Caracterización epidemiológica del Linfoma de Hodgkin en pacientes atendidos en el hospital de SOLCA - Guayaqui / Epidemiological characterization of Hodgkin lymphoma in patients treated at the SOLCA - Guayaquil

Introducción: El linfoma de Hodgkin es una enfermedad en la que se forman células malignas en el sistema linfático y que en los últimos años ha venido aumentando su presencia en la población. Objetivo: Caracterizar epidemiológicamente los linfomas de Hodgkin atendidos en el hospital de SOLCA - Guayaquil durante el periodo 2010-2021. Materiales y métodos: Se realizó un estudio de datos abiertos de diseño observacional, descriptivo, de corte transversal, de casos nuevos atendidos con linfoma de Hodgkin diagnosticados en el hospital de SOLCA Guayaquil, entre 2010 y 2021. Resultados: Las atenciones del linfoma de Hodgkin en el hospital de SOLCA Guayaquil fueron del 4 % en el 2010 y del 12 % en el 2021. Se tuvo sobre todo el linfoma de Hodgkin con esclerosis nodular, en hombres; los grupos etarios más frecuentes fueron hombres entre 0 y 19 años (37,7 %) y mujeres entre 20 y 29 años (45,3 %), procedentes de la provincia del Guayas. Conclusiones: Durante este periodo incrementaron las atenciones por linfoma de Hodgkin, en las que se observó más la esclerosis nodular en los pacientes, en hombres de 0 a 19 años y en mujeres de 20 a 39 años, similar al estándar de comportamiento de esta enfermedad

Introduction: Hodgkin lymphoma is a disease in which malignant cells form in the lymphatic system; its presence in the population has been increasing in recent years. Objective: Epidemiological characterization of the Hodgkin lymphomas treated at the SOLCA - Guayaquil hospital during the period 2010­2021. Material and methods: A study was carried out with open data from a cross-sectional observational descriptive design of the new cases of Hodgkin Lymphoma diagnosed and treated at the SOLCA - Guayaquil hospital between 2010 and 2021. Results: Hodgkin lymphoma care at the SOLCA - Guayaquil Hospital went from 4% in 2010 to 12% in 2021. Hodgkin lymphoma with nodular sclerosis was mostly observed in men; however, by age group, it was more frequent in men between 0 and 19 years old (37.7%) and in women between 20 and 29 years old (45.3%) from the Guayas province. Conclusions: During this period, Hodgkin lymphoma has garnered more attention due to an increase in nodular sclerosis cases observed in men aged 0 to 19 and women aged 20 to 39, which aligns with the standard behavior of this disease

Application of the Central Nervous System International Prognostic Index (CNS-IPI) score in daily practice: a retrospective analysis apart from the clinical trial at two centers in Brazil

ABSTRACT Introduction: The diffuse large B-cell lymphoma (DLBCL) is the most common type of non-Hodgkin lymphoma (NHL) and, despite all the progress in this field, central nervous system infiltration (CNSi) still occurs at an incidence of 2-10%. The objective of the present study was to evaluate the Central Nervous System International Prognostic Index (CNS-IPI) score in daily practice regarding the reproducibility in a heterogeneous cohort apart from a clinical trial. Methods: Primary DLBCL patients were eligible for this study, between January 2007 and January 2017. All patients were treated with rituximab-based chemotherapy, mostly R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone). The CNSi was diagnosed by liquor (positive cytology and/or immunophenotype), computerized tomography, magnetic resonance image and/or fluorodeoxy-glucose-positron emission tomography, requested only in symptomatic patients when the CNSi was clinically suspected. The CNS-IPI was assessed by graphical comparison and calibration. Results: After applying the inclusion/exclusion criteria, 322 patients were available for the analysis. The median follow-up was 60 months and the median age was 58 years. Seven patients experienced CNSi, characterizing an incidence of 2.17% (7/322). Comparing groups of patients with and without CNSi, we observed that the lactate dehydrogenase (LDH), number of extranodal sites, IPI, kidney/adrenal and absence of complete response were statistically different. The CNS-IPI model stratified patients in a three-risk group model as low-, intermediate- and high-risk. In our cohort, using the same stratification, we obtained an equivalent the 2-year rate of CNS relapse of 0.0%, 0.8% and 13.8%, respectively. Conclusion: Our study reinforces the reproducibility of the CNS-IPI, specifically apart from clinical trials, and suggests the CNS-IPI score as a tool to guide therapy.

All about blinatumomab: the bispecific T cell engager immunotherapy for B cell acute lymphoblastic leukemia

ABSTRACT Introduction: B cell acute lymphoblastic leukemia-lymphoma (B-ALL) accounts for approximately 75% of ALL cases and is observed in children and adults. Recent advances in disease diagnosis, stratification and prognostication have led to a better characterization of different subgroups of ALL. Notwithstanding the significant improvement in the complete remission rate of B-ALL, patients with minimal residual disease (MRD) and relapsed/refractory (R/R) settings suffer from poor outcomes. Hypothesis: However, novel therapies, such as agents targeting tyrosine kinases or the CD20 molecule, combination therapies and improved supportive care, have changed the treatment landscape of B-ALL. Method and results: Meanwhile, blinatumomab has been FDA-approved for MRD-positive or R/R B-ALL patients. Blinatumomab is a bispecific T cell engager containing the CD3 and CD19 that recognize domains redirecting cytotoxic T cells to lyse B cells. Promising outcomes, including long-term overall survival and improved MRD-negative response rates, have been reported in patients who received this drug. Adding blinatumomab to new ALL regimens seems promising for achieving better outcomes in poor prognosis B-ALL patients. Nevertheless, the neurotoxicity and cytokine release syndrome are the two major adverse events following the blinatumomab therapy. Conclusion: This review summarizes the function and effectiveness of blinatumomab in R/R and MRD positive B-ALL patients. Furthermore, blinatumomab's positive and negative aspects as a novel therapy for B-ALL patients have been briefly discussed.

Avaliação das manifestações clínicas e microscópicas dos linfomas em glândulas salivares maiores / Assessment of clinical and microscopic manifestations of lymphomas in major salivary glands

Os subtipos de linfomas não Hodgkin representam 2,8% de todos os novos casos de câncer no mundo, sendo o terceiro grupo mais comum de neoplasias malignas da região de cabeça e pescoço. As glândulas salivares maiores representam o terceiro sítio extranodal mais acometido pelo linfoma na região da cabeça e pescoço; entretanto, nas glândulas salivares maiores é muito raro, representando aproximadamente 1,7­3,1% de todas as neoplasias das glândulas salivares, acometendo a maioria dos casos as glândulas parótidas (79%), seguidas pelas glândulas submandibulares (18%) e sublinguais (1%). Os subtipos mais comuns são linfoma do tecido linfoide associado à mucosa (MALT), o linfoma folicular (FL) e o linfoma difuso de grandes células B (DLBCL), e a frequência destas neoplasias está associado com a ocorrência simultânea de condições sistêmicas que predispõem ao desenvolvimento de neoplasias linfoides como a Síndrome de Sjögren (SS). Entretanto, a literatura sobre linfomas em glândulas maiores permanece muito escassa e impede que conheçamos de forma apropriada as características destes pacientes. Assim, o objetivo deste estudo é avaliar as manifestações clínicas e microscópicas dos linfomas em glândulas salivares maiores. Para isto, foram recuperados de forma retrospectiva dos arquivos de patologia de algumas instituições todos os casos diagnosticados como linfomas acometendo estes sítios anatômicos. Foram coletados os dados clínicos referentes ao sexo, idade, localização, apresentação clínica, tempo de evolução, status, estadiamento e ocorrência da SS, e as informações histopalógicas foram coletadas de blocos de parafina e lâminas em hematoxilina e eosina e imuno-histoquímicas acessíveis. Os resultados obtidos foram avaliados de forma descritiva. As séries compreenderam de 7 casos de linfomas em glândula sublingual, 16 casos em glândula submandibular e 12 casos em glândula parótida. Clinicamente, os linfomas apresentam-se como aumento de volume assintomático, sendo os subtipos mais frequentes os de células B maduras de baixo grau (MALT, FL, MCL), mas subtipos de alto grau também foram observados (LDGCB, SOE). Dois pacientes, um de linfoma de células do manto (LCM) e outro de LDGCB,SOE em glândula sublingual apresentaram como doença disseminada, e apenas três casos de linfoma MALT em glândula parótida apresentam a SS. O tratamento dependeu do microscópico e estágio do tumor, variando de cirurgia, regimes quimioterápicos com R-CHOP e radioterapia. O prognóstico foi favorável principalmente para os casos de baixo grau (MALT,FL), e apenas dois pacientes de sublingual (LDGCB,SOE, MCL) e três de submandibular (LDGCB,SOE, linfoma plasmablastico e MALT) faleceram após o diagnóstico. Neste estudo concluímos que os linfomas em glândulas salivares maiores são afetados principalmente por neoplasias de células B maduras de baixo grau (MALT, FL, MCL) e esses pacientes devem passar por uma avaliação sistêmica criteriosa para determinar se a doença se trata de uma neoplasia primária ou disseminada.

Non-Hodgkin's lymphomas account for 2.8% of all new cancer cases worldwide and are the third most common group of malignant neoplasms in the head and neck region. The major salivary glands represent the third most common extranodal site affected by lymphoma in the head and neck region; however, in the major salivary glands it is very rare, representing approximately 1.7-3.1% of all salivary gland neoplasms, affecting most cases in the parotid glands (79%), followed by the submandibular glands (18%) and sublingual glands (1%). The most common subtypes are mucosa- associated lymphoid tissue lymphoma (MALT), follicular lymphoma (FL) and diffuse large B-cell lymphoma (DLBCL), and the frequency of these neoplasms is associated with the simultaneous occurrence of systemic conditions that predispose to the development of lymphoid neoplasms such as Sjögren's Syndrome(SS). However, the literature on lymphomas in major glands remains very scarce and prevents us from properly understanding the characteristics of these patients. Therefore, the aim of this study was to evaluate the clinical and microscopic manifestations of lymphomas in the major salivary glands. To this end, all cases diagnosed as lymphomas affecting these anatomical sites were retrospectively retrieved from the pathology archives of a number of institutions. Clinical data was collected on gender, age, location, clinical presentation, time of evolution, status, staging and occurrence of SS, and histopathological information was collected from paraffin blocks and slides in hematoxylin and eosin and accessible immunohistochemistry. The results obtained were evaluated descriptively. The series comprised 7 cases of lymphomas in the sublingual gland, 16 cases in the submandibular gland and 12 cases in the parotid gland. Clinically, the lymphomas presented as asymptomatic enlargement, with the most frequent subtypes being low-grade mature B-cells (MALT, FL, MCL), but high- grade subtypes were also observed (LDGCB, SOE). Two patients, one with mantle cell lymphoma (MCL) and the other with LDGCB,SOE in the sublingual gland presented with disseminated disease, and only three cases of MALT lymphoma in the parotid gland presented with SS. Treatment depended on the microscopic subtype and stage of the tumor, ranging from surgery to chemotherapy regimens with R-CHOP and radiotherapy. Prognosis was mainly favorable for low-grade cases (MALT,FL), and only two sublingual patients (LDGCB,SOE, MCL) and three submandibular patients (LDGCB,SOE, plasmablastic lymphoma and MALT) died after diagnosis. In this study we conclude that lymphomas in the major salivary glands are mainly affected by low- grade mature B-cell neoplasms (MALT, FL, MCL) and these patients should undergo a careful systemic evaluation to determine whether the disease is a primary or disseminated neoplasm.

Microorganismos aislados de hemocultivos en niños con leucemia y linforma: Seis años de seguimiento / Microorganisms isolated from blood cultures in children with leukemia and lymphoma: six years of follow-up

Las Leucemias y linfomas constituyen las enfermedades oncológicas más frecuentes en pediatría y las bacteriemias representan infecciones graves en estos pacientes. Objetivos: describir los microorganismos aislados de sangre en pacientes con leucemia aguda o linfoma pediátrico; comparar la incidencia de aislamientos según enfermedad de base; detallar las variaciones en la incidencia de dichos aislamientos y la evolución de su resistencia antimicrobiana. Estudio retrospectivo, observacional. Se incluyeron 823 episodios de bacteriemia en 467 pacientes pediátricos, entre julio-2016 y junio-2022, dividido en tres períodos (período-1: años 2016- 2018, período-2: años 2018-2020, período-3: años 2020-2022). Se aislaron 880 microorganismos: 55,3% gram negativos (GN), 40% gram positivos (GP) y 4,7% levaduras. En GN predominaron: enterobacterias (72%) y en GP: estreptococos del grupo viridans (SGV) (34,1%). Se encontró asociación entre LLA-enterobacterias (p=0,009) y LMA-SGV (p<0,001). Hubo aumento de GN entre los períodos 1 y 3 (p=0,02) y 2 y 3 (p=0,002) y disminución de GP entre 2 y 3 (p=0,01). Se registraron los siguientes mecanismos de resistencia: BLEE (16,4%), carbapenemasas: KPC (2,5%); MBL (2,7%) y OXA (0,2%); meticilinorresistencia en Staphylococcus aureus (20%) y estafilococos coagulasa negativos (95%), vancomicina resistencia en Enterococcus spp. (39%), SGV no sensibles a penicilina (44%) y a cefotaxima (13%). Hubo aumento de MBL entre los períodos 1 y 2 (p=0,02) y una tendencia en disminución de sensibilidad a penicilina en SGV entre el 1 y 3 (p=0,058). El conocimiento dinámico y análisis de estos datos es esencial para generar estadísticas a nivel local, fundamentales para el diseño de guías de tratamientos empíricos (AU)

Leukemias and lymphomas are the most common cancers in children and bacteremia is a severe infection in these patients. Objectives: to describe the microorganisms isolated from blood in pediatric patients with acute leukemia or lymphoma; to compare the incidence of isolates according to the underlying disease; and to detail the variations in the incidence of these isolates and the evolution of their antimicrobial resistance. Retrospective, observational study. We included 823 episodes of bacteremia in 467 pediatric patients seen between July-2016 and June-2022, divided into three periods (period-1: 2016- 2018, period-2: 2018-2020, period-3: 2020-2022). A total of 880 microorganisms were isolated: 55.3% were gram-negative (GN), 40% gram-positive (GP) and 4.7% yeasts. In GN there was a predominance of: enterobacteria (72%) and in GP viridans group streptococci (VGS) (34.1%). An association was found between ALL-enterobacteria (p=0.009) and AML-VGS (p<0.001). There was an increase in GN between periods 1 and 3 (p=0.02) and 2 and 3 (p=0.002) and a decrease in GP between 2 and 3 (p=0.01). The following resistance mechanisms were recorded: BLEE (16.4%), carbapenemases: KPC (2.5%), MBL (2.7%), and OXA (0.2%); methicillin resistance in Staphylococcus aureus (20%) and coagulase negative staphylococci (95%), vancomycin resistance in Enterococcus spp. (39%), VGS resistant to penicillin (44%) and to cefotaxime (13%). There was an increase in MBL between periods 1 and 2 (p=0.02) and a decreasing trend in penicillin sensitivity in VGS between periods 1 and 3 (p=0.058). Dynamic knowledge and analysis of these data is essential to generate statistics at the local level, which is fundamental for the design of empirical treatment guidelines (AU)

Neurotoxicidad subaguda por metotrexato en pacientes con enfermedad oncohematológica. Reporte de tres casos / Subacute methotrexate neurotoxicity in patients with oncohematological disease. A report of 3 cases

El metotrexato es un fármaco análogo del ácido fólico ampliamente utilizado en el tratamiento de enfermedades autoinmunes, leucemias y linfomas. Su uso puede ocasionar la aparición de múltiples efectos adversos entre los que se encuentran aquellos relacionados con la presencia de toxicidad neurológica, que puede presentarse de forma aguda, subaguda o crónica. La neurotoxicidad subaguda es aquella que ocurre típicamente entre los 2 y los 14 días posteriores a la administración y puede manifestarse con una amplia gama de síntomas neurológicos. En la mayoría de los casos, no recurre con futuras exposiciones al medicamento. Presentamos tres casos de neurotoxicidad subaguda por metotrexato con manifestaciones clínicas diferentes en pacientes oncohematológicos que se internaron entre los años 2018 y 2020. Dos de ellos presentaron recurrencia frente a la nueva administración del fármaco y todos evidenciaron lesiones en resonancia magnética nuclear.

Methotrexate is a folic acid analogue widely used in the treatment of autoimmune diseases, leukemias, and lymphomas. Methotrexate use may cause multiple adverse effects, including those related to the presence of neurological toxicity, which may be acute, subacute, or chronic. Subacute neurotoxicity typically occurs between 2 and 14 days after administration and may present as a wide range of neurological symptoms. In most cases, it does not recur with future exposures to the drug. Here we describe 3 cases of subacute methotrexate neurotoxicity with different clinical manifestations in patients with oncohematological disease who were hospitalized between 2018 and 2020. Two of them showed recurrence with a new drug administration. Lesions were observed in the magnetic resonance imaging tests of all of them.

Linfoma em cão da raça cane corso: relato de caso / Lymphoma in a cane corso dog: case report / Linfoma en un perro cane corso: relato de un caso

Os linfócitos são células de defesa do organismo que funcionam como barreira contra infecções e células cancerígenas, elas circulam pelo sistema linfático e estão presentes por todo o organismo do animal, podem se proliferar de forma maligna, caracterizando o linfoma. Acometem em sua maioria, cães de raças de grande porte, animais de meia idade e idosos. Sendo uma doença de etiologia desconhecida, vários fatores podem contribuir para sua evolução, como deficiências autoimunes, bem como hábitos alimentares ao longo da vida do animal, ou até por predisposição genética. O presente relato de caso, tem o objetivo de mostrar a evolução gradual da doença, quais sinais clínicos o animal poderá apresentar, e como os exames laboratoriais podem nos auxiliar em seu diagnóstico.(AU)

The lymphocytes are defense cells of the body that act as a barrier against infection and cancer cells, they circulate through the lymphatic system and are present throughout the animal's body, and can proliferate in a malignant way, characterizing the lymphoma. They mostly affect large breed dogs, middle-aged and elderly animals. Being a disease of unknown etiology, several factors may contribute to its evolution, such as autoimmune deficiencies, as well as food habits throughout the animal's life, or even genetic predisposition. The present case report has the objective of showing the gradual evolution of the disease, which clinical signs the animal may present, and how laboratory tests can help us in its diagnosis.(AU)

Los linfocitos son células de defensa del organismo que actúan como barrera contra infecciones y células cancerígenas, circulan por el sistema linfático y están presentes en todo el organismo del animal, pudiendo proliferar de forma maligna, caracterizando el linfoma. Afectan sobre todo a perros de razas grandes, animales de mediana edad y ancianos. Siendo una enfermedad de etiología desconocida, varios factores pueden contribuir a su evolución, como deficiencias autoinmunes, así como hábitos alimentarios a lo largo de la vida del animal, o incluso predisposición genética. El presente caso clínico tiene como objetivo mostrar la evolución gradual de la enfermedad, qué signos clínicos puede presentar el animal y cómo las pruebas de laboratorio pueden ayudarnos en su diagnóstico.(AU)

Brentuximab vedotin in ALK-negative anaplastic large cell lymphoma presenting with alar mass: A case report

@#Anaplastic large cell lymphoma (ALCL) is a rare subtype of T-cell non-Hodgkin lymphoma (NHL) primarily involving the lymph nodes; however, extra-nodal manifestations are also common. Diagnosis can be confirmed by a combination of histopathology and immunohistochemical staining. Complete workup and staging include imaging and bone marrow examination. This presents a case of a 55-year-old male with anaplastic lymphoma kinase (ALK) - negative ALCL presenting with an alar mass. ALCL patients often present with rapidly progressing lymphadenopathy. Extra-nodal manifestations commonly involve the skin, liver, lung, and gastrointestinal tract. Biopsy of the mass showed small to medium-sized anaplastic lymphoid cells that stained positive for CD30, LCA (CD45), CD99, and negative for CD20, ALK (CD246), neuron-specific enolase, CD34, CD5, PAX5, TdT, MPO, CD138, EMA, pancytokeratin, CD3 and synaptophysin. These findings were most compatible with an ALK-negative ALCL. The patient was started on a combination of brentuximab vedotin, cyclophosphamide, doxorubicin, and prednisone (BV + CHP) every 21 days for 6 cycles. There was a progressive decrease in the size of the mass, and a resolution was noted after the 5th cycle. FDG-PET/CT scan was done after the 6th cycle of chemotherapy and 6 months after completion of treatment. Both scans showed no evidence of metabolically active nodal or extra-nodal lymphomatous disease. This case showed a unique extra-nodal manifestation of an ALK-negative ALCL presenting as an alar mass with a good response to BV + CHP. However, more evidence is necessary to further establish the role of BV as the first-line treatment regimen for CD30-positive peripheral T-cell lymphoma (PTCL), including ALK-negative ALCL.

Unmasking tumors: A case of a CD30-Negative MycosisFungoides masquerading as Erythema NodosumLeprosumin a filipino male

@#Mycosis Fungoides is the most common type of primary cutaneous lymphoma. Early in its course, it usually presents as erythematous patches and plaques similar to a lot of cutaneous conditions. A 68-year-old male presented with a 13-year history of multiple erythematous patches and plaques on the arms and trunk. The lesions were pruritic and chronically relapsing over the years, temporarily relieved by topical corticosteroids. Thereafter, there was onset of multiple nodules on the trunk, extremities and face. Initial biopsy was done by a different dermatologist, revealed Hansen’s Disease, Borderline Borderline spectrum. Fitefaraco stain was positive but no bacillary index was given. The patient was subsequently started on rifamcpicin 600mg/capsule once a day, Ofloxacin 400mg/tablet once a day, and Minocycline 100mg/capsule once a day. The lesions were noted to worsen, eventually developing ulcerations over the trunk and extremities prompting referral to our institution. The biopsy results were as follows: Cutaneous T-Cell Lymphoma, diffuse cluster of differentiation (CD) 3 staining, focal CD20 staining, and negative FiteFaraco stain. The histopathologic findings combined with the clinical presentation led to the diagnosis of Mycosis Fungoides. He was then referred to medical oncology for proper staging and definitive management. The prognosisof Mycosis Fungoides is generally dependent on the stage as determined by the extent of skin involvement as well as presence of lymph node invasion and/or metastasis.

Clinicopathological characteristics and prognosis of patients with small bowel tumors: A single center analysis of 220 cases / 中华胃肠外科杂志

Objective: To analyze the clinicopathological characteristics and prognosis of patients with small bowel tumors. Methods: This was a retrospective, observational study. We collected clinicopathological data of patients with primary jejunal or ileal tumors who had undergone small bowel resection in the Department of Gastrointestinal Surgery, West China Hospital, Sichuan University between January 2012 and September 2017. The inclusion criteria included: (1) older than 18 years; (2) had undergone small bowel resection; (3) primary location at jejunum or ileum; (4) postoperative pathological examination confirmed malignancy or malignant potential; and (5) complete clinicopathological and follow-up data. Patients with a history of previous or other concomitant malignancies and those who had undergone exploratory laparotomy with biopsy but no resection were excluded. The clinicopathological characteristics and prognoses of included patients were analyzed. Results: The study cohort comprised 220 patients with small bowel tumors, 136 of which were classified as gastrointestinal stromal tumors (GISTs), 47 as adenocarcinomas, and 35 as lymphomas. The median follow-up for all patient was 81.0 months (75.9-86.1). GISTs frequently manifested as gastrointestinal bleeding (61.0%, 83/136) and abdominal pain (38.2%, 52/136). In the patients with GISTs, the rates of lymph node and distant metastasis were 0.7% (1/136) and 11.8% (16/136), respectively. The median follow-up time was 81.0 (75.9-86.1) months. The 3-year overall survival (OS) rate was 96.3%. Multivariate Cox regression-analysis results showed that distant metastasis was the only factor associated with OS of patients with GISTs (HR=23.639, 95% CI: 4.564-122.430, P<0.001). The main clinical manifestations of small bowel adenocarcinoma were abdominal pain (85.1%, 40/47), constipation/diarrhea (61.7%, 29/47), and weight loss (61.7%, 29/47). Rates of lymph node and distant metastasis in patients with small bowel adenocarcinoma were 53.2% (25/47) and 23.4% (11/47), respectively. The 3-year OS rate of patients with small bowel adenocarcinoma was 44.7%. Multivariate Cox regression-analysis results showed that distant metastasis (HR=4.018, 95%CI: 2.108-10.331, P<0.001) and adjuvant chemotherapy (HR=0.291, 95% CI: 0.140-0.609, P=0.001) were independently associated with OS of patients with small bowel adenocarcinoma. Small bowel lymphoma frequently manifested as abdominal pain (68.6%, 24/35) and constipation/diarrhea (31.4%, 11/35); 77.1% (27/35) of small bowel lymphomas were of B-cell origin. The 3-year OS rate of patients with small bowel lymphomas was 60.0%. T/NK cell lymphomas (HR= 6.598, 95% CI: 2.172-20.041, P<0.001) and adjuvant chemotherapy (HR=0.119, 95% CI: 0.015-0.925, P=0.042) were independently associated with OS of patients with small bowel lymphoma. Small bowel GISTs have a better prognosis than small intestinal adenocarcinomas (P<0.001) or lymphomas (P<0.001), and small bowel lymphomas have a better prognosis than small bowel adenocarcinomas (P=0.035). Conclusions: The clinical manifestations of small intestinal tumor are non-specific. Small bowel GISTs are relatively indolent and have a good prognosis, whereas adenocarcinomas and lymphomas (especially T/NK-cell lymphomas) are highly malignant and have a poor prognosis. Adjuvant chemotherapy would likely improve the prognosis of patients with small bowel adenocarcinomas or lymphomas.

Efficiency and safety analysis of Plerixafor combined with granulocyte colony-stimulating factor on autologous hematopoietic stem cell mobilization in lymphoma / 中华血液学杂志

Objective: To evaluate the advantages and safety of Plerixafor in combination with granulocyte colony-stimulating factor (G-CSF) in autologous hematopoietic stem cell mobilization of lymphoma. Methods: Lymphoma patients who received autologous hematopoietic stem cell mobilization with Plerixafor in combination with G-CSF or G-CSF alone were obtained. The clinical data, the success rate of stem cell collection, hematopoietic reconstitution, and treatment-related adverse reactions between the two groups were evaluated retrospectively. Results: A total of 184 lymphoma patients were included in this analysis, including 115 cases of diffuse large B-cell lymphoma (62.5%) , 16 cases of classical Hodgkin's lymphoma (8.7%) , 11 cases of follicular non-Hodgkin's lymphoma (6.0%) , 10 cases of angioimmunoblastic T-cell lymphoma (5.4%) , 6 cases of mantle cell lymphoma (3.3%) , and 6 cases of anaplastic large cell lymphoma (3.3%) , 6 cases of NK/T-cell lymphoma (3.3%) , 4 cases of Burkitt's lymphoma (2.2%) , 8 cases of other types of B-cell lymphoma (4.3%) , and 2 cases of other types of T-cell lymphoma (1.1%) ; 31 patients had received radiotherapy (16.8%) . The patients in the two groups were recruited with Plerixafor in combination with G-CSF or G-CSF alone. The baseline clinical characteristics of the two groups were basically similar. The patients in the Plerixafor in combination with the G-CSF mobilization group were older, and the number of recurrences and third-line chemotherapy was higher. 100 patients were mobilized with G-CSF alone. The success rate of the collection was 74.0% for one day and 89.0% for two days. 84 patients in the group of Plerixafor combined with G-CSF were recruited successfully with 85.7% for one day and 97.6% for two days. The success rate of mobilization in the group of Plerixafor combined with G-CSF was substantially higher than that in the group of G-CSF alone (P=0.023) . The median number of CD34(+) cells obtained in the mobilization group of Plerixafor combined with G-CSF was 3.9×10(6)/kg. The median number of CD34(+) cells obtained in the G-CSF Mobilization group alone was 3.2×10(6)/kg. The number of CD34(+) cells collected by Plerixafor combined with G-CSF was considerably higher than that in G-CSF alone (P=0.001) . The prevalent adverse reactions in the group of Plerixafor combined with G-CSF were grade 1-2 gastrointestinal reactions (31.2%) and local skin redness (2.4%) . Conclusion: The success rate of autologous hematopoietic stem cell mobilization in lymphoma patients treated with Plerixafor combined with G-CSF is significantly high. The success rate of collection and the absolute count of CD34(+) stem cells were substantially higher than those in the group treated with G-CSF alone. Even in older patients, second-line collection, recurrence, or multiple chemotherapies, the combined mobilization method also has a high success rate of mobilization.

Intrathoracic masses in children and adolescents: a single tertiary pediatric institution experience

OBJECTIVES@#The Cancer and Hematology Division of the PCMC receives an average of 24 cases of pediatric intrathoracic masses annually. Comprehensive data on the demographic status, clinical profile, management, and outcome are still not available. This study aims to determine the clinical features, diagnosis, management and outcome of children and adolescents with intrathoracic masses from 2017 to 2019.@*MATERIALS AND METHODS@#Descriptive study design was utilized. Data were collected by doing a chart review. Possible associations between the clinical features and outcome were described.@*RESULTS@#Sixty-eight (68) cases were referred from January 2017 to December 2019. Mean age at diagnosis is 8.8 years with a 2.4:1 male to female ratio. Severe wasting was seen in 21%. All subjects were symptomatic at presentation, 50% with respiratory compromise. Anterior mediastinal lesions are observed at 82% of cases. Elevated LDH was seen in 50% of the patients. Malignant hematologic lesions are the most common etiology. Steroid pretreatment was given in 40% of patients. Only a small percentage (<20%) underwent definitive treatment. Patients were symptomatic for 18 days on average before consult. It took an average of 18 days for a case to be diagnosed definitively, and 10 days from the diagnosis to start of directed treatment. Mortality rate was high at 57.4%@*CONCLUSION@#Patients with intrathoracic mass and malnutrition are 1.4x more likely to die. Diagnosis is the most significant factor associated with death. Observed data can be used as basis to formulate protocols which can streamline the diagnostic and therapeutic approach in these patients.

Development and validation of a CT-based radiomics model for differentiating pneumonia-like primary pulmonary lymphoma from infectious pneumonia: A multicenter study / 中华医学杂志(英文版)

BACKGROUND@#Pneumonia-like primary pulmonary lymphoma (PPL) was commonly misdiagnosed as infectious pneumonia, leading to delayed treatment. The purpose of this study was to establish a computed tomography (CT)-based radiomics model to differentiate pneumonia-like PPL from infectious pneumonia.@*METHODS@#In this retrospective study, 79 patients with pneumonia-like PPL and 176 patients with infectious pneumonia from 12 medical centers were enrolled. Patients from center 1 to center 7 were assigned to the training or validation cohort, and the remaining patients from other centers were used as the external test cohort. Radiomics features were extracted from CT images. A three-step procedure was applied for radiomics feature selection and radiomics signature building, including the inter- and intra-class correlation coefficients (ICCs), a one-way analysis of variance (ANOVA), and least absolute shrinkage and selection operator (LASSO). Univariate and multivariate analyses were used to identify the significant clinicoradiological variables and construct a clinical factor model. Two radiologists reviewed the CT images for the external test set. Performance of the radiomics model, clinical factor model, and each radiologist were assessed by receiver operating characteristic, and area under the curve (AUC) was compared.@*RESULTS@#A total of 144 patients (44 with pneumonia-like PPL and 100 infectious pneumonia) were in the training cohort, 38 patients (12 with pneumonia-like PPL and 26 infectious pneumonia) were in the validation cohort, and 73 patients (23 with pneumonia-like PPL and 50 infectious pneumonia) were in the external test cohort. Twenty-three radiomics features were selected to build the radiomics model, which yielded AUCs of 0.95 (95% confidence interval [CI]: 0.94-0.99), 0.93 (95% CI: 0.85-0.98), and 0.94 (95% CI: 0.87-0.99) in the training, validation, and external test cohort, respectively. The AUCs for the two readers and clinical factor model were 0.74 (95% CI: 0.63-0.83), 0.72 (95% CI: 0.62-0.82), and 0.73 (95% CI: 0.62-0.84) in the external test cohort, respectively. The radiomics model outperformed both the readers' interpretation and clinical factor model ( P <0.05).@*CONCLUSIONS@#The CT-based radiomics model may provide an effective and non-invasive tool to differentiate pneumonia-like PPL from infectious pneumonia, which might provide assistance for clinicians in tailoring precise therapy.

The Risk and Survival Analysis of Multiple Malignancies in Hematologic Malignancy Patients: A Single Chinese Center Retrospective Study, 2009 through 2017 / 中国实验血液学杂志

OBJECTIVE@#To explore the risk and location of multiple malignancies in patients with hematologic malignancies who were followed up for 9 years in Jiangsu Province Hospital and to evaluate the impact of the second primary malignancy on survival of patients.@*METHODS@#The incidence and survival of multiple malignancies in 7 921 patients with hematologic malignancies from 2009 to 2017 were analyzed retrospectively.@*RESULTS@#A total of 180 (2.3%, 180/7 921) patients developed second malignancy, of whom 58 patients were diagnosed with hematologic malignancies as the first primary malignancy, and 98 patients developed hematologic malignancies as second primary malignancy, and the other 24 cases were diagnosed with the second malignancy within 6 months after the first primary malignancy was diagnosed, which was difined as multiple malignancies occurring simultaneously. In 180 patients, 18 cases developed two hematologic malignancies successively, and 11 patients developed more than 3 primary cancers (among them, 2 female patients were diagnosed with 4 primary cancers). Patients with lymphoma and multiple myeloma (MM) as the second primary malignancy had poorer survival than patients with lymphoma and MM as the first primary malignancy. Patients with chronic myeloid leukemia as the second primary malignancy were also associated with inferior overall survival.@*CONCLUSION@#In this study, 2.3% of hematologic malignancy patients had multiple mali-gnancies, lymphoma and MM as the second primary malignancy had poor survival.

Effect of Nucleolin on Lymphoma Proliferation by Regulating Thymidine Kinase 1 / 中国实验血液学杂志

OBJECTIVE@#To investigate the mechanism of nucleolin (NCL) involved in lymphoma proliferation by regulating thymidine kinase 1 (TK1).@*METHODS@#Twenty-three patients with diffuse large B-cell lymphoma (DLBCL) were selected and divided into initial treatment group (14 cases) and relapsed/refractory group (9 cases). Serum TK1 and C23 protein in peripheral blood mononuclear cells were detected. Cell models of CA46-NCL-KD (CA46-NCL-knockdown) and CA46-NCL-KNC (CA46-NCL-knockdown negative control) were established by lentivirus vector mediated transfection in Burkitt lymphoma cell line CA46. The half maximal inhibitory concentration (IC50) of CA46-NCL-KD, CA46-NCL-KNC, and CA46 to adriamycin were detected by cell proliferation assay (MTS). The expression of NCL mRNA and protein in CA46-NCL-KD and CA46-NCL-KNC cells were dectected by Q-PCR and Western blot, respectively. The cell cycle of CA46-NCL-KD, CA46-NCL-KNC, and CA46 cells were detected by flow cytometry. The expression of TK1 protein in CA46-NCL-KD and CA46-NCL-KNC cells was detected by an enhanced chemiluminescence (ECL) dot blot assay.@*RESULTS@#The level of serum TK1 in the initial treatment group was 0.43(0-30-1.01) pmol/L, which was lower than 10.56(2.19-14.99) pmol/L in the relapsed/refractory group (P<0-01), and the relative expression level of NCL protein in peripheral blood was also significantly lower. The IC50 of CA46-C23-KD cells to adriamycin was (0.147±0.02) μg/ml, which was significantly lower than (0.301±0.04) μg/ml of CA46-C23-KNC cells and (0.338±0.05) μg/ml of CA46 cells (P<0.05). Compared with CA46-NCL-KNC cells, the expression of NCL mRNA and protein, TK1 protein decreased in CA46-NCL-KD cells, and the proportion of S phase and G2/M phase also decreased, while G0/G1 phase increased in cell cycle.@*CONCLUSION@#The increased expression of NCL in DLBCL and CA46 cells indicates low sensitivity to drug. NCL may participate in regulation of lymphoma proliferation by affecting TK1 expression, thereby affecting the drug sensitivity.

Multiomics and Multidimensional Testing for Efficacy Monitoring of Patients with Lymphoma / 中国实验血液学杂志

OBJECTIVE@#To explore the role of a new blood-based, multiomics and multidimensional method for evaluating the efficacy of patients with lymphoma.@*METHODS@#10 ml peripheral blood was extracted from each patient, and the genomic copy number aberrations (CNA) and fragment size (FS) were evaluated by low-depth whole genome sequencing of cfDNA, and the level of a group of plasma tumor marker (PTM) were detected at the same time. The cancer efficacy score (CES) was obtained by standardized transformation of the value of above three numerical indexes, and the changes of CES before and after treatment were compared to evaluate the patient's response to the treatment regimen.@*RESULTS@#A total of 35 patients' baseline data were collected, of which 23 cases (65.7%) had elevated CES values. 18 patients underwent the first time test. The results showed that the CES value of 9 patients with positive baseline CES decreased significantly at the first test, and the efficacy evaluation was PR, which was highly consistent with the imaging evaluation results of the same period. At the same time, the CNA variation spectrum of all patients were evaluated and it was found that 23 patients had partial amplification or deletion of chromosome fragments. The most common amplification site was 8q24.21, which contains important oncogenes such as MYC. The most common deletion sites were 1p36.32, 4q21.23, 6q21, 6q27, 14q32.33, and tumor suppressor-related genes such as PRDM1, ATG5, AIM1, FOXO3 and HACE1 were expressed in the above regions, so these deletions may be related to the occurrence and development of lymphoma.@*CONCLUSION@#With the advantages of more convenience, sensitivity and non-invasive, this multiomics and multidimensional efficacy detection method can evaluate the tumor load of patients with lymphoma at the molecular level, and make more accurate efficacy evaluation, which is expected to serve the clinic better.

The Molecular Mechanism of LncRNA HOTAIR Regulating the Proliferation, Invasion and Migration of Lymphoma Cells through Target Gene miR-20a-5p / 中国实验血液学杂志

OBJECTIVE@#To investigate the effects of lncRNA HOTAIR on the proliferation, invasion and migration of lymphoma cells through target gene miR-20a-5p and its molecular mechanism.@*METHODS@#After synthesizing HOTAIR siRNA and siRNA NC plasmids, they were transfected into lymphoma Raji cells, respectively. The expression of HOTAIR mRNA was detected by RT-qPCR. The proliferation, invasion and migration of lymphoma Raji cells were detected by CCK-8 assay, Transwell assay and cell scratch healing assay, respectively. The target gene of lncRNA HOTAIR was predicted by miRcode software, and the relationship between HOTAIR and target gene was analyzed by dual luciferase assay. After synthesis of miR-20a-5p inhibitor and inhibitor NC, Raji cells were transiently transfected. The expression of miR-20a-5p was detected by RT-qPCR, and the effects of down-regulation of miR-20a-5p on the proliferation, invasion and migration of Raji cells were analyzed. The overexpression plasmid of lncRNA HOTAIR and miR-20a-5p mimics were transfected into Raji cells simultaneously to analyze the proliferation, invasion and migration ability of Raji cells. After overexpression or down-regulation of miR-20a-5p, the expression of JAK/STAT3 signaling pathway related proteins was analyzed.@*RESULTS@#HOTAIR expression in Raji cells was decreased after transfection of HOTAIR siRNA (P<0.01), and miR-20a-5p expression was also decreased after transfection of miR-20a-5p inhibitor (P<0.01). HOTAIR had a targeting and negative regulation relationship with miR-20a-5p (r=-0.826). Silencing HOTAIR promoted the expression of miR-20a-5p and inhibited the proliferation, invasion and migration of Raji cells. Down-regulation of miR-20a-5p expression promoted the proliferation, invasion and migration of Raji cells. Effect of HOTAIR overexpression on the proliferation, invasion and migration of Raji cells could be reversed by up-regulation of miR-20a-5p. Down-regulation of miR-20a-5p expression activated the intracellular JAK/STAT3 signaling pathway.@*CONCLUSION@#HOTAIR affects the proliferation, invasion and migration of lymphoma cells by targeting miR-20a-5p, and its mechanism may be related to the activation of JAK/STAT3 signaling pathway.

Gene Polymorphisms of Patients with Lymphoma-Associated Hemophagocytic Syndrome in Longyan area, Fujian Province / 中国实验血液学杂志

OBJECTIVE@#To analyze the gene polymorphisms of patients with lymphoma-associated hemophagocytic syndrome in Longyan area, Fujian province.@*METHODS@#A total of 125 patients with lymphoma-associated hemophagocytic syndrome in Longyan, Fujian province, admitted to Longyan First Hospital from May 2017 to November 2020 were selected. Peripheral venous blood was collected from all the patients, and the genotypes of perforin 1 (PRF1) and interleukin-10 (IL-10) gene loci were detected by PCR-fluorescence probe method, and the correlation between PRF1 and IL-10 gene polymorphisms and lymphoma-associated hemophagocytic syndrome was analyzed.@*RESULTS@#The mutation frequencies of PRF1 gene loci rs885821 (C>T), rs885822 (C>T), rs1889490 (G>A) in patients with lymphoma-associated hemophagocytic syndrome were 10.40%, 78.8% and 64.4%, respectively. The mutation frequencies of rs1800872 (A>C), rs1800871 (C>T) and rs1800896 (G>A) of IL-10 loci were 56.0%, 45.2% and 77.6%, respectively.@*CONCLUSION@#PRF1 and IL-10 gene loci were polymorphic in patients with lymphoma-associated hemophagocytic syndrome in Longyan area, Fujian province. Alleles C and G of PRF1 and IL-10 were risk factors, and alleles T and A were protective factors.