Watch-and-wait policy versus rituximab-combined chemotherapy in Japanese patients with intestinal follicular lymphoma.

BACKGROUND AND AIM: Few reports have demonstrated the effectiveness of treatments for intestinal follicular lymphoma (FL) because of the limited number of patients who undergo comprehensive small intestinal examinations. This study compared the efficacy of rituximab-combined chemotherapy in patients with asymptomatic and low tumor burden (LTB) intestinal FL, according to the criteria of the Groupe d'Etude des Lymphomes Folliculaires, with that of a "watch and wait" (W&W) approach. METHODS: The endoscopic examination for entire gastrointestinal tracts was performed in 29 Japanese patients with intestinal FL. These patients had CD21-positive follicular dendritic cells arranged in a duodenal pattern. In a prospective, two-center, open-label trial, this study evaluated the efficacy of rituximab-combined chemotherapy ([cyclophosphamide, doxorubicin, vincristine, and prednisone] or [cyclophosphamide, vincristine, and prednisone]) and prolonged treatment with rituximab (R-Chemo+prolongedR) in 14 patients and compared their outcomes with those of 15 patients managed with a W&W approach. RESULTS: Four patients managed with the W&W plan showed worsening macroscopic findings, lesion area enlargement, or clinical stage progression but stayed on this plan because they had LTB and experienced no changes in bowel function. In the R-Chemo+prolongedR group, all patients achieved complete remission; recurrence occurred in one patient, who was subsequently managed with the W&W plan because of LTB. There were no significant differences in progression-free survival between the two groups (P = 0.1045). Overall survival was 100% in both groups. CONCLUSIONS: The prognoses of patients with asymptomatic intestinal FL and LTB who were managed with a W&W strategy were comparable with those of patients receiving R-Chemo+prolongedR.

[Successful treatment with bortezomib for a patient with plasma cell leukemia accompanied by severe hyperbilirubinemia].

A 59-year-old woman was admitted to our hospital with jaundice, renal dysfunction, anemia and hypercalcemia. Primary plasma cell leukemia (PCL) was diagnosed based on findings of IgA-λ type M-protein, 22% plasma cells in the bone marrow and 23.1% plasma cells of WBC in the peripheral blood. Because the total bilirubin (T.Bil) level increased even after the administration of prednisolone (PSL), dexamethasone and methylprednisolone, the patient was started on bortezomib (0.7 mg/m(2) on days 1, 4, 8 and 11 for 3 weeks) combined with PSL (40 mg/day). The level of T.Bil decreased and the patient's condition remarkably improved. We then increased the dose of bortezomib to 1.0 mg/m(2) in the second course, but discontinued treatment just after starting the third course because NCI-CTCAE Grade 3 peripheral neuropathy developed. According to the criteria of the International Myeloma Working Group, the response category was VGPR (=very good partial response) at 1 month after pausing treatment. We recommend these novel agents for PCL, which is an aggressive form of extramedullary plasma cell cancer.

[Angioimmunoblastic T-cell lymphoma complicated with EBV-associated B-cell lymphoma].

A 71-year-old man with high fever and enlargement of the bilateral submandibular, cervical and inguinal lymph nodes was hospitalized at Hiroshima University Hospital. The immunohistochemical and pathologic findings from the biopsy specimens led to the diagnosis of angioimmunoblastic T-cell lymphoma (AILT) with a cluster of CD20-positive cells. Flow cytometry analysis by two-color staining did not reveal any neoplastic B cells. Southern blot analysis showed rearrangement of both the IgH gene and the TCR gene. Furthermore, PCR of the IgH gene using DNA extracted from purified CD19-positive cells from the lymph nodes showed a monoclonal band, and it was different from that of purified CD138-positive cells from the bone marrow. Furthermore, monoclonal Epstein-Barr virus (EBV) infection was detected with PCR using the SL18 and SL19 primers of the LMP-1 gene. Numerous EBER-positive cells were detected diffusely in the lymph nodes. These findings indicated a diagnosis of angioimmunoblastic T-cell lymphoma complicated with EBV-associated B-cell lymphoma, and that immunodeficiency in AILT led to an expansion of EBV infected B-cells.