Hamid Zargar A, Ahmad Laway B, Alam Bhat K, Shah A, Ahmad M, Aejaz Aziz S, Iftikhar Bashir M, Iqbal Wani A, Hayat Bhat M
Department of Endocrinology, Sher-i-kashmir Institute of Medical Sciences, Srinagar, Kashmir, India.
Exp Clin Endocrinol Diabetes. 2004 Sep;112(8):462-4. doi: 10.1055/s-2004-821231.
A case of primary adrenal insufficiency, secondary to primary bilateral adrenal lymphoma is reported. A 50-year-old woman presented with features of primary adrenal insufficiency (darkening of skin, asthenia, anorexia, constipation) for at least 8 months. Clinical examination was unremarkable except for low body mass index and generalized skin and buccal mucosal pigmentation. Routine investigations including complete hemogram, serum chemistry, urine analysis, chest radiograph and electrocardiogram were normal; serum lactate dehydrogenase was moderately elevated. Primary adrenal insufficiency was confirmed on cortisol dynamics (very low basal and peak cortisol) after insulin-induced hypoglycemia. Routinely detected adrenal masses on ultrasonography were confirmed by contrast enhanced CT abdomen. A diagnosis of primary adrenal non- Hodgkin's lymphoma (B-cell) was made after exploratory laprotomy and further staging. The patient was put on combination chemotherapy (CHOP) protocol, but was lost to follow-up after receiving two cycles of treatment. Primary adrenal lymphoma, although a rare entity, needs to be suspected in patients with features of primary adrenal insufficiency who have evidence of bilateral adrenal masses on imaging.
报告了一例继发于原发性双侧肾上腺淋巴瘤的原发性肾上腺功能不全病例。一名50岁女性出现原发性肾上腺功能不全症状(皮肤变黑、乏力、厌食、便秘)至少8个月。除体重指数低以及全身皮肤和颊黏膜色素沉着外,临床检查无异常。包括全血细胞计数、血清化学、尿液分析、胸部X线片和心电图在内的常规检查均正常;血清乳酸脱氢酶中度升高。胰岛素诱发低血糖后,通过皮质醇动力学检查(基础和峰值皮质醇水平极低)确诊为原发性肾上腺功能不全。超声检查常规发现的肾上腺肿块经腹部增强CT得以证实。经剖腹探查和进一步分期后,诊断为原发性肾上腺非霍奇金淋巴瘤(B细胞型)。该患者接受了联合化疗(CHOP方案),但在接受两个周期治疗后失访。原发性肾上腺淋巴瘤虽然罕见,但对于有原发性肾上腺功能不全症状且影像学检查有双侧肾上腺肿块证据的患者,应怀疑此病。