Large B-cell Lymphoma Presenting Research Articles

Lacrimal sac diffuse large B-cell lymphoma presenting as sudden-onset binocular diplopia

Introduction: Diffuse large B-cell lymphoma (DLBCL) is a prevalent subtype of non-Hodgkin lymphoma (NHL) affecting predominantly elderly individuals. Case description: A 68-year-old man with a history of hypertension, hyperlipidaemia and a small pituitary gland tumour presented with sudden-onset binocular diplopia and right-eye blurry vision. A magnetic resonance imaging (MRI) of the brain revealed enhancing soft tissue in the right superolateral orbit inseparable from the lacrimal gland, extending medially to the right superior rectus muscle and soft tissue. Further scanning showed widespread metastasis to the bilateral retroperitoneal lymph nodes, adrenal gland, spine and lymph nodes in the neck. A biopsy of the lacrimal gland confirmed DLBCL. Conclusion: Primary lacrimal gland DLBCL is a rare and delayed diagnosis that often stems from the resemblance of its clinical manifestations to more benign conditions such as dacryocystitis, dacryostenosis or mucocele. Timely recognition and accurate diagnosis are essential for initiating appropriate treatment and improving patient outcomes.

Diffuse Large B-cell Lymphoma Presenting as Bronchial Mass.

Diffuse Large B-cell Lymphoma Presenting as Bronchial Mass.

Intravascular large B-cell lymphoma presenting in the prostate

Intravascular large B-cell lymphoma presenting in the prostate

Primary Extranodal Diffuse Large B-cell Lymphoma Presenting in the Lips: A Case Report and Literature Review.

Approximately 30-40% of malignant lymphomas are classified as diffuse large B-cell lymphoma (DLBCL), with 30% of DLBCL cases manifesting as extranodal lymphomas. Among these extranodal DLBCLs, primary DLBCL in oral lesions, particularly in the lips, is rare. While the treatment methods, chemotherapy assessment, and prognosis for nodal and extranodal DLBCLs are generally similar, diagnostic challenges can lead to delayed therapeutic intervention. We herein present a recent case of primary extranodal DLBCL in the lips that was swiftly diagnosed and managed using rituximab-containing chemotherapies. Our experience underscores the important role that hematologists play in identifying the possibility of oral hematological tumors, thereby allowing for a rapid diagnosis and timely intervention.

Colonic polyposis with underlying diffuse large B-cell lymphoma presenting with ileocolic intussusception

Colonic polyposis with underlying diffuse large B-cell lymphoma presenting with ileocolic intussusception

Primary central nervous system diffuse large B-cell lymphoma presenting as homonymous hemianopia

Post-transplant lymphoproliferative disorder rarely affects the central nervous system although it can have visually significant consequences depending on the location of intracranial involvement.

Diffuse large B-cell lymphoma presenting as sternal mass in a patient with ankylosing spondylitis: a case report study.

The authors present a rare case of primary diffuse large B-cell lymphoma of the sternum in a 41-year-old Iranian woman with ankylosing spondylitis (AS). Physical examination revealed a 7×7.5cm firm swelling of the anterior midline chest wall above the breasts, and MRI showed a lesion within the sternal marrow with an associated soft-tissue mass in the anterior aspect of the sternum. Following core-needle biopsy under ultrasound guidance, a histopathological study demonstrated diffuse sheets of large noncleaved atypical cells with large multilobated prominent nuclei and fine chromatin compatible with diffuse large B-cell lymphoma. Primary and exclusive involvement of the sternum is an uncommon presentation of lymphoma. Radiological, histological, and clinical characteristics of primary bone lymphoma can resemble those of other medical disorders. Although infrequent, existing evidence shows that AS seems to be associated with a small but significant risk for malignancy. Even though inflammatory involvement of the anterior chest wall could be a common clinical finding in patients with AS, it is recommended that anterior chest wall pain or any mass almost always needs comprehensive assessment and imaging evaluation in such patients to avoid any delayed diagnosis, misdiagnosis, and ensuing morbidity or mortality.

Intravascular Large B-cell Lymphoma Presenting as Rapidly Progressive Encephalopathy

Intravascular large B-cell lymphoma (IVLBCL) is a rare form of non-Hodgkin’s lymphoma that selectively involves the lumina of vessels, and may present as ischemic stroke. A 62-year-old man presented with progressive aphasia, dementia, seizure and stroke-like symptom. Brain magnetic resonance imaging revealed dynamic progressive fluid attenuated inversion recovery lesions and multiple punctate microbleeds in the bilateral subcortical region. A kidney biopsy revealed IVLBCL. We report a patient with IVLBCL presenting as rapidly progressive encephalopathy.

Intravascular Large B-cell Lymphoma Presenting as Sudden Death.

Intravascular large B-cell lymphoma is a rare subset of non-Hodgkin lymphoma composed of mature B lymphoma cells confined to the intravascular space. This disease remains elusive because it lacks a discrete tumor mass, can affect any part of the body, and has vague symptoms paired with heterogeneous clinical findings resulting in delayed or missed accurate diagnosis, even at postmortem examination. This is a case of a woman who died within hours of presenting to the emergency department with a diagnosis of intravascular large B-cell lymphoma made through autopsy examination, adding to the knowledge of this rare disease and bringing it to the attention of practicing autopsy and forensic pathologists.

Clinicopathological features of primary central nervous system diffuse large B-cell lymphoma presenting with diffuse white matter lesions

目的： 分析以弥漫性脑白质病变为表现的原发中枢神经系统弥漫性大B细胞淋巴瘤（又称大脑淋巴瘤病）的临床及病理学特征，探讨其鉴别诊断、治疗及预后。 方法： 选取2020年6月至2022年6月首都医科大学附属北京天坛医院病理科诊断的2例大脑淋巴瘤病患者资料，分析临床、磁共振成像（MRI）及病理学表现。 结果： 2例患者均为男性，年龄分别53岁（例1）、69岁（例2），临床表现为头晕乏力、记忆力减退，均伴有言语错乱；MRI均未见肿块形成，表现为弥漫性脑白质病变，呈T1稍低信号、T2及T2-FLAIR高信号、增强病灶未见明显强化，其中例2患者于2个月后复查MRI，病变范围较前扩大，病灶出现部分强化；送检穿刺组织镜下白质结构基本完整，可见弥漫浸润的异型淋巴细胞，核分裂象可见，核仁明显，免疫组织化学染色CD20、CD79α及PAX5强阳性，CD3弱阳性，Ki-67阳性指数高；例1进行以甲氨蝶呤为基础的化疗，就诊后5个月存活；例2未行特殊治疗，就诊后13个月死亡。 结论： 大脑淋巴瘤病临床罕见、预后不佳，临床表现为快速进展的认知功能障碍、影像学表现为弥漫性脑白质病变，没有明确肿块形成，没有外科手术指征，所以要尽快进行组织病理活检明确诊断，积极治疗以改善预后。.

Intravascular Large B-Cell Lymphoma (IVLBCL) Presenting with CNS Involvement in Patient with Chronic Lymphocytic Leukemia

Background: Intravascular lymphoma (IVL) is a rare, often fatal disease characterized by intraluminal proliferation of lymphoid cells within blood vessels. Intravascular Large B-Cell Lymphoma (IVLBCL) is frequently found in different organs, but the skin and central nervous system (CNS) are the most affected ones. Routine radiological examination and additional diagnostic tools, such as high-resolution, three-dimensional (3D) FLAIR sequence, T2-weighted (T2W) and T1-weighted (T1W) contrast-enhanced magnetic resonance imaging (MRI), MRI diffusion and perfusion imaging studies are useful to confirm the diagnosis in cases where it is not easy to perform histopathological examinations [1]. 1.2. Case Report: We present a clinical case of a 63-year-old female patient who presented with progressive cognitive deficit as mild motor aphasia, acalculia, agraphia, dyslexia and mild ideomotor apraxia without any signs of other organ involvement. It was known that the patient had 5-year anamnesis of chronic lymphocytic leukemia, stage II and splanchnic venous thrombosis 2 months ago. Blood biochemical analysis without any significant change, except leukocytosis in full blood count (leukocytes 15,2 x 10*9/L, where neutrophils were in normal range, but lymphocytes were increased). Head magnetic resonance imaging (MRI) showed pathological signal areas on the left parietal lobe with hypointense signal on contrast-enhanced T1-weighted, hyperintense signal on contrast-enhanced T2-weighted and FLAIR-weighted MRI images with restricted diffusion and slightly lower ADC value. Magnetic resonance perfusion imaging studies did not show an increase in cerebral blood flow or in cerebral blood volume. In this case, the patient’s diagnosis was made based on typical radiological features. 1.3. Conclusions: Intravascular large B-cell lymphoma is a rare subtype of extra nodal large B-cell lymphoma with an aggressive clinical course. The diagnosis is challenging, as it presents without any obvious tumour mass or lymphadenopathy. [2] As the clinical presentation is variable without specific characteristics, mostly biopsy or even autopsy confirms the definite diagnosis. But in some cases, the diagnosis can be made by typical radiological findings in the restricted availability of brain biopsy.

Molecular and clinical diversity in primary central nervous system lymphoma

Primary central nervous system lymphoma (PCNSL) is a rare and distinct entity within diffuse large B-cell lymphoma presenting with variable response rates probably to underlying molecular heterogeneity. To identify and characterize PCNSL heterogeneity and facilitate clinical translation, we carried out a comprehensive multi-omic analysis [whole-exome sequencing, RNA sequencing (RNA-seq), methylation sequencing, and clinical features] in a discovery cohort of 147 fresh-frozen (FF) immunocompetent PCNSLs and a validation cohort of formalin-fixed, paraffin-embedded (FFPE) 93 PCNSLs with RNA-seq and clinico-radiological data. Consensus clustering of multi-omic data uncovered concordant classification of four robust, non-overlapping, prognostically significant clusters (CS). The CS1 and CS2 groups presented an immune-cold hypermethylated profile but a distinct clinical behavior. The 'immune-hot' CS4 group, enriched with mutations increasing the Janus kinase (JAK)-signal transducer and activator of transcription (STAT) and nuclear factor-κB activity, had the most favorable clinical outcome, while the heterogeneous-immune CS3 group had the worse prognosis probably due to its association with meningeal infiltration and enriched HIST1H1E mutations. CS1 was characterized by high Polycomb repressive complex 2 activity and CDKN2A/B loss leading to higher proliferation activity. Integrated analysis on proposed targets suggests potential use of immune checkpoint inhibitors/JAK1 inhibitors for CS4, cyclin D-Cdk4,6 plus phosphoinositide 3-kinase (PI3K) inhibitors for CS1, lenalidomide/demethylating drugs for CS2, and enhancer of zeste 2 polycomb repressive complex 2 subunit (EZH2) inhibitors for CS3. We developed an algorithm to identify the PCNSL subtypes using RNA-seq data from either FFPE or FF tissue. The integration of genome-wide data from multi-omic data revealed four molecular patterns in PCNSL with a distinctive prognostic impact that provides a basis for future clinical stratification and subtype-based targeted interventions.

Diffuse large B-cell lymphoma presenting as transverse myelitis

Diffuse large B-cell lymphoma (DLBCL) is the most common type of non-Hodgkin’s lymphoma. Although progressive lymphadenopathy is a typical feature, extranodal involvement may also occur, including the gastrointestinal tract, skin, bone, thyroid, and testes. Central nervous system invasion is rare, so differentiating it from diseases such as inflammatory demyelinating disorder or infection is essential. DLBCL is therefore a challenge to diagnose, especially when the first findings are neurological symptoms. We report an unusual case of DLBCL that presented as transverse myelitis.

Upper Digestive Tract Bleeding as Presentation of Diffuse Large B-Cell Lymphoma.

Upper Digestive Tract Bleeding as Presentation of Diffuse Large B-Cell Lymphoma.

Spontaneous splenic rupture as initial presentation of diffuse large B-cell lymphoma.

Spontaneous splenic rupture as initial presentation of diffuse large B-cell lymphoma.

S2606 Hematochezia as a Rare Presentation of Primary Colonic Diffuse Large B-Cell Lymphoma

Introduction: Diffuse large B cell lymphoma is the most common subtype of non-Hodgkin's lymphoma with localization seen only in approximately 20% of patients. Gastrointestinal localization of extranodal disease is extremely rare. We present a case of an 82-year-old female who presented with occult lower gastrointestinal bleeding as the primary presentation leading to the diagnosis of diffuse large B cell lymphoma (DLBCL). Case Description/Methods: An 82-year-old female with a past medical history of partial colectomy with colostomy reversal secondary to diverticulitis presented to the hospital for bright red blood per rectum with the passage of blood clots. She had a negative screening colonoscopy 5 years prior to admission. Upon evaluation, the patient was found to be in hemorrhagic shock resulting from acute blood loss. Colonoscopy and EGD showed mild gastritis, colitis, and a deformed cecum due to previous postoperative changes. Despite packed red blood cell transfusions, the patient remained anemic and developed pancytopenia on hospital day 4. Abdominal CT imaging demonstrated thickened loop of bowel with small to borderline-enlarged mesenteric lymph nodes adjacent to the area of bowel wall thickening. Given her history, lymphadenopathy was considered to be reactive vs. metastatic in nature while biopsies from the colonoscopy were pending. Once hemodynamically stable, the patient was discharged with appropriate f. Final biopsy results of the ascending colon confirmed a diagnosis of diffuse large B cell lymphoma (DLBCL), negative for the t(8;14) translocation or MYC/IGH fusion per FISH analysis. Chemotherapy with cyclophosphamide, vincristine, and prednisolone (CVP) was initiated. Discussion: Although hematochezia in an elderly patient is a cause for malignant workup, it is a rare presentation of hematologic malignancies. Most commonly, extranodal DLBCL is seen to involve the gastric mucosa in the event that the gastrointestinal system is involved at all. Common sites of manifestation include the spleen, thymus, and lymphoid aggregate tissue in the neck. Studies have demonstrated that extranodal disease is correlated with older patient populations as well as a poorer performance score when compared to nodal disease. This case illustrates the need of considering a broad differential inclusive of hematologic malignancy when assessing a patient with lower gastrointestinal bleeding, particularly in elderly patients with associated transfusion-resistant anemia.

Primary Testicular Diffuse Large B-cell Lymphoma Presenting in a Young Adult

Primary testicular lymphoma is a rare type of extranodal non-Hodgkin lymphoma (NHL), its positive diagnosis is based on histopathological findings. Treatment modalities consist of surgical excision, chemotherapy, and radiation therapy but the accurate procedures are not standardized. The authors report a new case of primary testicular lymphoma, and we discuss its diagnostic and therapeutic aspects. Sperm cryopreservation was carried out. The patient was started on chemotherapy with cyclophosphamide, hydroxydaunorubicin, oncovin, and prednisone (CHOP) regime.

Primary diffuse large b-cell lymphoma presenting as a chest wall mass: A case report

Chest wall lymphoma is a rare entity and often confused with the more common sarcoma. We report a case of diffuse large B-cell non-Hodgkin lymphoma patient presented with a sole chest wall mass. Chest computed tomography revealed an abnormal mass with well-defined borders below the pectoralis major muscle with contrast enhancement. The excisional biopsy revealed diffuse large B-cell non-Hodgkin lymphoma, confirmed by immunohistochemistry. The patient was then treated with six cycles of R-CHOP and achieved complete response. She is currently free from malignancy for 12 months after treatment. This paper illustrates the rarity of this entity, the need for distinguishing it from sarcoma, and briefly reviews its current management.

Diffuse large B-cell lymphoma presenting with paraparesis.

The development of paraparesis in the setting of back pain and lassitude in an immunocompetent patient was diagnosed as the presenting feature of diffuse large B-cell lymphoma. MRI imaging and surgical biopsy were diagnostic, despite the absence of lymphadenopathy or splenomegaly, and near-normal laboratory workup. Different possible pathological mechanisms of paraparesis in large B-cell lymphoma are discussed.

Unusual presentation of primary ovarian diffuse large B-cell lymphoma: a case report

BackgroundPrimary ovarian lymphoma has been difficult to diagnose clinically and pathologically due to its rare incidence and non-specific clinical symptoms.Case presentationA 75-year-old female patient was reported in this study. The patient had a six-month history of changes in bowel habits, with occasional black feces and paroxysmal pain in the abdomen. The computed tomography scan of the pelvic cavity illustrated that rectal cancer and sigmoid colon adenocarcinoma invaded the lower part of the right-side ureter. The patient was once treated with excision of part of small intestine, fallopian tube and ovary, and uterus. The pathological examination of these excised tissues, combined with the immunohistochemistry, confirmed that the female patient suffered from primary ovarian diffuse large B-cell lymphoma (DLBCL), and the lymphoma had invaded the entire right-side ovary tissues, serous membranes on the posterior surface of the uterus, and the wall of small intestine.ConclusionFew reports were available regarding the primary ovarian DLBCL. The initial symptom of the patient was the changes in bowel habits, which had not been reported beforehand. Hopefully, this case could helpfully render the early diagnosis possible, and increase clinical understanding of primary ovarian DLBCL, which would thereby reduce the chance of misdiagnosis.