Dermatology Service, Department of Medicine, Memorial Sloan Kettering Cancer Center and Weill Cornell Medical College, New York, New York; Department of Dermatology, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel.
Dermatology Service, Department of Medicine, Memorial Sloan Kettering Cancer Center and Weill Cornell Medical College, New York, New York; Drexel University College of Medicine, Philadelphia, Pennsylvania.
J Am Acad Dermatol. 2019 Jun;80(6):1704-1711. doi: 10.1016/j.jaad.2019.01.062. Epub 2019 Feb 1.
Follicular mucinosis (FM), which is defined by mucin accumulation within follicular epithelium, may occur in mycosis fungoides (MF). FM without MF is occasionally reported in systemic hematologic malignancies and may be diagnostically challenging.
To describe clinicopathologic characteristics of FM in patients with hematologic malignancies other than MF.
Clinical data and histopathology features were analyzed in patients with FM and hematologic malignancies diagnosed between 1994 and 2017.
A total of 18 patients with FM and systemic hematologic malignancies without cutaneous T-cell lymphoma (CTCL) were identified; 9 of them were discovered after hematopoietic stem cell transplantation. No patients with non-CTCL-associated FM (n = 46 [37 biopsy specimens]) developed CTCL during a mean follow-up of 4.3 years. Of the cases of CTCL associated with FM (n = 44 [31 biopsy specimens]), MF was the most common subtype (n = 38), although other CTCLs were identified. FM in patients with non-CTCL hematologic malignancies differed clinically from those with MF-associated FM, presenting most frequently with erythematous papules (P < .0001), without plaques (P <.0001), without alopecia (P = .001), and without histopathologically identified epidermal exocytosis (P = .013).
A retrospective study in a single cancer center.
FM can present in systemic hematologic malignancies, including after hematopoietic stem cell transplantation. Papular lesional morphologic and histopathologic features may help to distinguish these cases from MF.
滤泡黏液病(FM)是指滤泡上皮内黏液积聚,可发生于蕈样真菌病(MF)中。MF 以外的系统性血液恶性肿瘤偶尔也会报告发生 FM,且诊断具有挑战性。
描述 MF 以外的血液恶性肿瘤患者中 FM 的临床病理特征。
分析了 1994 年至 2017 年间诊断为 FM 伴血液系统恶性肿瘤且无皮肤 T 细胞淋巴瘤(CTCL)的患者的临床数据和组织病理学特征。
共发现 18 例 FM 伴系统性血液恶性肿瘤而无 CTCL 的患者,其中 9 例在造血干细胞移植后发现。在平均 4.3 年的随访中,46 例(37 例活检标本)非 CTCL 相关 FM 患者无一例发展为 CTCL。在与 FM 相关的 CTCL 中(n=44 [31 例活检标本]),MF 是最常见的亚型(n=38),尽管也发现了其他 CTCL。非 CTCL 血液恶性肿瘤患者的 FM 在临床上与 MF 相关 FM 不同,最常表现为红斑丘疹(P<.0001),无斑块(P<.0001),无脱发(P=.001),且无组织病理学确认的表皮外溢(P=.013)。
在单一癌症中心进行的回顾性研究。
FM 可发生于系统性血液恶性肿瘤,包括造血干细胞移植后。丘疹样病变形态学和组织病理学特征有助于将这些病例与 MF 区分开来。