RESUMEN
Patients with immunodeficiency, whether congenital or acquired, have a significantly higher incidence of malignancies, especially mature lymphoid neoplasms and lymphoproliferative disorders. We present the case of a 50-year-old patient with a history of dermatomyositis and antisynthetase syndrome on immunosuppressive therapy, who consulted due to increased volume of the lacrimal gland in the upper left eyelid, associated with weight loss and night sweats. He was admitted for an elective biopsy. The day after the postoperative period, she evolved with an acute abdomen. Computed axial tomography revealed multiple hypodense lesions in the liver, spleen, kidneys, and adrenal glands associated with a perforated tumor in the transverse colon and free fluid in the peritoneal cavity. In this scenario, an infectious, neoplastic, or rheumatological etiology was considered a differential diagnosis, especially in the context of our patient. Finally, the biopsies evidenced extensive necrosis with angiocentric and angiodestructive lymphoid infiltration associated with positive EBV. After extensively reviewing the symptoms, histology, and new classifications of mature B-lymphoid neoplasms, the diagnosis of polymorphic B-lymphoproliferative disorder, lymphomatoid granulomatosis-type was made, an uncommon entity rarely associated with iatrogenic immunosuppression.
Asunto(s)
Granulomatosis Linfomatoide , Humanos , Persona de Mediana Edad , Granulomatosis Linfomatoide/patología , Femenino , Enfermedad Iatrogénica , Biopsia , Masculino , Inmunosupresores/efectos adversos , Inmunosupresores/uso terapéutico , Diagnóstico DiferencialRESUMEN
BACKGROUND: Philadelphia-negative myeloproliferative neoplasms (Ph-MPN) are chronic hematological disorders characterized by the overproduction of one or more mature myeloid blood cell lineages. Classical Ph-MPN are polycythemia vera (PV), essential thrombocytopenia (ET) and primary myelofibrosis (PMF). AIM: To assess the epidemiological, clinical and diagnostic characteristics of Ph-MPN in Chile. Material and Methods: Retrospective review of medical records of all patients referred as MPN from 2012 to 2017. Patients with (9;21) translocation were excluded. RESULTS: Data of 462 cases with a median age of 69 years from 10 public hospitals was reviewed. ET was the most frequently Ph-MNP found. The incidence of Ph-MPN was 1.5 x 100.000 cases. The JAK2 V617F mutation study was performed in 96% of patients and only 30% had a bone marrow biopsy. Thrombotic events were observed in 29% of patients. Bleeding events were observed in 7%. Five-year overall survival was 87%. Conclusions: ET is the most frequent Ph-MPN. The mean incidence was lower than reported in the literature, in part because of a sub diagnosis.
RESUMEN
BACKGROUND: Philadelphia-negative myeloproliferative neoplasms (Ph-MPN) are chronic hematological disorders characterized by the overproduction of one or more mature myeloid blood cell lineages. Classical Ph-MPN are polycythemia vera (PV), essential thrombocytopenia (ET) and primary myelofibrosis (PMF). AIM: To assess the epidemiological, clinical and diagnostic characteristics of Ph-MPN in Chile. MATERIAL AND METHODS: Retrospective review of medical records of all patients referred as MPN from 2012 to 2017. Patients with (9;21) translocation were excluded. RESULTS: Data of 462 cases with a median age of 69 years from 10 public hospitals was reviewed. ET was the most frequently Ph-MNP found. The incidence of Ph-MPN was 1.5 x 100.000 cases. The JAK2 V617F mutation study was performed in 96% of patients and only 30% had a bone marrow biopsy. Thrombotic events were observed in 29% of patients. Bleeding events were observed in 7%. Five-year overall survival was 87%. CONCLUSIONS: ET is the most frequent Ph-MPN. The mean incidence was lower than reported in the literature, in part because of a sub diagnosis.
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Trastornos Mieloproliferativos , Policitemia Vera , Mielofibrosis Primaria , Trombocitemia Esencial , Anciano , Chile/epidemiología , Humanos , Janus Quinasa 2/genética , Mutación , Trastornos Mieloproliferativos/diagnóstico , Trastornos Mieloproliferativos/epidemiología , Trastornos Mieloproliferativos/genética , Policitemia Vera/diagnóstico , Policitemia Vera/epidemiología , Policitemia Vera/genética , Mielofibrosis Primaria/diagnóstico , Mielofibrosis Primaria/epidemiología , Mielofibrosis Primaria/genética , Trombocitemia Esencial/diagnóstico , Trombocitemia Esencial/genéticaRESUMEN
BACKGROUND: Philadelphia-negative myeloproliferative neoplasms (Ph-MPN) are chronic hematological disorders characterized by the overproduction of one or more mature myeloid blood cell lineages. Classical Ph-MPN are polycythemia vera (PV), essential thrombocytopenia (ET) and primary myelofibrosis (PMF). AIM: To assess the epidemiological, clinical and diagnostic characteristics of Ph-MPN in Chile. MATERIAL AND METHODS: Retrospective review of medical records of all patients referred as MPN from 2012 to 2017. Patients with (9;21) translocation were excluded. RESULTS: Data of 462 cases with a median age of 69 years from 10 public hospitals was reviewed. ET was the most frequently Ph-MNP found. The incidence of Ph-MPN was 1.5 x 100.000 cases. The JAK2 V617F mutation study was performed in 96% of patients and only 30% had a bone marrow biopsy. Thrombotic events were observed in 29% of patients. Bleeding events were observed in 7%. Five-year overall survival was 87%. CONCLUSIONS: ET is the most frequent Ph-MPN. The mean incidence was lower than reported in the literature, in part because of a sub diagnosis.
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Trastornos Mieloproliferativos , Policitemia Vera , Mielofibrosis Primaria , Trombocitemia Esencial , Anciano , Chile/epidemiología , Humanos , Janus Quinasa 2/genética , Mutación , Trastornos Mieloproliferativos/diagnóstico , Trastornos Mieloproliferativos/epidemiología , Trastornos Mieloproliferativos/genética , Policitemia Vera/diagnóstico , Policitemia Vera/epidemiología , Policitemia Vera/genética , Mielofibrosis Primaria/diagnóstico , Mielofibrosis Primaria/epidemiología , Mielofibrosis Primaria/genética , Trombocitemia Esencial/diagnóstico , Trombocitemia Esencial/genéticaRESUMEN
PURPOSE: Increasing numbers of reports have shown acceptable short-term mortality of patients with cancer admitted into the intensive care unit (ICU). The aim of this study was to determine the mortality of critically ill patients with cancer admitted to the ICU in a general hospital in Chile. MATERIALS AND METHODS: This was a prospective cohort trial in which we included all patients with cancer admitted to the ICU between July 2015 and September 2016. Demographic, physiologic, and treatment data were registered, and survival at 30 days and 6 months was evaluated. A prespecified subgroup analysis considering the admission policy was performed. These subgroups were (1) ICU admission for full code management and (2) ICU trial (IT). RESULTS: During the study period, 109 patients with cancer were included. Seventy-nine patients were considered in the full code management group and 30 in the IT. The mean age of patients was 60 years (standard deviation [SD], 15), and 56% were male. Lymphoma was the most frequent malignancy (17%), and 59% had not received cancer treatment because of a recent diagnosis. The mean Acute Physiology and Chronic Health Evaluation and Sequential-Related Organ Failure Assessment scores were 22.2 (SD, 7.3) and 7 (SD, 3), respectively. There were no differences in vasopressor, fluid, or transfusion requirements between subgroups. Lactate levels, Sequential-Related Organ Failure Assessment scores (day 1, 3, and 5), complications, and ICU length of stay were similar. In the entire cohort, 30-day and 6-month mortality was 47% and 66%, respectively. There was no difference in mortality between subgroups according to the admission policy. CONCLUSION: Patients admitted to the ICU in a developing country are at high risk for short-term mortality. However, there is a relevant subgroup that achieves 6-month survival, even among patients who undergo an IT.
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Neoplasias/mortalidad , Chile , Estudios de Cohortes , Humanos , Unidades de Cuidados Intensivos , Persona de Mediana Edad , Estudios ProspectivosRESUMEN
BACKGROUND: Pathological complete response (pCR) after neoadjuvant chemotherapy (NCT) in breast cancer (BC) identifies patients with good prognosis. AIM: To assess if the clinico-pathological subtype, determined by classic immunohistochemical (IHC) markers, is able to predict pCR and prognosis in BC patients treated with NCT. MATERIAL AND METHODS: One hundred thirty three BC patients aged 24-80 years, were treated with NCT. Clinico-pathological subtype was defined based on classic IHC markers. pCR was defined as the absence of invasive neoplastic cells in the breast and lymph nodes, on final breast surgery. RESULTS: pCR was achieved in 8.2% of patients, 3.5 and 19.5% in luminal and hormonal receptor (HR) negative tumors respectively (p < 0.01). Median follow-up was 72.6 months (3.5-190). Patients who achieved pCR had higher overall survival (OS) (p = 0.04). A univariate analysis revealed that size of the tumor, ratio of metastatic to examined lymph nodes and absence of HR were significant predictors of pCR. These findings were not replicated in the multivariate analyses. CONCLUSIONS: Clinico-pathological subtypes were independent prognostic factors for pCR and OS in BC patients in our cohort. These findings support using classic and cheap biomarkers as a predictive tool for NCT in BC.
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Biomarcadores de Tumor/análisis , Neoplasias de la Mama/patología , Terapia Neoadyuvante , Adulto , Anciano , Anciano de 80 o más Años , Neoplasias de la Mama/química , Neoplasias de la Mama/tratamiento farmacológico , Neoplasias de la Mama/mortalidad , Femenino , Humanos , Inmunohistoquímica , Persona de Mediana Edad , Reacción en Cadena de la Polimerasa , Valor Predictivo de las Pruebas , Pronóstico , Estudios Retrospectivos , Análisis de Supervivencia , Carga Tumoral , Adulto JovenRESUMEN
Background: Pathological complete response (pCR) after neoadjuvant chemotherapy (NCT) in breast cancer (BC) identifies patients with good prognosis. Aim: To assess if the clinico-pathological subtype, determined by classic immunohistochemical (IHC) markers, is able to predict pCR and prognosis in BC patients treated with NCT. Material and Methods: One hundred thirty three BC patients aged 24-80 years, were treated with NCT. Clinico-pathological subtype was defined based on classic IHC markers. pCR was defined as the absence of invasive neoplastic cells in the breast and lymph nodes, on final breast surgery. Results: pCR was achieved in 8.2% of patients, 3.5 and 19.5% in luminal and hormonal receptor (HR) negative tumors respectively (p < 0.01). Median follow-up was 72.6 months (3.5-190). Patients who achieved pCR had higher overall survival (OS) (p = 0.04). A univariate analysis revealed that size of the tumor, ratio of metastatic to examined lymph nodes and absence of HR were significant predictors of pCR. These findings were not replicated in the multivariate analyses. Conclusions: Clinico-pathological subtypes were independent prognostic factors for pCR and OS in BC patients in our cohort. These findings support using classic and cheap biomarkers as a predictive tool for NCT in BC.