Pain as an emergent issue in thalassemia.
Pain as an emergent issue in thalassemia.
复制标题
疼痛是地中海贫血中的一个紧急问题。
DOI：
10.1002/ajh.21670
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发表时间：
2010
影响因子：
12.8
通讯作者：
T
中科院分区：
文献类型：
--
作者：
Trachtenberg,Felicia;Foote,Dru;Martin,Marie;Carson,Susan;Coates,Thomas;Beams,Owen;Vega,Olivia;Merelles-Pulcini,Manuela;Giardina,PatriciaJ;Kleinert,DorothyA;Kwiatkowski,Janet;Thompson,AlexisA;Neufeld,EllisJ;Schilling,Leann;T
Natural killer (NK)-cell lymphomas are aggressive and relapses occur early. Late relapses are exceptional. Ten relapses 17.5 (11–29) years after first complete remission (CR1) were analyzed. Initial diseases were stage-I (nasal, n 5 8; tonsil, n 5 1; ileum, n 5 1), treated with radiotherapy (n 5 6), combined radiotherapy/chemotherapy (n 5 3), and chemotherapy (n 5 1). Relapse occurred at the same (nasal, n 5 6; tonsil, n 5 1), adjacent (initial: nasal, relapse: palate; n 5 1) or distant (n 5 2) sites. Five patients died soon afterwards. Four patients remitted with chemotherapy, two remaining in CR2 (3, 14 years). This series documented the rare late relapses in NK-cell lymphomas, which may still respond to salvage therapy.NK-cell lymphomas show a specific geographical predilection for Asian and South American countries, and are extremely uncommon in other populations [1]. Another peculiar feature is the consistent presence of Epstein-Barr virus (EBV) infection in the lymphoma cells [1–3]. Stage I/II NK-cell lymphomas respond well to combined radiotherapy and chemotherapy, with longterm survivals of up to 50% of patients [2]. However, stage III/IV diseases have poor prognoses with few survivors. Most relapses of NK-cell lymphoma occur within the first two years of remission [1]. However, an exceptional case of relapse of nasal NK-cell lymphoma has been described after 38 years of remission [3]. Such late relapses are so rare that practically nothing is known about them. We analyzed a group of patients with NK-cell lymphomas relapsing after prolonged remissions to define their clinicopathological features and treatment outcome. Nine cases of late relapses were identified from a cohort of 40 patients followed up long-term in Hong Kong, comprising 26 men and 14 women (Table I). Two patients had been reported briefly before [4]. One additional case was identified from a Seoul series. There were five men and five women, at a median presentation age of 34.5 (17–68) years. All patients had stage I disease, with the nasal area involved in eight cases, the tonsil in one case, and the ileum in one case. The initial treatment was radiotherapy in six cases, combined radiotherapy and chemotherapy in three cases, and chemotherapy alone in one case. The median duration of CR1 was 17.5 (11–29) years before disease relapsed. In eight cases, first relapse (R1) occurred at the same (nasal, n 5 6; tonsil, n 5 1) or adjacent (initial: nasal, relapse: palate; n 5 1) sites. In two other cases, the relapse was systemic. Five patients (including two systemic relapses) died soon afterwards, so that the impact of treatment could not be evaluated. However, for four patients, who could complete the planned chemotherapy, CR2 was achieved. Two patients had remained in CR2 for 3 and 14 years. One patient had R2 again at the same nasal site a year afterwards, and achieved a durable CR3 with further radiotherapy. The remaining patient had R2 12 years later at the age of 80 years, opted for conservative treatment and died. This series documented the rare occurrence of late relapses in NK-cell lymphoma. Late relapses are uncommon in aggressive lymphomas. For high-grade B-cell lymphomas, survival curves start to plateau at 3–5 years, with relapses after 5–10 years accounting for only about 20% of treatment failures [5, 6]. Relapses, after 10 or more years, are considered exceptionally unusual, and documented cases are few. In contrast, lowgrade B-cell lymphomas relapse continuously for decades [7]. Interestingly, it has been shown that late relapses of aggressive B-cell lymphomas (> 10 years) might in fact be due to an undiagnosed underlying lowgrade lymphoma [5].
