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Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is a disease known from a few months, caused by a recently arisen virus and, consequently, it is little known. The disease has a benign course in most infected subjects (children and young adults), is often symptomatic in adults over the age of 50 and often serious and life threatening in people with comorbidities and the elderly. The few data published on coronavirus disease-2019 (COVID-19) in the blood-oncology field report a serious clinical presentation, a serious course of the disease, and a high mortality rate, as has also been reported for other cancer contexts. The current strategy for treating patients with SARS-CoV-2 includes antivirals that are effective against other viral infections and drugs that can moderate the cytokine storm. There is no specific vaccine and consequently all possible precautions must be taken to prevent SARS-CoV-2 infection in the areas of oncology, oncohematology, and bone marrow transplantation. In this reviewer's article, we report the information currently available on SARS-CoV-2 infection to help young doctors and hematologists to successfully manage patients with COVID-19.
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COVID-19/sangue , COVID-19/patologia , SARS-CoV-2 , Anticorpos Antivirais/sangue , COVID-19/diagnóstico , COVID-19/terapia , Humanos , RNA Viral/sangue , SARS-CoV-2/genética , SARS-CoV-2/imunologiaAssuntos
Neoplasias , Envio de Mensagens de Texto , Humanos , Neoplasias/terapia , Neoplasias/psicologiaRESUMO
AIMS: This study, conducted in a 'field-practice' scenario, investigates the effectiveness and safety of everolimus in the second-line treatment of metastatic renal cell carcinoma (mRCC) patients. PATIENTS & METHODS: mRCC patients, who started everolimus 10 mg/day after failure of first-line VEGF receptor-targeted tyrosine kinase inhibitor, were included in this study. Study end points were treatment response, progression-free survival and tolerability. RESULTS: In total, 100 patients were assessed; the median duration of everolimus treatment was 7.1 months (95% CI: 5.7-8.5). A total of 19% of patients experienced a partial response and 62% of patients reached a stable disease. Median progression-free survival was 8 months (95% CI: 6.7-9.3). A total of 75% of patients experienced adverse events; no grade 4 adverse events were reported. CONCLUSION: These findings, obtained in a 'field-practice' scenario, support the use of everolimus for mRCC patients who failed one VEGF receptor-targeted tyrosine kinase inhibitor.
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Antineoplásicos/uso terapêutico , Carcinoma de Células Renais/tratamento farmacológico , Neoplasias Renais/tratamento farmacológico , Sirolimo/análogos & derivados , Adulto , Idoso , Carcinoma de Células Renais/mortalidade , Carcinoma de Células Renais/secundário , Intervalo Livre de Doença , Everolimo , Feminino , Humanos , Estimativa de Kaplan-Meier , Neoplasias Renais/mortalidade , Neoplasias Renais/patologia , Masculino , Pessoa de Meia-Idade , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Sirolimo/uso terapêuticoRESUMO
Mycosis fungoides is the most common primary cutaneous T-cell lymphoma, characterized by skin-homing CD4+ T cells derivation, indolent course, and low-grade of malignancy. Mycosis fungoides's classic type typically onsets with cutaneous erythematous patches, plaque, and tumor. In WHO-EORTC classification, folliculotropic mycosis fungoides, pagetoid reticulosis, and granulomatous slack skin are recognized as distinct variants of mycosis fungoides, because of their clinical and histological features, behavior, and /or prognosis. Mycosis fungoides often shows diagnostic difficulties, due to its absence of specific features and lesional polymorphism. A patient's treatment requires staging. In about 10% of cases, mycosis fungoides can progress to lymph nodes and internal organs. Prognosis is poor at advanced stage and management needs a multidisciplinary team approach. Advanced stage disease including tumors, erythroderma, and nodal, visceral, or blood involvement needs skin directed therapy associated with systemic drugs. Skin directed therapy includes steroids, nitrogen mustard, bexarotene gel, phototherapy UVB, and photochemiotherapy, i.e., total skin electron radiotherapy. Systemic therapies include retinoids, bexarotene, interferon, histone deacetylase inhibitors, photopheresis, targeted immunotherapy, and cytotoxic chemotherapy. Complexity of mycosis fungoides associated with long-term chronic evolution and multiple therapy based on disease stage need a multidisciplinary team approach to be treated.
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BACKGROUND: The t (2; 5) chromosomal rearrangement of the ALK gene with nucleophosmin 1 gene (NPM1), resulting in an NPM1-ALK fusion, was first demonstrated in 1994 in anaplastic large cell lymphoma, (ALCL), a T-cell lymphoma responsive to cyclophosphamide, abriblastine, vincristine and prednisone in approximately 80% of cases; refractory cases usually respond favorably to brentuximab vedotin. These treatments are regarded as a bridge to allogeneic hematopoietic stem cell transplantation (allo-SCT). Nowadays, transplant procedures and the monitoring of chemotherapy patients proceed very slowly because the SARS-CoV-2 pandemic has heavily clogged the hospitals in all countries. RESULTS: A 40-year-old Caucasian woman was first seen at our clinical center in June 2020. She had ALCL ALK+, a history of failure to two previous therapeutic lines and was in complete remission after 12 courses of brentuximab, still pending allo-SCT after two failed donor selections. Facing a new therapeutic failure, we requested and obtained authorization from the Italian drug regulatory agency to administer 250 mg of crizotinib twice a day, a drug incomprehensibly not registered for ALCL ALK +. CONCLUSIONS: The response to crizotinib was optimal since no adverse event occurred, and CT-PET scans persisted negative; this drug has proved to be a valid bridge to allo-SCT.
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BACKGROUND: The incidence of cancer is higher in transplant patients than in the normal population, mostly due to the assumption of immunosuppressants able to reduce the possibility of rejection. In addition, immunocompromised patients have a greater susceptibility to EBV, HPV and HIV, infectious agents that by themselves may favor the onset of malignancies. Post-transplant lymphoproliferative diseases (PLDs) are among the most frequent neoplasms in transplant patients which like other aggressive neoplasms may be identified by the [18f] fluoro-D-deoxyglucose positron emission tomography/computed tomography (FDG-PET/CT). METHODS: We evaluated the clinical use of FDG-PET/CT in detecting PTLDs and other neoplasms performed at the lowest clinical or laboratory suspicion of malignancy in 127 consecutive subjects who underwent heart transplantation. RESULTS: A SUV>4 more confirmed the suspect of malignancy and induced us to further investigations. Of the 127 transplant subjects who underwent FDG-PET/CT, 64 showed a SUV value >4. Of these 64, 8 had PTLDs, 49 other neoplasms (urinary tract tumors, thyroid cancer, HPV cancer related, Kaposi' sarcoma and EBV related head and neck neoplasms) and 7 patients with chronic non-neoplastic inflammatory diseases. CONCLUSIONS: In the present study, FDG-PET/CT examination was of great use for an early identification and for an early treatment of PTLDs and other neoplasms.
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Fluordesoxiglucose F18 , Transplante de Coração/efeitos adversos , Transtornos Linfoproliferativos/diagnóstico por imagem , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada/métodos , Complicações Pós-Operatórias/diagnóstico por imagem , Compostos Radiofarmacêuticos , Adulto , Idoso , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Feminino , Transplante de Coração/estatística & dados numéricos , Doença de Hodgkin/diagnóstico por imagem , Doença de Hodgkin/tratamento farmacológico , Doença de Hodgkin/etiologia , Humanos , Hiperplasia/diagnóstico por imagem , Hiperplasia/tratamento farmacológico , Hiperplasia/etiologia , Hospedeiro Imunocomprometido , Linfoma não Hodgkin/diagnóstico por imagem , Linfoma não Hodgkin/tratamento farmacológico , Linfoma não Hodgkin/etiologia , Transtornos Linfoproliferativos/tratamento farmacológico , Transtornos Linfoproliferativos/etiologia , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias/tratamento farmacológico , Complicações Pós-Operatórias/etiologiaRESUMO
Chest pain following a trans-thoracic biopsy often has multiple etiologies, especially in patients with lymphomas. Pathological neuronal mechanisms integrate with an overproduction of IL-6, TNF-α, IL1-ß by macrophages and monocytes, which amplifies inflammation and pain. In consideration of this complex pathogenesis, international guidelines recommend diversified analgesia protocols: thoracic epidural, paravertebral block, and systemic administration of opioids. This study reports an attempt to reduce chest pain and prevent chronic pain in 51 patients undergoing trans-thoracic biopsy for mediastinal lymphoma. The entity of pain, measured 72nd hour after biopsy by the Numerical Rating Scale (NRS), was compared with that seen at a 6th month checkpoint in 46 patients. The pain decreased in all cases. At the 6th month checkpoint, among 31 opioid-treated patients, none of the 16 patients with NRS < 6 within the 72nd hour post biopsy had developed chronic chest pain, while 8 of the 15 with higher values did (p < 0.01). Of 10 patients undergoing thoracotomy and treated with opioids, eight had a NRS of no more than 2, of which six had no chronic pain. Of the twenty-one patients who underwent VATS biopsy and were treated with opioids, fifteen had NRS no greater than 2, of which ten had no chronic pain. Subgroups of patients biopsied under mediastinotomy or video-assisted thoracoscopic surgery (VATS) and treated with thoracic epidural analgesia (TEA) or PVB were too small for such analysis.
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Chronic lymphatic leukemia (CLL) is the most frequent type of leukemia in western countries and when association with del(11q) is correlated with a worse prognosis. We reported the clinical case of an 80-year-old patient with CLL related to del(11q) and a BMI of 16.4 kg/m2, who presented a voluminous mass in abdominal cavity (23 × 14 × 4 cm) which occupied the whole of the mesentery and the retroperitoneal space, treated with ibrutinib, adequate nutrition, and a program of physical activity. He showed a great improvement under ibrutinib therapy and took to artificial nourishment and adequate muscle rehabilitation until he recovered his autonomy. In August 2018, a 5-days-a-week training program was started: Physical activity for at least 20 min consisting of a fast walk in the open air three times a week and a moderate physical activity in the remaining two days of at least 20 consecutive minutes (cycling at a regular pace, carrying light weights). The exercise program included also aerobic, upper and lower limb resistance training, chore stability and stretches. The physical condition further improved and remained excellent throughout the follow-up period. In December 2018, his clinical condition was quite normal; a CT showed a great decrease of all lymphoadenomegaly, and FISH test did not show del(11q). He continued to cultivate his land, while still being treated with ibrutinib. The combination of the right therapy, adequate nutrition, and muscle rehabilitation is the best solution to improve the clinical condition of old cachectic CLL del(11q) patient.
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Leucemia Linfocítica Crônica de Células B , Pirazóis , Pirimidinas , Adenina/análogos & derivados , Idoso de 80 Anos ou mais , Deleção Cromossômica , Cromossomos Humanos Par 11 , Exercício Físico , Humanos , Leucemia Linfocítica Crônica de Células B/tratamento farmacológico , Leucemia Linfocítica Crônica de Células B/genética , Masculino , Piperidinas , Pirazóis/uso terapêutico , Pirimidinas/uso terapêuticoRESUMO
INTRODUCTION: In the last months of 2019, the advent of a new virus called SARS-CoV-2 caused the spread of a pandemic disease, COVID-19, that has afflicted patients with chronic pain. CASE PRESENTATION: We describe a COVID-19 patient recently implanted with a spinal cord stimulator for FBSS, treated with Tocilizumab for cytokine storm complicating SARS-COV-2 infection. This patient developed a delayed hyperimmune reaction, causing an inflammatory reaction in the surgical pocket site, well treated with total remission. The total resolution of this local reaction occurred after the resolution of systemic COVID-19 infection by Tocilizumab. CONCLUSIONS: We discuss the balance between damage-associated molecular patterns (DAMPs) and pathogen-recognition receptors (PRRs), and the putative role of polymorphism in the IL-6/174 position of the promoter region.
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Even patients with lymphoproliferative diseases may develop a persistent chronic pain not responsive to usual treatments due to changes in antibody production and to some treatments like radiotherapy, chemotherapy, and the administration of monoclonal antibodies, which further impair the immune defense and induce chronic inflammatory phenomena acting as a substrate for a persistent chronic pain. Five patients with indolent lymphoproliferative diseases were treated for severe pain nonresponsive to other pain reliever treatments with SCS applied with an All-in-One Shot (OS) procedure. For all patients, the estimated survival time was of 5 years or more. All patients showed a significant reduction of the intensity of pain: the mean Numerical Rating Scale was 7.4 before treatment and 2.2 after. No patient developed adverse events. Supported by the data of this study, we believe that the habit to deprive patients with an indolent form of lymphoproliferative diseases of the possibility to reduce the intensity of chronic pain by SCS treatment is extremely reductive and frustrating.
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Primary cutaneous anaplastic large cell lymphoma (pcALCL) is part of a spectrum of cutaneous CD30+ lymphoproliferative disease that also includes lymphomatoid papulosis. It often occurs in elderly patients, presenting at a median age of 60 years, although it may occur at any age. It is a CD30+ T-cell neoplasm composed of large cells with anaplastic, pleomorphic, or immunoblastic morphology, with exclusively cutaneous onset and localization. The clinical course of pcALCL is predominantly indolent. Most elderly patients with lymphoma tend to have a sedentary lifestyle, which has a negative effect on their quality of life (QoL) and survival. Several studies indicate that exercise has a positive impact on QoL because it reduces peak oxygen consumption, improves physical capacity, increases self-esteem, reduces accumulated stress, and promotes relaxation. Therefore, particularly in indolent lymphomas, it is necessary to indicate a program of physical activity to be practiced systematically. Complete surgical excision and local radiotherapy are the first line gold standard in pcALCL with a solitary lesion.
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Exercício Físico , Linfoma Anaplásico Cutâneo Primário de Células Grandes/terapia , Papulose Linfomatoide/terapia , Neoplasias Cutâneas/terapia , Idoso , Humanos , Antígeno Ki-1 , Estilo de Vida , Pessoa de Meia-Idade , Qualidade de Vida , Comportamento SedentárioRESUMO
Lymphomatoid papulosis (LyP) is a non-aggressive skin disorder characterized by papulonodular injuries, sometimes necrotic, often scattered, relapsing, which frequently regress spontaneously. LyP represents about 12% of cutaneous lymphomas. The etiology of LyP is unknown. Based on its histopathology, in 2018, the World Health Organization (WHO) classified LyP into six types with similar prognosis (A,B,C,D,E and DUSP22). Once the diagnosis of LyP has been made, having an excellent prognosis, this pathology must be managed mainly with a "watch and wait" strategy. Treatment should be given only in the presence of diffuse, symptomatic lesions with disfiguring evolution, with the aim of reducing time of resolution and preventing recurrences or the formation of new lesions.
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Papulose Linfomatoide/patologia , Papulose Linfomatoide/terapia , Neoplasias Cutâneas/patologia , Neoplasias Cutâneas/terapia , Dermoscopia , Humanos , Papulose Linfomatoide/classificação , Papulose Linfomatoide/imunologia , Prognóstico , Neoplasias Cutâneas/classificação , Neoplasias Cutâneas/imunologia , Linfócitos TRESUMO
Ruxolitinib, an orally bioavailable and selective inhibitor of Janus kinase 1 (JAK1) and JAK2, significantly reduces splenomegaly and disease-related symptoms in patients with myelofibrosis (MF). However, no clear survival benefit has been demonstrated, which may in part reflect suboptimal drug exposure related to lower dosages needed to minimize hematological toxicity, specifically cytopenias. Furthermore, the optimal management of specific conditions such as leukocytosis or thrombocytosis in patients under ruxolitinib therapy is still undefined. In these cases, combining ruxolitinib with a cytoreductive agent like hydroxyurea might improve hematological response. This observational multi-center study enrolled 20 adult patients with intermediate- or high-risk primary MF, post- polycythemia vera MF, or postessential thrombocythemia MF with hyperproliferative manifestations of the disease and WBC and/or platelet counts not controlled by ruxolitinib therapy. The patients received treatment with a combination of ruxolitinib and hydroxyurea. A clinical response of any type was obtained in 8 patients (40%) during ruxolitinib monotherapy and in 17 patients (85%) during ruxolitinib-hydroxyurea combination (P = 0.003). After a median duration of 12.4 months of combination therapy, 16/20 patients had a hematological response; 14/17 patients who had started combination therapy to control WBC count and 2/3 who started in order to reduce platelets count. The number of patients requiring ruxolitinib dosage reduction or discontinuations was lower during combination therapy and, at the end of follow-up the median ruxolitinib dose was increased in 50% of patients. In conclusion, the combination of hydroxyurea with ruxolitinib yielded a high clinical response rate and increased ruxolitinib exposure in patients with hyperproliferative forms of MF.
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Hidroxiureia/uso terapêutico , Mielofibrose Primária/diagnóstico , Mielofibrose Primária/tratamento farmacológico , Pirazóis/uso terapêutico , Adulto , Idoso , Idoso de 80 Anos ou mais , Antineoplásicos/administração & dosagem , Antineoplásicos/efeitos adversos , Antineoplásicos/uso terapêutico , Biomarcadores , Quimioterapia Combinada , Feminino , Humanos , Hidroxiureia/administração & dosagem , Hidroxiureia/efeitos adversos , Inibidores de Janus Quinases/administração & dosagem , Inibidores de Janus Quinases/efeitos adversos , Inibidores de Janus Quinases/uso terapêutico , Masculino , Pessoa de Meia-Idade , Nitrilas , Mielofibrose Primária/metabolismo , Pirazóis/administração & dosagem , Pirazóis/efeitos adversos , Pirimidinas , Estudos RetrospectivosRESUMO
Administration of rituximab, one of the basic drugs for the therapy of B-cell lymphoproliferative diseases, during pregnancy has been suspected to cause developmental fetal events, particularly if given during the first trimester of pregnancy. Therefore, use in pregnancy is not permitted. Howe ver, several cases of pregnant women being treated with rituximab are reported herein; an exception is often made in cases with grave illness. We describe an exceptional case of a woman with non-Hodgkin lymphoma of the mucosa-associated lymphoid tissue type where rituximab was given as a single agent without interruption during two consecutive pregnancies. This case can certainly supply important indications on the safety of rituximab.
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The prevalence of chronic pain is between 33% to 64% and is due to cancer pain, but it has also been observed in non-cancer patients. Chronic pain is associated with lower quality of life and higher psychological distress and depressive/anxiety disorders in patients without a history of disorder. In this study we evaluated in clinical practice the effectiveness of the intrathecal pump in 140 patients who underwent pain therapy at our Center. These patients were consecutively enrolled from January 2010 to July 2018. Follow-up was carried out over these eight years regarding the infusion modalities. Pain relief was obtained in 71 (50,7%) patients out of the 140 that experienced satisfactory pain control globally. Intrathecal therapy is one of the best options for chronic severe refractory pain. The greatest advantage of this therapy is due to the possibility of treating the pain with minimal dosages of the drug, avoiding the appearance of troublesome side effects.
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Retroperitoneal fibrosis is a connective disease of the auto-inflammatory/auto-immune type of the retroperitoneum with unknown etiology and pathological mechanism. The manifestations of the pathology can be local or systemic. Amongst the local symptoms, the dull and constant pain in the hips, back or abdomen is the most frequent. We report here a case of a 47-year-old woman, whose pathogenic mechanism could be related to an "IgG4-related disease" disorder as suggested by an increased serum level of this subclass of IgG and the positive immunohistochemistry. The diagnosis is not easy, because this pathology generates masses; adenomegalies with retro peritoneal development, that makes it similar to lymphomas or metastases from ovarian tumors.
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Sunitinib is the most commonly prescribed drug for advanced renal cell carcinoma in the first-line setting and has been associated with multiple adverse events related to its on-and off-target effects, including hand and foot syndrome and fatigue. It was hypothesized that sunitinib-induced fatigue may be related to off target inhibition of the AMPK enzyme, which results in impairment of energy-producing processes at a systemic level. Quercetin is a naturally occurring flavonol with established AMPK-stimulating activity. While clinical use of quercetin is limited by its poor bio-availability, quercetin-3-O-ß-d-glucopyranoside, that is isoquercetin, has an improved pharmacokinetic profile. On the grounds of the in vitro stimulatory activity with respect to AMPk, we hypothesized that oral isoquercetin could improve fatigue in kidney cancer patients receiving sunitinib. Given the lack of data on the safety of isoquercetin given concomitantly with sunitinib, we conducted a phase I trial to assess the safety of GMP manufactured isoquercetin given at two dose levels (450 and 900 mg a day). In the 12-patient study cohort included in this study, isoquercetin was administered concomitantly with 50 mg sunitinib for a median 81 days (IQR, 75.5, 86.5). None of the 12 patients required isoquercetin suspension or isoquercetin dose reduction because of adverse events. No abnormalities in ECG, heart or lower limbs doppler ultrasound were detected. A statistically significant improvement was reported for the FACIT fatigue score (6.8 points; 95% CI: 2.8-10.8; p = 0.002) and for the FACIT Adverse Events score (18.9 points; 95% CI: 9.1-28.8; p < 0.001) after isoquercetin consumption vs. baseline. In this phase I trial, isoquercetin was remarkably safe, with a preliminary signal of activity in terms of improvement of sunitinib adverse events.
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Primary cardiac tumors do not occur frequently, and only one quarter of them, chiefly sarcomas, are malignant. Patients with angiosarcoma typically have a shorter survival time than do patients with other sarcomas, and the prognosis for survival depends strictly on the stage of the disease at the time of diagnosis and the possibility of complete surgical excision. Chemotherapy and radiotherapy have well-established postoperative roles because of the high probability of metastasis. We report the case of a 25-year-old man who presented with pericardial effusion and echocardiographic evidence of an intracavitary right atrial mass but without the bulky, infiltrative growth typical of this location of the disease. Malignancy was suggested by the clinical presentation, the location of the mass in the right side of the heart, and the absence of conditions favoring thrombus formation. After complete surgical excision, the mass was confirmed to be an angiosarcoma. Conventional adjuvant chemotherapy and maintenance therapy with inhibitors of CD117 (c-kit) and vascular endothelial growth factor relieved the patient's clinical symptoms and enabled his long-term, disease-free survival. In addition to reporting this case, we discuss aspects of the diagnosis and treatment of angiosarcoma.