临床表现似系统性红斑狼疮的大 B 细胞淋巴瘤骨髓受累 1 例

Bone marrow infiltration of large B-cell lymphoma with clinical manifestations similar to systemic lupus erythematosus ：A case report

摘要

淋巴结或脏器肿大是淋巴瘤的常见临床表现，当患者无上述异常，仅以水肿、乏力等非特异性表现为主诉时易被误诊、漏诊。本例患者就诊期间发现贫血、血小板减少、乳酸脱氢酶升高、凝血功能异常、尿蛋白阳性、补体 C3 和 C4 降低、抗核抗体阳性，经影像学检查未见肿瘤迹象，初诊为系统性红斑狼疮，经激素治疗后部分症状及实验室指标有所改善，但乏力症状进行性加重，乳酸脱氢酶一过性下降后再次升高，血小板、凝血指标无改善，系统性红斑狼疮不能解释疾病的全貌。为进一步确定病因，行骨髓穿刺检查，明确诊断为大 B 细胞淋巴瘤骨髓受累。临床诊疗中，系统性红斑狼疮的诊断需充分排除其他疾病，特别是存在不能解释的临床表现或对治疗应答不佳，以避免误诊、误治。

Abstract

Systemic lupus erythematosus（SLE）and lymphoma are two different diseases.However， they might mimic each other sometimes.Here，we reported a case of bone marrow infiltration as initial manifestation of large B-cell lymphoma that mimiced SLE.The patient was an elderly female whose chief complaints were edema and bilateral lower limb fatigue for one month.Initially，she was prescribed thy-roid hormone supplementation and albumin infusion for hypothyroidism and hypoproteinemia，respective-ly.The edema was improved，but fatigue worsened and the patient was bedridden.Then further examina- tions revealed anemia，thrombocytopenia，elevated lactate dehydrogenase（LDH），abnormal coagulation function，positive urinary protein，reduced complement C3 and C4，and positive antinuclear antibodies. Based on the above manifestations and laboratory findings，the diagnosis of SLE was considered.After administering 40 mg/d of methylprednisolone，the patient＇s edema，platelet count and LDH levels were improved，whereas her fatigue worsened to the point where she had difficulty turning in bed.When the dose of glucocorticoid tapered to 40 mg/d of prednisone，the LDH levels elevated and platelet counts de- creased.The treatment response was inconsistent with SLE.In order to clarify and confirm the diagnosis ， bone marrow puncture was performed and showed large B-cell lymphoma with bone marrow infiltration of 27.5％tumor cells.Enlargement of superficial lymph node or visceral organ is common clinical manifes- tation of lymphoma.However，our present case was bone marrow infiltration lonely without enlargement of lymph node and organ，and it is referred to as bone marrow lymphoma（BML）.BML can be classified into primary bone marrow lymphoma（PBML）and secondary bone marrow lymphoma（SBML）.PBML is a rare and primary tumor in the bone marrow without involvement of lymph nodes，liver，spleen and any other organs.It is easy to be misdiagnosed or missed diagnosis due to the absence of specific clinical presentation.Bone marrow examination is required and important to make the diagnosis of PBML.Diffuse large B-cell lymphoma is the most common type of PBML.SBML is caused by lymphoma in other parts of the body and invasion of bone tissue.Most of BML patients have a poor prognosis and require rapid diag- nosis and treatment.The patient we reported only took two months from onset of symptoms to death.This case indicated that it was crucial to revisit the diagnostic and therapeutic strategies when treatment re- sponse was not consistent with the diagnosis.

关键词

B 细胞淋巴瘤 / 系统性红斑狼疮 / 鉴别诊断 / 水肿 / 疲劳

Key words

B-cell lymphoma / Systemic lupus erythematosus / Differential diagnosis / Edema / Fatigue

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基金资助

北京大学首钢医院首颐医疗科技发展基金重点创新项目(SGYYZ202403)