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毛细胞白血病中腹部淋巴结病的发生率及意义

Incidence and significance of abdominal lymphadenopathy in hairy cell leukaemia.

作者信息

Mercieca J, Puga M, Matutes E, Moskovic E, Salim S, Catovsky D

机构信息

Academic Department of Haematology and Cytogenetics and Diagnostic Radiology, Royal Marsden Hospital, London, UK.

出版信息

Leuk Lymphoma. 1994;14 Suppl 1:79-83.

PMID:7820058
Abstract

We have investigated the incidence and significance of abdominal lymphadenopathy in hairy cell leukemia (HCL) by routinely CT scanning 88 patients. These included 70 men and 18 women with a median age of 51 years (range 25-83). Abdominal CT scans were performed at diagnosis in 29 patients and in 59 during the course of the disease. Abdominal lymphadenopathy was documented in 25 patients (28%) overall; the incidence of abdominal lymphadenopathy was higher in relapse (56%) than at diagnosis (17%). All patients with lymphadenopathy had bone marrow disease. There is no association with age or sex but lymphadenopathy tends to be more common in patients with long-standing disease (median duration of disease 6 years v one year in those without nodes) and in patients with bulky disease, particularly in the relapse group (splenomegaly/splenectomy in 95% vs 40%). The presence of abdominal nodes is also associated with relative resistance to treatment, with more treatment failures and fewer complete responses seen in this group. Most patients with lymphadenopathy had large, immature-looking hairy cells present in both the bone marrow and lymph nodes, when these were examined. Abdominal lymphadenopathy in HCL is more common than previously recognised, particularly in relapsed patients, and is always associated with active disease. The presence of large hairy cells and the relative resistance to treatment suggest that this phenomenon represents a form of transformation of the disease. Longer follow up is required to confirm this.

摘要

我们通过对88例毛细胞白血病(HCL)患者进行常规CT扫描,研究了腹部淋巴结病的发生率及其意义。其中包括70名男性和18名女性,中位年龄为51岁(范围25 - 83岁)。29例患者在诊断时进行了腹部CT扫描,59例在疾病过程中进行了扫描。总体上,25例患者(28%)有腹部淋巴结病记录;腹部淋巴结病的发生率在复发时(56%)高于诊断时(17%)。所有有淋巴结病的患者均有骨髓疾病。腹部淋巴结病与年龄或性别无关,但在病程较长的患者(有淋巴结病患者的疾病中位持续时间为6年,无淋巴结病患者为1年)以及有大块病变的患者中更常见,尤其是在复发组(95%有脾肿大/脾切除,无淋巴结病患者为40%)。腹部淋巴结的存在还与相对治疗抵抗有关,该组治疗失败更多,完全缓解更少。在检查时,大多数有淋巴结病的患者骨髓和淋巴结中都有大的、外观不成熟的毛细胞。HCL中的腹部淋巴结病比以前认识到的更常见,尤其是在复发患者中,并且总是与活动性疾病相关。大毛细胞的存在和相对治疗抵抗表明这种现象代表了疾病的一种转化形式。需要更长时间的随访来证实这一点。

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