Division of Rheumatology, Department of Internal Medicine, NTT West Osaka Hospital, Osaka, Japan.
Department of Respiratory Medicine and Clinical Immunology, Graduate School of Medicine, Osaka University, Osaka, Japan.
Mod Rheumatol Case Rep. 2020 Jan;4(1):28-33. doi: 10.1080/24725625.2019.1650697. Epub 2019 Sep 5.
A 66-year-old woman presented with severe anaemia, thrombocytopenia and lymphopenia. The bone marrow biopsy demonstrated hypocellular marrow with myelofibrosis (MF); there was no evidence of malignancy, but infiltration of peripheral T and B cells were noticed. Magnetic resonance imaging (MRI) revealed that bone marrow of the spine exhibited low signal intensity (SI) with spotty high SI in T1- and T2-weighted images. Because there was evidence of autoimmune abnormality, she had fulfilled the classification criteria for systemic lupus erythematosus (SLE). She was diagnosed with autoimmune myelofibrosis (AIMF) associated with SLE and was treated with corticosteroid. Cytopenia improved after 1 month of corticosteroid therapy. A repeated bone marrow biopsy demonstrated that cellularity had increased and that the amount of reticulin fibre had reduced after treatment. Compared with primary MF, AIMF has generally a favourable prognosis and is often associated with autoimmune diseases, especially SLE. Bone marrow biopsy, but not aspiration, was useful for diagnosing bone marrow fibrosis. Although the association between SLE and MF has been rarely reported, we should pay attention to MF as a possible cause of pancytopenia.
一位 66 岁女性因严重贫血、血小板减少和淋巴细胞减少就诊。骨髓活检显示骨髓增生低下伴骨髓纤维化（MF）；无恶性肿瘤证据，但注意到外周 T 和 B 细胞浸润。磁共振成像（MRI）显示脊柱骨髓在 T1 和 T2 加权图像上表现为低信号强度（SI），伴有斑点状高 SI。由于存在自身免疫异常的证据，她符合系统性红斑狼疮（SLE）的分类标准。她被诊断为与 SLE 相关的自身免疫性骨髓纤维化（AIMF），并接受了皮质类固醇治疗。皮质类固醇治疗 1 个月后，血细胞减少症得到改善。重复骨髓活检显示，治疗后细胞数量增加，网状纤维数量减少。与原发性 MF 相比，AIMF 一般预后良好，常与自身免疫性疾病相关，尤其是 SLE。骨髓活检而非抽吸对于诊断骨髓纤维化是有用的。虽然 SLE 和 MF 之间的关联很少有报道，但我们应该注意 MF 作为全血细胞减少的可能原因。
