Byrd J C, Edenfield W J, Shields D J, Dawson N A
Department of Medicine, Walter Reed Army Medical Center, Washington, DC 20307, USA.
J Clin Oncol. 1995 Jul;13(7):1800-16. doi: 10.1200/JCO.1995.13.7.1800.
To discuss the predisposing risk factor for all forms of extramedullary leukemia (EML) and to review the clinical features, prognostic significance, and treatment strategies for primary EML and leukemia cutis (LC)/granulocytic sarcomas (GS) in the setting of acute nonlymphocytic leukemia (ANLL).
A review of all reports published since 1965 related to all forms of extramedullary leukemia (LC, GS, gingival hypertrophy, and meningeal leukemia [ML]).
Several factors, including chromosomal abnormalities [t(8;21), inv(16)], cell-surface markers (CD56, CD2, CD4, CD7), French-American-British (FAB) subtype (M2, M4, M5), blast differentiation and maturation, patient nutritional status, age, cellular immune dysfunction, high presenting leukocyte count, and decreased blast Auer rods, have been associated with a higher incidence of EML. Of 154 published cases of primary EML identified, 71 (46%) were initially misdiagnosed. The addition of immunohistochemical stains can assist in preventing such misdiagnoses and should be included in all atypical lymphoma/carcinoma cases. Only one of the patients (3%) with primary EML did not progress to ANLL in the absence of chemotherapy. In contrast, 66% of patients who received chemotherapy for the primary EML never developed ANLL. The prognostic significance of EML at presentation and medullary relapse of ANLL is uncertain. Isolated extramedullary recurrence of ANLL always heralds bone marrow relapse and should be treated with reinduction chemotherapy. Close clinical follow-up observation is necessary to insure resolution of EML. Radiation therapy is an effective local treatment for resistant or symptomatic EML.
Many advances in diagnoses and treatment of EML have been made. Future investigations are needed to define the clinical significance of EML in patients with ANLL treated with modern chemotherapy or bone marrow transplantation.
探讨各种形式的髓外白血病（EML）的易感危险因素，并回顾急性非淋巴细胞白血病（ANLL）背景下原发性EML及皮肤白血病（LC）/粒细胞肉瘤（GS）的临床特征、预后意义及治疗策略。
对1965年以来发表的所有与各种形式的髓外白血病（LC、GS、牙龈肥大和脑膜白血病[ML]）相关的报告进行综述。
包括染色体异常[t(8;21)、inv(16)]、细胞表面标志物（CD56、CD2、CD4、CD7）、法美英（FAB）亚型（M2、M4、M5）、原始细胞分化和成熟、患者营养状况、年龄、细胞免疫功能障碍、初诊时白细胞计数高以及原始细胞奥氏小体减少等多种因素与EML的较高发生率相关。在已发表的154例原发性EML病例中，71例（46%）最初被误诊。免疫组织化学染色的加入有助于预防此类误诊，应纳入所有非典型淋巴瘤/癌病例中。在未进行化疗的情况下，原发性EML患者中只有1例（3%）未进展为ANLL。相比之下，接受原发性EML化疗的患者中有66%从未发生ANLL。EML在初诊时及ANLL髓内复发的预后意义尚不确定。ANLL孤立的髓外复发总是预示着骨髓复发，应采用再诱导化疗进行治疗。密切的临床随访观察对于确保EML消退是必要的。放射治疗是治疗耐药或有症状EML的有效局部治疗方法。
EML的诊断和治疗已取得许多进展。未来需要进行研究以明确EML在接受现代化疗或骨髓移植治疗的ANLL患者中的临床意义。
