CAJ | 학술논문

患儿女,13岁.臀部、四肢反复起斑块6个月,加重伴间断发热5个月.体检:各系统检查未见明显异常.皮肤科情况:臀部及双下肢散在分布直径3～ 11 cm质硬、浸润性、紫红色皮下斑块,界不清,表面少许脱屑,无明显压痛及溃疡形成.实验室检查:白细胞(0.03 ～ 3.7)×109/L,红细胞(2.8～ 4.4)×1012/L,血小板计数正常;纤维蛋白原1.79 g/L,NK细胞比例4.6％;骨髓涂片示骨髓增生活跃,粒系比例降低,网织细胞易见,并见少量分类不明细胞及噬血现象.皮损组织病理学检查:表皮、真皮未见明显异常,皮下脂肪组织内可见大量淋巴样细胞浸润,以脂肪小叶为主,部分小叶间隔受累;浸润细胞体积中等偏大,胞质少,核多形,部分核扭曲;可见坏死及吞噬核碎片、淋巴细胞现象;免疫组化:CD3、颗粒酶B、T细胞细胞内抗原-1(TIA-1)、TCRγδ阳性,CD5、CD7部分阳性,CD4、CD8、TCRαβ、CD56阴性,Epstein-Barr病毒编码RNA原位杂交检测阴性.诊断:原发性皮肤γδ-T细胞淋巴瘤合并噬血细胞综合征.
환인녀,13세.둔부、사지반복기반괴6개월,가중반간단발열5개월.체검:각계통검사미견명현이상.피부과정황:둔부급쌍하지산재분포직경3～ 11 cm질경、침윤성、자홍색피하반괴,계불청,표면소허탈설,무명현압통급궤양형성.실험실검사:백세포(0.03 ～ 3.7)×109/L,홍세포(2.8～ 4.4)×1012/L,혈소판계수정상;섬유단백원1.79 g/L,NK세포비례4.6％;골수도편시골수증생활약,립계비례강저,망직세포역견,병견소량분류불명세포급서혈현상.피손조직병이학검사:표피、진피미견명현이상,피하지방조직내가견대량림파양세포침윤,이지방소협위주,부분소협간격수루;침윤세포체적중등편대,포질소,핵다형,부분핵뉴곡;가견배사급탄서핵쇄편、림파세포현상;면역조화:CD3、과립매B、T세포세포내항원-1(TIA-1)、TCRγδ양성,CD5、CD7부분양성,CD4、CD8、TCRαβ、CD56음성,Epstein-Barr병독편마RNA원위잡교검측음성.진단:원발성피부γδ-T세포림파류합병서혈세포종합정.
A 13-year-old girl presented with multiple recurrent cutaneous plaques for more than six months,which had been aggravated with intermittent fever for five months.No obvious systemic abnormality was found.Dermatological examination revealed multiple,non-ulcerative,painless,infiltrative,indurated,poorly marginated,purple subcutaneous plaques measuring 3-1 1 cm in diameter with slight squamation in bilateral buttocks and lower limbs.Laboratory investigations showed bicytopenia with the white blood cell count being (0.03-3.7) × 109/L and red blood cell count being (2.8-4.4) × 1012/L,a normal platelet count,hypofibrinogenemia (1.79 g/L) and low proportion of natural killer cells (4.6％).Bone marrow smear showed active proliferation of cells,decreased proportion of granulocytes,presence of a few indefinitely classified cells,and phagocytosis.Reticulocytes were easily seen in the bone marrow smear.Pathologically,no obvious abnormality was observed in the epidermis or dermis,while the subcutaneous adipose tissue,especially fat lobules and some interlobular septa,was extensively infiltrated by large-to medium-sized lymphoid cells with pleomorphic and twisted nuclei as well as a small amount of cytoplasm; necrosis and phagocytosis of nuclear debris and lymphocytes were visible.The atypical lymphoid cells stained positive for CD3,T-cell intracellular antigen-1,granzyme B and TCRγδ with partial loss of CD5 and CD7,but negative for CD56,CD4,CD8 and TCRαβ.No Epstein-Barr virus-encoded RNA (EBER) was detected by in situ hybridization.Based on these findings,a diagnosis of primary cutaneous γδ-T cell lymphoma with hemophagocytic syndrome was made.