Rafiei Rana, Eftekhari Hojat, Rafiee Behnam
Skin Research Center, Department of Dermatology, Razi Hospital, School of Medicine, Guilan University of Medical Sciences, Rasht, Iran.
Department of Pathology, NYU Winthrop Hospital, 222 Station Plaza, No. 620, Mineola, NY 11501, USA.
Caspian J Intern Med. 2020 May;11(3):340-342. doi: 10.22088/cjim.11.3.340.
Waldenstrom macroglobulinemia is a lymphoplasmacytic lymphoma with elevated serum immunoglobulin M and multi-organ involvement. Primary systemic amyloidosis usually develops due to immunoglobulin light chains depositions in different organs due to an underlying gammopathy.
Our patient was an 86-year-old man with macroglossia, ecchymotic patches and bullous lesions associated with a skin laxity on the periorbital, palmar, and glans penis areas. Skin biopsy confirmed dermal amyloid depositions. In serum immunofixation electrophoresis, prominent monoclonal immunoglobulin-M lambda light chains were detected associated with prominent lymphoplasmacytic infiltration in bone marrow biopsy which was diagnosed as Waldenstrom macroglobulinemia.
Skin involvement presenting as cutaneous amyloidosis could be the first manifestation of Waldenstrom macroglobulinemia. We should think about an underlying gammopathy in an old patient with skin laxity and ecchymosis.
华氏巨球蛋白血症是一种伴有血清免疫球蛋白M升高和多器官受累的淋巴浆细胞淋巴瘤。原发性系统性淀粉样变性通常由于潜在的丙种球蛋白病导致免疫球蛋白轻链在不同器官沉积而发生。
我们的患者是一名86岁男性，有巨舌、瘀斑和大疱性病变，伴有眶周、手掌和阴茎头部位皮肤松弛。皮肤活检证实真皮有淀粉样沉积。血清免疫固定电泳检测到显著的单克隆免疫球蛋白M λ轻链，骨髓活检显示有显著的淋巴浆细胞浸润，诊断为华氏巨球蛋白血症。
表现为皮肤淀粉样变性的皮肤受累可能是华氏巨球蛋白血症的首发表现。对于有皮肤松弛和瘀斑的老年患者，我们应考虑潜在的丙种球蛋白病。
