Al-Riyami Nafila, Al-Farsi Khalil, Al-Amrani Khalfan, Al-Harrasi Sameera, Al-Huneini Mohammed, Al-Kindi Salam
Department of Hematology, Sultan Qaboos University Hospital, Muscat, Oman.
Oman Med J. 2015 May;30(3):216-8. doi: 10.5001/omj.2015.45.
Monoclonal gammopathies are frequently seen in B-cell malignancies. Monoclonal proteins are seen in a significant proportion of patients with chronic lymphocytic leukemia (CLL), which is a clonal disorder of mature B cells. The use of more sensitive laboratory methods has enabled the detection of monoclonal proteins or light chains in the serum and/or urine in the majority of these patients. The presence of some of these monoclonal proteins may explain the different autoimmune phenomena that are associated with this disease. Some reports indicate that the finding of monoclonal proteins has a negative impact on patients' survival. The presence of two different monoclonal proteins (i.e. biclonal gammopathy) is on the other hand rare. Most of the reported cases in the literature are of patients with plasma cell disorders. In this report, we describe a rare occurrence of biclonal gammopathy in a patient with CLL. Serum protein electrophoresis and immunofixation, which were negative at the time of initial diagnosis, showed biclonal immunoglobin A (IgA) kappa and IgA lambda during the course of the disease. The patient's disease showed steady progression, despite multiple treatments. Although this could just be the result of using more sensitive laboratory techniques, biclonal gammopathy in this patient likely reflects the evolution of another clone, which would explain the encountered resistance to therapy. Because of paucity of reports, the impact of biclonal gammopathies in such patients is not known and an effort to collectively report the presentation and outcome of these patients is needed to further understand the pathophysiology and clinical significance of such a finding.
单克隆丙种球蛋白病常见于B细胞恶性肿瘤。在相当一部分慢性淋巴细胞白血病(CLL)患者中可检测到单克隆蛋白,CLL是一种成熟B细胞的克隆性疾病。使用更敏感的实验室方法能够在大多数此类患者的血清和/或尿液中检测到单克隆蛋白或轻链。其中一些单克隆蛋白的存在可能解释了与该疾病相关的不同自身免疫现象。一些报告表明,单克隆蛋白的发现对患者的生存有负面影响。另一方面,存在两种不同的单克隆蛋白(即双克隆丙种球蛋白病)的情况很少见。文献中报道的大多数病例是浆细胞疾病患者。在本报告中,我们描述了一例CLL患者罕见的双克隆丙种球蛋白病。血清蛋白电泳和免疫固定在初诊时为阴性,但在疾病过程中显示出双克隆免疫球蛋白A(IgA)κ和IgAλ。尽管进行了多种治疗,该患者的病情仍呈稳定进展。虽然这可能仅仅是使用更敏感实验室技术的结果,但该患者的双克隆丙种球蛋白病可能反映了另一个克隆的演变,这可以解释所遇到的治疗抵抗。由于报告较少,双克隆丙种球蛋白病对此类患者的影响尚不清楚,需要努力汇总报告这些患者的表现和转归,以进一步了解这一发现的病理生理学和临床意义。