). After a 3-year remission of the PCNSL, the patient’s lymphoma recurred, involving the small intestine. 18F-FDG-PET/CT upon the recurrence before chemotherapy showed multiple nodular lesions in the patient’s gastrointestinal tract. Central nervous system lesions were not detected. We administered intensive salvage chemotherapy consisting of cyclophosphamide, high-dose AraC, methyl-prednisolone, etoposide, and rituximab. The response was a rapid partial response, but on day 10 after the initiation of salvage chemotherapy, she complained of abdominal pain with tenderness. The contrast-enhanced (CE)-CT revealed transmural ischemia of the intestine. On the 7th day after the onset of urgent abdominal symptoms, follow-up CE-CT showed that the ischemic lesion had become thin. We conducted elective surgery after waiting for the complete recovery of the patient’s white blood cell count. The pathological findings of resected intestine confirmed the elimination of the majority of lymphoma cells and concomitant partial necrotic tissue.ConclusionsWe were able to avoid the neutropenic period and safely conducted the surgical treatment for the subclinical perforation by using CE-CT. The combination of 18F-FDG-PET/CT before chemotherapy and CE-CT scanning for the targeted involvement site helped us evaluate the surgical indications and optimal timing of surgery in a lymphoma patient with gastrointestinal involvement.