F. Sicre de Fontbrune

Fecal calprotectin and α1-antitrypsin dynamics in gastrointestinal GvHD

In a previous study, the fecal biomarkers calprotectin and α1-antitrypsin (α1-AT) at symptom onset were reported to be significantly associated with the response to steroids in gastrointestinal GvHD (GI-GvHD). The purpose of this trial was to evaluate the dynamics of the fecal biomarkers calprotectin and α1-AT throughout the course of GvHD. Patients who were refractory to steroids had initially higher biomarker levels and in the course of GvHD demonstrated a continuous increase in fecal biomarkers. In contrast, the dynamics of calprotectin and α1-AT demonstrated low and decreasing levels in cortico-sensitive GvHD. In steroid-refractory patients who received a second line of treatment, the biomarker levels at the beginning of second-line treatment did not predict the subsequent response. Nevertheless, calprotectin levels progressively decreased in subsequent responders, whereas non-responders demonstrated continuously high levels of calprotectin. α1-AT values correlated to a lesser extent with the response to second-line treatment and remained elevated in both non-responders and responders. In conclusion, calprotectin monitoring can be of use in the management of immunosuppressive treatment in GI-GvHD.

Efficacy and tolerance of ruxolitinib in refractory sclerodermatous chronic graft-versus-host disease

DEAR EDITOR, Chronic graft-versus-host disease (cGVHD) occurs frequently following allogeneic haematopoietic stem cell transplantation (AHSCT). It can often be severe and there is currently an unmet therapeutic need. A recent retrospective study has shown the potential efficacy of ruxolitinib, a selective Janus kinase (JAK)1/2 inhibitor, for the treatment of acute graft-versus-host disease (aGVHD) (n = 54) and cGVHD (n = 41), with an overall response rate of 82% and 85%, respectively. There are no specific studies evaluating the efficacy of ruxolitinib in sclerodermatous cGVHD of the skin, a rare and difficult to treat form of cGVHD. This monocentric retrospective study enrolled patients (n = 12) with severe sclerodermatous cGVHD treated with ruxolitinib between May 2015 and July 2016. Patients with severe sclerodermatous cGVHD were defined as patients who had a modified Rodnan skin score (mRSS) ≥ 10 and were refractory to corticosteroids and at least one other immunosuppressive drug. All of the patients had a stable or worsening mRSS

KIR3DL2 expression in patients with adult T-cell lymphoma/leukaemia

Adult T-cell lymphoma/leukemia (ATLL) is a hematologic malignancy of mature CD4-positive T-cells associated with chronic infection by human T-lymphotropic virus 1 (HTLV-1). Four different subtypes of ATLL have been described: acute, lymphomatous, chronic and smoldering forms. ATLL prognosis remains poor, with overall survival duration depending on the subtype, because there is no curative treatment of ATLL and patients often suffer from opportunistic infections. This article is protected by copyright. All rights reserved.

Cutaneous presentation of adult T-cell leukemia/lymphoma (ATLL). Single-center study on 37 patients in metropolitan France between 1996 and 2016

INTRODUCTION Adult T-cell leukemia/lymphoma (ATLL) is a hematological malignancy associated with chronic HTLV-1 infection. AIM To describe skin lesions in ATLL. METHODS A descriptive, retrospective study between 1996 and 2016, including all patients diagnosed with ATLL at Saint-Louis Hospital (Paris, France). RESULTS Thirty-seven ATLL patients were included. Fifteen patients (41%) had a cutaneous localization of the disease, which was present from the beginning of the disease for two thirds of them. ATLL types in patients with cutaneous localization of the disease were as follows: lymphoma, n=5, chronic, n=4, smoldering, n=4, acute, n=2. Half the patients had 2 or more cutaneous manifestations. The cutaneous localizations observed were as follows: nodulotumoral (n=8), plaques (n=7), multipapular (n=6), macular (n=4), purpuric (n=2). Among the 15 patients with cutaneous localization, median overall survival was significantly shorter in the acute and lymphoma types compared to the smoldering and chronic types (8.7 months vs. 79 months, P=0.003). DISCUSSION ATLL is a hematologic malignancy with variable expression that is diagnosed only very rarely in metropolitan France, but that should be sought in patients from countries with high HTLV-1 prevalence in the event of a chronic eruption with patches, papules, plaques and/or tumors. The chronic and smoldering types are relatively indolent, whereas the acute and lymphoma forms have a poor prognosis.

GVH cutanées chroniques ulcérées : spectre clinique et évolutif sur une cohorte rétrospective de 25 patients

— 9 (deux hommes et sept femmes) (5,7 %) avaient des antécédents d’IST ; — 54,1 % ont déclaré avoir une couverture vaccinale vis-à-vis du VHB. La prévalence de la séropositivité VIH était de 0,66 % (1/151) (0,49 % au CDAG en 2012). Aucun test n’a été positif pour le VHB (0/32), le VHC (0/11) et la syphilis (0/30). Un dépistage systématique par PCR sur un auto-écouvillonnage vaginal a trouvé 13 (17,6 %) chlamydioses et six (8,1 %) gonococcies. Discussion.— Le nombre important d’IST dépistées chez les patients mineurs confirme l’intérêt du CDAG. L’anonymat et la gratuité leur permettent d’éviter que leurs parents soient informés. La faible prévalence de la couverture contraceptive ainsi que les antécédents d’IVG justifient une optimisation de la coordination entre CDAG et les structures CPEF (Planning Familial). Conclusion.— La proportion importante de patients mineurs souffrant d’IST et à risque de grossesse est une situation particulièrement préoccupante, qui peut être en partie liée à une éducation sexuelle insuffisante. L’anonymat qui est la clef de voûte du CDAG présente un intérêt très important pour les mineurs. Déclaration d’intérêt.— Aucun.