We conducted a multicenter, retrospective study to determine the anatomical distribution and prognostic factors of gastrointestinal (GI) follicular lymphoma (FL). This study included 125 patients with stage I and II 1 GI-FL. Of the 125 patients, the small intestine was examined in 70 patients, with double-balloon endoscopy and ⁄ or capsule endoscopy. The most frequently involved GI-FL site was the duodenal second portion (DSP) (81%), followed by the jejunum (40%); 85% of patients with involvement of the DSP also had jejunal or ileal lesions. The absence of abdominal symptoms and macroscopic appearance of multiple nodules were significantly present in the DSP-positive group. During a median follow up of 40 months, six patients showed disease progression. Patients with involvement of the DSP had better progression-free survival (PFS) than those without such involvement (P = 0.001). A multivariate analysis revealed that male sex, the presence of abdominal symptoms, and negative involvement of the DSP were independently associated with poor PFS. In conclusion, most patients with GI-FL have duodenal lesions associated with multiple jejunal or ileal lesions. Gastrointestinal follicular lymphomas involving the DSP might be a distinct entity showing a favorable clinical course. (Cancer Sci 2011; 102: 1532-1536

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Pregnancy with paroxysmal nocturnal hemoglobinuria (PNH) is associated with significant risk of complications, such as life-threatening thrombosis. Recently, eculizumab has come into clinical use and revolutionized the treatment of PNH. However, clinical information regarding eculizumab use for PNH during pregnancy is limited. The present report describes pregnancies with PNH treated with eculizumab that were registered with the Japan PNH study group and reviews the literature. In case 1, the patient received eculizumab throughout pregnancy and delivered a healthy neonate at term, although breakthrough hemolysis occurred at 20 weeks of gestation. In case 2, the patient discontinued eculizumab before pregnancy and developed preeclampsia at 27 weeks of gestation. She received eculizumab and delivered a preterm, but healthy, neonate by cesarean section. In case 3, the patient received eculizumab from 18 weeks of gestation and delivered a healthy neonate at term without any complications. Reports of 11 pregnant women treated with eculizumab were identified in the literature. Of 14 pregnancies, including our own cases, breakthrough hemolysis and preeclampsia occurred in five and two cases, respectively. There were no thrombotic complications, maternal or neonatal deaths, or fetal structural abnormalities. Thus, eculizumab appears to be safe and effective for managing PNH during pregnancy.

SummaryFunctional studies of the interleukin 2 receptor (IL2R) of two (ED515-D and Kit225) IL-2-dependent and three (ED515-I, 3T3-mill, and Hut102) IL-2-independent cell lines were done . All of these cell lines appeared to express high as well as low affinity IL-2R. However, ED515-1 and 3T3-ao11, which expressed the IL-2R 0 chain, did not bind IL-2 at all when IL-2 binding to their IL-2R a chain was blocked with anti-Tac monoclonal antibody, whereas the intermediate affinity binding in ED515-D, Kit225, and Hut102 cells remained. We tentatively called the high affinity IL-2R of the former cells pseudo-high affinity IL-2R. The dissociation constant of pseudo-high affinity IL-2R was higher than that of ordinary high affinity IL-2R. Internalization of cell-bound 125 1-IL2 into ED515-I and 3T3-ao11 cells was less efficient than that into ED515-D cells . The addition of IL-2 neither promoted cell growth nor upregulated IL-2R a chain expression in ED515-1 and 3T3-x/311 cells . Furthermore, tyrosine phosphorylation of the cellular proteins (p120, p98, p96, p54, and p38) was induced or enhanced in response to the addition of IL-2 in ED515-D and Kit225 cells, but not in the cell lines expressing pseudo-high affinity IL-2R. Finally, 125 1-IL2 crosslinking followed by SDS-PAGE analysis showed an 80-kD band corresponding to p65 + IL-2, in addition to bands corresponding to IL-2R a and ,0 chain + IL-2 in cells bearing ordinary high affinity IL-2R but not in cells with pseudo-high affinity IL-2R. Taken together, we consider that another protein whose molecular mass is approximately 65 kD is functionally important in IL-2 binding and subsequent signal transduction and may be the third component of IL-2R.L-2 is a lymphokine produced by T cells that induces proliferation and differentiation of T, B, and NK cells, as well as thymocytes and monocytes . Cells bind IL-2 with three different affinities, designated as low (Kd = 10 -8 M), intermediate (Kd = 10-9 M), and high (Kd = 10-11 M) (1-9). Studies using mAbs and affinity crosslinking with radiolabeled IL-2 have identified and characterized the heterodimeric structure of IL-2R (6-15) . A low affinity IL2R consists of an a chain (p55, Tac) (2, 12), and association and dissociation of IL-2 for the low affinity IL-2R are very rapid (t112 = 5 and 6 s, respectively) (16, 17) . The IL-2R a chain with a short intracytoplasrnic portion does not solely transduce a growth signal (3,4,18) . In contrast, the IL-2R 0 chain binds IL-2 with an intermediate affinity when expressed solely and appears to be more important in IL-2 signal transduction . The association and dissociation ofIL-2 for the intermediate affinity IL-2R are much slower (t1i2 = 45 and 290 min, respectively) . Because IL-2 binding to the high affinity IL2R takes on the characteristics ofthe low affinity IL-2R for its association and of the intermediate affinity IL-2R for its dissociation (612 = 37 s and 285 min, respectively), IL-2/high affinity IL-2R complexes are most stable (16,17). In cell lines that bind IL-2 with only in...