Department of Dermatology, Hôpital Henri-Mondor, AP-HP, 94220 Créteil, France.
Department of Dermatology, Inselspital Bern University Hospital, University of Bern, 3010 Bern, Switzerland.
Hum Pathol. 2018 Jan;71:100-108. doi: 10.1016/j.humpath.2017.10.018. Epub 2017 Oct 28.
Eruption of lymphocyte recovery (ELR) may occur during bone marrow aplasia after chemotherapies. We reviewed the clinical and pathologic features of 12 patients (male-female ratio, 7:5; median age, 61 years) with an atypical ELR histologically mimicking a primary cutaneous T-cell lymphoma such as Sézary syndrome or CD30+ T-cell lymphoproliferative disorder. All the patients displayed an erythematous maculopapular eruption on the trunk and the limbs associated with fever. All but one had received a polychemotherapy for an acute myeloid leukemia (n=10) or a urothelial carcinoma (n=1) before the occurrence of the skin eruption. One had an autoimmune lymphoproliferative syndrome causing chronic agranulocytosis requiring granulocyte colony-stimulating factor injection. In all patients, the skin eruption was associated with a slight increase of white blood cell count followed by bone marrow recovery within the next weeks. All skin biopsies showed a dermal perivascular lymphocytic infiltrate containing atypical medium- to large-sized CD3+, CD4+ and CD8+, CD25+, ICOS+, PD1- lymphocytes with a strong CD30 expression in most instances (n=10), suggesting the recruitment of strongly activated T cells in the skin. In 6 patients, a diagnosis of CD30+ lymphoproliferative disorder or Sézary syndrome was proposed or suspected histopathologically, and only the clinical context allowed the diagnosis of ELR with a peculiar presentation with atypical lymphocytes. We describe a series of patients with an unusual form of ELR characterized by the presence of atypical activated T cells in the skin. On a practical ground, pathologists should be aware of this distinctive and misleading presentation.
淋巴细胞恢复性发疹(ELR)可能发生于化疗后骨髓衰竭期。我们回顾了 12 例(男女比例 7:5;中位年龄 61 岁)患者的临床和病理特征,这些患者的组织学表现为类似原发性皮肤 T 细胞淋巴瘤,如蕈样肉芽肿或 CD30+T 细胞淋巴增生性疾病的非典型 ELR。所有患者均表现为躯干部和四肢红斑性斑丘疹性发疹,伴有发热。除 1 例外,所有患者在皮肤发疹前均因急性髓系白血病(n=10)或尿路上皮癌(n=1)接受了多化疗。1 例患者患有自身免疫性淋巴增生综合征,导致慢性粒细胞减少症,需要粒细胞集落刺激因子注射。所有患者的皮肤发疹均伴有白细胞计数轻度增加,随后在接下来的几周内骨髓恢复。所有皮肤活检均显示真皮小血管周围淋巴细胞浸润,其中含有中等至大的 CD3+、CD4+和 CD8+、CD25+、ICOS+、PD1-的非典型淋巴细胞,大多数情况下 CD30 表达强烈(n=10),提示在皮肤中募集了强烈活化的 T 细胞。在 6 例患者中,组织病理学上提出或怀疑为 CD30+淋巴增生性疾病或蕈样肉芽肿的诊断,仅临床背景允许诊断为 ELR,表现为具有非典型淋巴细胞的独特表现。我们描述了一组具有非典型 ELR 的患者,其特征为皮肤中存在非典型活化的 T 细胞。从实际角度来看,病理学家应注意到这种独特且具有误导性的表现。