Helm K F, Peters M S, Tefferi A, Leiferman K M
Department of Dermatology, Mayo Clinic, Rochester, MN 55905.
J Am Acad Dermatol. 1992 Nov;27(5 Pt 2):868-71. doi: 10.1016/0190-9622(92)70269-l.
Large granular lymphocytic leukemia refers to a clonal expansion of lymphocytes that have abundant cytoplasm and azurophilic granules. The disease is characterized clinically by chronic neutropenia and it may be associated with recurrent pyogenic infections. Except for these infections, cutaneous manifestations of this disease have not been well characterized. We describe a patient with large granular lymphocytic leukemia, which was confirmed by molecular genetics studies, who had a pyoderma gangrenosum-like ulcer on his leg. Results of an evaluation of the histologic characteristics and the leukocytic immunophenotype of a skin biopsy specimen from the ulcer demonstrated large granular lymphocytes within the blood vessels. Cutaneous ulceration may be a manifestation of large granular lymphocytic leukemia, and this disease should be considered when diagnosing patients with otherwise unexplained pyoderma gangrenosum-like ulcers of the skin.
大颗粒淋巴细胞白血病是指具有丰富细胞质和嗜天青颗粒的淋巴细胞的克隆性扩增。该疾病临床上以慢性中性粒细胞减少为特征，可能与复发性化脓性感染有关。除了这些感染外，该疾病的皮肤表现尚未得到充分描述。我们描述了一名经分子遗传学研究确诊为大颗粒淋巴细胞白血病的患者，其腿部有坏疽性脓皮病样溃疡。对该溃疡皮肤活检标本的组织学特征和白细胞免疫表型评估结果显示血管内有大颗粒淋巴细胞。皮肤溃疡可能是大颗粒淋巴细胞白血病的一种表现，在诊断患有其他原因不明的坏疽性脓皮病样皮肤溃疡的患者时应考虑这种疾病。
