Kanuganti Deepthi, Nagarjunakonda Venkata Sundarachary, Bandarupalli Pranathi, Gorijala Vamsi Krishna, Konagalla Venkata Lakshman Sai Ram, Kowtha Praveen
Neurology, Dr. Ramesh Cardiac and Multispeciality Hospital, Guntur, IND.
Neurology, Guntur Medical College, Guntur, IND.
Cureus. 2022 Jul 19;14(7):e27001. doi: 10.7759/cureus.27001. eCollection 2022 Jul.
Polyneuropathy, organomegaly, endocrinopathy, monoclonal protein elevation, and skin changes (POEMS) syndrome is a rare multisystem disorder that occurs due to an underlying plasma cell dyscrasia. A diagnosis is made with the presence of two mandatory criteria and at least one major and one minor criterion. We present a case of a 28-year-old patient who presented with weakness of bilateral arms and legs, thinning of hands, and swelling of bilateral lower limbs and abdomen. The patient also reported weight loss and loss of appetite. Examination revealed areflexic quadriparesis with sensory loss, diffuse lymphadenopathy, pleural effusion, ascites, and pulmonary hypertension. Investigations showed elevated erythrocyte sedimentation rate (ESR). Nerve conduction studies revealed severe axonal polyneuropathy of all nerves. Lymph node biopsy showed Castleman disease. A diagnosis of POEMS syndrome was made and he was sent for a stem cell transplant, which is the definitive treatment in patients eligible for stem cell transplant.
多神经病、器官肿大、内分泌病、单克隆蛋白升高和皮肤改变(POEMS)综合征是一种罕见的多系统疾病,由潜在的浆细胞发育异常引起。诊断需具备两项必备标准以及至少一项主要标准和一项次要标准。我们报告一例28岁患者,其表现为双侧手臂和腿部无力、手部变细以及双侧下肢和腹部肿胀。患者还自述体重减轻和食欲不振。检查发现四肢腱反射消失伴感觉丧失、弥漫性淋巴结病、胸腔积液、腹水和肺动脉高压。检查显示红细胞沉降率(ESR)升高。神经传导研究显示所有神经均有严重的轴索性多神经病。淋巴结活检显示为卡斯特曼病。诊断为POEMS综合征,患者被送去接受干细胞移植,这是适合干细胞移植患者的确定性治疗方法。