Lembersky B C, Ratain M J, Golomb H M
Department of Medicine, University of Chicago, Pritzker School of Medicine, IL.
J Clin Oncol. 1988 Aug;6(8):1280-4. doi: 10.1200/JCO.1988.6.8.1280.
We identified eight patients with skeletal complications associated with hairy cell leukemia (HCL). The median time from diagnosis of HCL to the diagnosis of skeletal complications was 20 months (range, 0 to 93). All patients complained of pain and all but one lesion were located in the axial skeleton, primarily the proximal femur. Lytic lesions were seen on radiographic examination in all but one patient, and one patient additionally had multiple osteoporotic vertebral compression fractures. Radionuclide technetium bone scan was abnormal in all patients examined. Although the peripheral blood counts were variable (only two patients had a leukemic phase of the disease), all patients examined had a hypercellular bone marrow biopsy with hairy cells comprising at least 90% of the hematopoietic elements. The skeletal abnormalities responded well to local radiation therapy. Seven patients were begun on systemic therapy with interferon alpha-2b after the diagnosis of the skeletal lesion. Four of five evaluable patients had a partial hematological response and a substantial improvement in the degree of hairy cell infiltration of the bone marrow. None of these patients has had a recurrence of skeletal complications at a median follow-up time of 29 months. One patient failed to respond hematologically and developed additional bone lesions after 1 year of treatment. Another patient developed a new skeletal lesion 3 months after the cessation of interferon therapy at which time the bone marrow was essentially packed with hairy cells. This retrospective study indicates that bone involvement is a rare complication of HCL and is associated with a high tumor burden in the bone marrow. In addition to local radiation therapy, systemic treatment with interferon should be considered.
我们确定了8例与毛细胞白血病（HCL）相关的骨骼并发症患者。从HCL诊断到骨骼并发症诊断的中位时间为20个月（范围为0至93个月）。所有患者均主诉疼痛，除1处病变外，所有病变均位于中轴骨骼，主要是股骨近端。除1例患者外，所有患者的X线检查均可见溶骨性病变，1例患者还伴有多发性骨质疏松性椎体压缩骨折。所有接受检查的患者放射性核素锝骨扫描均异常。尽管外周血细胞计数各不相同（只有2例患者处于疾病的白血病期），但所有接受检查的患者骨髓活检均显示细胞增多，毛细胞占造血细胞的至少90%。骨骼异常对局部放射治疗反应良好。7例患者在骨骼病变诊断后开始接受α-2b干扰素全身治疗。5例可评估患者中有4例出现部分血液学反应，骨髓毛细胞浸润程度有显著改善。在中位随访时间29个月时，这些患者均未出现骨骼并发症复发。1例患者血液学无反应，治疗1年后出现额外的骨病变。另1例患者在停止干扰素治疗3个月后出现新的骨骼病变，此时骨髓基本被毛细胞填满。这项回顾性研究表明，骨骼受累是HCL的一种罕见并发症，且与骨髓中的高肿瘤负荷相关。除局部放射治疗外，应考虑使用干扰素进行全身治疗。
