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1.
Curr Opin Oncol ; 2024 Sep 09.
Artigo em Inglês | MEDLINE | ID: mdl-39246175

RESUMO

PURPOSE OF REVIEW: Health disparities or inequities, which are defined as differences in the quality of medical and healthcare between populations among racial, ethnic, and socioeconomic groups, have been validated in numerous studies as determinants of survival and quality of life in different diseases, including cancer.Compared to the improvement in overall survival in developed countries in relation to better diagnostic opportunity and novel therapeutic approaches, low and middle-income countries still have significant barriers in accessing these therapies.The potential impact of overcoming these barriers is immense and offers hope for better outcomes. RECENT FINDINGS: There is great heterogeneity in the diagnostic and therapeutic approach to multiple myeloma among different latitudes. Latin America has been characterized by important limitations in using the best technologies currently available in developed countries. SUMMARY: Overcoming health disparities in multiple myeloma in LMICs could help improve survival and quality of life outcomes. Likewise, it is necessary to increase the representation of the Latin population in clinical studies, primarily in our region.

3.
Acta Haematol ; : 1-9, 2024 Aug 10.
Artigo em Inglês | MEDLINE | ID: mdl-39128463

RESUMO

INTRODUCTION: POEMS syndrome is a rare paraneoplastic syndrome caused by an underlying plasma cell disorder. The acronym refers to the following features: polyradiculoneuropathy, organomegaly, endocrinopathy, monoclonal paraproteinemia, and skin changes. METHODS: The study was conducted at 24 hematological centers across 8 Latin-American countries. The study included a total of 46 patients {median age was 52 years (interquartile range [IQR]: 42-61.5), 30 males and 16 females} fulfilling the POEMS syndrome criteria diagnosed over a period of 12 years (January 1, 2011, through July 31, 2023). Epidemiological and clinical data were collected in an ad hoc database sent to the members of GELAMM, as well as the Kolmogorov-Smirnov test and Kaplan-Meier estimates. RESULTS: All patients had polyneuropathy and monoclonal gammopathy; 89% had bone marrow plasma cell infiltration, 33% had sclerotic bone lesions. Only 10 patients underwent vascular endothelial growth factor (VEGF) testing in plasma samples. The paraproteinemia was IgG λ in 32% and IgA λ in 30%. 59% patients presented with cutaneous changes, mainly hyperpigmentation, 54% had organomegaly, and 74% endocrinopathy. The median interval from symptom onset to diagnosis was 7.7 months (IQR: 4.0-12.6). 69% of patients received a single line of treatment. The median follow-up period was 25 months (IQR: 9.37-52.0) and the 2-year overall survival rate was 100%. All patients who underwent transplantation (43%) are alive, with a median follow-up of 45.62 months (IQR: 15.46-70). CONCLUSION: This study investigates POEMS syndrome in Latin America and presents an initial overview of the disease in the region. VEGF usage is recommended for accurate diagnosis, but only 7 hematology centers in the region used it. Survival rate in Latin America is comparable with those observed internationally.

4.
Support Care Cancer ; 31(6): 337, 2023 May 15.
Artigo em Inglês | MEDLINE | ID: mdl-37183206

RESUMO

PURPOSE: Dermatologic adverse events (dAEs) occur frequently in hospitalized patients and can significantly reduce quality of life. Physicians grade dAEs using the Common Terminology Criteria of Adverse Events (CTCAE). However, they often underestimate symptom frequency and severity. The patient-reported outcomes (PRO) version of the CTCAE (PRO-CTCAE) was developed to assess symptoms from the patient's perspective. In this study, we assessed the patient-reported burden of dAEs via the PRO-CTCAE questionnaire and compared results with dAE assessment by treating oncologists and dermatologists. METHODS: Patients admitted to Memorial Sloan Kettering Cancer Center from 6/1/2018 to 4/30/2019 and received a dermatology consultation were eligible. Once enrolled, participants completed a PRO-CTCAE questionnaire on 14 dermatologic symptoms. CTCAE grades assigned by oncology and dermatology were obtained from clinical notes, and kappa statistics were calculated to evaluate the level of agreement between physician and patient evaluations. RESULTS: A total of 100 patients (mean age 59.4, 55% male) were prospectively enrolled. The most common patient-reported dAEs were rash (72%), swelling (67%), pruritus (64%), bruising (53%), and hives (37%). Oncologists and dermatologists underreported dAEs except for rash (median kappa values 0.3 [0.02-0.84] and 0.32 [0.02-0.87], respectively). Oncologists and dermatologists were concordant with each other's documented assessment of dAEs (median kappa value 0.985 [0.55-1]). CONCLUSION: Oncology patient-reported dAEs in a tertiary academic oncologic referral center were under-recognized by providers. PRO-CTCAE may be a useful tool to optimize inpatient dermatologic care for cancer patients by detecting and allowing management of patient-reported dAEs.


Assuntos
Exantema , Neoplasias , Humanos , Masculino , Pessoa de Meia-Idade , Feminino , Qualidade de Vida , Neoplasias/tratamento farmacológico , Medidas de Resultados Relatados pelo Paciente , Inquéritos e Questionários
5.
Rev Med Chil ; 151(9): 1201-1206, 2023 Sep.
Artigo em Espanhol | MEDLINE | ID: mdl-39093157

RESUMO

BACKGROUND: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare malignant tumor with a dismal prognosis, with isolated case reports in Chile. The BPDCN can present skin and bone marrow compromise, and its diagnosis is frequently confused with other pathologies. This study aimed to evaluate the clinical and immunophenotypical features of BPDCN in the Chilean population. METHODS: We performed a retrospective study from 2013 to 2021 in clinical records of 2 public Chilean referral hospitals, including ten patients, 80% male, with a median age of 66 years (15-81). RESULTS: The most frequent initial referral diagnoses were T-cell lymphoma (4/10) and acute myeloblastic leukemia (3/10). Seven patients presented skin and bone marrow involvement; we found a lower frequency of adenopathies (5/10), splenomegaly (2/10), and hepatomegaly (2/10). The complete blood count revealed anemia and leukopenia, with blasts in 5/10. Nine patients received induction therapy. CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) was indicated in 8/10 cases with remission in 5/8, and 1 patient received HyerCVAD (cyclophosphamide, vincristine, doxorubicin and dexamethasone, methotrexate, cytarabine) and an allogeneic bone marrow transplant. The median survival was 10 months (95% CI 4.2-15.8 months) with 9/10 deaths. Relapse in the central nervous system was documented in 2 cases. CONCLUSIONS: Our study found that BPDCN, a rare pathology in the Chilean population, shows a similar clinical presentation compared to previous studies. It is susceptible to respond to initial systemic and intrathecal chemotherapy.


Assuntos
Células Dendríticas , Humanos , Masculino , Pessoa de Meia-Idade , Feminino , Estudos Retrospectivos , Idoso , Chile/epidemiologia , Adulto , Adolescente , Idoso de 80 Anos ou mais , Células Dendríticas/patologia , Adulto Jovem , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Neoplasias Cutâneas/patologia , Neoplasias Hematológicas/patologia , Vincristina/uso terapêutico , Ciclofosfamida/uso terapêutico , Prednisona/uso terapêutico
6.
Rev Med Chil ; 151(1): 15-22, 2023 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-37906742

RESUMO

BACKGROUND: Patients with Coronavirus Disease 2019 (COVID-19) frequently experience a hyperinflammatory syndrome leading to unfavorable outcomes. This condition resembles Secondary Hemophagocytic Lymphohistiocytosis (sHLH) described in neoplastic, rheumatic and other infectious diseases. A scoring system (HScore) that evaluates underlying immunosuppression, temperature, organomegaly, cytopenias, ferritin, triglycerides, fibrinogen and AST was validated for sHLH, and recently proposed to evaluate hyperinflammation in COVID-19. AIM: To assess the presence of sHLH among patients with COVID-19 admitted for hospitalization and to evaluate Hscore as a prognostic tool for poor outcomes. MATERIAL AND METHODS: One hundred forty-three patients aged 21-100 years (64% males) admitted because of COVID-19 were enrolled in a prospective study. HScore was calculated within 72 hours admission. The incidence of sHLH during hospitalization was evaluated. Additionally, the relationship between a HScore ≥ 130 points and either the requirement of mechanical ventilation or 60-days mortality was explored. RESULTS: The median HScore was 96 (33-169). A SHLH was diagnosed in one patient (incidence 0.7%), whose HScore was 169. After adjusting for age, sex, comorbidities and obesity, HScore ≥ 130 was independently associated with the composite clinical outcome (Hazard rartio 2.13, p = 0.022). CONCLUSIONS: sHLH is not frequent among COVID-19 patients. HScore can be useful to predict the risk for poor outcomes.


Assuntos
COVID-19 , Linfo-Histiocitose Hemofagocítica , Masculino , Humanos , Feminino , COVID-19/complicações , Estudos Prospectivos , Linfo-Histiocitose Hemofagocítica/etiologia , Comorbidade , Hospitalização
7.
Rev Med Chil ; 151(11): 1532-1537, 2023 Nov.
Artigo em Espanhol | MEDLINE | ID: mdl-39270121

RESUMO

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.


Assuntos
Granulomatose Linfomatoide , Humanos , Pessoa de Meia-Idade , Granulomatose Linfomatoide/patologia , Feminino , Doença Iatrogênica , Biópsia , Masculino , Imunossupressores/efeitos adversos , Imunossupressores/uso terapêutico , Diagnóstico Diferencial
8.
Rev Med Chil ; 151(11): 1501-1505, 2023 Nov.
Artigo em Espanhol | MEDLINE | ID: mdl-39270117

RESUMO

Philadelphia negative myeloproliferative neoplasms [MPN Ph (-)] and monoclonal gammopathies (MG) stem from different hematopoietic progenitor lines. The association between both has a frequency between 3 to 14%, and it has been associated with a higher risk of thrombosis. This study aimed to describe the clinical characteristics of patients with both entities at our center. METHODS: Retrospective observational study of case series. The MPN Ph (-) database of our center between 2015 and 2020 was consulted. The clinical records were reviewed, obtaining demographic, clinical, and management determinants. Descriptive statistical analysis was performed. RESULTS: Among 144 patients, 6 patients diagnosed with MG and MPN were found, all of them female. The median age was 71 years at diagnosis of MPN Ph (-) and 70 years at diagnosis of MG. Two were diagnosed concomitantly with both pathologies, in 2 the MG preceded the MPN, and in 2 the MPN was previously diagnosed. No patient has progressed to acute leukemia or myelofibrosis. Regarding the treatments received, all received Hydroxicarbamide, some with aspirin and one with anticoagulation. Of the MGs, one patient with solitary bone plasmacytoma progressed to multiple myeloma, requiring treatment after 2 years. Two had a thrombotic event, both arterials. CONCLUSION: The observed MG frequency of 4% was similar to what was expected for the age of the patients. Although it is noteworthy that 2 had thrombotic events, further studies are needed to evaluate this association.


Assuntos
Transtornos Mieloproliferativos , Humanos , Feminino , Estudos Retrospectivos , Idoso , Pessoa de Meia-Idade , Transtornos Mieloproliferativos/complicações , Paraproteinemias/complicações , Idoso de 80 Anos ou mais , Masculino
9.
Am J Hematol ; 97(7): 877-884, 2022 07.
Artigo em Inglês | MEDLINE | ID: mdl-35389534

RESUMO

Monoclonal gammopathy of renal significance (MGRS) is a recognized clinical entity. Literature regarding treatment and its outcomes in MGRS is sparse due to the rarity and misdiagnosis of MGRS. We retrospectively analyzed 280 adults with an MGRS diagnosis from 2003 to 2020 across 19 clinical centers from 12 countries. All cases required renal biopsy for the pathological diagnosis of MGRS. Amyloidosis-related to MGRS (MGRS-A) was present in 180 patients; nonamyloidosis MGRS (MGRS-NA), including a broad spectrum of renal pathologies, was diagnosed in 100 patients. The median overall survival in the studied cohort was 121.0 months (95% CI: 105.0-121.0). Patients with MGRS-A had a shorter overall survival than patients with MGRS-NA (HR = 0.41, 95%CI: 0.25-0.69; p = 0.0007). Both hematologic and renal responses were associated with longer survival. Achievement of ≥VGPR was generally predictive of a renal response (OR = 8.03 95%CI: 4.04-115.96; p < 0.0001), one-fourth of patients with ≥VGPR were renal nonresponders. In MGRS-A, factors associated with poor prognosis included elevated levels of creatinine, beta-2-microglobulin, and hemodialysis at diagnosis. In MGRS-NA, only age >65 years was associated with increased risk of death. Treatments provided similar hematologic response rates in both types of MGRS. Autologous stem cell transplantation led to better response than other treatments. This multicenter and international effort is currently the largest report on MGRS.


Assuntos
Transplante de Células-Tronco Hematopoéticas , Nefropatias , Gamopatia Monoclonal de Significância Indeterminada , Paraproteinemias , Lesões Pré-Cancerosas , Adulto , Idoso , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Humanos , Nefropatias/etiologia , Nefropatias/patologia , Nefropatias/terapia , Gamopatia Monoclonal de Significância Indeterminada/complicações , Gamopatia Monoclonal de Significância Indeterminada/diagnóstico , Gamopatia Monoclonal de Significância Indeterminada/terapia , Paraproteinemias/diagnóstico , Prognóstico , Estudos Retrospectivos , Transplante Autólogo/efeitos adversos
10.
Rev Med Chil ; 150(10): 1401-1406, 2022 Oct.
Artigo em Espanhol | MEDLINE | ID: mdl-37358100

RESUMO

Sulfhemoglobin (SulfHb) is formed by hemoglobin (Hb) oxidation by sulfur compounds. Sulfhemoglobinemia is mainly associated with drugs or intestinal bacterial overgrowth. Patients present with central cyanosis, an abnormal pulse oximetry and normal arterial oxygen partial pressure. These features are shared with methemoglobinemia (MetHb) whose diagnosis requires an arterial co-oximetry. Depending on the device used, SulfHb may produce interference with this technique. We report two females aged 31 and 43 years, consulting at the emergency room with cyanosis. Both had a history of acute and chronic, high dose zopiclone ingestion. Pulse oximetry showed desaturation but with normal arterial oxygen partial pressure. Cardiac and pulmonary diseases were ruled out. Co-oximetry in two different analyzers showed interference or normal MetHb percentages. No other complications ensued, and cyanosis decreased over days. Since MetHb was discarded among other causes of cyanosis in a compatible clinical context, the diagnosis of sulfhemoglobinemia was made. The confirmatory method is not available in Chile. The presence of SulfHb is difficult to diagnose, confirmatory tests are not readily available, and it frequently interferes with arterial co-oximetry. This is attributed to a similar absorbance peak of both pigments in arterial blood. Venous co-oximetry can be useful in this context. SulfHb is a self-limited condition in most cases, however it must be differentiated from methemoglobinemia to avoid inappropriate treatments like methylene blue.


Assuntos
Metemoglobinemia , Sulfemoglobinemia , Feminino , Humanos , Metemoglobinemia/induzido quimicamente , Metemoglobinemia/diagnóstico , Sulfemoglobinemia/complicações , Oximetria/efeitos adversos , Cianose/complicações , Oxigênio
11.
Rev Med Chil ; 150(9): 1260-1265, 2022 Sep.
Artigo em Espanhol | MEDLINE | ID: mdl-37358138

RESUMO

Hereditary transthyretin amyloidosis is a multisystemic autosomal dominant genetic disorder characterized by progressive distal sensory-motor polyneuropathy or restrictive cardiomyopathy, secondary to amyloid deposits. Its pathogenesis lies in the TTR gene mutation, and the Val50Met mutation is the most frequent. Patients have significant differences in the onset and severity of clinical presentation according to their country of origin. The diagnosis of this pathology is complex, even more in countries where it is not considered endemic. However, early suspicion and management are essential to improve survival and avoid unnecessary diagnostic and therapeutic strategies. We report a 69-year-old woman who presented a sensory-motor polyneuropathy, predominantly sensory, associated with distal neuropathic pain and bilateral vitritis. The history of her Italian father with polyneuropathy of unspecified etiology stood out. A vitreous biopsy identified amyloid substance deposits (congo red positive). These were also confirmed on a superficial peroneal nerve biopsy. During the etiological study of her polyneuropathy, an increased Kappa/Lambda index of 2.55 mg/L stood out. Therefore, light chain amyloidosis was suspected, and chemotherapy treatment was indicated without favorable response. After 10 years of progressive neurological and ophthalmological involvement, a genetic study confirmed the first case of late-onset hereditary transthyretin amyloidosis Val50Met with polyneuropathy in Chile.


Assuntos
Neuropatias Amiloides Familiares , Polineuropatias , Humanos , Feminino , Idoso , Pré-Albumina/genética , Neuropatias Amiloides Familiares/complicações , Neuropatias Amiloides Familiares/diagnóstico , Neuropatias Amiloides Familiares/genética , Mutação , Polineuropatias/etiologia , Polineuropatias/genética
12.
Rev Med Chil ; 149(11): 1532-1538, 2021 Nov.
Artigo em Espanhol | MEDLINE | ID: mdl-35735314

RESUMO

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.


Assuntos
Transtornos Mieloproliferativos , Policitemia Vera , Mielofibrose Primária , Trombocitemia Essencial , Idoso , Chile/epidemiologia , Humanos , Janus Quinase 2/genética , Mutação , Transtornos Mieloproliferativos/diagnóstico , Transtornos Mieloproliferativos/epidemiologia , Transtornos Mieloproliferativos/genética , Policitemia Vera/diagnóstico , Policitemia Vera/epidemiologia , Policitemia Vera/genética , Mielofibrose Primária/diagnóstico , Mielofibrose Primária/epidemiologia , Mielofibrose Primária/genética , Trombocitemia Essencial/diagnóstico , Trombocitemia Essencial/genética
13.
Rev Med Chil ; 149(12): 1687-1693, 2021 Dec.
Artigo em Espanhol | MEDLINE | ID: mdl-35735335

RESUMO

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.


Assuntos
Transtornos Mieloproliferativos , Policitemia Vera , Mielofibrose Primária , Trombocitemia Essencial , Idoso , Chile/epidemiologia , Humanos , Janus Quinase 2/genética , Mutação , Transtornos Mieloproliferativos/diagnóstico , Transtornos Mieloproliferativos/epidemiologia , Transtornos Mieloproliferativos/genética , Policitemia Vera/diagnóstico , Policitemia Vera/epidemiologia , Policitemia Vera/genética , Mielofibrose Primária/diagnóstico , Mielofibrose Primária/epidemiologia , Mielofibrose Primária/genética , Trombocitemia Essencial/diagnóstico , Trombocitemia Essencial/genética
14.
Nephrology (Carlton) ; 25(6): 442-449, 2020 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31825549

RESUMO

BACKGROUND: Monoclonal gammopathy of renal significance (MGRS)-related lesions are infrequent entities. There are no publications on these disorders in Latin America (LA). The aim of this study was to describe epidemiological and clinical characteristics of these patients in LA. METHODS: We performed a multicentre retrospective study. Patients with diagnosis of MGRS between 2012 and 2018 were included. Epidemiological and clinical data were collected from clinical records. RESULTS: Twenty-seven patients from Chile, Argentina, Ecuador and Uruguay were included. Half debuted with a nephrotic syndrome, and 32% required dialysis. Proliferative glomerulonephritis with monoclonal immunoglobulin deposits was found in 33%, amyloidosis in 26% and monoclonal immunoglobulin deposition disease also in 26%. The immunoglobulin most frequently found in renal biopsies was IgG kappa. In 67% a paraprotein was found. Twenty patients received an anti-plasma cell regimen, and 3 a rituximab-based regimen (IgM-MGRS). Renal response (RR) was achieved in 56%. Early treatment (≤3 months) was associated with higher RR (75% vs 43%). Three patients relapsed within 21.5 months, and 3 progressed: 1 to multiple myeloma, 1 to systemic amyloidosis and another to systemic light-chain deposition disease. Two patients died, both due to infection during induction treatment. CONCLUSION: There was a higher than expected frequency of patients requiring dialysis. The most common MGRS-related lesion was PGNMD. Early treatment was associated with better response. As a rare disease, increasing awareness and promoting early diagnosis are necessary in LA to improve outcomes. SUMMARY AT A GLANCE A collection of 27 cases of MGRS from Latin America with information on epidemiology, clinical characteristics, treatment and outcome of patients diagnosed of MGRS-related renal lesions.


Assuntos
Nefropatias/epidemiologia , Paraproteinemias/complicações , Adulto , Idoso , Progressão da Doença , Feminino , Glomerulonefrite/epidemiologia , Glomerulonefrite/terapia , Humanos , Nefropatias/terapia , América Latina/epidemiologia , Masculino , Pessoa de Meia-Idade , Paraproteinemias/terapia , Diálise Renal , Estudos Retrospectivos
15.
Hematol Oncol ; 37(1): 47-53, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30117170

RESUMO

The aim of the study was to describe the clinical and epidemiological characteristics, anatomic and histologic distribution, and treatment results of extranodal lymphomas (ENLs), diagnosed and treated in the public health system in Chile. We included patients with ENL diagnosed from 1998 to 2014, in 17 cancer centers, registered prospectively in the database of the National Adult Cancer Program (PANDA) of the Ministry of Health. Treatment was based on the local protocols for each lymphoma subtype. Extranodal lymphoma was documented in 1215 of 4907 non-Hodgkin lymphomas diagnosed in that period (25%). Median age was 59 years (range, 16-95), and 55% were female. The gastrointestinal (GI) tract was the most common location (38%), followed by the head and neck (24%) and the skin (15%). B-cell lymphomas accounted for 78% of cases, diffuse large B-cell lymphoma being the most common histologic subtype (68%). Mycosis fungoides/Sezary syndrome was the most frequent T-cell subtype (36%), followed by NK/T-cell lymphomanasal type (24%). In comparison with western countries, Chile showed a significantly high prevalence of NK/T-cell lymphoma nasal type, while the frequency of B-cell ENL and the anatomic distribution appeared similar, being GI the most commonly involved site.


Assuntos
Atenção à Saúde , Linfoma Extranodal de Células T-NK/epidemiologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Biomarcadores , Biópsia , Chile/epidemiologia , Feminino , Humanos , Linfoma Extranodal de Células T-NK/diagnóstico , Linfoma Extranodal de Células T-NK/mortalidade , Masculino , Pessoa de Meia-Idade , Imagem Multimodal/métodos , Estadiamento de Neoplasias , Avaliação de Resultados em Cuidados de Saúde , Vigilância em Saúde Pública , Sistema de Registros , Adulto Jovem
16.
Rev Med Chil ; 147(8): 1036-1041, 2019 Aug.
Artigo em Espanhol | MEDLINE | ID: mdl-31859969

RESUMO

Hematological neoplasms are tumors of cells in different states of maturation and differentiation. Since monoclonal gammopathies (MG) refer to B mature lymphocyte neoplasms, lymphogenesis should be well known. We must keep in mind that the last stage of maturation of these lymphocytes is the plasma cell. This is how a MG could appear in the context of a plasma cell neoplasm, such as multiple myeloma or amyloidosis, but also in relation to a lymphoma. A monoclonal peak is produced by mature B lymphocytes or plasma cells that secrete a monoclonal protein (Immunoglobulin), and represents a MG. But it must be emphasized that, in the correct clinical context, a hypogammaglobulinemia can represent a MG as well. Another important point is the understanding and interpretation of requested tests, such as protein electrophoresis (PEP), immunofixation (IFx) or serum free light chains (sFLC). The current MG screening panel includes these three studies (PEF, IFx, sFLC), although a simpler panel measuring PEF and sFLC has also been proposed, but not yet formally validated. Therefore, screening done only with PEP is insufficient.


Assuntos
Neoplasias de Plasmócitos/sangue , Paraproteinemias/sangue , Paraproteínas/análise , Linfócitos B/metabolismo , Eletroforese das Proteínas Sanguíneas/métodos , Humanos , Neoplasias de Plasmócitos/diagnóstico , Paraproteinemias/diagnóstico
17.
Rev Med Chil ; 147(3): 275-280, 2019 Mar.
Artigo em Espanhol | MEDLINE | ID: mdl-31344163

RESUMO

BACKGROUND: Waldenström macroglobulinemia (WM) is an uncommon indolent B-cell lymphoma, due to the proliferation of lymphoplasmacytic cells, and secretion of a monoclonal IgM protein. AIM: To evaluate the clinical characteristics, management and results of treatment of patients with WM at a public hospital in Chile. PATIENTS AND METHODS: Review of medical records of 31 patients aged 43 to 85 years (16 males) with WM diagnosed between 2002 and 2017. Clinical features and survival were recorded. RESULTS: All patients had bone marrow compromise, and 31%, extranodal involvement. According to the International Prognostic Score System for WM (IPSSWM) 16, 58 and 26% were at low, intermediate and high risk, respectively. Twenty-five patients (81%) were treated, 32% with plasmapheresis and 36% with rituximab. Four cases (16%) achieved complete remission. Median follow up was 35 months (range 6-159). Estimated overall survival (OS) at 5 and 10 years was 74% and 53%, respectively. According to IPSSWM, the estimated five-year OS was 80, 92 and 39%, for low, intermediate and high-risk patients, respectively. CONCLUSIONS: OS was similar to that reported abroad, except for low risk patients, probably due to the low number of cases and short follow up. An improved survival should be expected with the routine use of immunochemotherapy.


Assuntos
Macroglobulinemia de Waldenstrom/diagnóstico , Adulto , Idoso , Idoso de 80 Anos ou mais , Antineoplásicos Imunológicos/uso terapêutico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Biópsia , Medula Óssea/patologia , Chile/epidemiologia , Ciclofosfamida/uso terapêutico , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prednisona/uso terapêutico , Estudos Retrospectivos , Rituximab/uso terapêutico , Taxa de Sobrevida , Resultado do Tratamento , Vincristina/uso terapêutico , Macroglobulinemia de Waldenstrom/tratamento farmacológico , Macroglobulinemia de Waldenstrom/mortalidade
18.
Rev Med Chil ; 147(7): 836-841, 2019 Jul.
Artigo em Espanhol | MEDLINE | ID: mdl-31859981

RESUMO

BACKGROUND: Autoimmune hemolytic anemia (AIHA) is an uncommon disease. In its presentation, it can be severe and even lethal. There is only one clinical report concerning this pathology in Chile. OBJECTIVE: To describe the clinical characteristics and evolution of adult AIHA inpatients. MATERIALS AND METHODS: Retrospective review of clinical records of adult AIHA inpatients between January 2010 and June 2018 was done. Demographic, clinical, laboratory and therapeutic information was analyzed. A descriptive, analytical and survival analysis was performed. RESULTS: Forty-three adult patients diagnosed with AHIA were hospitalized in a period of 8 years. Median age was 63 years (range 22-86 years), mostly women (72%). Warm antibodies were detected in 36 cases (84%) and cold antibodies in seven. Seventy two percent of the patients had an underlying cause, and 58% were secondary to lymphoproliferative neoplasms. All patients except two, received steroids as initial treatment, with response in 37 (90%) of them. Three refractory patients received rituximab, with response in all of them, and relapse in one. Median follow-up was 38 months (range 2-98 months). Five year overall survival was 72%. CONCLUSION: AHIA in adults inpatients is a heterogeneous disease, mainly due to warm antibodies, and to secondary etiology.


Assuntos
Anemia Hemolítica Autoimune/diagnóstico , Adulto , Idoso , Idoso de 80 Anos ou mais , Anemia Hemolítica Autoimune/mortalidade , Anemia Hemolítica Autoimune/terapia , Azatioprina/administração & dosagem , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Rituximab/administração & dosagem , Esplenectomia , Análise de Sobrevida , Adulto Jovem
19.
Rev Med Chil ; 147(11): 1374-1381, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-32186597

RESUMO

Background Renal failure (RF) is a common complication in patients with newly diagnosed multiple myeloma (NDMM). Aim To evaluate the frequency of RF in NDMM patients, and the prognostic impact of its reversibility. Material and Methods A retrospective study evaluating demographic and clinical characteristics of 154 consecutive patients with NDMM was carried out. Estimated glomerular filtration rate (eGFR) was calculated at the beginning and at the end of the induction therapy. In addition, we evaluated renal responses (RR) according to the International Myeloma Working Group (IMWG) criteria. The induction regimen was based on thalidomide in all cases. Results Fifty-three patients had RF (34.4%). Complete renal response (RR) was achieved in 51%. Three years overall survival in patients without RF, with RF and complete RR, and patients with RF and any other RR, was 66, 47 and 13%, respectively. Median survival was 53, 27 and 6 months, respectively (p < 0.01). In the multivariate analysis, RF and hypercalcemia were independent predictors of a worse outcome. Conclusions Achieving a complete RR in patients with NDMM, is associated with a better survival.


Assuntos
Mieloma Múltiplo/complicações , Insuficiência Renal/etiologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Protocolos de Quimioterapia Combinada Antineoplásica , Feminino , Taxa de Filtração Glomerular , Humanos , Masculino , Pessoa de Meia-Idade , Mieloma Múltiplo/tratamento farmacológico , Estadiamento de Neoplasias , Prognóstico , Indução de Remissão , Estudos Retrospectivos , Análise de Sobrevida
20.
Rev Med Chil ; 147(1): 61-64, 2019.
Artigo em Espanhol | MEDLINE | ID: mdl-30848766

RESUMO

BACKGROUND: Cytogenetic abnormalities observed in the bone marrow of patients with multiple myeloma (MM) are an important prognostic factor for risk stratification. AIM: To investigate karyotype characteristics and frequency of the high-risk cytogenetic abnormalities t(4;14), t(14;16) and del(17p) in Chilean patients with MM. MATERIAL AND METHODS: We studied 30 patients with MM by conventional cytogenetics (CC) and fluorescent in situ hybridization of plasma cells selected using cytoplasmic immunoglobulin staining (cIg-FISH). RESULTS: Overall, the two techniques in combination allowed us to identify clonal genetic abnormalities in 47% of patients. The t(4;14) abnormality was observed in 19% of patients, del(17p) was observed in 10% of patients, and t(14;16) was not detected. CONCLUSIONS: Our results showed frequencies of high-risk abnormalities similar to those reported abroad. Cytogenetic studies should be performed routinely for all MM patients at the moment of diagnosis.


Assuntos
Aberrações Cromossômicas , Mieloma Múltiplo/genética , Adulto , Idoso , Chile , Análise Citogenética/métodos , Feminino , Humanos , Hibridização in Situ Fluorescente/métodos , Cariótipo , Masculino , Pessoa de Meia-Idade , Valores de Referência , Medição de Risco/métodos , Fatores de Risco
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