CAJ | 학술논문

目的探讨伴Kasabach-Merritt现象(KMP)的卡波西形血管内皮瘤(KHE)和丛状血管瘤(TA)临床病理特征.方法对7例伴KMP的KHE和TA病例进行临床及病理学分析.结果诊断为KHE 5例,TA 2例,均伴有血小板降低.7例均在出生后1月内发病,病灶均位于躯干或四肢.2例快速增大,2例缓慢进展,3例稳定或逐渐消退.二者病灶外观多样,典型表现为暗红或紫红色肿块或斑片.组织学上,KHE和TA均由结节状梭形血管内皮细胞团构成.区别在于KHE内皮细胞团边界不清,红细胞淤滞,含铁血黄素沉积;TA内皮细胞团界限清晰,呈弹坑样分布.口服泼尼松治疗2例,切除植皮1例,效果满意;随访观察4例,病情无进展.结论 KMP病理性质为KHE或TA,前者为中间性肿瘤,具局部侵袭性,后者为良性肿瘤,二者均可部分白行退化,需与其他类型血管性肿瘤鉴别.
목적 탐토반Kasabach-Merritt현상(KMP)적잡파서형혈관내피류(KHE)화총상혈관류(TA)림상병리특정.방법 대7례반KMP적KHE화TA병례진행림상급병이학분석.결과 진단위KHE 5례,TA 2례,균반유혈소판강저.7례균재출생후1월내발병,병조균위우구간혹사지.2례쾌속증대,2례완만진전,3례은정혹축점소퇴.이자병조외관다양,전형표현위암홍혹자홍색종괴혹반편.조직학상,KHE화TA균유결절상사형혈관내피세포단구성.구별재우KHE내피세포단변계불청,홍세포어체,함철혈황소침적;TA내피세포단계한청석,정탄갱양분포.구복발니송치료2례,절제식피1례,효과만의;수방관찰4례,병정무진전.결론 KMP병이성질위KHE혹TA,전자위중간성종류,구국부침습성,후자위량성종류,이자균가부분백행퇴화,수여기타류형혈관성종류감별.
Objective To investigate the clinical and pathological features of Kasabach-Merritt phenomenon(KMP)associated with Kaposiform hemangioendothelioma(KHE)and tufted angioma(TA).Methods Clinical presentations and pathological features of seven patients with KHE or TA complicated by KMP were analyzed retrospectively and a literature review was made.Results Five patients were diagnosed with KHE and two with TA,and all of them exhibited a decrease in platelet count(less than 100×109/L).In all patients,lesions were present or developed on the trunk or limbs at birth or wimin one month after birth.The lesions showed a rapid growth in two patients,slow expansion in two patients,kept stable or regressed gradually in three patients.Lesion appearance varied in KHE and TA,and characteristic manifestation was dark-erythematous or prunosus tumors or patches involving the skin or subcutis.Histopathologically.both KHE and TA were composed of numerous fusiform endothellal cell clumps arranged in a lobular fashion.The nodules of KHE had an indistinct boundary usually with erythrocyte stasis or hemosiderin deposition,and TA was characterized by rounded,well-defined nodules distributed in a crater-like fashion within the dermis.A satisfactory outcome was achieved in two patients treated with oral dehydrocortisone and one patient with surgical resection plus skin grafting;the other four patients remained untreated and no progress was observed during follow-up.Conclusions KMP is pathologically associated with KHE or TA.KHE is an intermediate tumor with local aggressiveness while TA is a benign tumor.Both of them can regress spontaneously and should be difierentiated from other types of vascular tumors.