O'Brien T R, Diamondstone L, Fried M W, Aledort L M, Eichinger S, Eyster M E, Hilgartner M W, White G, Di Bisceglie A M, Goedert J J
Viral Epidemiology Branch, National Cancer Institute, Rockville, MD 20852, USA.
Am J Hematol. 1995 Jul;49(3):201-6. doi: 10.1002/ajh.2830490305.
Persons with hemophilia or other HIV-1 risk factors may be more likely to have idiopathic CD4+ T-lymphocytopenia (ICL). We determined the frequency of ICL in prospectively followed cohorts of HIV-1 seronegative hemophilic men and seronegative female sex partners of HIV-1 infected hemophilic men, and examined factors potentially associated with ICL. Seven of 304 (2.3%) seronegative hemophilic men and one of 160 (0.6%) female partners met the ICL definition, but the condition resolved for two of the men and for the sole female partner. All five men with persistent ICL had lymphocytopenia (< 1,200 total lymphocytes/microliters) and < 300 total CD4+ lymphocytes/microliters; only one had a low CD4+ percentage. On the most recent measurement, 14.5% of the 304 seronegative hemophilic men had lymphocytopenia. Compared with matched hemophilic controls, men with persistent ICL more often had a history of liver disease (3/5 cases, 0/21 controls, P = 0.007) or splenomegaly (3/5 cases, 4/21 controls; P = 0.04), but not severe hemophilia, greater clotting factor concentrate exposure, high alanine aminotransferase levels, hepatitis B virus antigenemia, or detectable hepatitis C virus RNA in plasma. All five cases and 20/21 controls had antibodies to hepatitis C virus present in their serum. In this cohort of hemophilic men, ICL was related to lymphocytopenia associated with liver disease rather than selective loss of CD4+ lymphocytes.
患有血友病或其他HIV-1风险因素的人可能更易患特发性CD4+T淋巴细胞减少症（ICL）。我们确定了前瞻性随访的HIV-1血清阴性血友病男性队列以及HIV-1感染血友病男性的血清阴性女性性伴侣队列中ICL的发生率，并研究了可能与ICL相关的因素。304名血清阴性血友病男性中有7名（2.3%），160名女性伴侣中有1名（0.6%）符合ICL定义，但其中2名男性和唯一的女性伴侣的病情得到缓解。所有5名患有持续性ICL的男性均有淋巴细胞减少症（总淋巴细胞<1200/微升）且总CD4+淋巴细胞<300/微升；只有1名的CD4+百分比低。在最近一次测量中，304名血清阴性血友病男性中有14.5%有淋巴细胞减少症。与匹配的血友病对照相比，患有持续性ICL的男性更常患有肝病（5例中的3例，21例对照中的0例，P = 0.007）或脾肿大（5例中的3例，21例对照中的4例；P = 0.04），但没有严重血友病、更高的凝血因子浓缩物暴露、高丙氨酸转氨酶水平、乙肝病毒抗原血症或血浆中可检测到的丙肝病毒RNA。所有5例患者和21例对照中的20例血清中均有丙肝病毒抗体。在这个血友病男性队列中，ICL与肝病相关的淋巴细胞减少症有关，而非CD4+淋巴细胞的选择性丢失。
