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The diagnosis of Sézary syndrome (SS) relies on the identification of blood Sézary cells (SC) by different markers via flow cytometry. Treatment of SS is challenging since its pathogenesis is characterized by cell death resistance rather than hyperproliferation. In this study, we establish an integrated approach that considers both the expression of SC markers and sensitivity to cell death both spontaneously and upon in vitro treatment. Peripheral blood mononuclear cells were isolated from 20 SS patients and analysed for the SC markers CD7 and CD26 loss as well as CD158k and PD1 gain. The cells were then treated with different established and experimental therapies in vitro and cell death was measured. Spontaneous and therapeutically induced cell death were measured and correlated to cellular marker profiles. In the marker‐positive cells, spontaneous cell death sensitivity was reduced. Different treatments in vitro managed to specifically induce cell death in the putative CTCL cell populations. Interestingly, a repeated analysis after 3 months of treatment revealed the CTCL cell death sensitivity to be restored by therapy. We propose this novel integrated approach comprising the evaluation of SC marker expression and analysis of cell death sensitivity upon treatment that can also enable a better therapy stratification. [ABSTRACT FROM AUTHOR]

Eosinophils are characteristic in ALHE with unknown function.[3] We depleted eosinophils with benralizumab, an antibody directed against the IL-5 receptor alpha chain.[4] However, eosinophils were not the driving force in ALHE in this patient. To further characterize the infiltrating lymphocytes, the ALHE skin biopsies were stained for the T cell marker CD4 (Figure 1c,e), the Th2-lineage marker GATA3[5] (Figure 1d,e) and the B cell lineage marker Pax5 (data not shown). [Extracted from the article]

Background: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, aggressive type of haematologic precursor malignancy primarily often manifesting in the skin. We sought to provide a thorough clinical characterization and report our experience on therapeutic approaches to BPDCN. Methods: In the present multicentric retrospective study, we collected all BPDCN cases occurring between 05/1999 and 03/2018 in 10 secondary care centres of the German–Swiss–Austrian cutaneous lymphoma working group. Results: A total of 37 BPDCN cases were identified and included. Almost 90% of the patients had systemic manifestations (bone marrow, lymph nodes, peripheral blood) in addition to skin involvement. The latter presented with various types of cutaneous lesions: nodular (in more than 2/3) and bruise‐like (in 1/3) skin lesions, but also maculopapular exanthema (in circa 1/6). Therapeutically, 22 patients received diverse combinations of chemotherapeutic regimens and/or radiotherapy. Despite initial responses, all of them ultimately relapsed and died from progressive disease. Eleven patients underwent haematopoietic stem cell transplantation (HSCT; autologous HSCT n = 3, allo‐HSCT n = 8). The mortality rate among HSCT patients was only 33.33% with a median survival time of 60.5 months. Conclusion: Our study demonstrates the clinical diversity of cutaneous BPDCN manifestations and the positive development observed after the introduction of HSCT. [ABSTRACT FROM AUTHOR]

Fixed Drug eruptions: an update, emphasizing the potentially lethal generalized bullous fixed drug eruption. Despite quinine's now diminished therapeutic importance and clinical use, the contribution of dietary quinine to clinical symptoms of a fixed drug eruption should be considered, and adequate testing should be performed. Two cases of quinine-induced fixed "drug" eruption induced by long drinks. [Extracted from the article]

Associated primary tumours in patients with primary cutaneous lymphoma: a single-centre retrospective cohort study Associated primary tumours were categorized into solid, skin and haematological/lymphoid neoplasms, and the time point of the occurrence in relation to the CL was documented. The present analysis confirms an increased occurrence of associated primary tumours in patients with CL, including patients with CBCL in northern Germany. [Extracted from the article]