Inflammatory Presentation of a Primary Extranodal Diffuse Large B-cell Lymphoma of the Maxillary Sinus.

Diffuse large B-cell lymphoma originating from the maxillary sinus is rarely reported in the literature. Diagnosis is challenging since the long absence of signs and symptoms allows it to grow undetected or be confused with benign inflammatory conditions. The purpose of this paper is to present an unusual manifestation of this rare pathology. A patient in his 50s, presented to his local emergency department with malar and left eye pain after local trauma. Physical examination showed infraorbital edema, palpebral ptosis, exophthalmos, and left ophthalmoplegia. CT scan showed a soft tissue mass measuring 43x31 mm in the left maxillary sinus. An incisional biopsy was performed, and results showed diffuse large B-cell lymphoma with positivity for CD10+, BCL6+, BCL2+, and Ki-67 index greater than 95%. Afterward, the patient promptly started treatment with rituximab-cyclophosphamide-hydroxydaunorubicin-Oncovin-prednisone (R-CHOP) chemotherapy. A good medical history, clinical and imaging evaluations, and anatomopathological studies are crucial to establish an early diagnosis of diffuse large B-cell lymphoma (DLBCL).

Fill in the blank for fashion complementary outfit product Retrieval: VISUM summer school competition.

Every year, the VISion Understanding and Machine intelligence (VISUM) summer school runs a competition where participants can learn and share knowledge about Computer Vision and Machine Learning in a vibrant environment. 2021 VISUM's focused on applying those methodologies in fashion. Recently, there has been an increase of interest within the scientific community in applying computer vision methodologies to the fashion domain. That is highly motivated by fashion being one of the world's largest industries presenting a rapid development in e-commerce mainly since the COVID-19 pandemic. Computer Vision for Fashion enables a wide range of innovations, from personalized recommendations to outfit matching. The competition enabled students to apply the knowledge acquired in the summer school to a real-world problem. The ambition was to foster research and development in fashion outfit complementary product retrieval by leveraging vast visual and textual data with domain knowledge. For this, a new fashion outfit dataset (acquired and curated by FARFETCH) for research and benchmark purposes is introduced. Additionally, a competitive baseline with an original negative sampling process for triplet mining was implemented and served as a starting point for participants. The top 3 performing methods are described in this paper since they constitute the reference state-of-the-art for this particular problem. To our knowledge, this is the first challenge in fashion outfit complementary product retrieval. Moreover, this joint project between academia and industry brings several relevant contributions to disseminating science and technology, promoting economic and social development, and helping to connect early-career researchers to real-world industry challenges.

[Venetoclax: A New Hope for Elderly Patients with Acute Myeloid Leukemia]. / Venetoclax: Uma Nova Esperança para o Doente Idoso com Leucemia Mielóide Aguda.

Acute myeloid leukemia (AML) is a hematopoietic malignancy characterized by clonal proliferation, with increased incidence with advancing age. AML with myelodysplasia-related changes (AML-MRC) represents an AML subtype with a poor prognosis and challenging treatment, particularly in elderly patients. We report the case of a 77-year-old patient diagnosed with high-risk AML-MRC, ineligible for intensive chemotherapy, with frequent need of transfusion of red cell concentrates. The authors present, to the best of their knowledge, the first patient in Portugal with AML-MRC treated with an hypomethylating agent, azacytidine, and a BCL2 inhibitor (venetoclax), and that association was essential in the treatment and overall survival, which was much higher than expected.

A leucemia mielóide aguda (LMA) é uma neoplasia hematológica caracterizada pela proliferação clonal, com incidência crescente com a idade. A LMA com alterações relacionadas com mielodisplasia (LMA-ARM) representa um subtipo de LMA com prognóstico adverso, cujo tratamento é desafiante, sobretudo nos doentes mais idosos. Descrevemos o caso de uma doente de 77 anos com o diagnóstico de LMA-ARM, de alto risco inelegível para quimioterapia intensiva, com necessidades transfusionais frequentes. Tanto quanto é do seu conhecimento, os autores apresentam o primeiro caso clínico de LMA-ARM, em Portugal, tratado com a associação do agente hipometilante, azaciditina, e o inibidor BCL2 (venetoclax), o que foi crucial para o tratamento e sobrevivência global do doente, muito além da sobrevivência esperada.

Interim FDG18-PET SUVmax Variation Adds Prognostic Value to Deauville 5-Point Scale in the Identification of Patients with Ultra-High-Risk Diffuse Large B Cell Lymphoma.

INTRODUCTION: Interim response evaluation by 18F-fluorodeoxyglucose positron emission tomography/computed tomography (iPET) in diffuse large B cell lymphoma (DLBCL) could be important to rule out disease progression and has been suggested to be predictive of survival. However, treatment guidance by iPET is not yet recommended for DLBCL in clinical practice. We aimed to compare the predictive value of iPET when utilizing the visual Deauville 5-point scale (DS) and the semiquantitative variation of maximum standardized uptake value (ΔSUVmax). MATERIALS AND METHODS: We included 85 patients diagnosed with DLBCL and uniformly treated with standard protocols. iPET with DS of 1-3 and/or ΔSUVmax ≥66% was defined as negative. Univariable and multivariable Cox regression analyses were performed to determine the independent factors affecting progression free survival (PFS) or overall survival (OS) and to estimate PFS and OS. RESULTS: iPET positivity, measured by DS or ΔSUVmax, showed predictive value of disease refractoriness, improved by combining DS and ΔSUVmax. After a median follow-up of 50.1 months, iPET was an independent predictor for both PFS and OS when interpreted by DS, but only for PFS by ΔSUVmax. Combined visual and semiquantitative analysis (D4-5 & ΔSUVmax<66%) was an independent predictor of PFS and OS, and allowed to identify an ultra-high-risk subgroup of patients with very dismal outcome, increasing the discriminating capacity for iPET. CONCLUSION: Our study suggests that combined DS and ΔSUVmax in iPET assessment predicts refractory disease and distinguishes ultra-high-risk DLBCL patients with a very dismal prognosis, who may benefit from PET-guided therapy adjustment.

Hodgkin's variant of Richter transformation during ibrutinib therapy: A case report and review of the literature.

Hodgkin's variant of Richter transformation is a rare complication of chronic lymphocytic leukemia and is associated with inferior outcomes compared to de novo Hodgkin lymphoma. Further data concerning prognosis and treatment of Hodgkin's variant of Richter transformation occurring in the setting of novel targeted therapies are needed.

Total Shoulder Reverse Arthroplasty in Alkaptonuria: An Effective Option to Treat a Complication of a Rare Disease.

Introduction: Alkaptonuria (AKU) is a rare autosomal recessive disorder in which tyrosine metabolism is altered. There is a deficiency of the Homogentisate 1,2-dioxygenase enzyme (homogentisic acid oxidase) This enzyme is responsible for the metabolism of the tyrosine - homogentisic acid - in maleylacetoacetic acid. Accumulation of homogenous acid occurs in urine and conjunctival tissue in the second with important deleterious effects on cartilage and bone. The most limiting complication is progressive degenerative arthropathy, especially in the axial and load load-bearing joints, with a lower tendency to affect the shoulder. Because of its rarity little is known about the results of arthroplasties in these patients. Case Report: A 60 -years -old man, retired, with a history of right knee arthroplasty 6 years ago (performed in another hospital). Sent to the orthopedic consultation due to intense shoulder pain. The case was revealed to be a rare disease: arthropathy due AKU. He was treated with total shoulder reverse arthroplasty. Currently with two 2 years of follow-up, with excellent functional results. Conclusion: The most commonly performed procedure, in this patients, is the hip prosthesis and there are few reported cases of shoulder arthroplasty. However, the results of our follow follow-up are good and encouraging.

Methotrexate-Associated Lymphoproliferative Disorder in a Patient with Psoriasis: A Case Report and Review of the Literature.

Iatrogenic immunodeficiency-associated lymphoproliferative disorders (LPDs) are heterogeneous clinicopathological entities developing in patients receiving immunosuppression. Outside the posttransplant setting, methotrexate (MTX), a drug commonly used for the treatment of autoimmune diseases, is an immunosuppressive agent frequently reported to be associated with LPD. MTX-associated LPD (MTX-LPD) includes a spectrum of lymphocytic proliferations, ranging from polyclonal hyperplasia to malignant lymphoma. MTX-LPD diagnosis can be challenging, as signs and symptoms are often nonspecific and may overlap with those of several other conditions, including exacerbation of the underlying autoimmune disease. Spontaneous regression of LPD after MTX discontinuation is characteristic of MTX-LPD, therefore avoiding chemotherapeutic intervention in a significant proportion of patients. Other cases, however, should receive chemotherapy.

Rare case of bilateral anterolateral and symmetrical bowing of tibia successfully treated with a distal tibial opening wedge osteotomy.

The anterolateral bowing of the tibia is closely associated with the development of its pseudarthrosis. Roughly, all deformities are unilateral so the shortening and angulation are easy to identify. We present a 6-year-old boy with an exuberant bilateral anterolateral bowing of tíbia. He has short stature, disturbed gait and callosity at the lateral border of the foot. Deformity was successfully treated by opening wedge tibia osteotomy and filled the remaining gap with structural fibular graft. It was fixed with two crossed K-wires and cast immobilization for 6 weeks. We decided to correct it before skeletal maturity due to the significant disturbance of the gait and esthetic impairment. It was obtained a satisfactory morphological and functional result with a simple and fast technique.