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Patients with hematological malignancies (HMs) are at a higher risk of developing severe form and protracted course of COVID-19 disease. We investigated whether the combination of viral replication inhibition with remdesivir and administration of anti-SARS-CoV-2 immunoglobulins with convalescent plasma (CP) therapy might be sufficient to treat B-cell-depleted patients with COVID-19. We enrolled 20 consecutive patients with various HMs with profound B-cell lymphopenia and COVID-19 pneumonia between December 2020 and May 2021. All patients demonstrated undetectable baseline anti-SARS-CoV-2 immunoglobulin levels before CP. Each patient received at least a complete course of remdesivir and at least one unit of CP. Previous anti-CD20 therapy resulted in a more prolonged SARS-CoV-2 PCR positivity compared to other causes of B-cell lymphopenia (p = 0.004). Timing of CP therapy showed a significant impact on the clinical outcome. Simultaneous use of remdesivir and CP reduced time period for oxygen weaning after diagnosis (p = 0.017), length of hospital stay (p = 0.007), and PCR positivity (p = 0.012) compared to patients who received remdesivir and CP consecutively. In addition, time from the diagnosis to CP therapy affected the length of oxygen dependency (p < 0.001) and hospital stay (p < 0.0001). In those cases where there were at least 10 days from the diagnosis to plasma administration, oxygen dependency was prolonged vs. patients with shorter interval (p = 0.006). In conclusion, the combination of inhibition of viral replication with passive immunization was proved to be efficient and safe. Our results suggest the clear benefit of early, combined administration of remdesivir and CP to avoid protracted COVID-19 disease among patients with HMs and B-cell lymphopenia.
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Tratamiento Farmacológico de COVID-19 , COVID-19 , Neoplasias Hematológicas , Linfopenia , Adenosina Monofosfato/análogos & derivados , Alanina/análogos & derivados , COVID-19/terapia , Neoplasias Hematológicas/complicaciones , Neoplasias Hematológicas/terapia , Humanos , Inmunización Pasiva/métodos , Linfopenia/etiología , Linfopenia/terapia , Oxígeno , SARS-CoV-2 , Sueroterapia para COVID-19RESUMEN
INTRODUCTION: Nowadays, more than 80% of newly diagnosed classical Hodgkin lymphoma (HL) patients can be cured and become long-term survivors due to risk and response-adapted treatment strategies. A well-known side effect is cognitive dysfunction that appears in HL patients after chemotherapy. In the present study, we aimed to measure cognitive dysfunction in our HL patients in this study and to find potential correlations between patient-related factors, the signs and symptoms of their diseases, or therapeutic factors. METHODS: We carried out a computer-assisted assessment (CANTAB) of cognitive dysfunction in 118 patients. We examined the domains of visual memory, attention, working memory, and planning. RESULTS: The median age of 64 females and 54 males at diagnosis was 29 (13-74) and 41 (21-81) years at the completion of CANTAB. Fifty-two percent of all patients showed cognitive impairment. Attention was impaired in 35% of patients, the working memory and planning were impaired in 25%, while visual memory was affected in 22%. All the three functions showed a significant association with inactive employments status. A close correlation was found between visual memory/working memory and planning, higher age at HL diagnosis or the completion of CANTAB test, and disability pensioner status. DISCUSSION: Our investigation suggests that patients with inactive employment status and older age require enhanced attention. Their cognitive function and quality of life can be improved if they return to work or, if it is not possible, they receive a cognitive training.
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Disfunción Cognitiva , Enfermedad de Hodgkin , Cognición , Disfunción Cognitiva/diagnóstico , Disfunción Cognitiva/epidemiología , Disfunción Cognitiva/etiología , Femenino , Enfermedad de Hodgkin/tratamiento farmacológico , Enfermedad de Hodgkin/psicología , Humanos , Hungría , Masculino , Memoria a Corto Plazo , Pruebas Neuropsicológicas , Calidad de Vida , Sobrevivientes/psicologíaAsunto(s)
Tratamiento Farmacológico de COVID-19 , COVID-19 , Dexametasona , Neoplasias Hematológicas , Sistema de Registros , SARS-CoV-2 , Humanos , Dexametasona/uso terapéutico , Neoplasias Hematológicas/mortalidad , Neoplasias Hematológicas/tratamiento farmacológico , COVID-19/mortalidad , COVID-19/complicaciones , Masculino , Femenino , Anciano , Persona de Mediana Edad , SARS-CoV-2/aislamiento & purificación , Adulto , Anciano de 80 o más AñosRESUMEN
Moderate thrombocytosis can accompany several diseases (bleeding, inflammation, iron deficiency, or autoimmune diseases), but hematologic examination is strongly recommended in a patient with persistent platelet count above 450 G/L unless reactive origin can be confirmed. The 47-year-old woman's medical history included hypertonia, asthma bronchiale, and endometriosis. In March 2015, she underwent laboratory examination due to weight loss and lack of appetite. Her results showed elevated thrombocyte count (617 G/L), but no iron deficiency. She presented in our clinic on 07. 04. 2015 with acute pain below her left hypochondrial region, but simple imaging examinations showed no difference to explain it. Abdominal CT revealed a 4.5 cm thrombus which protruded into the left renal artery, blocking it. We started APTI- (activated partial thromboplastin time) monitored continuous intravenous treatment with unfractionated heparin. The JAK2V617F mutation analysis came back positive. Subsequent bone marrow examination revealed prefibrotic/early stage myelofibrosis, prompting treatment with hydroxyurea. The applied treatments led to the disappearance of the patient's symptoms accompanied by the gradual normalisation of the thrombocyte count. Moderate thrombocytosis is often secondary, but if it persists and is accompanied by mainly thromboembolic events, the risk of diseases of the haematopoietic system, primarily Philadelphia chromosome negative chronic myeloproliferative disease should also be considered. Clinically, essential thrombocythaemia and the prefibrotic/early stage of myelofibrosis can be very similar. Differential diagnosis is only possible through the histological examination of the bone marrow, which becomes indispensible due to the difference in prognosis and treatment options. Orv Hetil. 2018; 159(15): 603-609.
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Trastornos Mieloproliferativos/diagnóstico , Mielofibrosis Primaria/diagnóstico , Trombocitopenia/diagnóstico , Médula Ósea/patología , Femenino , Humanos , Persona de Mediana Edad , Trastornos Mieloproliferativos/complicaciones , Trastornos Mieloproliferativos/terapia , Inhibidores de Agregación Plaquetaria/uso terapéutico , Mielofibrosis Primaria/complicaciones , Mielofibrosis Primaria/terapia , Trombocitopenia/etiología , Trombocitopenia/terapiaRESUMEN
BACKGROUND: Due to risk and response adapted treatment strategies, more than 80% of newly diagnosed classical Hodgkin lymphoma (HL) patients can be cured, and become long-term survivors. However, a high proportion of survivors suffer from treatment-related long-term side effects such as secondary malignancy, organ failure, persistent fatigue and psychological distress. The aim of this study was to evaluate psychological distress and its risk factors among our HL survivors. METHODS: One hundred sixty-three (50% female) adult HL survivors were contacted between January 1, 2012 and march 31, 2015 in our outpatient centre. The patients were asked to complete a standardized, validated, self-administered Hungarian questionnaire with demographic questions and the following scales: Hospital anxiety and depression scale (HADS14), general health questionnaire (GHQ12), sense of coherence (SOC13) perceived stress scale (PSS4), dysfunctional attitude scale (DAS17). Disease and treatment data were acquired from hospital records. RESULTS: Majority of HL survivors are in early adulthood, our most important goal should be to return them to normal life after their lymphoma is cured. The employment status at the time of survey seemed to be crucial so patients were divided into either active (n = 93) or inactive (n = 47) group. Retired survivors (n = 19) were excluded from the subgroup analysis. Psychological distress was significantly lower in active patients. Multiple logistic regression analysis showed significant differences between the inactive and active subgroups, such as age at diagnosis (≥30 years or below, p = 0.001), education level (below college vs. college, p = 0.032) and treatment related long-term side effects (yes vs. no, p < 0.001). Predictors for treatment-related long-term side effects are female gender (p = 0.011), chemotherapy protocol (ABVD vs. other, p < 0.001). CONCLUSIONS: Our data suggest that employment status and treatment-related long-term side effects play a critical role in the health related quality of life outcome among Hungarian HL survivors.
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Empleo/psicología , Enfermedad de Hodgkin/psicología , Calidad de Vida , Estrés Psicológico , Sobrevivientes/psicología , Adulto , Anciano , Empleo/estadística & datos numéricos , Femenino , Humanos , Hungría , Masculino , Persona de Mediana Edad , Estrés Psicológico/psicología , Encuestas y Cuestionarios , Adulto JovenRESUMEN
Approximately 10-30% of Hodgkin lymphoma patients relapses or experience refractory disease after first line treatment. Nowadays, autologous stem cell transplantation can successfully salvage half of these patients, median overall survival is only 2-2.5 years. Several prognostic factors determine success of autologous stem cell transplantation. Result of transplantation can be improved considering these factors and using consolidation treatment, if necessary. Patients who relapse after autologous transplantation had worse prognosis, treatment of this patient population is unmet clinical need. Several new treatment options became available in the recent years (brentuximab vedotin and immuncheckpoint inhibitors). These new treatment options offer more chance for cure in relapsed/refractory Hodgkin patients. Outcome of allogenic stem cell transplantation can be improved by using haploidentical donors. New therapeutic options will be discussed in this review. Orv Hetil. 2017; 158(34): 1338-1345.
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Antineoplásicos/uso terapéutico , Trasplante de Células Madre Hematopoyéticas , Enfermedad de Hodgkin/terapia , Inmunoconjugados/uso terapéutico , Brentuximab Vedotina , Quimioterapia de Consolidación/métodos , Enfermedad de Hodgkin/tratamiento farmacológico , Enfermedad de Hodgkin/inmunología , Humanos , Inducción de Remisión , Terapia Recuperativa/métodos , Análisis de Supervivencia , Trasplante AutólogoRESUMEN
Objective Non-Hodgkin lymphoma (NHL) arising as a secondary malignancy in patients treated for classical Hodgkin lymphoma (cHL) is an infrequent and challenging clinical scenario. NHL can be presented synchronously with cHL or may develop later, sequentially, up to years after treatment for cHL. The relationship between the two lymphomas is unclear, and there are no clear guidelines for the management of these patients. We would like to find a better clinical understanding of this issue so this study investigates the occurrence and clinical characteristics of secondary NHL. Materials and methods In this retrospective cohort examination, we collected cHL cases when NHL occurred during or after the course of treating cHL. We performed the histopathologic revisions of the samples, and in every case where the quality of the sample was lower, we performed molecular examinations to find the association between cHL and NHL. We performed next-generation genome sequencing (NGS) and immunoglobulin heavy-chain variable region gene (IgHV) clonality testing. Results In a cohort of 164 cHL patients diagnosed between 2011 and 2020, six patients were identified with NHL during rebiopsy prompted by lymphoma relapse or progression. Among these, five patients were diagnosed with post-germinal center-originated diffuse large B-cell lymphoma (post-GC DLBCL), and one patient presented high-grade B-cell lymphoma (HG-BCL). The NHL manifestation differed in its timing: three cases emerged after successful cHL treatment, with at least 18 months of complete remission, while the other three patients faced primary refractory cHL. Notably, the primary refractory cases did not exhibit a confirmed clonal relationship between cHL and NHL, but NGS data raised the possibility of synchronous NHL in one case. In contrast, among the patients with sequentially occurring NHL, polymerase chain reaction (PCR) testing of the IgHV gene affirmed a clonal connection between cHL and secondary DLBCL in one case, while the high morphological similarity suggested a potential clonality between the two lymphomas in another case. Conclusion This study reveals that secondary NHL may manifest both synchronously and sequentially following cHL. Our results suggest that synchronous NHL has a worse prognosis compared to sequential cases when the different lymphomas are not recognized at the time of diagnosis. As our data showed, in some cases, mutations that accompany the tumor cells throughout their clonal evolution can be identified, with additional mutations later on. In the future, next-generation sequencing (NGS)-based processing of liquid biopsy samples can overcome the limitations resulting from the spatial heterogeneity of lymphoid malignancies. Over the long term, this identification could lead to early patient selection and alternative treatment strategies, ultimately leading to improved prospects for cure.
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Introduction: Erdheim-Chester disease (ECD) is a rare disease that belongs to the group of Dendritic and histiocytic neoplasms. Only 2000 cases have been reported worldwide. It can present with a wide range of symptoms, making a differential diagnosis especially difficult. The primary and most important diagnostic tool is a biopsy of the affected organ/tissue. Nowadays the analysis of different mutations affecting the BRAF and MAPK pathways makes it possible to use targeted treatments, such as vemurafenib, dabrafenib, or cobimetinib. Objective: Our aim is to present the results of three male patients treated in our hematology department. Results: Our BRAF mutation-positive patient presented with retroperitoneal tissue proliferation and diabetes insipidus. The initial therapy of choice was dabrafenib. After 3 months of treatment, 18F-fluoro-deoxyglucose positron emission tomography (FDG-PET)/computed tomography (CT) scans showed regression, and after 2 years of treatment, no disease activity was detected. In our second patient, a recurrent febrile state (not explained by other reasons) and diabetes insipidus suggested the diagnosis. A femoral bone biopsy confirmed BRAF-negative ECD. The first-line therapy was interferon-alpha. After 3 months of treatment, no response was observed on 18FDG-PET/CT, and treatment with cobimetinib was started. The control 18FDG-PET/CT imaging was negative. Our third patient was evaluated for dyspnea, and a CT scan showed fibrosis with hilar lymphadenomegaly. A lung biopsy confirmed BRAF-negative ECD. We started treatment with interferon-alpha, but unfortunately, no improvement was observed. Second-line treatment with cobimetinib resulted in a partial metabolic response (PMR) according to control 18FDG-PET/CT. Conclusions: Our results demonstrate that an appropriately chosen treatment can lead to a good therapeutic response, but dose reduction may be necessary due to side effects. With advanced targeted therapeutic treatment options, survival and quality of life are significantly improved.
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Treating relapsed and refractory diffuse large B-cell lymphoma is still challenging for clinicians, but the available CAR-T and bispecific antibodies have revolutionized therapy. Autologous stem cell transplantation was the most effective treatment modality previously. The authors reported data from a single center over ten years. The retrospective study included 116 patients, with 53 relapsed cases, 39 primary refractory cases, 19 who had CNS involvement, and 5 who had received primary consolidation transplants. The median duration of follow-up was 46 months. The median event-free survival was 75 months, and the median overall survival was 105 months for all cases. Five-year overall survival was 59%, and event-free survival was 54%. Pretreatment prognostic factors at diagnosis had no effect on the outcome of transplantation. The authors found no difference between survival in relapsed or refractory cases, and the number of salvage lines or the germinal center/activated B-cell type also did not influence the results. Complete metabolic response before transplantation confirmed by 18FDG PET/CT strongly affected survival. The pre-transplant creatinine and CRP levels significantly influenced the long-term outcome. The number of stem cells infused did not affect survival, but engraftment within nine days did result in a longer survival. These data support the finding that the response to salvage therapy did facilitate the identification of a better prognostic group who may still benefit from autologous transplantation.
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Transfusion medicine is traditionally a strong/fundamental part of clinical practice, saving hundreds of millions of lives. However, blood-borne or transmitted infections are a well-known and feared possibility, a risk we relentlessly mitigate. Pathogens are continuously and rather quickly changing, so during the last decade, many, sometimes exotic, new pathogens and diseases were recorded and analyzed, and some of them were proved to be transmitted with transfusions. Blood or blood component transfusions are carried out after cautious preparative screening and inactivation maneuvers, but in some instances, newly recognized agents might escape from standard screening and inactivation procedures. Here, we try to focus on some of these proven or potentially pathogenic transfusion-transmitted agents, especially in immunocompromised patients or bone marrow transplantation settings. These pathogens are sometimes new challenges for preparative procedures, and there is a need for more recent, occasionally advanced, screening and inactivation methods to recognize and eliminate the threat a new or well-known pathogen can pose. Pathogen transmission is probably even more critical in hemophiliacs or bone marrow transplant recipients, who receive plasma-derived factor preparations or blood component transfusions regularly and in large quantities, sometimes in severely immunosuppressed conditions. Moreover, it may not be emphasized enough that transfusions and plasma-derived product administrations are essential to medical care. Therefore, blood-borne transmission needs continued alertness and efforts to attain optimal benefits with minimized hazards.
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Only few studies have analyzed the efficacy of tixagevimab/cilgavimab to prevent severe Coronavirus disease 2019 (COVID-19) and related complications in hematologic malignancies (HM) patients. Here, we report cases of breakthrough COVID-19 after prophylactic tixagevimab/cilgavimab from the EPICOVIDEHA registry). We identified 47 patients that had received prophylaxis with tixagevimab/cilgavimab in the EPICOVIDEHA registry. Lymphoproliferative disorders (44/47, 93.6%) were the main underlying HM. SARS-CoV-2 strains were genotyped in 7 (14.9%) cases only, and all belonged to the omicron variant. Forty (85.1%) patients had received vaccinations prior to tixagevimab/cilgavimab, the majority of them with at least two doses. Eleven (23.4%) patients had a mild SARS-CoV-2 infection, 21 (44.7%) a moderate infection, while 8 (17.0%) had severe infection and 2 (4.3%) critical. Thirty-six (76.6%) patients were treated, either with monoclonal antibodies, antivirals, corticosteroids, or with combination schemes. Overall, 10 (21.3%) were admitted to a hospital. Among these, two (4.3%) were transferred to intensive care unit and one (2.1%) of them died. Our data seem to show that the use of tixagevimab/cilgavimab may lead to a COVID-19 severity reduction in HM patients; however, further studies should incorporate further HM patients to confirm the best drug administration strategies in immunocompromised patients.
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COVID-19 , Neoplasias Hematológicas , Humanos , COVID-19/prevención & control , SARS-CoV-2 , Neoplasias Hematológicas/complicaciones , Neoplasias Hematológicas/tratamiento farmacológico , Anticuerpos Monoclonales , Inmunización Pasiva , Sistema de RegistrosRESUMEN
OBJECTIVES: Treatment options for relapsing Hodgkin lymphoma (HL) are controversial after autologous hemopoietic stem cell transplantation (HSCT). Nevertheless, allogeneic HSCT may be curative if it is performed in complete remission. CASE REPORT: In 2007, a 22-year-old female patient was diagnosed with nodular sclerosis subtype of classical HL. Her clinical stage was IIAX with unfavorable prognosis. Eight courses of doxorubicin, bleomycin, vinblastine and dacarbazine chemotherapy and involved field irradiation were applied, but after 3 months of complete remission, disseminated relapse was recognised by (18)FDG-PET/CT. After two cycles of salvage dexamethasone, cisplatinum, and cytosine arabinoside therapy, further progression was noticed, so the treatment was modified to ifosfamide, gemcitabine, vinorelbine, and prednisone (IGEV) regimen. After two cycles of IGEV regimen, she achieved a complete metabolic remission, which was confirmed by a (18)FDG-PET/CT scan again. She was referred for autologous-HSCT, and a successful stem cell collection was performed in August 2008. However, a rapid progression was detected again, so total body irradiation was applied before the conditioning therapy with R-mini-BEAM regimen. The (18)FDG-PET/CT scan performed 100 days after the autologous-HSCT was still positive. In December 2009, multiple nodal and extranodal progression was detected, so ifosfamide, carboplatine, etoposide, mesna protection rescue treatment was started, but it was ineffective. Based on sporadic data of the literature, rituximab-bendamustine therapy was started in March 2010. After four cycles, she achieved complete metabolic remission, which was verified by (18)FDG-PET/CT. The patient has been referred for an allogeneic HSCT with reduced intensity conditioning. CONCLUSIONS: Based on our experience, bendamustine-rituximab salvage therapy can be a suitable option for the treatment of post-transplant progression or relapse of HL.
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Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Trasplante de Células Madre Hematopoyéticas , Enfermedad de Hodgkin/terapia , Adulto , Anticuerpos Monoclonales de Origen Murino/administración & dosificación , Clorhidrato de Bendamustina , Femenino , Humanos , Compuestos de Mostaza Nitrogenada/administración & dosificación , Recurrencia , Rituximab , Terapia Recuperativa , Trasplante AutólogoRESUMEN
INTRODUCTION: Rituximab treatment may induce a long-term B-cell depletion, which can be accompanied with an increased infection risk. AIMS: To examine the changes of the white blood cell, CD19+ B-cell and CD4+ T-cell counts and the levels of immunoglobulin G, A, M after rituximab containing chemotherapy and to explore the infectious complications in our patients and review of the literature. PATIENTS AND METHODS: Thirty-five diffuse large B-cell lymphoma patients were examined, who were treated with rituximab-cyclophosphamide-vincristine-doxoribicine-prednisolone (R-CHOP). The B- and T-cell populations were analyzed with flow-cytometry while the immunoglobulin levels were measured by nephelometry. RESULTS: CD19+ B-lymphocytes were undetectable after the treatment and their count only increased from the post-therapeutic 12th month. Infection did not occur in this group of patients. CONCLUSIONS: Rituximab induced B-cell depletion was appreciable also in this group of patients, while serious or unexpected infection did not occur. Increased infectious risk primarily can be observed after long-term, maintenance rituximab treatment.
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Anticuerpos Monoclonales de Origen Murino/efectos adversos , Anticuerpos Monoclonales de Origen Murino/inmunología , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Linfoma de Células B Grandes Difuso/tratamiento farmacológico , Linfoma de Células B Grandes Difuso/inmunología , Adulto , Anciano , Anticuerpos Monoclonales de Origen Murino/administración & dosificación , Protocolos de Quimioterapia Combinada Antineoplásica/administración & dosificación , Protocolos de Quimioterapia Combinada Antineoplásica/efectos adversos , Linfocitos B/efectos de los fármacos , Linfocitos B/inmunología , Ciclofosfamida/administración & dosificación , Supervivencia sin Enfermedad , Doxorrubicina/administración & dosificación , Femenino , Citometría de Flujo , Hepatitis B/diagnóstico , Humanos , Inmunoglobulinas/sangre , Incidencia , Infecciones/diagnóstico , Infecciones/etiología , Leucoencefalopatía Multifocal Progresiva/diagnóstico , Leucoencefalopatía Multifocal Progresiva/etiología , Recuento de Linfocitos , Masculino , Persona de Mediana Edad , Nefelometría y Turbidimetría , Neutropenia/inducido químicamente , Neutropenia/complicaciones , Prednisona/administración & dosificación , Recurrencia , Estudios Retrospectivos , Medición de Riesgo , Rituximab , Linfocitos T/efectos de los fármacos , Linfocitos T/inmunología , Factores de Tiempo , Resultado del Tratamiento , Vincristina/administración & dosificaciónRESUMEN
BACKGROUND: Acquired von Willebrand syndrome (AVWS) is a rare, frequently underdiagnosed and underestimated bleeding disorder. Careful personal and family history and late-onset mucocutaneous bleeding could help clarify the etiology of bleeding deficiency. CASE PRESENTATION: An 82-year-old male patient was admitted to our clinic with a severe nosebleed on 30.05.2018. Laboratory results revealed thrombocytosis, elevated white blood cell count and high LDH. Basic coagulation parameters were normal. He was referred to our clinic, where a bone marrow biopsy was taken. His personal and family history had no mention of bleeding disorders, nor was he on anticoagulant therapy. We detected elevated VWF antigen and decreased VWF ristocetin cofactor activity. Loss of high molecular weight multimers was detected by using agarose gel electrophoresis. These laboratory results were indicative of AVWS. Hydroxyurea treatment was initiated, leading to a gradual decrease in platelet count. The histological examination revealed essential thrombocytosist while mutation analysis was JAK2/CALR/MPL negative. However, due to severe nosebleeds, the patient was hospitalized and needed blood transfusion. A cardiological check-up revealed the progression of aortic valve stenosis. After, balloon-dilation a transcatheter aortic valve implantation was performed. As a result, VWF activity and activity to antigen ratio returned to normal as did multimeric structure. In July 2019, the follow-up examination showed that the patient was in a satisfactory condition, with normal hematological parameters, and no new nosebleed episode occurred. CONCLUSIONS: The patient complained of recurring nosebleeds, which stopped completely after the resolution of both underlying conditions successful cytoreductive treatment of triple-negative ET and transcatheteric aortic valve replacement.
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Enfermedades de von Willebrand/diagnóstico , Enfermedades de von Willebrand/terapia , Factor de von Willebrand/análisis , Anciano de 80 o más Años , Transfusión Sanguínea , Inhibidores Enzimáticos/uso terapéutico , Hemorragia/sangre , Hemorragia/complicaciones , Hemorragia/terapia , Humanos , Hidroxiurea/uso terapéutico , Masculino , Trombocitosis/sangre , Trombocitosis/complicaciones , Trombocitosis/terapia , Enfermedades de von Willebrand/sangre , Enfermedades de von Willebrand/complicacionesRESUMEN
AIMS AND BACKGROUND: Quality of life and survival of patients with malignant diseases are improving thanks to the development in diagnostics and therapy. METHODS: We determined the quality of life and frequency and severity of fatigue with an EORTC QLQ-C30 questionnaire in 168 Hodgkin's lymphoma patients (85 women, 83 men). We scored all functional and symptom scales in cured patients (who were in complete remission for at least 10 years; mean period of survival after the treatment(s) was 16.61 years) and in those who suffered from late complications. RESULTS: The global health status score (QL2) was significantly lower in patients who had late complications (mean QL2, 45.53) than in patients with no complications (mean QL2, 67.57, P < 0.001) and in cured patients (mean QL2, 52.5) than in those who were not disease free 10 years after the treatment or who were treated actively (mean QL2, 67.48, P < 0.001). We found that fatigue level (FA) was significantly higher in patients who had been treated more than 20 years before (FA score, 53.37) than in those who were undergoing treatment (FA score, 29.35, P = 0.03). A significantly higher FA score (FA, 48.72) was observed in patients who suffered from late complications of the treatment than in those who had no complications (FA, 31.88; P = 0.001). More comorbidity can cause higher fatigue scores than observed in these groups of Hodgkin's lymphoma patients. CONCLUSIONS: Fatigue is more frequent than we think, and it has a strong effect on quality of life, so its early recognition and treatment is important and needs multidisciplinary cooperation.
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Fatiga/etiología , Enfermedad de Hodgkin/complicaciones , Calidad de Vida , Adulto , Anciano , Femenino , Humanos , Incidencia , Masculino , Persona de Mediana Edad , Índice de Severidad de la Enfermedad , Encuestas y Cuestionarios , Análisis de Supervivencia , Adulto JovenRESUMEN
Treating patients with DLBCL remains a challenge, as the response to first-line immunochemotherapy is somewhat unpredictable. The International Prognostic Index (IPI) is one of the most widely used methods for assessing prognosis. Interim PET/CT (iPET/CT) can play an important role in the early identification of 'non-responder' patients before the end of treatment examination. In this study, we retrospectively analyzed 104 newly diagnosed DLBCL patients treated with R-CHOP-like regimens who underwent iPET/CT imaging during therapy. There was a significant difference in 2-year OS between patients with negative iPET/CT and those with positive iPET/CT. Patients who had positive iPET/CT showed inferior 2-year PFS compared to those with negative iPET/CT. According to IPI, there was a statistically significant difference in 2-year OS and PFS between patients in the lower and higher risk groups. However, these patients can be further subdivided according to iPET/CT. The iPET/CT results in the present study clearly separate good- and poor-prognosis patients according to differences in 2-year OS, both in the lower and higher IPI risk groups. These results are in agreement with those of previous studies that demonstrated that iPET/CT has high negative predictive value, clearly identifying good-prognosis patients even within the poor-prognosis IPI group.
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Protocolos de Quimioterapia Combinada Antineoplásica/administración & dosificación , Linfoma de Células B Grandes Difuso/diagnóstico por imagen , Linfoma de Células B Grandes Difuso/tratamiento farmacológico , Linfoma de Células B Grandes Difuso/mortalidad , Tomografía Computarizada por Tomografía de Emisión de Positrones , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Ciclofosfamida/administración & dosificación , Supervivencia sin Enfermedad , Doxorrubicina/administración & dosificación , Femenino , Estudios de Seguimiento , Humanos , Masculino , Persona de Mediana Edad , Prednisona/administración & dosificación , Estudios Retrospectivos , Rituximab/administración & dosificación , Tasa de Supervivencia , Vincristina/administración & dosificaciónRESUMEN
OBJECTIVE AND IMPORTANCE: Hodgkin's lymphoma (HL) is a well-curable disease. The treatment usually includes combined multiagent conventional chemotherapy and radiotherapy. One-fifth of the patients need repeated treatments because of relapse or primary progressive disease. Those HL patients, who cannot be cured at least with salvage therapy (including autologous haemopoietic stem cell transplantation (auto-HSCT)), have really unfavourable prognosis. INTERVENTION: For this heavily pretreated subset of HL patients, novel but less toxic treatment strategies should be considered. Brentuximab-vedotin (BV) is a novel targeted treatment option, which was administered after the failure of two different regimens in patients, who were ineligible for auto-HSCT or after the failure of auto-HSCT. Moreover, there are favourable data with chemotherapeutical regimens supplemented with rituximab not only in relapsed but also in newly diagnosed classical HL patients. Bendamustine, an almost forgotten 50-year-old drug, lives its renaissance in the twenty-first century, which can be administered in refractory HL as well. Combination of the 'new' and 'old' drugs might be also helpful. CONCLUSION: Our data suggest that rituximab plus bendamustine (supplemented with or without BV) could be a suitable alternative bridging salvage therapy for heavily pretreated HL patients.
Asunto(s)
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Enfermedad de Hodgkin/tratamiento farmacológico , Adulto , Brentuximab Vedotina , Femenino , Enfermedad de Hodgkin/patología , Humanos , Inmunoconjugados/administración & dosificación , Masculino , Persona de Mediana Edad , Recurrencia Local de Neoplasia/tratamiento farmacológico , Recurrencia Local de Neoplasia/patología , Rituximab/administración & dosificación , Terapia Recuperativa , Adulto JovenRESUMEN
The B-cell lymphoma, unclassifiable, showing intermediate features typical for both diffuse large B-cell lymphoma (DLBCL) and classical Hodgkin lymphoma (HL) is a novel category of diffuse large B-cell lymphomas (DLBCL/HL), which has been described by the WHO classification in 2008. This rare type of lymphomas, previously called as gray zone lymphoma presents peculiar clinical, morphological and immunophenotypical patterns. In December 2011 a 17-year old male was diagnosed with mixed cellularity subtype of classical HL. His clinical stage was IV/BXS (abdominal bulky) with unfavourable prognosis. Because of the unusually extended disease (nodal-extranodal-bulky) a histological revision was performed. After an incomplete course of ABVD chemotherapy the patient's symptoms disappeared and regression was detected in the size of peripheral lymph nodes. The diagnosis changed into DLBCL/HL, so the treatment was modified to R-CHOP-14 regimen. After the administration of 3 cycles of R-CHOP-14, he achieved a complete metabolic remission (CMR), which was confirmed by a (18)FDG-PET/CT scan. Receiving further 4 cycles of R-CHOP-14 therapy the patient was still in CMR, but a PET negative large mass (70 × 30 mm) still remained visible in the abdominal region. Considering this residuum and the aggressive subtype of lymphoma he was referred for an autologous hemopoietic stem cell transplantation (AHSCT). After 2 cycles of R-DHAP regimen, successful CD34 positive stem cell collection was performed in August 2012. In September 2012, he underwent a R-BEAM conditioning followed by AHSCT. The next (18)FDG-PET/CT still detected CMR 100 days after the AHSCT. The patient was in excellent clinical condition and also in complete remission 15 months after the AHSCT. Upon this case, it should be underlined that the diagnosis may need revision if a patient represents atypical clinical signs and behavior, and the importance of cooperation between clinicians and pathologists is also strongly emphasized.
Asunto(s)
Enfermedad de Hodgkin/patología , Linfoma de Células B Grandes Difuso/patología , Adolescente , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Ciclofosfamida/uso terapéutico , Doxorrubicina/uso terapéutico , Fluorouracilo/uso terapéutico , Trasplante de Células Madre Hematopoyéticas/métodos , Enfermedad de Hodgkin/tratamiento farmacológico , Humanos , Linfoma de Células B Grandes Difuso/tratamiento farmacológico , Masculino , Metotrexato/uso terapéutico , Vinblastina/uso terapéutico , Vincristina/uso terapéuticoRESUMEN
Autoimmune diseases and malignant lymphomas have numerous similarities in their etiology and pathogenesis. Patients with autoimmune disorders have increased risk to develop non-Hodgkin's lymphomas, yet little is known about the occurrence of autoimmune features within lymphoma patients. Our aim was to examine the prevalence of autoimmune diseases among patients with non-Hodgkin's (NHL) and Hodgkin's lymphoma (HL). We reviewed 352 patients' charts with malignant lymphomas to assess the rate of associated autoimmune diseases. Of 231 NHL patients, 30 (12.9%) had autoimmune disorders, while there were 11 patients who suffered from more than one disease entity. It was Sjögren's syndrome that occurred in the largest number (eight cases), other frequent entities were undifferentiated connective tissue disease (seven), thyroiditis (six), rheumatoid arthritis (four), and systemic vasculitis (four). The female/male ratio was significantly different between patients with or without autoimmune diseases, while no other clinical features differed significantly between the two groups. Ten patients (33.3%) were initially diagnosed with lymphoma, 13 (43.3%) of them had already been diagnosed with autoimmune disease at the time of lymphoma occurrence. Six patients (20%) with previously diagnosed immunological disorder developed new autoimmune condition after the treatment of lymphoma. Lymphoma and autoimmune disease occurred simultaneously in one patient. Among the 121 HL patients, 14 (11.5%) had associated autoimmune disease. Ten patients developed thyroiditis after the lymphoma treatment, two had immune thrombocytopenia, and one had autoimmune hemolytic anemia. One female patient was diagnosed with systemic sclerosis 10 years before the onset of HL. Our results highlight that an increased risk for the development of autoimmune diseases should be considered in patients both with NHL and HL.