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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/psicologíaRESUMEN
OBJECTIVE: We report an extension study of patients with essential thrombocythaemia (ET) in the Hungarian Myeloproliferative Neoplasm (HUMYPRON) Registry, which demonstrated that over 6 years anagrelide significantly decreased the number of patients experiencing minor arterial and minor venous thrombotic events (TEs) vs hydroxyurea+aspirin. METHODS: Data on patients with ET were collected through completion of a questionnaire developed according to 2008 WHO diagnostic criteria and with regard to Landolfi, Tefferi and IPSET criteria for thrombotic risk. Data were entered into the registry from 14 haematological centres. TEs, secondary malignancies, disease progression and survival were compared between patients with ET treated with anagrelide (n = 116) and with hydroxyurea+aspirin (n = 121). RESULTS: Patients were followed for (median) 10 years. A between-group difference in the number of patients with TEs was observed (25.9% anagrelide vs 38.0% hydroxyurea+aspirin; P = .052). Minor arterial events were more frequently reported in the hydroxyurea+aspirin group (P < .001); there were marginally more reports of major arterial events in the anagrelide group (P = .049). TE prior to diagnosis was found to significantly influence TE incidence (P > .001). Progression-free survival (P = .004) and survival (P = .001) were significantly increased for the anagrelide group vs hydroxyurea+aspirin. CONCLUSIONS: Anagrelide reduced TEs, and increased progression-free and overall survival vs hydroxyurea+aspirin over (median) 10 years.
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Trombocitemia Esencial/complicaciones , Trombocitemia Esencial/mortalidad , Trombosis/etiología , Trombosis/mortalidad , Aspirina/administración & dosificación , Aspirina/uso terapéutico , Quimioterapia Combinada , Encuestas de Atención de la Salud , Humanos , Hungría , Hidroxiurea/administración & dosificación , Hidroxiurea/uso terapéutico , Quinazolinas/administración & dosificación , Quinazolinas/uso terapéutico , Sistema de Registros , Trombocitemia Esencial/epidemiología , Trombosis/epidemiología , Trombosis/prevención & control , Resultado del TratamientoRESUMEN
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
OBJECTIVE: To evaluate the reduction in thrombotic events (TE) in patients with essential thrombocythaemia (ET) treated with anagrelide versus hydroxyurea + aspirin (HU + ASA). METHODS: A questionnaire was developed using 2008 WHO diagnostic criteria, and thrombotic risk factors were stratified according to Landolfi criteria. Through questionnaire completion, clinicians at Hungarian haematological centres entered data into the Hungarian MPN Registry on patients with myeloproliferative neoplasms. Based on ET registry data, TEs in anagrelide-treated patients (n = 139) were compared with HU + ASA-treated patients (n = 141). RESULTS: Patients were followed up for (median) 6 yr. TEs were reported in significantly fewer anagrelide-treated patients versus HU + ASA (15.1% versus 49.6%; P < 0.001). Numbers of major arterial and major venous events were similar between the groups, although there were over fivefold more minor arterial and minor venous events in the HU + ASA group (P < 0.001). While median age at diagnosis was older and length of follow-up shorter in the HU + ASA group (P < 0.05), this did not influence TE incidence; medication and TE before diagnosis only influenced TE incidence. CONCLUSIONS: Anagrelide significantly decreased the number of patients experiencing minor arterial and minor venous TEs versus HU + ASA over 6 yr. Risk of TE after diagnosis was significantly increased if the patient had TE before diagnosis.
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Aspirina/uso terapéutico , Hidroxiurea/uso terapéutico , Inhibidores de Agregación Plaquetaria/uso terapéutico , Quinazolinas/uso terapéutico , Trombocitemia Esencial/complicaciones , Trombosis/etiología , Trombosis/prevención & control , Adulto , Anciano , Anciano de 80 o más Años , Quimioterapia Combinada , Femenino , Estudios de Seguimiento , Humanos , Hungría , Masculino , Persona de Mediana Edad , Riesgo , Trombosis/diagnóstico , Trombosis/epidemiologíaRESUMEN
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
INTRODUCTION: In order to establish and use a national registry, several Hungarian hematology centers collected data of myeloproliferative neoplasia patients. AIM: The recent publication is an analysis of the data of registered essential thrombocythaemic patients. METHOD: an online electronic registry has been established, using 2008 World Health Organization's diagnostic criteria and thrombotic risk was evaluated according to Landolfi stratification. RESULTS: Data of 350 essential thrombocythaemic patients from 15 Hungarian hematology centers entered up to the date of June 30, 2015 were used for analysis. Patients were followed up to (median) 6 years. The epidemiologic data (age, gender) and thrombotic events prior and after the diagnosis, were similar to the literature. The thrombotic events of anagrelide treated patient (n = 139) and the hydroxyurea + aspirin treated patients (n = 141) have been compared. The major arterial and venous events were similar between the groups, but there were fivefold less minor arterial and venous events in the anagrelide group (p<0.001). Thrombotic incidence after diagnosis were influenced only by medication and thrombotic events before the diagnosis. CONCLUSIONS: Anagrelide significantly decreased the number of patients experiencing minor arterial and minor venous thrombosis, vs hydroxyurea + aspirin. Despite of the treatment the risk of thrombotic events after diagnosis remained high, and was significantly increased in patients with thrombosis before diagnosis. Orv. Hetil., 2017, 158(3), 111-116.
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Trastornos Mieloproliferativos/tratamiento farmacológico , Trastornos Mieloproliferativos/epidemiología , Cromosoma Filadelfia , Inhibidores de Agregación Plaquetaria/uso terapéutico , Quinazolinas/uso terapéutico , Sistema de Registros , Femenino , Humanos , Hungría , MasculinoRESUMEN
Intruduction and aim: The Hungarian National Registry for Philadelphia chromosome negative myeloproliferative neoplasms has been developed. The aim of the recent study is to assess the clinical characteristics of Hungarian patients with polycythemia vera. METHOD: Data of 351 JAK2V617F and exon 12 mutation positive polycythemia vera patients were collected online from 15 haematology centres reporting epidemiologic, clinical characteristics, diagnostic tools, therapeutic interventions, thromboembolic complications, disease transformations. Vascular events prior to and after diagnosis were evaluated upon the Landolfi risk assessment scale. RESULTS: 116 thromboembolic events were reported in 106 PV patients prior to diagnosis and 152 occasions in 102 patients during follow-up. The frequency of major arterial events were significantly reduced (p<0.0001) and the minor venous events were significantly elevated (p<0.0001) after the diagnosis. Major hemorrhagic complications were found in 25 and transformation in 26 cases. CONCLUSIONS: Our registry allows to collect and evaluate the features of patients with polycythemia vera. The Landolfi risk stratification was proven to be useful. Based on evaluated data, accuracy of diagnostic criteria and compliance to risk-adapted therapeutic guidelines are needed. Orv Hetil. 2017; 158(23): 901-909.
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Policitemia Vera/epidemiología , Mielofibrosis Primaria/epidemiología , Sistema de Registros , Distribución por Edad , Anciano , Femenino , Humanos , Hungría , Masculino , Persona de Mediana Edad , Cromosoma Filadelfia , Medición de Riesgo , Factores de Riesgo , Distribución por SexoRESUMEN
Polycythaemia vera (PV), a condition characterized by blood hyperviscosity due to the expansion of the erythrocyte mass is the most common entity among all Philadelphia chromosome-negative myeloproliferative neoplasms. Arterial and venous thrombotic events are leading determinants of morbidity and mortality but impairment of quality of life due to vasomotor symptoms (erythromelalgia, pruritus) and disease-associated symptoms (tiredness, fatigue, pruritus, night sweats, vision problems, headache, concentration loss, abdominal discomfort, early satiety, fever, weight loss) are also present. The review of polycythaemia vera is actual as the updated WHO 2016 classification of myeloid neoplasms has changed the diagnostic criteria and a new second-line treatment option - JAK1/JAK2 inhibitor ruxolitinib - has been approved for patients who had an inadequate response to or are intolerant of hydroxyurea, which represents a breakthrough in the treatment of this patient population. Orv. Hetil., 2016, 157(44), 1743-1751.
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Policitemia Vera/clasificación , Policitemia Vera/diagnóstico , Anticoagulantes/administración & dosificación , Medicina Basada en la Evidencia , Humanos , Nitrilos , Inhibidores de Agregación Plaquetaria/administración & dosificación , Policitemia Vera/tratamiento farmacológico , Pirazoles/administración & dosificación , PirimidinasRESUMEN
Primary myelofibrosis is one of the Philadelphia negative chronic myeloproliferative neoplasms. It is a rare disease featured by cytopenias and hepatosplenomegaly. Although the etiology of the disease is still unknown, our knowledge about its pathology and prognosis has been improving in the last few years. Furthermore, the JAK2 inhibitor ruxolitinib has become available in Hungary since 2015. Beside its high efficacy in spleen volume and in reduction of myelofibrosis-associated symptoms, this novel therapy also exerts a disease-modifying effect and, therefore, ruxolitinib may improve the life expectancy too. Treatment approach of myelofibrosis has been changed these years, which gives a reason for this summary. Orv. Hetil., 2016, 157(39), 1547-1556.
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INTRODUCTION: The establishment and operation of disease registry can be used to collect data on epidemiology cases. In addition, the registry can help to work out medical and health economical and political decisions for longer term. AIM: The aim of the authors was to collect and analyse data of patients with Philadelphia negative neoplasia in Hungary and draw conclusions about the basic types and features of the relevant disease. METHOD: An online electronic data collection system has been established, based on the permission of the Regional and Institutional Committee of Science and Research Ethics obtained in April 8, 2013. Data collection has been initiated by hematology centres in Hungary. In addition to collection of the epidemiologic data, blood and bone marrow analysis data have been collected, as well. Also, based on cardiovascular factors, risk stratification has been established. Finally, the authors have investigated the method and practice of patient treatment in Hungary. RESULTS: Data of 901 patients from 15 Hungarian haematology centres have been recorded up to the date of June 30, 2015. After clarification of the data, 426 polycythaemia vera, 350 essential thrombocythaemia and 82 myelofibrosis cases were used for analysis. CONCLUSIONS: An online registry has been established which helps to clarify and analyse the basic features of certain medical cases and their treatment in Hungary. Including additional medical centres could help to improve the accuracy of medical analysis.
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Policitemia Vera/epidemiología , Mielofibrosis Primaria/epidemiología , Sistema de Registros , Trombocitemia Esencial/epidemiología , Adulto , Distribución por Edad , Anciano , Enfermedad Crónica , Femenino , Humanos , Hungría/epidemiología , Masculino , Persona de Mediana Edad , Cromosoma Filadelfia , Policitemia Vera/terapia , Mielofibrosis Primaria/terapia , Medición de Riesgo , Factores de Riesgo , Distribución por Sexo , Trombocitemia Esencial/terapiaRESUMEN
Acute myelogenous leukemia is a heterogeneous disease. Recent molecular mutational analysis techniques have shed more light on different, genetically well characterised types of the disease. Treatment approach is uniform except for acute promyelocytic leukemia. Application of the "3 + 7" induction treatment has been the gold standard in the past 40 years. While the dose of cytarabine has not been changed, escalating daunorubicine dose in younger (<60 years) patients with good performance status to 90 mg/m(2) had a positive impact on overall survival. High dose chemotherapy is tolerated poorly in patients older than 60 years of age and, as treatment is not curative in the elderly, improvement of overall survival and quality of life remains the main goal of management in these patients. Low intensity treatment is beneficial and can provide additional advantage over supportive care. Innovative and targeted therapy approaches might give promise to better management of patients with acute myelogenous leukemia.
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Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Quimioterapia de Inducción , Leucemia Mieloide Aguda/tratamiento farmacológico , Factores de Edad , Anciano , Citarabina/administración & dosificación , Daunorrubicina/administración & dosificación , Esquema de Medicación , Humanos , Quimioterapia de Inducción/métodos , Estado de Ejecución de Karnofsky , Persona de Mediana Edad , Terapia Molecular Dirigida , Calidad de Vida , Análisis de Supervivencia , Resultado del TratamientoRESUMEN
INTRODUCTION: Hodgkin lymphoma is a curable lymphoma with an 80-90% long-term survival, however, 30% of the patients develop relapse. Only half of relapsed patients can be cured with autologous stem cell transplantation. AIM: The aim of the authors was to analyze survival rates and incidence of relapses among Hodgkin lymphoma patients who were treated between January 1, 1980 and December 31, 2014. Novel therapeutic options are also summarized. METHOD: Retrospective analysis of data was performed. RESULTS: A total of 715 patients were treated (382 men and 333 women; median age at the time of diagnosis was 38 years). During the studied period the frequency of relapsed patients was reduced from 24.87% to 8.04%. The numbers of autologous stem cell transplantations was increased among refracter/relapsed patients, and 75% of the patients underwent transplantation since 2000. The 5-year overall survival improved significantly (between 1980 and 1989 64.4%, between 1990 and 1999 82.4%, between 2000 and 2009 88.4%, and between 2010 and 2014 87.1%). Relapse-free survival did not change significantly. CONCLUSIONS: During the study period treatment outcomes improved. For relapsed/refractory Hodgkin lymphoma patients novel treatment options may offer better chance for cure.
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Antineoplásicos/uso terapéutico , Trasplante de Células Madre Hematopoyéticas , Enfermedad de Hodgkin/epidemiología , Enfermedad de Hodgkin/terapia , Recurrencia Local de Neoplasia/epidemiología , Recurrencia Local de Neoplasia/terapia , Acondicionamiento Pretrasplante/métodos , Adulto , Antígeno B7-H1/efectos de los fármacos , Antígeno B7-H1/metabolismo , Clorhidrato de Bendamustina/administración & dosificación , Brentuximab Vedotina , Supervivencia sin Enfermedad , Femenino , Trasplante de Células Madre Hematopoyéticas/métodos , Enfermedad de Hodgkin/mortalidad , Enfermedad de Hodgkin/patología , Humanos , Hungría/epidemiología , Inmunoconjugados/administración & dosificación , Masculino , Persona de Mediana Edad , Recurrencia Local de Neoplasia/mortalidad , Recurrencia , Estudios Retrospectivos , Rituximab/administración & dosificación , Terapia Recuperativa/métodos , Esclerosis , Análisis de Supervivencia , Tasa de Supervivencia , Trasplante Autólogo , Trasplante Homólogo , Resultado del TratamientoRESUMEN
Treatment of autoimmune haemolytic anaemia is still a challenge to clinicians. Even today it may be lethal. Half of the cases are secondary due to an underlying disease, and the others are primary or idiopathic cases. According to the specificity and type of autoantibodies there are warm and cold type forms of autoimmune haemolytic anaemia. The hallmark of the diagnosis is to detect the presence of haemolysis by clinical and laboratory signs and detect the underlying autoantibodies. Treatment of autoimmune haemolytic anaemia is still a challenge to clinicians. We still loose patients due to excessive haemolysis or severe infections caused by immunosuppression. First line treatment is corticosteroids. Other immunosuppressive agents like: cyclophosphamide, azathioprine, cyclosporine or the off label rituximab can be used in case of corticosteroid refractoriness. Splenectomy is a considerable option in selective cases. The authors discuss treatment options and highlight difficulties by presenting 4 cases.
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Corticoesteroides/uso terapéutico , Anemia Hemolítica Autoinmune/diagnóstico , Anemia Hemolítica Autoinmune/tratamiento farmacológico , Autoanticuerpos/sangre , Hemoglobinas/metabolismo , Inmunosupresores/uso terapéutico , Corticoesteroides/administración & dosificación , Anciano , Anemia Hemolítica Autoinmune/sangre , Anemia Hemolítica Autoinmune/inmunología , Anticuerpos Monoclonales de Origen Murino/uso terapéutico , Azatioprina/uso terapéutico , Ciclofosfamida/uso terapéutico , Ciclosporina/uso terapéutico , Femenino , Humanos , Inmunosupresores/administración & dosificación , Masculino , RituximabRESUMEN
Successful treatment of lymphomas requires classical morphological diagnosis and also immunophenotyping as well as cytogenetics and molecular genetic examinations. The expansion of pathogenetic knowledge resulted in new classifications, new disease entities, and subtypes that are identified with distinct genetic aberrations bearing prognostic value as well and also serving as potential targets for new targeted therapies. The more detailed molecular background is explored the more precise personalized treatment options can be utilized nowadays and in the near future. Thus new up-to-date hematopathology is the key to successful oncohematological treatment. The imaging diagnosis has also evolved during the last decade in the diagnosis of lymphomas. This can also help us to monitor response to therapy by using positron emission tomography. This enables us to monitor therapy and make decisions based on efficiency of the therapeutic regimen. Survival data of lymphoma patients can further be improved by avoiding under- and overtreatment, which is achieved by using PET/CT as a "biomarker" or response indicator besides conventional biologic and clinical prognostic markers.
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Biomarcadores de Tumor , Linfoma/diagnóstico , Tomografía de Emisión de Positrones , Tomografía Computarizada por Rayos X , Biomarcadores de Tumor/análisis , Biomarcadores de Tumor/genética , Fluorodesoxiglucosa F18 , Humanos , Linfoma/tratamiento farmacológico , Linfoma/genética , Linfoma/patología , Terapia Molecular Dirigida , Tomografía de Emisión de Positrones/métodos , PronósticoRESUMEN
OBJECTIVE: Follicular lymphoma (FL) is an indolent, lymphoproliferative disease of B-cell origin that has a heterogeneous disease course with varying outcomes. Certain patients may undergo autologous stem cell transplantation. We investigated the outcome of autologous stem cell transplantation in patients with FL. METHODS: Patients who received autologous stem cell transplantation at the University of Debrecen's Department of Hematology between 2004 and 2021 were retrospectively analyzed. The overall survival (OS) and progression-free survival (PFS) after transplantation of patients with FL were examined. Prognostic factors that may influence the course of the disease were chosen. RESULTS: Data were collected from 49 patients. OS was influenced only by age, whereas PFS was affected by age and the lymphocyte/monocyte ratio. The combination of age and lymphocyte/monocyte ratio defined a patient population with a particularly unfavorable prognostic risk profile: patients over 47 years of age with a pre-transplant lymphocyte/monocyte ratio greater than or equal to 2.675. CONCLUSION: Age and lymphocyte/monocyte ratio were identified as useful prognostic factors for PFS in patients with FL following autologous stem cell transplantation.
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Trasplante de Células Madre Hematopoyéticas , Linfoma Folicular , Humanos , Trasplante Autólogo/efectos adversos , Trasplante de Células Madre Hematopoyéticas/efectos adversos , Pronóstico , Estudios Retrospectivos , Monocitos/patología , Linfocitos/patología , Protocolos de Quimioterapia Combinada Antineoplásica , Supervivencia sin EnfermedadRESUMEN
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.
RESUMEN
TP53 aberrations predict chemoresistance and represent a contraindication for the use of standard chemoimmunotherapy in chronic lymphocytic leukaemia (CLL). Recent next-generation sequencing (NGS)-based studies have identified frequent low-burden TP53 mutations with variant allele frequencies below 10%, but the clinical impact of these low-burden TP53 mutations is still a matter of debate. In this study, we aimed to scrutinise the subclonal architecture and clinical impact of TP53 mutations using a sensitive, NGS-based mutation analysis in a 'real-world' cohort of 901 patients with CLL. In total, 225 TP53 mutations were identified in 17.5% (158/901) of the patients; 48% of these alterations represented high-burden mutations, while 52% were low-burden TP53 mutations. Low-burden mutations as sole alterations were identified in 39% (62/158) of all mutated cases with 82% (51/62) of these being represented by a single low-burden TP53 mutation. Patients harbouring low-burden TP53 mutations had significantly lower time to first treatment compared to patients with wild-type TP53. Our study has expanded the knowledge on the frequency, clonal architecture, and clinical impact of low-burden TP53 mutations. By demonstrating that patients with sole low-burden TP53 variants represent more than one-third of patients with TP53 mutations and have an increased risk for treatment initiation, our findings strengthen the need to redefine the threshold of TP53 variant reporting to below 10% in the routine diagnostic setting.