RESUMO
We present a patient with autoimmune autonomic ganglionopathy (AAG) who had persistently positive ganglionic nicotinic acetylcholine receptor antibody levels despite immunosuppressive therapy. Rituximab-based therapy for an incidental lymphoma was associated with prolonged symptomatic and serological control of AAG.
Assuntos
Doenças Autoimunes do Sistema Nervoso/diagnóstico , Doenças Autoimunes do Sistema Nervoso/tratamento farmacológico , Gânglios Autônomos/patologia , Fatores Imunológicos/uso terapêutico , Rituximab/uso terapêutico , Humanos , Masculino , Pessoa de Meia-IdadeRESUMO
Non-verbal communication is an important component of the physician-patient interaction. Oncology patients face specific emotional and psychological issues requiring additional physician emotional support. Multiple studies in oncology patients have revealed that patients perceive physicians seated during the medical interview to be more compassionate, caring, and likely to spend more time with the patients. These are all associated with improved patient outcomes. Barriers to sitting may be due to those imposed by time, space, and reduced perceived benefit of sitting by the physician. Although a sitting posture alone is unlikely to compensate for poor communication skills, assessing patient preference to physician posture, and following their preference, can be a simple way of improving communication, and thus patient outcomes, especially in oncology patients. The widespread introduction of the electronic medical record (EMR) system over the last decade has added a "third wheel" to the original dyadic physician-patient relationship. Physician posture and eye gaze towards to the EMR and its components has a deleterious effect on communication. Appropriate training and sensitization in this regard should be provided for physicians.
Assuntos
Oncologia , Comunicação não Verbal , Satisfação do Paciente , Pacientes/psicologia , Relações Médico-Paciente , Médicos , Postura , Empatia , Humanos , PercepçãoRESUMO
INTRODUCTION: Long-term data regarding kidney transplantation (KTx) patients with monoclonal gammopathy of undetermined significance (MGUS) are scarce. We evaluated the long-term outcomes of these patients in a single-center retrospective study from the Mayo Clinic, Rochester, Minn., USA. METHODS: Patients who had an MGUS before transplant or developed one after KTx were selected. Monoclonal protein was screened as part of the KTx evaluation by serum protein electrophoresis. Screening for posttransplant lymphoproliferative disorder (PTLD) or MGUS after transplant was not required by protocol. Patients with multiple myeloma, dysproteinemia-related kidney disease or no pretransplant serum protein electrophoresis were excluded. RESULTS: Between 1963 and 2006, 3,518 patients underwent KTx. MGUS was identified in 42 patients, with 23 before transplant and 19 after transplant. Median follow-up for these patients was 8.5 years (range 0.3-37). Four (17.4%) pretransplant MGUS patients developed a hematologic malignancy: 2 smoldering multiple myeloma and 2 PTLD - an Epstein-Barr virus-positive diffuse large cell lymphoma and a Hodgkin lymphoma. None of the 19 patients who developed an MGUS after transplant progressed to multiple myeloma, but 2 (10.5%) developed Epstein-Barr virus-negative T cell lymphoproliferative disorders at 16 and 26 years after transplant. Median survival was 26.1 and 28.0 years for the pretransplant and posttransplant MGUS groups, respectively. CONCLUSION: Progression from true MGUS to multiple myeloma is rare after KTx. KTx appears safe in true MGUS patients if the monoclonal gammopathy was not the cause of the kidney disease. None of the patients progressed to multiple myeloma, but 2 developed smoldering multiple myeloma and several developed PTLD. Further studies are needed to explain the relationship between MGUS and PTLD.
Assuntos
Progressão da Doença , Falência Renal Crônica/complicações , Transplante de Rim , Transtornos Linfoproliferativos/epidemiologia , Gamopatia Monoclonal de Significância Indeterminada/complicações , Gamopatia Monoclonal de Significância Indeterminada/epidemiologia , Adulto , Idoso , Antígenos Virais/sangue , Infecções por Vírus Epstein-Barr/complicações , Infecções por Vírus Epstein-Barr/epidemiologia , Feminino , Seguimentos , Herpesvirus Humano 4/imunologia , Doença de Hodgkin/epidemiologia , Humanos , Imunoglobulina A/sangue , Imunoglobulina G/sangue , Cadeias Pesadas de Imunoglobulinas/sangue , Imunoglobulina M/sangue , Falência Renal Crônica/cirurgia , Linfoma Difuso de Grandes Células B/epidemiologia , Masculino , Pessoa de Meia-Idade , Gamopatia Monoclonal de Significância Indeterminada/sangue , Mieloma Múltiplo/epidemiologia , Estudos Retrospectivos , Fatores de TempoAssuntos
Histiocitose de Células de Langerhans/diagnóstico , Imageamento por Ressonância Magnética , Celulite Orbitária/etiologia , Tomografia Computadorizada por Raios X , Adulto , Feminino , Histiocitose de Células de Langerhans/complicações , Histiocitose de Células de Langerhans/diagnóstico por imagem , Humanos , Celulite Orbitária/diagnóstico por imagem , Celulite Orbitária/tratamento farmacológico , Recusa do Paciente ao TratamentoAssuntos
Cardiopatias/diagnóstico por imagem , Neoplasias Cardíacas/diagnóstico por imagem , Trombose/diagnóstico por imagem , Diagnóstico Diferencial , Evolução Fatal , Cardiopatias/complicações , Neoplasias Cardíacas/complicações , Humanos , Masculino , Pessoa de Meia-Idade , Neoplasias , Trombose/complicaçõesRESUMO
Inherited thrombocytopenias, including inherited giant platelet disorders (IGPD) or macro thrombocytopenias are relatively rare, but their prevalence is likely underestimated from complexities of diagnosis and a spectrum of subclinical phenotypes. Harris platelet syndrome (HPS) is the most common IGPD reported from the Indian subcontinent. Of note there are an increased number of hemoglobinopathies reported from the geographic location. We analysed red blood cell and platelet indices of blood donors with HPS from the north eastern part of India and compared them with blood indices of blood donors of south India. We found a statistically significant lower platelet count in blood donors with HPS (median, range) 132 (71-267) vs. 252 (160-478) as compared to donors from south India (P < 0.001). Mean platelet volume (MPV) was higher in donors with HPS 13.1, (range 12-21.9 fl) as compared to donors from south India 7.35 (range 6-9.2 fl) (P < 0.001). This study showed that blood donors with HPS had a low median platelet bio-mass 0.17 (0.10-0.38%) vs. 0.19 (0.13-0.28%) in donors from south India. The platelet distribution width (PDW) was 17.4 (14.9-19.6) in donors with HPS vs. 16.38 (15.2-18.5) in south Indian blood donors (P < 0.001). Thirty-three donors with HPS had a normal platelet count with MPV more than 12 fL. Only donors with HPS had giant platelets and thrombocytopenia on peripheral blood smear examination. None of these donors had Dohle body inclusion in their leukocytes. Compared to donors from south India, donors with HPS had a significantly lower hemoglobin 13.8 (12-16.3 gm/dL) vs. 14.8 (12-18) respectively (P < 0.001) while red distribution width (RDW) was higher in HPS 13.6 (11.5-16.7) vs. 12.8 (11.4-15.1). However we did not find any statistically significant difference in MCV, MCH, MCHC between the two groups. Peripheral blood smear did not show any obvious abnormal red blood cell morphology. In the blood donors with HPS we found a statistically higher MPV, RDW and a lower platelet count and platelet biomass. A population-based study will be helpful in determining the existence of any hemoglobinopathies among subjects with HPS.
Assuntos
Transtornos Plaquetários/sangue , Índices de Eritrócitos , Contagem de Plaquetas , Adulto , Transtornos Plaquetários/fisiopatologia , Plaquetas/citologia , Plaquetas/metabolismo , Eritrócitos/citologia , Eritrócitos/metabolismo , Humanos , Índia , Síndrome , Adulto JovemAssuntos
Leucemia Mieloide Aguda/diagnóstico , Neoplasias Pulmonares/diagnóstico , Mesotelioma/diagnóstico , Idoso , Vazamento Acidental em Bhopal , Feminino , Humanos , Leucemia Mieloide Aguda/tratamento farmacológico , Leucemia Mieloide Aguda/genética , Neoplasias Pulmonares/patologia , Mesotelioma/patologia , SobreviventesRESUMO
Granulocyte colony-stimulating factor (GCSF) is currently the most widely used cytokine for stem cell mobilization. There are few studies suggesting GCSF administration may induce activation of both coagulation and endothelial cells that could favor the developing of thrombotic events. We report a 58-year-old female with vasculitis and renal impairment. She was found to have an underlying monoclonal gammopathy of unknown significance (MGUS). The monoclonal protein was felt to play a role in her underlying renal disease and peripheral neuropathy. She was considered a candidate for peripheral blood stem cell transplantation to manage the monoclonal protein. During stem cell mobilization with GCSF, she developed worsening of anemia; thrombocytopenia and worsening of renal function. She was diagnosed with thrombotic microangiopathy (TMA) which was successfully treated with therapeutic plasma exchange and rituximab. It is possible that GCSF may have directly (activating endothelial cells) or indirectly (activation of underlying autoimmune disorder) contributed to TMA in this patient.
Assuntos
Fator Estimulador de Colônias de Granulócitos/efeitos adversos , Mobilização de Células-Tronco Hematopoéticas/efeitos adversos , Gamopatia Monoclonal de Significância Indeterminada/terapia , Transplante de Células-Tronco de Sangue Periférico/efeitos adversos , Microangiopatias Trombóticas/etiologia , Feminino , Humanos , Pessoa de Meia-IdadeAssuntos
Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Asparaginase/efeitos adversos , Trombose Intracraniana/induzido quimicamente , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamento farmacológico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Antitrombina III/uso terapêutico , Asparaginase/administração & dosagem , Criança , Irradiação Craniana , Humanos , Trombose Intracraniana/prevenção & controleAssuntos
Antraciclinas/administração & dosagem , Antineoplásicos/administração & dosagem , Transplante de Células-Tronco Hematopoéticas , Cobertura do Seguro , Leucemia Mielomonocítica Aguda/tratamento farmacológico , Citarabina/administração & dosagem , Humanos , Revisão da Utilização de Seguros , Leucemia Mielomonocítica Aguda/terapia , Condicionamento Pré-TransplanteRESUMO
Acute lymphoblastic leukemia (ALL) is a haematological malignancy that can involve the central nervous system (CNS). Less than 10 % of patients with ALL have CNS involvement at presentation. The cranial nerve most commonly affected is cranial nerve VII although bilateral involvement is rare. Management and outcomes of these patients are not well understood. Moreover bilateral Bells palsy as a presenting symptom of ALL is extremely uncommon. We report a very unusual presentation of ALL with bilateral facial nerve palsy, and discuss the management strategies and outcomes for patients with ALL that present with cranial nerve palsies.
RESUMO
BACKGROUND: Primary effusion lymphoma (PEL) is a rare malignancy usually associated with HIV infection. Management and outcomes are poorly understood. METHODS: The medical records of all patients diagnosed with HIV-associated PEL at our institution between 1999 and 2014 were reviewed. Patients were followed till death, treatment failure or loss of follow-up. RESULTS: Twelve patients with PEL were identified during the 15 year study period; 9 had HIV infection. All 9 were male; median age was 45 years. All presented with local symptoms and were diagnosed with PEL a median of 11 years after HIV diagnosis. Location was pleural (3), pericardial (3), peritoneal (1) and extracavitatory (2). By definition, all had Ann Arbor stage 4 at diagnosis. Median follow-up was 34 months. Two patients had poor performance status and were unable to get chemotherapy. Seven patients had a complete remission (CR) and two died within 1 month of diagnosis. The median CD4 levels at PEL diagnosis in patients with poor versus good outcomes were 54 cells/mm3 (range, 26-82 cells/mm3) and 211 cells/mm3 (range, 73-800 cells/mm3). In contrast, the median lactate dehydrogenase (LDH) levels at PEL diagnosis with poor versus good prognosis were 1074 U/L (range, 703-1445 U/L) and 283 U/L (range, 156-760 U/L). CONCLUSIONS: Given its rarity, our knowledge of PEL relies solely on case reports and case series. Prompt HAART and chemotherapy may be effective in HIV- associated PEL and good outcomes are possible. LDH and CD4 may be possible prognostic factors in PEL.