Chernova N G, Soboleva N P, Mariina S A, Sidorova Y V, Sinitsyna M N, Dvirnyk V N, Badmazhapova D S, Vinogradova Y E, Zvonkov E E, Savchenko V G
National Research Center for Hematology, Russian Federation, Moscow, Russia.
The State Education Institution of Higher Professional Training The First Sechenov Moscow State Medical University under Ministry of Health of the Russian Federation, Moscow, Russia.
Ter Arkh. 2018 Aug 17;90(7):51-56. doi: 10.26442/terarkh201890751-56.
The aim of the study was to characterize quantitative and qualitative immunoglobulinopathies in patients with AITL at the onset of the disease.
55 patients with newly diagnosed AITL were enrolled in the study, the male/female ratio was 30/25; median age was 61 (29-81) years. Diagnosis was based on standard WHO criteria. Immunochemical studies of blood serum included serum protein electrophoresis/immunofixation, nephelometric quantification of total immunoglobulins, serum free light chain assay.
Quantitative and qualitative immunoglobulinopathies were determined in 49 (89,1%) of 55 pts. Quantitative immunoglobulinopathies were revealed in 47 (85.5%) of 55 cases, qualitative - in 14 (25,5%). Combination quantitative and qualitative immunoglobulinopathies was observed in 12 (21,8%) of 55 pts. The detected immunoglobulinopathies were divided into 4 groups: polyclonal hypergammaglobulinaemia, hypogammaglobulinaemia, oligoclonal gammapathy, and monoclonal gammapathy. Polyclonal hypergammaglobulinaemia was marked in 41 (74.5%) of 55 pts, elevated level of IgG was determined in 27 (49,15%) of 55 cases, IgM - in 18 (32,7%) and IgA - in 21 (38.2%). Interestingly, polyclonal IgE hypergammaglobulinaemia was detected in 12 (48,0%) of 25 cases of performed studies. Hypogammaglobulinaemia was detected in 8 (14,5%) of 55 cases. Oligoclonal gammapathy was determined in 4 (7.3%) of 55 pts. Monoclonal gammapathy was revealed in 11 (20,0%) of 55 cases. The amount of monoclonal immunoglobulin varied from 2.6 to 14.1 g/l. Monoclonal immunoglobulin Gk was detected in 5 of 11 pts, Gλ - in 2, Mλ - in 2, Mk - in 2. Monoclonal gammapathy was accompanied by polyclonal hypergammaglobulinaemia in 9 of 11 cases, hypogammaglobulinaemia - in 2.
Quantitative and qualitative immunoglobulinopathies are observed in most patients at the onset of AITL. Quantitative abnormalities were determined more often than qualitative. Monoclonal gammapathy can be a manifestation of lymphoproliferation and other concomitant disorders. The prognostic value of immunochemical parameters is still unclear and requires dynamic observation and study.
本研究旨在对AITL患者疾病初发时的定量和定性免疫球蛋白病进行特征描述。
55例新诊断的AITL患者纳入研究，男女比例为30/25；中位年龄为61（29 - 81）岁。诊断依据WHO标准。血清免疫化学研究包括血清蛋白电泳/免疫固定、总免疫球蛋白的散射比浊定量、血清游离轻链检测。
55例患者中有49例（89.1%）检测到定量和定性免疫球蛋白病。55例中有47例（85.5%）出现定量免疫球蛋白病，14例（25.5%）出现定性免疫球蛋白病。55例患者中有12例（21.8%）同时出现定量和定性免疫球蛋白病。检测到的免疫球蛋白病分为4组：多克隆高丙种球蛋白血症、低丙种球蛋白血症、寡克隆丙种球蛋白病和单克隆丙种球蛋白病。55例患者中有41例（74.5%）表现为多克隆高丙种球蛋白血症，55例中有27例（49.15%）IgG水平升高，18例（32.7%）IgM升高，21例（38.2%）IgA升高。有趣的是，在25例已进行研究的病例中有12例（48.0%）检测到多克隆IgE高丙种球蛋白血症。55例中有8例（14.5%）检测到低丙种球蛋白血症。55例患者中有4例（7.3%）出现寡克隆丙种球蛋白病。55例中有11例（20.0%）出现单克隆丙种球蛋白病。单克隆免疫球蛋白量在2.6至14.1 g/l之间。11例患者中有5例检测到单克隆免疫球蛋白Gk，2例为Gλ，2例为Mλ，2例为Mk。11例中有9例单克隆丙种球蛋白病伴有多克隆高丙种球蛋白血症，2例伴有低丙种球蛋白血症。
大多数AITL患者在疾病初发时可观察到定量和定性免疫球蛋白病。定量异常比定性异常更常见。单克隆丙种球蛋白病可能是淋巴增殖及其他伴随疾病的一种表现。免疫化学参数的预后价值仍不清楚，需要动态观察和研究。
