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Background: Congenital neutropenia is a primary immunodeficiency characterized by quantitative anomalies in neutrophil counts. It is classified as mild, moderate, or severe. Hematopoietic stem cell transplantation stands as a potential therapeutic intervention; nevertheless, graft-versus-host disease emerges as a main complication. Case report: We report the case of a 2-year-old female patient, with a history of congenital neutropenia. She had scalp abscesses and preseptal cellulitis, which were satisfactorily treated. After a multidisciplinary approach, a protocol was initiated to receive an allogenic hematopoietic progenitor cell transplant; graft versus host disease was subsequently diagnosed, ultimately resulting in death of the patient. Conclusion: Graft-versus-host disease remains a significant complication in patients who undergo allogeneic hematopoietic stem cell transplants. Its recognition, management, and treatment are clinically relevant aspects given the substantial degree of associated morbidity and mortality. [ABSTRACT FROM AUTHOR]

Graft-versus-host disease is a common complication after stem cell transplantation. The digestive tract is affected in many patients who suffer from it, with consequences that can be fatal. The proper approach, which includes endoscopic studies, allows ruling out differential diagnoses and managing the disease early. [ABSTRACT FROM AUTHOR]

Chronic cutaneous graft-versus-host disease (GVHD) is most frequently expressed in the skin. The sclerotic type presents difficulty in its management, since the multiple immunosuppressive systemic therapies to improve it are not always effective and increase the risk of infections. CLINICAL CASE: A 57-year-old male patient, in whom in January 2013 the diagnosis of acute lymphoblastic leukemia was established, which achieved complete remission after three cycles of chemotherapy with the HyperCVAD scheme. Five months later an allogeneic transplant of hematopoietic stem cells from related living donor (sibling) was performed as consolidation treatment. He received oral tacrolimus and mycophenolate oral as a prophylaxis of GVHD. Subsequently, Epstein-Barr virus infection (positive serology and PCR) was documented, so immunosuppressive management was discontinued. Ten months after the transplant, the patient started with generalized musculoskeletal pain, as well as oral, genital and ocular mucositis, for which he received treatment with unspecified creams and eye drops. Two months later he began with exulceration on the back of the neck, as well as maculo-papular rash on the head and trunk, remaining unchanged for four months. The diagnosis of graft disease against chronic host was established. CONCLUSION: Through a case with extensive sclerosis, we illustrate the beneficial management and proper tolerance of UVA phototherapy associated with oral psoralen (PUVA), avoiding other therapies and their adverse concomitant effects. [ABSTRACT FROM AUTHOR]

The acute graft-versus-host disease is a common complication of allogeneic transplants with impact in morbidity and mortality. The dermatological and gastrointestinal manifestations are usually the most frequent and classical presentation; however, the dermatological signs are usually the earliest which is important for a timely diagnosis; the maculopapular rash is the most common dermatological manifestation. They have been described in the literature as lichenoid or alopecic plaques or just dyshidrosis. A case of hyperpigmented macules on the face is presented and it represents an atypical clinical form of this disease. [ABSTRACT FROM AUTHOR]

Introduction: Chronic graft-versus-host disease (cGVHD) is the most important cause of late non-relapse mortality after allogeneic hematopoietic stem cell transplantation. Sclerodermatous cGVHD is usually steroid refractory and remains a therapeutic challenge. Activating antibodies against the PDGFR have been reported in patients with sclerodermatous cGVHD. These antibodies induce PDGFR phosphorylation and lead to fibrosis. There is increasing evidence of successful treatment of sclerodermatous cGVHD with imatinib, a tyrosine kinase inhibitor., Objective: To evaluate the response of cutaneous sclerodermatous cGVHD to imatinib., Materials and Methods: Retrospective study of 18 patients with sclerodermatous cGVHD refractory to immunosuppressants treated with imatinib in a single center. Evaluation of treatment response was performed by clinicians' assessment and patients' subjective response at one, 3, 6, 9, 12 and 18 months after initiation of imatinib. Response was assessed as complete, partial, significant, no change or progression. Tapper off steroids was complete, partial or not possible., Results: In our series, 4 (22%) patients achieved complete response, 9 (50%) patients partial response, 2 (11%) patients significant response, 2 (11%) patients had no change and one (6%) patient progressive disease at last follow-up. Mean time from initiation of imatinib to any degree of response was 2,75 months (range 1-9 months)., Conclusions: This study provides further evidence of the role of imatinib for the treatment of steroid refractory sclerodermatous cGVHD., (Copyright © 2017 AEDV. Publicado por Elsevier España, S.L.U. All rights reserved.)