Abstract:
A 72-year-old woman with no significant past medical history was admitted to the hospital for new-onset of leukocytosis with neutropenia, anemia, and thrombocytopenia, as well as a pruritic skin eruption. She was found to have acute myeloid leukemia (AML) with myelomonocytic differentiation. Her skin eruption consisted of widespread hemorrhagic crusted papules on the scalp and trunk. A skin biopsy was performed, which revealed a proliferation of mononuclear cells in the dermis with prominent epidermotropism and positive expression of CD1a and langerin (CD207), supporting a diagnosis of Langerhans cell histiocytosis (LCH). LCH is an uncommon proliferative disorder of activated Langerhans cells, which generally presents in children. In adults, it is exceptionally infrequent. Associated malignancies and rare reports of AML developing in subsequent years after an initial presentation of LCH have been described. Here we present an unusual concurrent presentation of LCH and AML in an adult.
摘要:
一名72岁无明显既往病史的女性因新发白细胞增多伴中性粒细胞减少症入院,贫血,和血小板减少症,还有皮肤瘙痒.她被发现患有急性髓细胞性白血病(AML)并伴有粒单核细胞分化。她的皮肤喷发包括头皮和躯干上广泛的出血性结壳丘疹。做了皮肤活检,这表明真皮中单个核细胞的增殖,具有突出的表皮性和CD1a和langerin(CD207)的阳性表达,支持朗格汉斯细胞组织细胞增生症(LCH)的诊断。LCH是激活的朗格汉斯细胞的一种罕见增殖性疾病,这通常表现在儿童身上。在成年人中,这是非常罕见的。已经描述了在最初出现LCH后的随后几年中发生的相关恶性肿瘤和AML的罕见报道。在这里,我们介绍了成人中LCH和AML的异常并发表现。