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An elevated HScore is associated with poor clinical outcomes in COVID-19
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Benavente, Rafael

; Peña, Camila

; Cid, Allyson

; Cabello, Nicolás

; Bustamante, Pablo

; Álvarez, Marco

; Henríquez, Elizabeth

; Soto, Andrés

; Rubilar, Erika

Revista médica de Chile

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Fev 2023, Volume 151 Nº 1 Páginas 15 - 22

Resumo: En Es | Texto: En Es | PDF: En | PDF: Es

ABSTRACT 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.

Antecedentes: Los pacientes con Enfermedad por Coronavirus 2019 (COVID-19), experimentan frecuentemente un síndrome hiperinflamatorio que lleva a resultados desfavorables. Esta situación se asemeja al Síndrome Hemofagocítico Secundario (sHLH) descrito en enfermedades neoplásicas, reumatológicas y por otros agentes infecciosos. Un sistema simple de puntaje (HScore) que evalúa inmunosupresión, temperatura organomegalia, citopenias, ferritina, triglicéridos, fibrinógeno y AST ha sido validado para el diagnóstico de sHLH y ha sido propuesto recientemente para evaluar la hiperinflamación en COVID-19. Objetivo: Medir la frecuencia de sHLH entre pacientes con COVID-19 hospitalizados, y evaluar a HScore como una herramienta pronóstica. Material y Métodos: Ciento cuarenta y tres pacientes de 21 a 100 años (64% hombres) fueron ingresados en este estudio de cohorte prospectivo, unicéntrico. Se calculó HScore dentro de las primeras 72 horas desde el ingreso, y se midió la incidencia de sHLH durante la hospitalización. Adicionalmente, se evaluó la relación entre HScore ≥ 130 puntos y un desenlace compuesto de ventilación mecánica o muerte a los 60 días. Resultados: La mediana de HScore fue 96 (33-169) puntos. Un paciente fue diagnosticado con sHLH (incidencia 0,7%). Luego de ajustar por edad, sexo, comorbilidades y obesidad, un HScore ≥ 130 se asoció de manera independiente con el desenlace compuesto. Conclusiones: El sHLH no es frecuente en los pacientes con COVID-19. El uso de HScore puede ser útil para predecir el riesgo de desenlaces clínicos desfavorables.

https://doi.org/10.4067/s0034-98872023000100015

Sulfohemoglobinemia secundaria a zopiclona. Casos clínicos
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Benavente, Rafael

; Parada, Natalia

; Sánchez, Bárbara

; Meneses, Rodrigo

; Torres, Sebastián

; Pineda, Camila

; Aguirre, Herman

; Peña, Camila

Revista médica de Chile

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Out 2022, Volume 150 Nº 10 Páginas 1401 - 1406

Resumo: Es En | Texto: Es En | PDF: Es | PDF: En

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.

Overall survival in Chilean patients with lymphoma and human immunodeficiency virus: A retrospective cohort study
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Benavente, Rafael

; Peña, Camila

; Soto, Andrés

; Valladares, Ximena

; Puga, Bárbara

; Cabrera, María Elena

Hematology, Transfusion and Cell Therapy

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Out 2022, Volume 44 Nº 4 Páginas 497 - 503

Resumo: En | Texto: En | PDF: En

ABSTRACT Introduction: People living with human immunodeficiency virus (PLWH) still face high morbidity and mortality resulting from lymphoma. Aim: To describe a population of PLWH and lymphoma in a Chilean public hospital and compare the overall survival (OS) with a previously reported cohort from the same institution. Methods: Retrospective single-center cohort study. All the patients diagnosed between 2010 and 2017 were included. Demographic and clinical variables were obtained from medical records. The overall survival (OS) was estimated in treated patients from diagnosis until death or October 2020. The OS was then compared with a cohort of patients diagnosed between 1992 and 2008. Main Results: Eighty-four patients were included. The most common histological types were Burkitt's lymphoma (BL), diffuse large B-cell lymphoma (DLBCL), Hodgkin's lymphoma (HL) and plasmablastic lymphoma (PBL) at 31%, 27%, 21% and 14%, respectively. The three-year OS for the whole cohort of BL, DLBCL, HL and PBL was 58.9%, 65.2%, 47.4%, 76.4% and 50%, respectively. Compared to the cohort of 1992 to 2008, a global increase in the OS was found after excluding HL and adjusting for age and clinical stage (HR 0.38, p = 0.002). However, when the main types were analyzed individually, the increase in the OS was statistically significant only in DLBCL (HR 0.29, p = 0.007). Most patients with DLBCL received CHOP chemotherapy, as in the previous cohort. Conclusion: The OS has improved in this population, despite no major changes in chemotherapy regimens, mainly due to the universal access to antiretroviral therapy.

https://doi.org/10.1016/j.htct.2021.06.007

Polineuropatía por amiloidosis por transtiretina de inicio tardío. Caso clínico
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Matamala, José Manuel

; Peña, Camila

; Moreno-Roco, Javier

; Álvarez, Jaime

; Villegas, Pablo

; Stuardo, Andres

; Puga, Bárbara

; Valjalo, Ricardo

; Correa, Gonzalo

; Jeraldo, Cecilia

; Méndez, Gonzalo

; Larrondo, Jorge

; Gosch, Marianne

; Carrasco, Rodrigo

Revista médica de Chile

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Set 2022, Volume 150 Nº 9 Páginas 1260 - 1265

Resumo: Es En | Texto: Es En | PDF: Es | PDF: En

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.

Características clínicas y epidemiológicas de las neoplasias mieloproliferativas Philadelphia negativas en el sistema público de salud de Chile
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Valladares, Ximena

; Benavente, Rafael

; Rojas, Christine

; Peña, Camila

; Valenzuela, Rodrigo

; Monardes, Virginia

; López, Hernán

; Abarca, Marcelo

; León, Pilar

; Osorio, Rocío

; Pérez, Erika

; Soto, Pablo

; Rocca, Gabriel La

; Cardemil, Daniela

; Torres, Vivianne

; Intriago, Marvila

; Cabrera, María Elena

; Undurraga, María Soledad

Revista médica de Chile

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Nov 2021, Volume 149 Nº 11 Páginas 1532 - 1538

Resumo: En Es | Texto: En Es | PDF: En | PDF: Es

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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Grupo de Estudio Latinoamericano de Linfoproliferativos (GELL) para el manejo del linfoma en estado de pandemia SARS CoV-2 / COVID 19
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Ospina-Idárraga, Juan Alejandro

; Martínez-Cordero, Humberto

; Idrobo-Quintero, Henry

; Ortiz-Alfaro, Conrad

; Martínez-Correa, Lina María

; García, Juan Esteban

; Malpica, Luis

; Castillo, Jorge

; Torres, María Alejandra

; Villela, Luis

; Candelaria, Myrna

; Glasenapp, Alana Von

; Beltrán, Brady

; Paredes, Sally

; Castro, Denisse

; Prates, Virginia

; Fiad, Lorena

; Di, Marcos

; Riva, Eloísa

; Abello-Polo, Virginia

; Choque, Juan Antonio

; Peña, Camila

; Rojas, Christine

; Valvert-Gamboa, Fabiola

Revista Colombiana de Cancerología

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Dez 2020, Volume 24 Supl. 1 Páginas 192 - 226

Resumo: Es En | Texto: Es En | PDF: Es | PDF: En

Resumen En diciembre de 2019 se detectó por primera vez en China la existencia del SARS-CoV2, causante de la enfermedad COVID-19. El virus rápidamente se propagó por Europa y Asia, tardándose un par de meses antes de llegar a América Latina. Se ha demostrado que los pacientes que desarrollan una enfermedad severa y que tienen mayor riesgo de mortalidad por COVID-19 son aquellos con edades avanzadas y que presentan por lo menos una enfermedad crónica, incluyendo el cáncer. Debido a lo anterior, surgen muchas dudas en el grupo de profesionales encargados de brindar tratamiento a pacientes con cáncer durante la pandemia, pues se debe equilibrar el riesgo-beneficio de proveer tratamiento a pacientes que se encuentran de base con un riesgo incrementado para tener manifestaciones severas por COVID-19. En este consenso planteamos recomendaciones para los profesionales en hematología que brindan tratamiento a pacientes que padecen de algún tipo de linfoma, con el fin de aclarar el panorama clínico durante la pandemia.

Abstract The existence of SARS-CoV2, the cause of COVID 19 disease, was detected for the first time in China in December 2019. The virus quickly spread across Europe and Asia, taking a couple months to reach Latin America. It has been shown that elderly patients and those with chronic diseases, including cancer, have a higher risk of mortality from COVID-19. Consequently, many doubts arise in the group of health professionals responsible for treating patients with cancer during the pandemic, as they must balance the risk-benefit of delivering treatment to patients with an increased risk for severe manifestations resulting from COVID-19. In this consensus we propose recommendations for hematology professionals who provide treatment to patients suffering from some type of lymphoma, with the aim of clarifying the clinical picture during the pandemic.

https://doi.org/10.35509/01239015.690 437 downloads

Double or triple-expressor lymphomas: prognostic impact of immunohistochemistry in patients with diffuse large B-cell lymphoma
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Peña, Camila

; Villegas, Pablo

; Cabrera, María Elena

Hematology, Transfusion and Cell Therapy

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Jun 2020, Volume 42 Nº 2 Páginas 192 - 193

Resumo: En | Texto: En | PDF: En

https://doi.org/10.1016/j.htct.2019.04.002 367 downloads

Leucemia/Linfoma T del adulto HTLV1, un desafío para el clínico
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Cabrera, María Elena

; Peña, Camila

Revista de la Facultad de Medicina Humana

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Abr 2020, Volume 20 Nº 2 Páginas 295 - 302

Resumo: En Es | Texto: En Es | PDF: En | PDF: Es

ABSTRACT Adult T-cell leukemia/lymphoma (ATL) is an aggressive disease of mature activated T cell caused by human T-cell lymphotropic virus type 1 (HTLV-1). ATL carries a bad prognosis due to intrinsic chemoresistance and severe immunosuppression. The aggressive ATL forms, acute and lymphoma, are treated with chemotherapy associated with antiretroviral agents (AZT/IFN) the acute form. However, they have failed to achieve an impact on survival, that ranges from 8-10 months, respectively. Patients with chronic and smoldering ATL forms, have a better prognosis, but long term survival is poor as well, when these patients are managed with a watchful waiting policy or with chemotherapy. Apparently, AZT/IFN seams to benefit these patients. Meanwhile, prevention of dissemination of HTLV-1, is a must in public health policies, performing screening in blood banks and a screening to pregnant women to reduce/avoid vertical transmission of the virus.

RESUMEN La leucemia / linfoma de células T en adultos (LLTA) es una enfermedad agresiva de células T maduras activadas causada por el virus linfotrópico de células T humano tipo 1 (HTLV-1). ATL tiene un mal pronóstico debido a la quimiorresistencia intrínseca y la inmunosupresión severa. Las formas agresivas de LLTA, aguda y linfoma, se tratan con quimioterapia asociada con agentes antirretrovirales (AZT / IFN). Sin embargo, no han logrado lograr un impacto en la supervivencia, que oscila entre 8 y 10 meses, respectivamente. Los pacientes con formas de LLTA crónicas y latentes tienen un mejor pronóstico, pero la supervivencia a largo plazo también es deficiente, tanto cuando estos pacientes se manejan con una política de espera vigilante o con quimioterapia. Aparentemente, las costuras AZT / IFN benefician a estos pacientes. Mientras tanto, la prevención de la diseminación del HTLV-1 es imprescindible en las políticas de salud pública, tanto por tamizaje del virus en bancos de sangre como a mujeres embarazadas para reducir / evitar la transmisión vertical del virus.

https://doi.org/10.25176/rfmh.v20i2.2919

Mieloma múltiple en Chile: Respuesta a tratamiento en pacientes con mieloma múltiple elegibles para trasplante autólogo de progenitores hematopoyéticos
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PEÑA, CAMILA

; ROJAS-VALLEJOS, JORGE

; ESPINOZA, MARCELA

; DONOSO, JAVIERA

; SOTO, PABLO

; CARDEMIL, DANIELA

; ARANDA, SANDRA

; CONTRERAS, CAROLINA

; VERGARA, CARMEN GLORIA

; LA ROCCA, GABRIEL

; OSORIO, ROCÍO

; LÓPEZ-VIDAL, HERNÁN

; CHANDÍA, MAURICIO

; ROJAS, CHRISTINE

Revista médica de Chile

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Dez 2019, Volume 147 Nº 12 Páginas 1561 - 1568

Resumo: En Es | Texto: En Es | PDF: En | PDF: Es

Background The treatment of choice of newly diagnosed multiple myeloma (NDMM) is an induction with proteasome inhibitors followed autologous stem cell transplantation (HSCT). Since 2013, the treatment of these patients in the public system is based on CTD (cyclophosphamide, thalidomide, and dexamethasone). Aim To evaluate the response rates achieved with CTD, and the results of HSCT in patients with NDMM in the public setting. Material and Methods Data from patients considered as candidates for HSCT from different centers of the National Adult Antineoplastic Drug Program (PANDA, for its acronym in Spanish), diagnosed between 2013 and 2017, was analyzed. The response to treatment of first and second lines of treatment was evaluated, in addition to the results of HSCT. An optimal Response was defined as the sum of strict complete remission, complete remission and very good partial response (sCR, CR and VGPR). Results One hundred and seventy-seven patients were analyzed, 54% women, and 53% with IgG multiple myeloma. Information about the international staging system was retrieved in 127 patients (71%). Seventeen percent were ISS I, 22% in ISS II and 32% ISS III. CTD was used as first treatment in 106 patients (60%), and cyclophosphamide, bortezomib and dexamethasone (CyBorD) in 13 (7%). As first line, CTD had an overall response of 50.9%, and CyBorD of 76.9%. Thirty patients were treated with bortezomib as second line treatment. Forty patients (22%) underwent HSCT. The 5-year Overall Survival (OS) in transplanted patients and non-transplanted patients was 100 and 62% respectively (p < 0.01). Conclusions The response rate achieved by CTD in these patients is suboptimal. The response to CyBorD was better.

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Prognostic impact of renal failure recovery in patients with newly diagnosed multiple mieloma
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PEÑA, CAMILA

; VALLADARES, XIMENA

; GAJARDO, CLAUDIA

; RUSSO, MOISÉS

; MORALES, ÁLVARO

; CORREA, GONZALO

; VALJALO, RICARDO

Revista médica de Chile

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Nov 2019, Volume 147 Nº 11 Páginas 1374 - 1381

Resumo: En Es | Texto: En Es | PDF: En | PDF: Es

ABSTRACT 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.

Antecedentes La falla renal (FR) es una complicación frecuente en pacientes con mieloma múltiple (MM). Objetivo Evaluar la frecuencia de FR en pacientes con reciente diagnóstico de MM y determinar la importancia pronóstica de su reversibilidad. Material y Métodos Se realizó un estudio retrospectivo de 154 pacientes consecutivos con MM. La función renal se evaluó mediante la tasa estimada de filtración glomerular al inicio y final de la terapia de inducción. Además, evaluamos las respuestas renales (RR) de acuerdo con los criterios del International Myeloma Working Group (IMWG). El régimen de inducción se basó en talidomida en todos los casos. Resultados Cincuenta y tres pacientes presentaron FR (34,4%) al diagnóstico. La RR completa se logró en 51%. La sobrevida global (SG) a 3 años en pacientes sin FR, con FR y RR completa, y pacientes con FR y cualquier otra RR, fue de 66, 47 y 13%, respectivamente. La SG media fue de 53, 27 y 6 meses (p < 0,01), respectivamente. En el análisis multivariado, la FR y la hipercalcemia fueron factores independientes de menor sobrevida. Conclusiones Lograr una RR completa en pacientes con MM recién diagnosticado se asocia con una mejor sobrevida.

1121 downloads

11.

AL amyloidosis in the Chilean public health system: a pending debt. Multicenter study of the Chilean Monoclonal Gammopathies Cooperative Group
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Peña, Camila

; González, José Tomás

; López-Vidal, Hernán

; Donoso, Javiera

; Contreras, Carolina

; Vergara, Carmen Gloria

; Hojas, Ricardo

; Soto, Pablo

; Correa, Gonzalo

; Valjalo, Ricardo

; Ríos, Álvaro

; Larrondo, Jorge

; Álvarez, Jaime

; Rojas, Christine

Revista médica de Chile

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Out 2019, Volume 147 Nº 10 Páginas 1239 - 1246

Resumo: En Es | Texto: En Es | PDF: En | PDF: Es

ABSTRACT Background: Immunoglobulin light chain (AL) amyloidosis is a rare and underdiagnosed entity. Aim: To characterize patients with AL amyloidosis in Chilean public health centers. Material and Methods: We conducted a retrospective, multicenter study. Public centers of the Chilean Monoclonal Gammopathies Cooperative Group were asked to search for patients with AL amyloidosis in their databases. Epidemiological, clinical and laboratory characteristics were evaluated. Results: Forty-two patients aged 22 to 84 years were found. Twenty four percent had localized AL amyloidosis; 64% had a lambda light chain clone; 47% were associated with multiple myeloma and 9% with non-Hodgkin lymphoma. The most commonly involved organ was the kidney (76%). Serum free light chains were measured in 31% and an echocardiogram was performed in 74% of patients. Seventeen percent of patients received only palliative care, 17% were treated with bortezomib, 21% with thalidomide, and 40% with melphalan. No patient was transplanted. The mean overall survival (OS) of the group was 19 months. The 5-year OS was 28%. Conclusions: It is important to obtain these realistic, national data to initiate strategies to improve early diagnosis and proper management of this disease.

La amiloidosis AL es una entidad poco frecuente y subdiagnosticada. Mientras todo el mundo discute sobre las nuevas herramientas diagnósticas y terapéuticas, en Chile y en América Latina en general, estamos lejos de esa realidad. El objetivo del presente estudio fue caracterizar a los pacientes con amiloidosis AL en centros del sistema público de nuestro país. Se realizó un estudio retrospectivo, multicéntrico, descriptivo. Los centros públicos del grupo cooperativo hematológico chileno buscaron en sus bases de datos pacientes diagnosticados con amiloidosis AL. Se evaluaron las características epidemiológicas, clínicas y de laboratorio. La edad media fue de 65 años. A 24% de los pacientes se les diagnosticó amiloidosis AL localizada; 64% tuvo paraproteína con cadena ligera lambda; 47% se asoció con mieloma múltiple y 9% con linfoma no Hodgkin. El órgano afectado con mayor frecuencia fue el riñón (76%). Las cadenas ligeras libres de suero se realizaron en 31% y ecocardiograma en 74%. El 17% recibió solo cuidados paliativos, 17% recibió tratamiento con bortezomib, 21% con talidomida y 40% con melfalán. Ningún paciente fue trasplantado. La media de sobrevida global (SG) del grupo fue de 19 meses. La SG a 5 años fue de 28%. Es importante reportar estos resultados nacionales para iniciar estrategias que mejoren tanto el diagnóstico temprano como el tratamiento de esta patología. Por lo tanto, mejorar la sospecha diagnóstica es crucial.

https://doi.org/10.4067/s0034-98872019001001239 1544 downloads

12.

Paraproteínas: claridad en la nebulosa
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Peña, Camila

Revista médica de Chile

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Ago 2019, Volume 147 Nº 8 Páginas 1036 - 1041

Resumo: En Es | Texto: En Es | PDF: En | PDF: Es

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.

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13.

Anemia hemolítica autoinmune en Chile: un análisis retrospectivo de 43 pacientes
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López-Vidal, Hernán

; Peña, Camila

; Gajardo, Claudia

; Valladares, Ximena

; Cabrera C., María Elena

Revista médica de Chile

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Jul 2019, Volume 147 Nº 7 Páginas 836 - 841

Resumo: En Es | Texto: En Es | PDF: En | PDF: Es

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.

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14.

Macroglobulinemia de Waldenström. Experiencia de 15 años en el Hospital del Salvador, Santiago, Chile
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Cardemil, Daniela

; León, Pilar

; Peña, Camila

; Valladares, Ximena

; Cabrera, María Elena

Revista médica de Chile

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Mar 2019, Volume 147 Nº 3 Páginas 275 - 280

Resumo: En Es | Texto: En Es | PDF: En | PDF: Es

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.

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15.

Características citogenéticas y detección de anormalidades de alto riesgo en mieloma múltiple
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Legües, María Eugenia

; Morales, Paulina

; Valenzuela, Mercedes

; Encina, Andrea

; Martí, María José

; Bascuñán, Claudia

; Cornejo, Paulina

; Peña, Camila

; Undurraga, María Soledad

Revista médica de Chile

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2019, Volume 147 Nº 1 Páginas 61 - 64

Resumo: En Es | Texto: En Es | PDF: En | PDF: Es

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.

2760 downloads

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