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继发于肾性骨营养不良的骨髓纤维化。

Myelofibrosis secondary to renal osteodystrophy.

作者信息

Nomura S, Ogawa Y, Osawa G, Katagiri M, Harada T, Nagahana H

机构信息

Department of Medicine, Kawasaki Medical School, Kurashiki, Japan.

出版信息

Nephron. 1996;72(4):683-7. doi: 10.1159/000188961.

DOI:10.1159/000188961
PMID:8730443
Abstract

A hemodialyzed women with secondary hyperparathyroidism who recovered well from myelofibrosis after a total parathyroidectomy with autotransplantation of parathyroid tissue to the forearm (PTx) is described. Before the operation, she had received regular transfusions to maintain an adequate hematocrit even under recombinant human erythropoietin (rhEpo) therapy. She showed splenomegaly and leukoerythroblastosis was present in her peripheral blood. A bone marrow biopsy and bone marrow scintigraphy confirmed the diagnosis of myelofibrosis. After PTx, her hematocrit gradually increased without any transfusion. It has been maintained around 35% now 16 months since the operation. Her spleen has also gradually decreased in size. In addition, no leukoerythroblastosis has been found in the peripheral blood. Serial follow-up scintigraphy of bone marrow revealed a decline in extramedullary hematopoiesis. These findings indicated that her myelofibrosis was the result of secondary hyperparathyroidism, and that this complication is potentially reversible if accurate treatment is given. Physicians dealing with the end-stage renal disease should be aware of this complication to avoid additional transfusions.

摘要

本文描述了一名接受血液透析的继发性甲状旁腺功能亢进女性患者,她在进行甲状旁腺全切并将甲状旁腺组织自体移植至前臂(PTx)后,骨髓纤维化恢复良好。术前,即使在接受重组人促红细胞生成素(rhEpo)治疗的情况下,她仍需定期输血以维持足够的血细胞比容。她出现脾肿大,外周血中存在幼稚粒-幼红细胞现象。骨髓活检和骨髓闪烁显像确诊为骨髓纤维化。PTx术后,她的血细胞比容逐渐升高,无需输血。自手术至今16个月以来,血细胞比容一直维持在35%左右。她的脾脏也逐渐缩小。此外,外周血中未再发现幼稚粒-幼红细胞现象。连续的骨髓闪烁显像随访显示髓外造血减少。这些发现表明她的骨髓纤维化是继发性甲状旁腺功能亢进的结果,并且如果给予准确的治疗,这种并发症可能是可逆的。处理终末期肾病的医生应意识到这种并发症,以避免额外输血。

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