The itch associated with cutaneous T-cell lymphoma (CTCL), including Mycosis Fungoides (MF) and Sézary syndrome (SS), is often severe and poorly responsive to treatment with antihistamines. Recent studies have highlighted the possible role of interleukins in nonhistaminergic itch. We investigated the role of IL-31 and IL-8 in CTCL, concerning disease severity and associated itch. Serum samples of 27 patients with CTCL (17 MF and 10 SS) and 29 controls (blood donors) were analyzed for interleukin- (IL-) 31 and IL-8; correlations with disease and itch severity were evaluated. IL-31 serum levels were higher in CTCL patients than in controls and higher in SS than in MF. Also, serum IL-31 levels were higher in patients with advanced disease compared to those with early disease, and they correlated positively with lactate dehydrogenase and beta 2-microglobulin levels, as well as with the Sézary cell count. Itch affected 67% of CTCL patients (MF: 47%; SS: 100%). Serum IL-31 levels were higher in itching patients than in controls and in patients without itching. There was no association between serum IL-8 and disease severity, nor with itching. Serum IL-8 levels correlated positively with peripheral blood leukocyte and neutrophil counts in CTCL patients. Our study suggests a role for IL-31 in CTCL-associated itch, especially in advanced disease and SS, offering a rational target for new therapeutic approaches. Increased serum IL-8 observed in some patients may be related to concomitant infections, and its role in exacerbating itch by recruiting neutrophils and promoting the release of neutrophil proteases deserves further investigation.
Our purpose was to evaluate the therapeutic effects of a natural product (propolis) on recurrent aphthous ulceration (RAU) of the minor type regarding the number of lesions, their duration and frequency of recurrence. Seventy patients with RAU composed the study group who were examined according to pre-established criteria. Forty patients presenting with RAU (mean age 38.5 years; 25 women and 15 men) were selected and medicated during the recurrence of their lesions using a purified propolis solution in a 5%propyleneglycol vehicle. Patients applied the topical solution three times a day from the first premonitory sign of RAU appearance and also during episodes of recurrence for a period of one year. A statistically significant reduction was observed in the number, frequency and duration of the lesions (p≤ 0.01). The natural propolis product utilized in this study for RAU therapy was without any adverse effects and proved beneficial.
Introduction: Mastocytosis is characterized by the clonal expansion of morphological and immunophenotypically abnormal mast cells in different organs. The skin is the most frequently affected tissue. Virtually all children and more than 80% of adult patients with mastocytosis show cutaneous lesions.Material and Methods: The present article describes the symptoms and signs in cutaneous mastocytosis, based on the review of recently published international consensus guidelines.Discussion: According to the 2016 World Health Organization classification, mastocytosis can be divided in cutaneous mastocytosis, systemic mastocytosis and mast cell sarcoma. Cutaneous mastocytosis is subclassified in three subtypes: maculopapular cutaneousmastocytosis, diffuse cutaneous mastocytosis and cutaneous astocytoma. Telangiectasia macularis eruptiva perstans is no longerconsidered a distinct entity.Conclusion: Based on the age of onset, cutaneous manifestations of mastocytosis can be variable. The classification of cutaneous mastocytosis has recently been updated. Typically, in patients with childhood-onset mastocytosis, the disease occurs as cutaneous mastocytosis and shows spontaneous resolution around puberty. In contrast, adult patients, despite having also cutaneous lesions, often show systemic involvement and the course of the disease is usually chronic.
Detailed knowledge about differentiation syndrome (DS) has remained limited. There are 2 large studies conducted by the Spanish workgroup PETHEMA (Programa Español de Tratamientos en Hematología; Spanish Program on Hematology Treatments) and the European group trial (LPA 96-99 and APL 93) in which the incidence, characteristics, prognostic factors and outcome of patients developing DS are evaluated. Both have described the median time of DS development between 10 and 12 days. The severity of the DS has been evaluated in the study conducted by PETHEMA, and severe DS usually occurs at the beginning of the treatment (median of 6 days), as compared with moderate DS (median of 15 days). We report here in two cases of late severe DS, with late diagnosis due to both time and form of presentation. We discuss the physiopathology, clinical presentation, prophylaxis and treatment of DS.
Introduction Mast cell (MC) leukemia (MCL) is extremely rare. We present a case of MCL diagnosed concomitantly with acute myeloblastic leukemia (AML). Case Report A 41-year-old woman presented with asthenia, anorexia, fever, epigastralgia, and diarrhea. She had a maculopapular skin rash, hepatosplenomegaly, retroperitoneal adenopathies, pancytopenia, 6% blast cells (BC) and 20% MC in the peripheral blood, elevated lactate dehydrogenase, cholestasis, hypoalbuminemia, hypogammaglobulinemia, and increased serum tryptase (184 μg/L). The bone marrow (BM) smears showed 24% myeloblasts, 17% promyelocytes, and 16% abnormal toluidine blue positive MC, and flow cytometry revealed 12% myeloid BC, 34% aberrant promyelocytes, a maturation blockage at the myeloblast/promyelocyte level, and 16% abnormal CD2−CD25+ MC. The BM karyotype was normal, and the KIT D816V mutation was positive in BM cells. The diagnosis of MCL associated with AML was assumed. The patient received corticosteroids, disodium cromoglycate, cladribine, idarubicin and cytosine arabinoside, high-dose cytosine arabinoside, and hematopoietic stem cell transplantation (HSCT). The outcome was favorable, with complete hematological remission two years after diagnosis and one year after HSCT. Conclusions This case emphasizes the need of an exhaustive laboratory evaluation for the concomitant diagnosis of MCL and AML, and the therapeutic options.
Between 1961 and 1976, 387 patients with Hodgkin disease were examined, evaluated, and treated at the Instituto Portugues de Oncologia de Francisco Gentil. After reviewing histological and clinical staging presentation, the authors retrospectively analyzed the results obtained with 303 patients classified in clinical stages I, II, and III (A, B) who were treated with or without chemotherapy in two time periods (before and after 1970) according to individual therapeutic modalities. The improvement of the 5-year survival rates in the last period was associated with the introduction of extended-field irradiation and multidrug chemotherapy (MOPP). However, the incidence of serious complications was higher in the group of patients subjected to combined field irradiation and MOPP. The authors suggest a stricter protocol based on the current recommendations for the treatment of Hodgkin disease in order to achieve better results with minimum possible hazards.
O brentuximab vedotina está aprovado pela Food and Drug Administration (FDA) e pela European Medicines Agency (EMA) para o tratamento de doentes adultos com linfomas cutâneos primários de células T CD30+, refratários a pelo menos uma linha terapêutica prévia. Os autores reportam dois casos clínicos de linfoma cutâneo primário de células grandes anaplásicas, tratados com brentuximab vedotina, com respostas clínicas distintas. Não obstante, o brentuximab vedotina poderá constituir uma alternativa terapêutica válida para o tratamento destas patologias.
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