Clinical features and prognostic factors of elderly patients with mantle cell lymphoma / 中华血液学杂志

Objective: To examine the clinical characteristics and prognostic factors of elderly patients with mantle cell lymphoma (MCL) and the impact of nutrition and underlying diseases on the prognosis of elderly patients with MCL. Methods: retrospectively analyzed 255 elderly patients with MCL from 11 medical centers, including Peking University Third Hospital between January 2000 and February 2021. We analyzed clinical data, such as age, gender, Mantle Cell Lymphoma International Prognostic Index score, and treatment options, and performed univariate and multivariate prognostic analysis. We performed a comprehensive geriatric assessment on elderly MCL patients with medical records that included retraceable underlying disease and albumin levels, and we investigated the impact of basic nutrition and underlying disorders on MCL prognosis in the elderly. Results: There were 255 senior individuals among the 795 MCL patients. Elderly MCL was more common in males (78.4%), with a median age of 69 yr (ages 65-88), and the majority (88.6%) were identified at a late stage. The 3-yr overall survival (OS) rate was 42.0%, with a 21.2% progression-free survival (PFS) rate. The overall response rate (ORR) was 77.3%, with a 33.3% total remission rate. Elderly patients were more likely than younger patients to have persistent underlying illnesses, such as hypertension. Multivariate analysis revealed that variables related with poor PFS included age of ≥80 (P=0.021), Ann Arbor stage Ⅲ-Ⅳ (P=0.003), high LDH level (P=0.003), involvement of bone marrow (P=0.014). Age of ≥80 (P=0.001) and a high LDH level (P=0.003) were risk factors for OS. The complete geriatric assessment revealed that renal deficiency was associated with poorer OS (P=0.047) . Conclusions: Elderly MCL patients had greater comorbidities. Age, LDH, renal function, bone marrow involvement, and Ann Arbor stage are all independent risk factors for MCL in the elderly.

Blastoid mantle cell lymphoma: cutaneous infiltration

Abstract Mantle cell lymphoma is an aggressive B-cell, non-Hodgkin's lymphoma, with lymph node or extranodal origin, and a mean survival of three to five years. Skin involvement is rare, secondary and indicates neoplasia dissemination. The authors report a case of a female patient, 69 years old, diagnosed previously, after lymph node and bone marrow biopsy. She was undergoing the second chemotherapy regimen when she showed infiltrated plaque-like lesions, nodules and tumors on the trunk and thigh root. Histopathology and immunohistochemistry demonstrated cutaneous infiltration of the blastoid lymphoma.

Resultados de pacientes con linfoma del manto: impacto de terapias basadas en citarabina y trasplante hematopoyético / Cytarabine and hematopoietic cell transplantation for mantle cell lymphoma: analysis of 20 patients

Background: Mantle cell lymphoma (MCL) has high relapse and mortality rates. There is a survival benefit when treatment is intensified with cytarabine (AraC), hematopoietic cell transplantation (HCT) and maintenance with rituximab. Aim: To assess the outcomes of patients with MCL treated in a university hospital. Material and Methods: Review of an oncology center database and medical records identifying patients with MCL treated between 2006 and 2017. Death dates were obtained from the death certificate database of the National Identification Service. We analyzed the response rate, overall survival (OS) and progression-free survival (PFS). As a secondary objective, the survival impact of AraC, HCT and maintenance with rituximab, was also analyzed. Results: Information on 20 patients aged 62 ± 11 years, followed for a median of 45 months was retrieved. Eighty-five percent were diagnosed at an advanced stage. The most used first-line regime was R-CHOP in 11 patients, followed by R-HyperCVAD in five. Only 47% achieved complete response. 4-year PFS and OS were of 30 and 77% respectively. Mantle Cell Lymphoma International Prognostic Index (MIPI) significantly predicted PFS and OS. Maintenance with rituximab or HCT was associated with better PFS (48 vs 21 months, p < 0.01). The exposure to AraC or HCT, in refractory or relapsed disease, was associated with an increase in PFS from 9 to 28 months (p = 0,02) and 4-year OS from 40 to 100% (p = 0.05). OS increased even more, from 25 to 100% in those with high-risk MIPI (p = 0.04). Conclusions: The incorporation of AraC, HCT and maintenance with rituximab in the therapeutic backbone of MCL, especially for high-risk cases, was associated with improved survival.

Eficacia y seguridad de ibrutinib en el tratamiento de pacientes con diagnóstico de linfoma de células del manto, con al menos dos líenas de tratamiento previo / Efficacy and safety of ibrutinib in the treatment of patients diagnosed with mantle cell lymphoma, with at least two previous treatment regimens

INTRODUCCIÓN: Antecedentes: El presente dictamen expone la evaluación de tecnología de la eficacia y seguridad del uso de ibrutinib en el tratamiento de pacientes con diagnóstico de linfoma de células del manto. Aspectos Generales: El linfoma de células del manto (LCM) es un tipo muy agresivo de linforma no-Hodgkin que se origina por la malignización de los linfocitos B en el margen externo del folículo de un ganglio linfático o zona del manto y se caracteriza por la translocación cromosomal t)11,14) y la sobre-expresión de la ciclina D1(1). Su incidencia se estima en0.51 casos por cada 100,000 habitantes, representando el 2-10% de todos los linforma no-Hodgkin. Epidemiológicamente se caracteriza por ser más frecuente en varones entre los 60-68 años de edad. Tecnología Sanitaria de Interés: Ibrutinib, un inhibidor covalente de primera generación de la tirosina quinasa de Bruton (BTK, por sus siglas en inglés(, es una molécula pequeña que se une de manera irreversible al residuo 481 de cisteína en el dominio de la quinasa de la tirosina de Bruton de las células B. Adicionalmente, ibrutinib también se puede unir a otros dominios de quinasa que contienen un residuo de cisteína homólogo, tales como HER2, BLK y JAK3 entre otros. El linforma no-Hodgkin de células del manto es un tipo de neoplasia de células B. La quinasa de Bruton se encuentra por debajo de la cascada de señalización del receptor de las células B. La señal enviada por el receptor de dichas células, ya sea dependiente o independiente de antígenos, es considerada una de los principales mecanismos detrás de la progresión de este tipo de neoplasias, ya que juega un rol critico en la supervivencia y proliferación de las células B. METODOLOGÍA: Estrategia de Búsqueda: Se realizó una búsqueda de la literatura con respecto a la eficacia y seguridad del uso de ibrutinib para el tratamiento de LCM en pacientes que han progresado a más de dos lineas de tratamiento previo. Esta búsqueda se realizó utilizando las bases de datos MEDLINES, EMBASE, SCOPUS, WEB OF SCIENCE, CINAHL, COCHRANE y TRIP DATABASE. Adicionalmente, se hizo una búsqueda dentro de la información generada por las principales agencias de tecnologías sanitarias que realizan revisiones sistemáticas (RS), evaluación de tecnologías sanitarias (ETS) y guías de práctica clínica (GPC). RESULTADOS: Sinopsis de la Evidencia: Se realizó la búsqueda bibliográfica y de evidencia científica hasta noviembre 2016 que sustente el uso de ibrutinib en el tratamiento de LCM para pacientes que han recibido dos o más líneas de tratamiento previo. CONCLUSIONES: En el presente dictamen se evaluó la evidencia científica publicada hasta noviembre 2016 en relación al uso de ibrutinib en pacientes con LCM que han recibido más de dos líenas de tratamiento. El Instituto de Evaluación de Tecnologías en Salud e Investigación (IETSI) no aprueba el uso de ibrutinib para el tratamiento de LCM en pacientes con al menos dos líneas de tratamiento.

Hyperinfection by Strongyloides stercoralis probably associated with Rituximab in a patient with mantle cell lymphoma and hyper eosinophilia / Hiperinfección por Strongyloides stercoralis probablemente asociada con Rituximab en una paciente con linfoma e hipereosinofilia

The first report to our knowledge, of hyperinfection by Strongyloides stercoralis (HS) and hypereosinophilia, associated to immune suppression by Rituximab (the only drug received for the last one year and 10 months), in a patient with mantle-cell lymphoma (MCL), is presented. The patient has a 3-year history of MCL, and developed two accesses of HS during 2008, including meningitis, pneumonia and presence of larvae of S. stercoralis in the lungs. We had a unique chance to look at cytotoxicity of filariform larvae in the expectoration after Ivermectin treatment, showing immobilization and death of larvae, associated with eosinophils attached to the cuticle of the parasite.

Se presenta el primer reporte, hasta donde tengamos información, de hiperinfección por Strongyloides stercoralis (HS) e hipereosinofilia asociados a inmunosupresión por Rituximab (el único medicamento recibido durante 1 año y 10 meses), en un paciente con linfoma de células del manto (LCM). La paciente tuvo una historia de 3 años con LCM, y desarrolló 2 accesos de HS durante el 2008, incluyendo meningitis, neumonía y presencia de larvas de S. stercoralis en los pulmones. Se tuvo la oportunidad única de observar la citotoxicidad contra las larvas filariformes en la expectoración, luego del tratamiento con Ivermectina, mostrando la inmovilización y muerte de las larvas, asociada a la presencia de eosinófilos adheridos a la cutícula del parásito.

Staphylococcus warneri meningitis in a patient with Strongyloides stercoralis hyperinfection and lymphoma: first report of a case / Staphylococcus warneri meningite em paciente com hiperinfecção com Strongyloides stercoralis e linfoma: primeiro relato de caso

A case of meningitis due to Staphylococcus warneri in a patient with a hyperinfection with Strongyloides stercoralis possibly associated with rituximab treatment for mantel cell lymphoma is reported for the first time in the literature. The patient was a 59-year-old woman, with a 3-year history of an apparently well controlled lymphoma after treatment with chemotherapy-immunotherapy and then immunotherapy alone, and diagnosis of strongyloidiasis. Meningitis was diagnosed by cerebrospinal fluid culture and tested with an automated plate system. The patient was successfully treated with vancomycin; although fever and productive cough persisted. Severe gastrointestinal symptoms and pneumonia developed three weeks later. Hyperinfection syndrome by S. stercoralis was diagnosed, with abundant larvae in feces and expectoration.

Caso de meningite por Staphylococcus warneri em paciente com hiperinfecção com Strongyloides stercoralis, possivelmente associada com tratamento de rituximab para linfoma de células do manto é relatada pela primeira vez na literatura. A paciente, mulher de 59 anos com história de três anos de linfoma aparentemente bem controlado com tratamento com quimioterapia-imunoterapia e, em seguida, somente imunoterapia e diagnóstico de estrongiloidíase. Meningite foi diagnosticada por cultura do líquido cefalorraquidiano e testada com sistema automatizado de placa. A paciente foi tratada com sucesso com vancomicina, embora a febre e a tosse produtiva não tenham desaparecido. Após graves sintomas gastrointestinais a paciente desenvolveu pneumonia três semanas mais tarde. Síndrome de hiperinfecção por S. stercoralis foi diagnosticada, com larvas abundantes nas fezes e expectoração.

Linfoma de células del manto: manifestación extranodal colorrectal / Mantle-cell lymphoma: extranodal manifestation colorectal

Antecedentes: Los linfomas gastrointestinales agrupan diferentes lesiones y son objeto de permanente revisión. Se los considera de acuerdo a su histología y el estadio evolutivo, hechos que suelen guardar estrecha relación con la clínica, las conductas terapéuticas y el pronóstico. El tubo digestivo está afectado entre un 5 a 15 por ciento en los pacientes que padecen Linfoma no Hodking. El colon y recto son los sitios de menor frecuencia. Representa entre 0,2 a 0,6 por ciento de los tumores malignos colorrectales. El linfoma de células del manto es un subtipo de linfoma de células B, con una frecuencia del 5 al 10 por ciento dentro de los linfomas no Hodking. Objetivo: Presentar el caso de una manifestación colorrectal de linfoma de células del manto, sus características fisiopatológicas y sus alternativas terapéuticas. Pacientes y método: Paciente de 82 años consultó por proctorragia, diarrea crónica, pérdida de peso y mucorrea de más de tres meses de evolución. Se realizó videocolonoscopía constatándose a 10 cm del margen anal lesión mamelonada, friable, ulcerovegetante, extendida hasta sigma con múltiples pólipos sesiles y subpediculados de gran tamaño en cantidad mayor de 100, extendidos en todo el colon. El hallazgo en correlación con la clínica y la endoscopia es compatible con linfoma de células del manto en su forma de afectación extranodal gastrointestinal, y poliposis linfomatosa múltiple. Resultados: Se realizó seis esquemas de quimioterapia bajo la modalidad CHOOP (ciclofosfamida, doxirrubicina, vincristina, prednisona) conjuntamente la aplicación al comienzo de los ciclos con Rituximab (Anticuerpo monoclonal). Se realizó un control endoscópico y tomográfico demostrando remisión completa del compromiso colorrectal, ganglios mesentéricos y retroperitoneales. Se observó persistencia de masas mediastinicas intercavo-aorticas derrame pleural bilateral y dilatación cardiaca...

Background: Gastrointestinal Lymphomas different injuries and are grouped under constant review. They are considered according to their histology and stage of development, events that are closely related to the clinic, the therapeutic behavior and prognosis. The gastrointestinal tract is affected between 5 to 15 per cent in patients with non-Hodgkin lymphoma. The colon and rectum are less frequent sites. It represents between 0.2 and 0.6 per cent of malignant colorectal tumors. The mantle cell lymphoma is a subtype of B-cell lymphoma, with a frequency of 5 to 10 per cent in non-Hodgkin lymphoma. Objective: To present the case of a manifestation colorectal mantle cell lymphoma, pathophysiological characteristics and treatment options. Patients and methods: Patient 82 years consulting with bloody diarrhea, chronic diarrhea, weight loss and mucorrea more than three months of evolution. A colonoscopy was performed, confirmed a 10 cm from the anal injury swellings, extended to multi-sigma subpediculados sessile polyps and large in quantity greater than 100, spread throughout the colon. The finding in correlation with clinical and endoscopy is compatible with mantle cell lymphoma, as extranodal gastrointestinal involvement and multiple lymphomatous polyposis. Results: we performed six schemes in the form CHOOP chemotherapy (cyclophosphamide, doxirrubicina, vincristine, prednisone) combined the application at the beginning of the cycles with Rituximab (monoclonal antibody). We undertook a endoscopy and CT demonstrated complete remission of colorectal disease, commitment, mesenteric and retroperitoneal lymph nodes. Showed persistence of mediastinal masses, bilateral pleural effusion and cardiac dilatation. Today is free from colorectal disease and active monitoring by their serial mediastinal and their heart failure...

A clinicopathologic study of mantle cell lymphoma in a single center study in India.

We present clinical features, histopathology and results of treatment in cases of mantle cell lymphoma (MCL) at our hospital. We had 93 cases (2.1%) of MCL out of total 4301 cases of non-Hodgkin's lymphoma (NHL) in a 4-year period. It included 68 cases (1.7%) of MCL from 3987 cases of NHL diagnosed on histopathology. Remaining 25 cases (7.9%) diagnosed solely on peripheral blood examination were excluded. Thirty-six (85%) patients had advanced-stage disease. Sixty-three were nodal and five were extranodal (all gastrointestinal tract). Common patterns were diffuse (64%), nodular (25%) and mantle zone type (11%). Sixty-two cases had lymphocytic while six had blastic morphology (all nodal). Tumor cells expressed CD20 (100%), CD43 (94%), CD5 (89%) and cyclin D1 (85%). Bone marrow was involved in 25 (59%) cases. Thirty-two patients could be treated. Median recurrence-free survival was 22.23 months. Diffuse pattern of nodal involvement had a lower overall survival.

Mantle cell lymphoma in Thai patients.

Mantle cell lymphoma (MCL) is a disease entity recently introduced into the new lymphoma classification, therefore, the clinical features as well as therapeutic outcomes in Thai patients with MCL has never been described The authors herein retrospectively analysed 21 newly diagnosed patients with MCL at King Chulalongkorn Memorial Hospital from January 1997 to December 2002. The median age of the patients was 54 years (range, 38-79). Male:female was 4:1. Generalized lymphadenopathy (67%) was the most common presenting feature. The majority of patients (85%) had advanced disease (stage III, IV, Ann Arbor system). Fifty-six percent of the patients were classified as the high- and high-intermediate risk group by the international prognostic index. Most patients were treated with CHOP (vincristine, cyclophosphamide, doxorubicin, prednisolone) or CVP (vincristine, cyclophosphamide, prednisolone) regimen. The overall complete remission rate was 59%. With a median follow-up of 13 months (range, 1-62 months), the rates of overall, progression-free and disease-free survivals were 32%, 9% and 20%, respectively. Sixty-seven percent of the patients had relapse/progressive disease after follow-up. In conclusion, the clinical features as well as the outcomes of Thai patients with MCL were comparable to patients in Western countries. Newly diagnosed patients should be treated with novel modalities other than conventional CHOP chemotherapy in order to improve the outcomes.