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粒细胞肉瘤:三例特殊患者。

Granulocytic sarcoma: three unusual patients.

作者信息

Gittin R G, Scharfman W B, Burkart P T

机构信息

Department of Medicine, Albany Medical College, New York 12208.

出版信息

Am J Med. 1989 Sep;87(3):345-7. doi: 10.1016/s0002-9343(89)80163-9.

Abstract

This report describes three unusual patients with lesions due to myeloblasts. In one instance, the patient presented with massive adenopathy. The second patient had bone lesions and a pathologic fracture. The third patient, with myelodysplasia, had diffuse skin lesions infiltrated with myeloblasts. These cases fit the diagnostic category of granulocytic sarcoma. Granulocytic sarcoma is a tumor of immature myeloid cells that may involve any site in the body but that most commonly affects the skin, soft tissues, lymph nodes, bone, and periosteum. Lesions can predate leukemia or occur late in an established chronic granulocytic leukemia or acute granulocytic leukemia. The most common presentation occurs late in the course of acute granulocytic leukemia or in chronic granulocytic leukemia as a herald to blastic transformation. Therapy for localized lesions is radiotherapy, which produces prompt shrinkage of the lesions but relapse occurs subsequently. Systemic chemotherapy also produces satisfactory clinical results. In all instances, therapy can only be considered palliative since virtually all patients have a short survival following the appearance of an extramedullary myeloblastic lesion. Recognition of this pathologic entity at an early stage may give us information on the best management for these patients.

摘要

本报告描述了三例因成髓细胞导致病变的特殊患者。其中一例患者表现为巨大淋巴结病。第二例患者有骨病变及病理性骨折。第三例患有骨髓发育异常的患者有弥漫性皮肤病变,有成髓细胞浸润。这些病例符合粒细胞肉瘤的诊断类别。粒细胞肉瘤是一种未成熟髓样细胞的肿瘤,可累及身体任何部位,但最常影响皮肤、软组织、淋巴结、骨骼和骨膜。病变可先于白血病出现,或在已确诊的慢性粒细胞白血病或急性粒细胞白血病晚期出现。最常见的表现是在急性粒细胞白血病病程晚期或慢性粒细胞白血病中作为急变的先兆出现。局限性病变的治疗方法是放疗,放疗可使病变迅速缩小,但随后会复发。全身化疗也能产生令人满意的临床效果。在所有病例中,治疗只能被视为姑息性的,因为几乎所有患者在出现髓外成髓细胞病变后存活期都很短。早期识别这种病理实体可能会为我们提供有关这些患者最佳治疗方法的信息。

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