Löffler H, Kosely F, Ho A D, Krämer A
Klinische Kooperationseinheit Molekulare Hämatologie/Onkologie, Deutsches Krebsforschungszentrum, Im Neuenheimer Feld 581, 69120 Heidelberg.
Dtsch Med Wochenschr. 2009 Sep;134(39):1927-30. doi: 10.1055/s-0029-1237532. Epub 2009 Sep 16.
A 70-year-old female patient developed a non-pruritic, indolent rash associated with infections and peripheral blood abnormalities. A skin biopsy was suggestive of malignant lymphoma of the skin.
The peripheral blood count showed mild pancytopenia and 12 % blasts. Additional immunohistochemistry of the skin specimen as well as cytologic and flow cytometric examination of the bone marrow revealed an immature cell population expressing CD4 and CD56 which infiltrated both the dermis and, with an infiltration grade of about 85 %, the bone marrow.
Blastic plasmacytoid dendritic cell neoplasm (formerly known as blastic NK cell lymphoma).
After the first course of induction chemotherapy with daunorubicin and cytarabin, both the rash and the hematologic findings of bone marrow and peripheral blood showed a complete remission.
The blastic plasmacytoid dendritic cell neoplasm is a rare, aggressive hematopoietic neoplasm most likely related to acute myeloid leukemia (AML). Since cutaneous involvement is regularly present at diagnosis, the differential diagnosis of unexplained skin lesions should include this disease entity, especially if peripheral blood abnormalities are present. Despite the initial response to cytostatic therapy being mostly excellent, the prognosis is poor. Hence, treatment as high-risk AML seems advisable.
一名70岁女性患者出现了一种与感染及外周血异常相关的非瘙痒性、进展缓慢的皮疹。皮肤活检提示为皮肤恶性淋巴瘤。
外周血细胞计数显示轻度全血细胞减少，原始细胞占12%。对皮肤标本进行的额外免疫组化以及对骨髓进行的细胞学和流式细胞术检查发现，有一群表达CD4和CD56的不成熟细胞，其浸润了真皮，并以约85%的浸润程度浸润了骨髓。
母细胞性浆细胞样树突状细胞肿瘤（以前称为母细胞性NK细胞淋巴瘤）。
在用柔红霉素和阿糖胞苷进行第一个诱导化疗疗程后，皮疹以及骨髓和外周血的血液学表现均显示完全缓解。
母细胞性浆细胞样树突状细胞肿瘤是一种罕见的、侵袭性造血肿瘤，很可能与急性髓系白血病（AML）相关。由于在诊断时通常存在皮肤受累情况，对于不明原因的皮肤病变进行鉴别诊断时应包括该疾病实体，尤其是在外周血存在异常的情况下。尽管对细胞毒性疗法的初始反应大多良好，但预后较差。因此，将其作为高危AML进行治疗似乎是可取的。
