OBJECTIVE: To analyze the clinical characteristics of patients with 11q23 rearrangement acute lymphoblastic leukemia (ALL) with non-KMT2A::AFF1 fusion genes. METHODS: The clinical data of 10 patients with KMT2A fusion gene positive and partner gene non-AFF1 ALL admitted to Henan Cancer Hospital from December 2016 to December 2024 were retrospectively summarized. The immunophenotype, molecular genetic characteristics, clinical manifestations and disease prognosis of these patients were analyzed. This research has been approved by the Medical Ethics Committee of Henan Cancer Hospital (Ethics No.: 2019342). RESULTS: Among the 10 patients, the fusion genes were KMT2A::MLLT1 in 7 cases, KMT2A::MLLT4, KMT2A::MLLT3 and KMT2A::MLLT10 in 1 case each. The European Group for the Immunological Classification of Leukemias (EGIL) classification included 6 cases of T-ALL, 2 cases of pro-B-ALL, 1 case of Common-B-ALL and 1 case of pre-B-ALL. 4 cases of B-ALL all expressed CD19, cCD79a, CD38 and HLA-DR, and some expressed CD34 and CD22, without expression or weak expression of CD10, without expression of CD20. One case was accompanied by myeloid marker CD15 expression. 6 cases of T-ALL all expressed CD34, CD7, most expressed CD38, and some expressed CD3, CD5, CD2, CD4 and CD8, and 1 case expressed CD4 and CD8 together. Chromosomal abnormalities were detected in 3 cases, 5 cases were positive for WT1 fusion gene, and 6 cases had gene alterations. 9 patients achieved the first complete remission (CR1) during chemotherapy, and 1 patient relapsed within 6 months after CR1. At the last follow up, 1 patient (the fusion gene was KMT2A::MLLT4) remained unrelieved. There were 2 cases of KMT2A rearrangement (KMT2A-r) persistent positive (+/+) and 8 cases of KMT2A-r negative (+/-). The overall survival (OS) rate and leukemia-free survival (LFS) rate of patients with KMT2A-r persistent positive were significantly lower than those of patients with negative change, and the differences were statistically significant (P values were all < 0.05). Among the 3 patients who received chemotherapy+allogeneic hematopoietic stem cell transplantation (allo-HSCT), no relapse was observed until the follow up day. The OS rate and LFS rate of patients with KMT2A::MLLT1 and chemotherapy+allo-HSCT were higher than those of non-KMT2A::MLLT1 and single chemotherapy patients, and the differences were not statistically significant (P values were all ≥ 0.05). There was no significant difference in OS rate and LFS rate between T-ALL and B-ALL patients (P values were all ≥ 0.05). The median LFS time of the 10 patients was 32 (0 ~ 100) months, and the median OS time was 36 (1 ~ 101) months. CONCLUSION The 11q23 rearrangement ALL with non-KMT2A::AFF1 transcript is mainly KMT2A::MLLT1, T-ALL is more common, and the rate of chromosomal karyotype detection is relatively low. Persistent positive KMT2A-r is unfavorable for patient survival, and allo-HSCT during the CR1 period may improve patient survival.
Humans ; Precursor Cell Lymphoblastic Leukemia-Lymphoma/genetics* ; Female ; Male ; Myeloid-Lymphoid Leukemia Protein/genetics* ; Histone-Lysine N-Methyltransferase/genetics* ; Adult ; Adolescent ; Chromosomes, Human, Pair 11/genetics* ; Child ; Transcriptional Elongation Factors/genetics* ; Gene Rearrangement ; Oncogene Proteins, Fusion/genetics* ; Retrospective Studies ; Young Adult ; Middle Aged ; Prognosis ; Child, Preschool ; DNA-Binding Proteins/genetics*

BACKGROUND AND OBJECTIVES

Non-Hodgkin Lymphoma (NHL) ranks 11th in cancer incidence and mortality in the Philippines with the combination chemotherapy composed of Cyclophosphamide, Doxorubicin, Vincristine, and Prednisone (CHOP) being commonly used as treatment. However, the addition of Rituximab to CHOP (R-CHOP) has been shown to exhibit higher response rates and longer remissions, potentially improving quality of life. Currently, there is conflicting evidence on the cost-utility of CHOP versus R-CHOP. The study aimed to describe the patient- and country-specific factors, and treatment modalities used for NHL and systematically review cost-utility evidence of R-CHOP versus CHOP in adult NHL patients.

METHODS

A systematic literature search of cost-utility studies on R-CHOP versus CHOP for NHL treatment was performed on eight databases: PubMed/MEDLINE, Scopus, Web of Science, EBSCOHost, Cochrane, York Research Database, Centre for Reviews and Dissemination Database, and HERDIN, where 607 studies were identified. Upon screening using an eligibility criteria, 10 studies were included and critically assessed using four appraisal tools: CHEERS, Drummond, Cooper, and ECOBIAS. These were performed independently by two authors with a third author assisting to help reach a consensus.

RESULTS

All studies from high-income countries (HICs) (n=8) and low-middle-income country (LMIC) (n=1) suggested that R-CHOP was more cost-effective for NHL treatment than CHOP in terms of utility outcomes. The study conducted in a low-income country (LIC) (n=1) suggested the opposite, favoring CHOP over R-CHOP. Methodological differences such as perspective, discount rate, willingness-to-pay (WTP), time horizon, and economic model were observed. Methodological limitations include completeness of data reported and credibility of sources used.

CONCLUSION

The results of this review shall be interpreted with caution as those favoring R-CHOP over CHOP for NHL treatment in terms of cost-utility were concentrated in HICs. More economic evaluations from LICs, LMICs, and upper-middle income countries (UMICs) are needed for a robust conclusion. Additionally, establishing a universally recognized guideline for economic evaluations is essential to guide researchers effectively.

Cost-benefit Analysis ; Hodgkin Disease ; Lymphoma ; Systematic Review ; Lymphoma, Non-hodgkin

OBJECTIVE: To explore the impact of age on the genetic variant spectrum and prognosis of patients with previously untreated Diffuse large B-cell lymphoma (DLBCL). METHODS: A retrospective analysis was conducted on the clinical data and follow-up information of 254 previously untreated DLBCL patients from 14 hospitals in the Jiangsu Cooperative Lymphoma Group (JCLG) enrolled from July 2018 and July 2023. Following extraction of DNA from tumor tissue samples, next-generation sequencing (NGS) technique was employed to analyze the genetic variant spectrum of the DLBCL patients, with an evaluation of the relationship between age and genetic variants as well as prognosis. This study was approved by the Medical Ethics Committee of the Affiliated Hospital of Nantong University (Ethics No.: 2023-K048-01). RESULTS: The median age of the 254 DLBCL patients was 62 years old, with 55% of patients aged 60 years or above. Clinical evaluation showed that younger (< 60 years) patients had higher complete response (CR) (70% vs. 59%), and objective response rate (ORR) (88% vs. 79%) than older patients, though the difference between the two groups was not statistically. Survival analysis indicated that both the five-year overall survival (OS) (82.7% vs. 71.7%, P = 0.006) and progression-free survival (PFS) (70.6% vs. 50.2%, P < 0.05) rates were significantly higher in younger patients. NGS showed that 99.6% of the patients harbored genetic variants, with PIM1, KMT2D, TP53, MYD88, and CD79B being the most common genes. Age significantly affected the variant frequency of certain genes, with MYC variants serving an adverse prognostic factor for OS in younger patients (P = 0.002), while TP53 (P = 0.024) and BCL2 (P = 0.002) variants significantly impacted OS in older patients. Prognostic analysis identified age ≥ 60 years (HR = 3.439, 95%CI: 1.318~9.874), presence of B symptoms (HR = 2.871, 95%CI = 1.133~7.307), and elevated lactate dehydrogenase (HR = 3.528, 95%CI = 1.231~10.66) as independent adverse prognostic factors. CONCLUSION Age, genetic variants, and clinical factors may significantly affect the prognosis of the DLBCL patients. Younger patients have better survival compared to older patients. Variants of the MYC, BCL2, and TP53 genes are closely associated with poor prognosis.
Humans ; Lymphoma, Large B-Cell, Diffuse/diagnosis* ; Middle Aged ; Female ; Male ; Retrospective Studies ; Aged ; Prognosis ; Adult ; Aged, 80 and over ; High-Throughput Nucleotide Sequencing ; Young Adult ; Adolescent ; Genetic Variation

Mantle cell lymphoma (MCL) is a rare B-cell lymphoma characterized by both the incurable nature of indolent lymphomas and the clinical course of aggressive lymphomas. The integration of high-dose cytosine arabinoside (Ara-C) and autologous hematopoietic stem cell transplantation (ASCT) has led to substantial improvement in the outcomes of MCL patients in the immunochemotherapy era. More recently, the widespread use of small molecule targeted agents, particularly Bruton tyrosine kinase inhibitor (BTKi), has re-shaped the therapeutic landscape of MCL patients and challenged the traditional role of high-dose Ara-C and ASCT. Novel immunotherapies including bi-specific antibodies and chimeric antigen receptor T-cell (CAR-T) therapy have emerged as important treatment options for MCL patients with relapsed or refractory disease. With advances in multi-omics profiling, the development of personalized, potentially curative strategies based on individual genetic and immune features is expected to become a major focus of future research on MCL. This article will delve into the latest research progress in the treatment and genetics and translational research on MCL patients, focusing on the latest progress of research on the treatment of newly diagnosed MCL patients, treatment of relapsed/refractory MCL patients, and the genetics and translational treatment of MCL patients, and explore the evolution and future direction of its treatment model.
Humans ; Lymphoma, Mantle-Cell/immunology* ; Translational Research, Biomedical ; Hematopoietic Stem Cell Transplantation ; Immunotherapy

OBJECTIVE

To report a case of Mucosa-Associated Lymphoid Tissue (MALT) lymphoma of the nasopharynx in a young woman, and its clinical presentation, laboratory findings and management.

METHODS

Design: Case Report Report

Setting: Tertiary Government Training Hospital

Patient: One

RESULTS

A 21-year-old woman with a four-month history of right lateral neck mass managed as a case of clinically diagnosed pulmonary tuberculosis consulted us. Nasal endoscopy showed an erythematous, non-ulcerating exophytic mass in the nasopharynx. Histopathologic, immunohistochemical and PET-CT imaging studies confirmed MALT. She completed seven sessions of chemotherapy and is currently in complete remission.

CONCLUSION

MALT lymphoma of the nasopharynx is rare and may mimic other head and neck tumors both clinically and histologically. A high index of suspicion, thorough examination of Waldeyer’s ring, and confirmatory immunohistochemistry are essential for timely and accurate diagnosis of this uncommon but treatable malignancy.

Human ; Female ; Young Adult: 19-24 Yrs Old ; Malt Lymphoma ; Lymphoma, B-cell, Marginal Zone ; Extranodal Marginal Zone Lymphoma ; Nasopharynx

OBJECTIVES

To report a case of triple primary malignant neoplasms in a 70-year-old woman diagnosed with follicular and papillary thyroid carcinoma and diffuse B-cell lymphoma of the right submandibular gland and cervical lymph nodes.

METHODS

Design:Case Report

Setting:Tertiary Government Training Hospital

Patient: One

RESULTS

A 70- year-old woman presented with a four-year history of gradually enlarging anterior neck mass, associated with a right submandibular mass and neck nodes for one year. The gradual progression of her symptoms made the patient think that it was a benign condition. This led to a delay in medical consultation. The patient underwent total thyroidectomy with functional neck dissection of the ipsilateral right neck. Histopathology revealed simultaneous follicular and papillary thyroid carcinoma, and diffuse B-cell lymphoma of the cervical lymph nodes. The patient was referred to medical oncology and nuclear medicine for further management.

CONCLUSION

Our patient was incidentally diagnosed with follicular and papillary thyroid carcinoma and diffuse B cell lymphoma of the cervical lymph nodes after surgery. Such triple primary malignant neoplasms in a single individual are rare, and as in our case, may only be diagnosed in hindsight.

Human ; Female ; Aged: 65-79 Yrs Old ; Carcinoma ; B-lymphocytes ; Adenocarcinoma, Follicular ; Neoplasms ; Submandibular Gland ; Lymph Nodes ; Lymphoma, B-cell ; Thyroid Gland ; Thyroid Cancer, Papillary ; Thyroidectomy

Intravascular large B-cell lymphoma (IVLBCL), a rare subtype of non-Hodgkin lymphoma, is classified as an independent subtype of extranodal diffuse large B-cell lymphoma (DLBCL) in the 2008 World Health Organization (WHO) Classification (Turner et al., 2010). The 5th edition of the World Health Organization (WHO 2022) classification of hematolymphoid tumors retains this subtype (Alaggio et al., 2022). IVLBCL, which is characterized by neoplastic lymphocyte proliferation within the lumen of small blood vessels, tends to invade organs, such as the nervous system, skin, bone marrow (BM), and lung (D'Angelo et al., 2019; Satoh et al., 2019; Vásquez et al., 2019; Fukami et al., 2020).
Humans ; Antineoplastic Combined Chemotherapy Protocols/therapeutic use* ; Lymphoma, Large B-Cell, Diffuse/drug therapy* ; Vascular Neoplasms/therapy*

Objective:To compare the clinical characteristics of nasal NK/T-cell lymphoma（NKTL） and diffuse large B-cell lymphoma（DLBCL） to improve the diagnosis and differential diagnosis of nasal lymphomas. Methods:A retrospective analysis of cases of nasal NKTL and DLBCL was conducted. The clinical symptoms, signs, and imaging features of both groups were compared and statistically analyzed. Results: The DLBCL group showed more symptoms like exophthalmos/diplopia and epiphora compared to the NKTL group （both P=0.040）. NKTL cases were more likely to be misdiagnosed as sinusitis（P=0.007）. In NKTL cases, nasal mucosal swelling（P<0.01）, destruction of nasal structures（P=0.002）, and external nasal structural abnormalities（P=0.003） were more prevalent. In imaging, the DLBCL group more commonly demonstrated worm-eaten destruction of sinus bones （P=0.004）, sinus masses （P=0.018）, and invasion of adjacent structures including the pterygopalatine fossa, infratemporal fossa （P<0.01）, orbit （P=0.039）, and skull base （P=0.011）. NKTL involved the turbinates（P=0.001）, nasal cavity and septum（P=0.016）, nasopharynx（P<0.01）, and "skip" infiltration of external nasal tissues（P=0.042） more frequently. No statistically significant differences were found in other clinical features between the two groups. Conclusion:For patients with nasal obstruction and discharge, it is essential to inquire about systemic B symptoms, such as fever, and eye symptoms, such as periorbital swelling, diplopia, and lacrimation. Lymphoma should be suspected if local examination reveals diffuse nasal swelling, destruction of turbinates or septum, and external nasal structural abnormalities. Worm-eaten bone destruction and "cast-like" changes of the turbinates, septum, and nasal cavity, as well as "skip" infiltration of the external nose, are more common in NKTL. Sinus masses with invasion of the pterygopalatine fossa, infratemporal fossa, skull base, and orbit are more typical of DLBCL.
Humans ; Retrospective Studies ; Lymphoma, Extranodal NK-T-Cell/diagnosis* ; Lymphoma, Large B-Cell, Diffuse/pathology* ; Nasal Cavity/pathology* ; Male ; Diagnosis, Differential ; Female ; Middle Aged ; Nose Neoplasms/diagnosis* ; Adult ; Aged

Objective:To analyze the clinical characteristics of malignant lymphoma of the head and neck in children, and to improve the understanding and diagnosis and treatment of this type of disease by otolaryngologists. Methods:Clinical data of 49 children with malignant lymphoma of the head and neck hospitalized in the Department of Otorhinolaryngology, Head and Neck Surgery of Kunming Children's Hospital from 2013-2021 were retrospectively analyzed and statistically analyzed according to gender, age distribution, duration of the disease, site of onset, type of pathology and survival status. Results:A total of 49 cases of malignant lymphoma of the head and neck in children were collected, of which, 39 were male and 10 female. The minimum age was 3 years, the maximum was 14 years and 4 months, the median age of onset was 7 years, and the largest percentage （51.02%） of children was in the school age（6-12 years）. The duration of the disease ranged from 5 days to 2 years, with a median of 1 month, and the site of the lesion was located in the neck in the majority of cases, 41（83.67%）. The pathologic types of hodgkin lymphoma（HL） were 25 cases（51.02%） and non-hodgkin lymphoma（NHL） were 24 cases（48.98%）, and among hodgkin lymphomas, mixed-cell classical hodgkin lymphoma was the most common, with 9 cases（18.37%）; among non-hodgkin lymphomas,originated from B-cells in 16 cases （32.65%） and from T-cells in 7 cases （14.29%）, with Burkitt's lymphoma being the most numerous of B-cell origin in 13 cases （26.53%）, and T-cell lymphoblastoid lymphoma being the most common of T-cell origin in 4 cases （8.16%）. The follow-up period was from 22 days to 6 years and 10 months, with 3 cases losing, 43 cases surviving, 3 cases dying, with a survival rate of 93 5%, and 3 cases relapsing, with a relapse rate of 6 5%. Conclusion:Children's head and neck malignant lymphoma has its own characteristics in terms of age of onset, gender distribution, site of onset, pathological type and treatment prognosis, which should be comprehensively assessed, diagnosed as early as possible, and multidisciplinary joint treatment actively carried out to propose personalized treatment plans.
Humans ; Male ; Female ; Child ; Head and Neck Neoplasms/therapy* ; Child, Preschool ; Retrospective Studies ; Adolescent ; Lymphoma/therapy* ; Survival Rate ; Hodgkin Disease ; Prognosis ; Infant ; Lymphoma, Non-Hodgkin

Objective:To development a deep learning（DL） model based on conventional MRI for automatic segmentation and differential diagnosis of nasopharyngeal carcinoma（NPC） and nasopharyngeal lymphoma（NPL）. Methods:The retrospective study included 142 patients with NPL and 292 patients with NPC who underwent conventional MRI at Renmin Hospital of Wuhan University from June 2012 to February 2023. MRI from 80 patients were manually segmented to train the segmentation model. The automatically segmented regions of interest（ROIs） formed four datasets: T1 weighted images（T1WI）, T2 weighted images（T2WI）, T1 weighted contrast-enhanced images（T1CE）, and a combination of T1WI and T2WI. The ImageNet-pretrained ResNet101 model was fine-tuned for the classification task. Statistical analysis was conducted using SPSS 22.0. The Dice coefficient loss was used to evaluate performance of segmentation task. Diagnostic performance was assessed using receiver operating characteristic（ROC） curves. Gradient-weighted class activation mapping（Grad-CAM） was imported to visualize the model's function. Results:The DICE score of the segmentation model reached 0.876 in the testing set. The AUC values of classification models in testing set were as follows: T1WI: 0.78（95%CI 0.67-0.81）, T2WI: 0.75（95%CI 0.72-0.86）, T1CE: 0.84（95%CI 0.76-0.87）, and T1WI+T2WI: 0.93（95%CI 0.85-0.94）. The AUC values for the two clinicians were 0.77（95%CI 0.72-0.82） for the junior, and 0.84（95%CI 0.80-0.89） for the senior. Grad-CAM analysis revealed that the central region of the tumor was highly correlated with the model's classification decisions, while the correlation was lower in the peripheral regions. Conclusion:The deep learning model performed well in differentiating NPC from NPL based on conventional MRI. The T1WI+T2WI combination model exhibited the best performance. The model can assist in the early diagnosis of NPC and NPL, facilitating timely and standardized treatment, which may improve patient prognosis.
Humans ; Nasopharyngeal Carcinoma/diagnostic imaging* ; Deep Learning ; Magnetic Resonance Imaging ; Retrospective Studies ; Nasopharyngeal Neoplasms/diagnostic imaging* ; Lymphoma/diagnostic imaging* ; Diagnosis, Differential ; ROC Curve ; Male ; Female ; Middle Aged ; Adult