自行消退的皮肤母细胞性浆细胞样树突细胞肿瘤

Spontaneous remission of blastic plasmacytoid dendritic cell neoplasm

报告1例皮肤母细胞性浆细胞样树突细胞肿瘤。患者女,33岁,孕32+1周。全身多发紫红色结节及斑块1个月,无痛痒,外周血WBC及单核细胞均增多。皮肤科检查:躯干及四肢散在紫红色结节及斑块,表面光滑,质韧。皮损组织病理检查:真皮全层胶原束间单个核细胞浸润,可见核异形。骨髓穿刺示骨髓增生Ⅱ级,粒系0.27,红系0.29,单核细胞比例偏高,原幼单细胞占0.31,可见Auer小体。外周血涂片:单核细胞比例偏高,可见中幼粒细胞。免疫组化示CD56(+)及CD4部分(+)。诊断:皮肤母细胞性浆细胞样树突细胞肿瘤。患者未行任何治疗,剖宫产后皮损、血象及骨髓象均暂时好转。3个月后病情复发,进展为急性单核细胞白血病。血液科行CLAG方案化疗达到完全缓解。

A case of blastic plasmacytoid dendritic cell neoplasm is reported. A 33-year-old pregnant woman presented with asymptomatic, purple nodules and plaques on the trunk and limbs for 1 month. Dermatologic examination showed firm, purple nod ules and plaques with smooth surface on the trunk and limbs. Her peripheral blood exhibited increased white blood cell and monocyte counts. Skin biopsy revealed infiltrates of mononuclear cells between collagen bundles in the dermis and nuclear atypia.Bone marrow aspiration showed myeloid hyperplasia grade Ⅱ, granulocyte=0.27, and erythroid cell=0.29. The proportion of mono cytes was high, with 0.31 of monoblasts/promonocytes. Auer body was also observed. Peripheral blood smear showed higher proportion of monocytes and presence of the Mesozoic cells. Immunohistochemistry showed CD56(+) and CD4(partially +). The diagnosis of blastic plasmacytoid dendritic cell neoplasm was made. After caesarean, skin lesions, and abnormalities of bone marrow and blood routine tests remitted spontaneously. Three months later disease became worsening. And she was diagnosed with acute monocytic leukemia. Complete remission was achieved following CLAG chemotherapy.