Nonhepatosplenic extramedullary hematopoiesis: Associated diseases, pathology, clinical course, and treatment
Nonhepatosplenic extramedullary hematopoiesis: Associated diseases, pathology, clinical course, and treatment
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DOI：
10.4065/78.10.1223
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发表时间：
2003-10-01
影响因子：
8.9
通讯作者：
Tefferi, A
中科院分区：
文献类型：
--
作者：
Koch, CA;Li, CY;Tefferi, A
目的：为了定义相关的临床条件，病理，自然史，和治疗结果的nonhepatosplenic extrauminal hematopoiesis（NHS-EMH）Patients and Methods：我们回顾性分析了所有患者的病历确定为有NHS-EMH从1975年至2002年。诊断是由组织活检，细针穿刺活检，或放射性核素骨髓scanning.Results：我们确定了27例NHS-EMH的死前诊断。最常见的相关疾病和疾病部位分别为骨髓纤维化伴髓样化生（MMM）（18例患者[67%]）和脊柱（7例患者[26%];均累及胸部）。在诊断NHS-EMH时，并发脾EMH（22例患者[82%]; 15例[56%]接受了脾切除术）和红细胞输血依赖（12例患者[44%]）是普遍的。在27例患者中，9例（33%）不需要特殊治疗。特异性治疗是放疗（7例患者，有效率为71%）和手术切除（6例患者，有效率为67%）。治疗相关并发症仅限于手术。非MMM组未使用放射治疗，但MMM组使用低剂量放射治疗治疗脊柱旁或脊柱内EMH（中位剂量，I戈伊;范围，1-10戈伊）、胸膜或肺部疾病（中位剂量，1.25戈伊;范围，1.00-1.50戈伊）和腹部或盆腔疾病（中位剂量，2.02戈伊;范围，1.50-4.50戈伊）。诊断为NHS-EMH后的中位生存期为13个月的MMM组和21个月的非MMM group.Conclusions：这项回顾性研究表明，NHS-EMH是罕见的，往往与MMM，并优先影响胸椎区域。无症状的疾病可能不需要特殊的治疗，而有症状的疾病最好用低剂量放射治疗。
Objective: To define associated clinical conditions, pathology, natural history, and treatment outcome of nonhepatosplenic extramedullary hematopoiesis (NHS-EMH).Patients and Methods: We retrospectively reviewed the medical charts of all patients identified as having NHS-EMH from 1975 to 2002. Diagnosis was made by tissue biopsy, fine-needle aspiration biopsy, or radionuclide bone marrow scanning.Results: We identified 27 patients with antemortem diagnosis of NHS-EMH. The most common associated condition and disease site were myelofibrosis with myeloid metaplasia (MMM) (in 18 patients [67%]) and the vertebral column (in 7 patients [26%]; all involving the thoracic region), respectively. At the time of diagnosis of NHS-EMH, concurrent splenic EMH (in 22 patients [82%]; 15 [56%] had undergone splenectomy) and red blood cell transfusion dependency (in 12 patients [44%]) were prevalent. Of,,the 27 patients, 9 (33%) required no specific therapy. Specific therapy was radiation (in 7 patients,with a 71% response rate) and surgical excision (in 6 patients with a 67% response). Treatment-associated complications were limited to surgery. Radiation therapy was not used in the non-MMM group, but low-dose radiation therapy was used in the MMM group for paraspinal or intraspinal EMH (median dose, I Gy; range, 1-10 Gy), pleural or pulmonary disease (median dose, 1.25 Gy; range, 1.00-1.50 Gy), and abdominal or pelvic disease (median dose, 2.02 Gy; range, 1.50-4.50 Gy). Median survival after the diagnosis of NHS-EMH was 13 months in the MMM group and 21 months in the non-MMM group.Conclusions: This retrospective study suggests that NHS-EMH is rare, is often associated with MMM, and preferentially affects the thoracic spinal region. Asymptomatic disease may require no specific treatment, whereas symptomatic disease is best managed with low-dose radiation therapy.