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A 55-year-old woman had remarkably generalized telangiectasia. Large atypical lymphoid cells were found within the lumen of small vessels, and CD20 was positive in her third skin biopsy. She was diagnosed with intravascular large B-cell lymphoma (IVLBCL) and went into remission of the skin manifestations after seven courses of R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone) and four courses of intravenous high-dose methotrexate (HD MTX). To our knowledge, this is the first case report of IVLBCL with generalized telangiectasia in China. From a review of the literature, we found that generalized telangiectasia is a remarkable manifestation of intravascular lymphoma, and the differential diagnosis of intravascular lymphoma and panniculitis or vasculitis is very important. When the diagnosis is confusing, multiple skin biopsies are useful. [ABSTRACT FROM AUTHOR]

Background: Intravascular large B‐cell lymphoma (IVLBCL) is a rare extranodal lymphoma that is characterized by the selective growth of neoplastic cells in blood vessels, representing a potentially treatable cause of rapidly progressive dementia (RPD). Given its diverse clinical and instrumental presentation, it is often misdiagnosed with more common RPD causes, for example, Creutzfeldt‐Jakob disease (CJD) or vascular dementia. Methods: This study presents the clinical and histopathological characteristics of four IVLBCL cases that we diagnosed post‐mortem over 20 years among over 600 brain samples received as suspected CJD cases at our prion disease reference center. Results: Our patients exhibited various presenting symptoms, including behavioral disturbances, disorientation, and alertness fluctuations. The diagnostic tests performed at the time, including blood work, cerebrospinal fluid (CSF) analyses, electroencephalography, and neuroimaging, yielded nonspecific and occasionally misleading results. Consequently, the patients were repeatedly diagnosed as variably having CJD, epilepsy, vascular dementia, and encephalitis. The stored CSF samples of two patients tested negative at prion real‐time quaking‐induced conversion (RT‐QuIC), which we performed afterwards for research purposes. Neuropathological analysis revealed a differential involvement of various brain areas, with frontotemporal neocortices being the most affected. Conclusions: Our results confirm the significant clinical and instrumental heterogeneity of IVLBCL. Neuropathological evidence of the preferential involvement of frontotemporal neocortices, potentially conditioning the clinical phenotype, could be relevant to reach an early diagnosis. Finally, given the therapeutic implications of its misdiagnosis with CJD, we emphasize the utility of prion RT‐QuIC as a test for ruling out CJD in these patients. [ABSTRACT FROM AUTHOR]

A 55-year-old woman had remarkably generalized telangiectasia. Large atypical lymphoid cells were found within the lumen of small vessels, and CD20 was positive in her third skin biopsy. She was diagnosed with intravascular large B-cell lymphoma (IVLBCL) and went into remission of the skin manifestations after seven courses of R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone) and four courses of intravenous high-dose methotrexate (HD MTX). To our knowledge, this is the first case report of IVLBCL with generalized telangiectasia in China. From a review of the literature, we found that generalized telangiectasia is a remarkable manifestation of intravascular lymphoma, and the differential diagnosis of intravascular lymphoma and panniculitis or vasculitis is very important. When the diagnosis is confusing, multiple skin biopsies are useful.

Pulmonary lymphomas are rare. With the current less invasive approaches used to obtain material for diagnosis, the diagnosis of pulmonary lymphoma is now frequently established in a small biopsy rather than in a resection. Therefore, the diagnosis has become more challenging and requires correlation with the clinico-radiologic presentation and with ancillary studies (immunohistochemistry, flow cytometry, cytogenetics, and/or molecular analysis). Due to the rarity of pulmonary lymphomas, clinical suspicion of a lymphomatous process is low at initial presentation, and material may be only submitted for histopathology. For this reason, herein, we provide recommendations to arrive at the correct diagnosis of the most common lung B-cell lymphomas (marginal zone lymphoma of mucosa-associated lymphoid tissue, diffuse large B-cell lymphoma, intravascular large B-cell lymphoma, lymphomatoid granulomatosis) in the setting of small biopsies, utilizing only immunohistochemistry. The differential diagnosis varies according to the lymphoma subtype and includes reactive conditions, solid tumors, and other hematolymphoid malignancies. Although morphology and immunohistochemistry may be sufficient to establish a diagnosis, in some cases, the best recommendation is to obtain additional tissue via a VATS biopsy/wedge resection with material submitted for flow cytometry, cytogenetics, and/or molecular studies to be able to properly classify a pulmonary lymphoid process. [ABSTRACT FROM AUTHOR]

Abstract Background Intravascular large B‐cell lymphoma (lVLBCL) is a very rare type of large B‐cell lymphoma. Methods We conducted a retrospective study on IVLBCL patients treated from 2000 to 2016 in LYSA cooperative group centers. Results Sixty‐five patients were identified in 23 centers. Median age at diagnosis was 69 years (range 23–92). Thirty‐four patients (64%) had an IPI score >3 and 40 patients (67%) had a performance status ≥2. The most frequent extra‐nodal locations were bone marrow (n = 34; 52%), central nervous system (n = 25; 39%), and skin (n = 21; 33%). Nodal involvement and endocrine system were observed in 34% (n = 22) and 18% (n = 12) of all cases, respectively. Twenty‐six patients (41%) had macrophage activation syndrome. Tumor cells were frequently CD5 positive (52%) with a non‐germinal center origin (86%). BCL2 was expressed in 87% of all samples analyzed (n = 20) and 43% of patients had a MYC/BCL2 double expression. Fifty‐six patients were treated with a regimen of chemotherapy containing rituximab, among whom 73% reached complete remission. The median progression‐free survival (PFS) and median overall survival (OS) were 29.4 months and 63.8 months, respectively. History of autoimmune disorder (Hazard ratio [HR] 3.3 [1.4–7.8]; p

Hemophagocytic lymphohistiocytosis (HLH) is a fatal disease characterized by a highly inflammatory state due to the abnormal activation of T lymphocytes and macrophages. Miliary tuberculosis (MTB) is a rare cause of HLH and its clinical appearances occasionally resembles that of intravascular lymphoma (IVL). A 76-year-old woman presented with persistent fever and fatigue. Abnormal laboratory findings showing thrombocytopenia (13,000/μL), hypofibrinogenemia (101 mg/dL), hyperferritinemia (2,312 ng/mL), and markedly elevated soluble interleukin-2 receptor (sIL-2R) level (32,200 U/mL), in addition, hemophagocytosis in the bone marrow (BM) smear, were suggestive of IVL-associated HLH. The pathology of the BM biopsy specimen showed granuloma with non-caseous necrosis, and culture tests using sputum, gastric fluid, urine, and peripheral and bone marrow blood revealed the presence of Mycobacterium tuberculosis , leading to the final diagnosis of MTB-associated HLH. Anti-TB medications and corticosteroids were administered, but thrombocytopenia, hypofibrinogenemia, and hyperferritinemia persisted. Concomitant use of recombinant thrombomodulin (rTM) enabled regression of clinical status. In this case, BM biopsy served as the diagnosis of MTB-associated HLH, although IVL-associated HLH is initially suspected by an extremely high level of sIL-2R. Furthermore, this case report informs that using rTM could improve the outcomes of MTB-associated HLH. [ABSTRACT FROM AUTHOR]

Background: Intravascular large B-cell lymphoma (IVLBCL) is a rare subtype of extranodal B-cell lymphoma, which has traditionally been associated with poor outcomes. Despite increasing recognition, IVLBCL requires a high degree of clinical suspicion on the part of the clinician for its diagnosis. Case Series: We present four patient cases: A 69-year-old female with constitutional symptoms and cognitive decline; a 78-year-old female with kidney injury and constitutional symptoms whose disease rapidly progressed to multiorgan failure and death; a 70-year-old asymptomatic female with an incidentally found, enlarged thyroid; and a 63-year-old male with cytopenia and constitutional symptoms. Retrospective chart analysis was performed on these four patients diagnosed with IVLBCL at our Institute to identify the pathognomonic features of the disease and compare these to the published evidence. IVLBCL has a heterogeneous presentation, as seen in our four patients. The disease is characterized by the exclusive presence of malignant cells inside the blood vessels and lack of organ infiltration. Traditional preliminary diagnostic modalities such as imaging are usually inconclusive, given the paucity of lymphomatous aggregates. A bone marrow biopsy, random skin biopsies, or a focal organ biopsy in appropriate cases is required for diagnosis. Immunosuppression might play a role in the pathogenesis. Timely initiation of aggressive cancer-directed therapy was associated with improved outcomes. Monitoring for disease response and relapse continues to be a challenge. Conclusion: Our mini-series highlights the significance of timely diagnosis and intervention in IVLBCL and emphasizes the importance of further research to determine its association with immunosuppression. [ABSTRACT FROM AUTHOR]

Intravascular lymphoma is a rare presentation of lymphoma with a predilection to the central nervous system (CNS). A 9‐year‐old male‐neutered Collie presented with a 3‐month history of lymphadenopathy and a 6‐day history of an acute onset, progressive, painful, symmetrical L4‐S3 myelopathy. Magnetic resonance imaging revealed multifocal extradural spinal cord compressions from L3 to L6 secondary to a bilaterally enlarged and occluded ventral vertebral venous plexus (VVVP). Histopathology revealed low‐grade lymphoma within the venous plexus in the lumbar vertebral column, tracheobronchial lymph nodes, and tonsils. Intravascular lymphoma should be considered a differential diagnosis for enlarged VVVP causing compression of the spinal cord. [ABSTRACT FROM AUTHOR]

Pulmonary lymphomas are rare. With the current less invasive approaches used to obtain material for diagnosis, the diagnosis of pulmonary lymphoma is now frequently established in a small biopsy rather than in a resection. Therefore, the diagnosis has become more challenging and requires correlation with the clinico-radiologic presentation and with ancillary studies (immunohistochemistry, flow cytometry, cytogenetics, and/or molecular analysis). Due to the rarity of pulmonary lymphomas, clinical suspicion of a lymphomatous process is low at initial presentation, and material may be only submitted for histopathology. For this reason, herein, we provide recommendations to arrive at the correct diagnosis of the most common lung B-cell lymphomas (marginal zone lymphoma of mucosa-associated lymphoid tissue, diffuse large B-cell lymphoma, intravascular large B-cell lymphoma, lymphomatoid granulomatosis) in the setting of small biopsies, utilizing only immunohistochemistry. The differential diagnosis varies according to the lymphoma subtype and includes reactive conditions, solid tumors, and other hematolymphoid malignancies. Although morphology and immunohistochemistry may be sufficient to establish a diagnosis, in some cases, the best recommendation is to obtain additional tissue via a VATS biopsy/wedge resection with material submitted for flow cytometry, cytogenetics, and/or molecular studies to be able to properly classify a pulmonary lymphoid process.

B-cell lymphoma, dermato-oncology, classic variant, IVLBCL, intravascular lymphoma, skin cancer, R-CHOP Intravascular large B-cell lymphoma presenting as an indurated subcutaneous plaque Keywords: B-cell lymphoma; classic variant; dermato-oncology; intravascular lymphoma; IVLBCL; R-CHOP; skin cancer EN B-cell lymphoma classic variant dermato-oncology intravascular lymphoma IVLBCL R-CHOP skin cancer 447 449 3 08/14/23 20230801 NES 230801 DATA AVAILABILITY STATEMENT The data that support the findings of this study are available from the corresponding author upon request. [Extracted from the article]

Jing Wang,1 Muhamad Alhaj Moustafa,2 Justin J Kuhlman,1 Karan Seegobin,2 Liuyan Jiang,3 Vivek Gupta,4 Han W Tun2 1Department of Internal Medicine, Mayo Clinic Florida, Jacksonville, FL, 32224, USA; 2Division of Hematology and Medical Oncology, Mayo Clinic Florida, Jacksonville, FL, 32224, USA; 3Department of Pathology and Laboratory Medicine, Mayo Clinic Florida, Jacksonville, FL, 32224, USA; 4Department of Radiology, Mayo Clinic Florida, Jacksonville, FL, 32224, USACorrespondence: Han W Tun, Mayo Clinic Florida Division of Hematology/Oncology, 4500 San Pablo Road S, Jacksonville, FL, 32224, USA, Tel +1 904-953-2000, Email tun.han@mayo.eduAbstract: Intravascular large B cell lymphoma (IVL) is a rare subtype of diffuse large B cell lymphoma confined to small blood vessels with a predilection for CNS involvement. The prognosis of IVL with CNS involvement (CNS-IVL) is extremely poor. The optimal treatment for CNS-IVL is not well defined. Thus, we report three patients with CNS-IVL successfully treated with a CNS-centric approach consisting of high-dose methotrexate (HDMTX) and high-dose Ara-C (HiDAC) based CNS-directed chemoimmunotherapy (CIT) alternating with anthracycline-based CIT. Our rationale for intensifying the CNS-directed therapy is the presence of intracerebral bleeding in two of our patients which would result in extravasation of lymphoma cells into the cerebral parenchyma with the development of CNS lymphoma. All three patients have achieved excellent therapeutic outcomes. Two patients with intracerebral bleeding have been in complete remission (CR) for about 11 years and 4 years. One patient was successfully induced into CR about 10 months ago and currently is in CR. This unique therapeutic approach should be further explored for CNS-IVL.Keywords: intravascular lymphoma, CNS involvement, CNS-directed chemoimmunotherapy, anthracycline-based chemoimmunotherapy, high-dose methotrexate, high-dose Ara-C

Background: Intravascular large B‐cell lymphoma (lVLBCL) is a very rare type of large B‐cell lymphoma. Methods: We conducted a retrospective study on IVLBCL patients treated from 2000 to 2016 in LYSA cooperative group centers. Results: Sixty‐five patients were identified in 23 centers. Median age at diagnosis was 69 years (range 23–92). Thirty‐four patients (64%) had an IPI score >3 and 40 patients (67%) had a performance status ≥2. The most frequent extra‐nodal locations were bone marrow (n = 34; 52%), central nervous system (n = 25; 39%), and skin (n = 21; 33%). Nodal involvement and endocrine system were observed in 34% (n = 22) and 18% (n = 12) of all cases, respectively. Twenty‐six patients (41%) had macrophage activation syndrome. Tumor cells were frequently CD5 positive (52%) with a non‐germinal center origin (86%). BCL2 was expressed in 87% of all samples analyzed (n = 20) and 43% of patients had a MYC/BCL2 double expression. Fifty‐six patients were treated with a regimen of chemotherapy containing rituximab, among whom 73% reached complete remission. The median progression‐free survival (PFS) and median overall survival (OS) were 29.4 months and 63.8 months, respectively. History of autoimmune disorder (Hazard ratio [HR] 3.3 [1.4–7.8]; p < 0.01), nodal involvement (HR 2.6 [1.4–5.1]; p < 0.01), lack of anthracycline (HR 0.1 [0–0.4] for use; p < 0.001), or no intensification at first‐line regimen (p = 0.02) were associated with worse PFS. High‐dose methotrexate use was not associated with better PFS or OS. Conclusions: Our study highlights the aggressive clinical picture of IVLBCL, in particular the frequency of macrophage activation syndrome, and the need for new therapies despite a response to R‐CHOP‐like regimen similar to non‐intravascular diffuse large B‐cell lymphomas. [ABSTRACT FROM AUTHOR]

Abstract Purpose We report a case of intravascular lymphoma with primary vitreoretinal lymphoma-like fundus findings. Case A 61-year-old man with a one-week history of temporal visual field defect in the left eye was referred by a local ophthalmologist to our department. A yellowish-white raised patchy lesion was found in the nasal fundus of the left eye. Vitreoretinal lymphoma was suspected, and vitrectomy was performed in the left eye for diagnostic purpose. However, vitreous interleukin-10 concentration was low and no significant result was obtained. He had fever of around 38 °C, and respiratory failure that started 2 weeks before ophthalmological examination, worsened. Intravascular lymphoma was diagnosed from the results of histopathological examinations of transbronchial lung biopsy, bone marrow biopsy and random skin biopsy. With the start of systemic chemotherapy, the subretinal lesions shrank gradually and systemic condition was stable. However, 5 months after the start of chemotherapy, spread to the central nervous system was observed, and chimeric antigen receptor T cell (CAR-T) therapy was started in another hospital. After the start of CAR-T therapy, the subretinal lesions shrank further. Conclusions Intravascular lymphoma may be accompanied by primary vitreoretinal lymphoma-like intraocular lesions. If intraocular lesions are accompanied by systemic symptoms such as fever of unknown origin, the possibility of intravascular lymphoma should be suspected and systemic work-up should be performed.

Key message There are more complications in transbronchial lung cryobiopsy than in a conventional transbronchial lung biopsy. Respiratory endoscopists should be aware of the potential complications, including rare complications such as hemothorax.

Intravascular large B-cell lymphoma (IVLBCL) is a rare hematological malignancy where its development in the intravascular environment is the main characteristic. Despite its ability to affect multiple organic systems, there is a tropism for the central nervous system, which may be related to several clinical syndromes, making this condition a great mimic and consequently a diagnostic challenge. Rapidly progressive dementia may be one of the presenting phenotypes of IVLBCL. This case report aims to highlight the main red flags, such as sustained elevation of lactate dehydrogenase, organomegaly and specific lesions with vasculitis-like bleeding, all that can be used as clinical clues to direct the differential diagnosis. In addition, it reinforces the role of early brain biopsy in this context, since IVLBCL is a treatable disease., Competing Interests: Declaration of competing interest The authors declare no conflict of interest., (Copyright © 2024 Elsevier B.V. All rights reserved.)

Chae Hong Lim,1 Sang Eun Yoon,2 Won Seog Kim,2 Kyung-Han Lee,3,4 Seok Jin Kim2,4 1Department of Nuclear Medicine, Soonchunhyang University College of Medicine, Seoul, Korea; 2Division of Hematology-Oncology, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea; 3Department of Nuclear Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea; 4Department of Health Sciences and Technology, Samsung Advanced Institute for Health Sciences and Technology, Sungkyunkwan University, Seoul, KoreaCorrespondence: Seok Jin KimDivision of Hematology-Oncology, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, 81 Irwon-ro, Gangnam-gu, Seoul, 06351, KoreaTel +82-2-3410-1766Fax +82-2-3410-1754Email kstwoh@skku.eduKyung-Han LeeDepartment of Nuclear Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, 81 Irwon-ro, Gangnam-gu, Seoul, 06351, KoreaTel +82-2-3410-2630Fax + 82-2-3410-2638Email khnm.lee@samsung.comPurpose: The clinical value of 18F-fluorodeoxyglucose positron emission tomography/computed tomography (FDG PET/CT) in intravascular large B-cell lymphoma (IVLBCL) is unknown. This study investigated the association between PET/CT features and prognosis in IVLBCL patients.Patients and Methods: Subjects were 30 newly diagnosed Asian variant IVLBCL patients at a single institution. Baseline PET/CT was analyzed for the distribution and intensity of FDG lesions, and PET/CT pattern groups were compared for the outcome.Results: Eight patients had hypermetabolic lymph node (LN) lesions (Nodal group). The remaining 22 patients with extranodal (EN) involvement were categorized into Deauville score 3– 4 (EN/DS3-4; n = 14) and DS5 groups (EN/DS5; n = 8). First-line therapy resulted in a complete or partial response in 75.0%, 64.3%, and 100% of the respective groups. Treatment-related deaths occurred in one nodal group and three EN/DS3-4 group cases, but none among the EN/DS5 group. During 56 months of follow-up, disease progression or relapse occurred in five, four, and one case of respective groups. Cancer-related death occurred more frequently in the Nodal (n = 6) and EN/DS3-4 groups (n = 7) than the EN/DS5 group (n = 1; P = 0.041). Nodal and EN/DS3-4 groups had worse 5-year event-free survival (EFS; 25.0% and 49.0%, respectively, P = 0.010 and 0.076) and overall survival (OS; 33.3% and 48.2%, P = 0.010 and 0.068) compared to the EN/DS5 group (87.5% EFS and 87.5% OS).Conclusion: In patients with Asian variant IVLBCL, the distribution and intensity of FDG uptake lesions on PET/CT can be useful for predicting treatment outcomes and survival.Keywords: intravascular lymphoma, 18F-fluorodeoxyglucose, PET/CT, prognosis

Intravascular large B-cell lymphoma (IVLBCL) is a very rare subtype of malignant lymphoma that is difficult to diagnose. Cases of myocardial infarction caused by IVLBCL are even rarer. Herein, we report a case presenting with heart failure and delayed enhancement in the hypokinetic cardiac septum on contrast-enhanced cardiac magnetic resonance imaging. Myocardial biopsy showed large B-cell lymphoma cells in the microvessels within the myocardium. To the best of our knowledge, this is the first report of imaging findings of cardiac involvement in IVLBCL.

Intravascular lymphoma (IVL) is a rare type of extranodal lymphoma that selectively affects small blood vessels. We report a patient who presented with dyspnea and weight loss as well as refractory shock and multiple‐organ dysfunction. The postmortem revealed disseminated involvement of an IVL but no evidence of infection. [ABSTRACT FROM AUTHOR]

Abstract Intravascular lymphoma (IVL) is a rare type of extranodal lymphoma that selectively affects small blood vessels. We report a patient who presented with dyspnea and weight loss as well as refractory shock and multiple‐organ dysfunction. The postmortem revealed disseminated involvement of an IVL but no evidence of infection.

A 59-year-old previously healthy woman presented with a six-month history of fever, nonproductive cough, and weight loss. The cause of these symptoms remained obscure despite a thorough, month-long hospitalization. On presentation, she was normotensive with a pulse of 98 beats/minute, respiratory rate of 20 breaths/minute, and a temperature of 39.4C. She was emaciated. Physical examination was notable for faint bibasilar crackles on lung auscultation. Initial laboratory testing revealed pancytopenia. Peripheral smear demonstrated normocytic, normochromic anemia without immature cells or schistocytes. Other notable laboratory findings included elevated levels of lactate dehydrogenase, elevated ferritin, and elevated levels of fasting serum triglycerides. A comprehensive laboratory evaluation for connective tissue disease was negative. Plain chest radiography was normal while computed tomography (CT) of the chest demonstrated sub-centimeter nodules in a branching centrilobular pattern as well as in a peri-lymphatic distribution without associated lymphadenopathy or organomegaly. The above constellation of laboratory abnormalities raised concern for hemophagocytic lymphohistiocytosis (HLH). Soluble IL-2 (CD25) receptor levels were markedly elevated. Bronchoscopy with transbronchial biopsies of the right lower lobe was performed, revealing intravascular lymphoma associated with HLH. Our case emphasizes the need for clinicians to consider vascular causes of tree - in-bud nodules in addition to the conventional bronchiolar causes. The case also is a reminder of the need to conduct an exhaustive search for malignancy, in patients with HLH. [ABSTRACT FROM AUTHOR]

A 67-year-old woman with paraplegia and acute painless vision loss in the right eye 6 months ago showed triangular regions of pigmentary change in both eyes suggestive of choroidal ischemia. Extensive workup showed negative results. The patient subsequently developed vision loss in her left eye. Brain biopsy revealed intravascular lymphoma. Purpose and Methods: To report a case of bilateral ocular ischemia caused by intravascular lymphoma with the presence of bilateral triangular sign of Amalric. Results: A 67-year-old woman was admitted to our hospital for progressive paraplegia and a 6-month history of acute painless vision loss in the right eye. Initial examination showed vision of counting fingers in the right eye and 20/20 in the left eye with normal anterior examination. Fundus examination revealed a likely previous central retinal artery occlusion in the right eye with pale nerve and attenuated vessels. Both fundi had triangular regions of pigmentary change known as the triangular sign of Amalric, indicative of choroidal ischemia. However, the left eye was asymptomatic. Neuroimaging revealed multifocal enhancing lesions throughout the central nervous system of unclear etiology. An extensive neurologic and systemic workup was unrevealing, including a brain biopsy, and empiric treatment for an unspecified inflammatory condition with intravenous corticosteroids was initiated. During her hospitalization, she developed acute painless vision loss in the left eye, and examination showed no light perception vision in both eyes with signs of acute retinal and choroidal ischemia in the left eye. A subsequent brain biopsy revealed intravascular lymphoma. Conclusion: Triangular pigmentary changes indicate choroidal ischemia and can be seen in many conditions. This patient presented with the triangular sign of Amalric in both eyes, including her asymptomatic left eye. Intravascular lymphoma should be considered in cases of concomitant inflammatory brain lesions and chorioretinal ischemia. [ABSTRACT FROM AUTHOR]

One of the rare variants of extranodal large B-cell lymphoma is intravascular large B-cell lymphoma (IVLBCL). Characteristics of IVLBCL include intraluminal selective proliferation of atypical lymphoid cells in small to medium-sized vessels. The etiologic of IVLBCL is unknown, but due to the growth pattern of this tumor, it is speculated that IVLBCL is caused by a defect in homing receptor of tumor cells. IVLBCL can involve any organ but central nervous system, lungs, and skin are the most involved sites. IVLBCL does not usually involve lymph nodes. IVLBCL mainly occurs in the middle aged to elderly population with a slight male predominance. Generally, IVLBCL is aggressive and rapidly fatal if left untreated. We here reported two cases of IVLBCL who succumbed to the disease at initial phase of treatment to emphasize the difficulty in diagnosis of IVLBCL due to its exclusive intravascular growth pattern and fulminant clinical course.

Purpose: We report a case of intravascular lymphoma with primary vitreoretinal lymphoma-like fundus findings. Case: A 61-year-old man with a one-week history of temporal visual field defect in the left eye was referred by a local ophthalmologist to our department. A yellowish-white raised patchy lesion was found in the nasal fundus of the left eye. Vitreoretinal lymphoma was suspected, and vitrectomy was performed in the left eye for diagnostic purpose. However, vitreous interleukin-10 concentration was low and no significant result was obtained. He had fever of around 38 °C, and respiratory failure that started 2 weeks before ophthalmological examination, worsened. Intravascular lymphoma was diagnosed from the results of histopathological examinations of transbronchial lung biopsy, bone marrow biopsy and random skin biopsy. With the start of systemic chemotherapy, the subretinal lesions shrank gradually and systemic condition was stable. However, 5 months after the start of chemotherapy, spread to the central nervous system was observed, and chimeric antigen receptor T cell (CAR-T) therapy was started in another hospital. After the start of CAR-T therapy, the subretinal lesions shrank further. Conclusions: Intravascular lymphoma may be accompanied by primary vitreoretinal lymphoma-like intraocular lesions. If intraocular lesions are accompanied by systemic symptoms such as fever of unknown origin, the possibility of intravascular lymphoma should be suspected and systemic work-up should be performed. [ABSTRACT FROM AUTHOR]

Background/Aims There are limited data about the influence of the central nervous system (CNS) involvement on the prognosis for patients with the Asian variant of intravascular large B-cell lymphoma (IVLBCL). Methods We analyzed 46 patients who were diagnosed with IVLBCL between 2001 and 2018. All patients were treated with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP). Results CNS involvement was diagnosed by cerebrospinal fluid analysis (n = 6) and brain imaging (n = 5) in 11 patients at diagnosis, and four cases with CNS relapse were found. Thus, 15 patients had CNS involvement (15/46, 33%). The clinical characteristics were not different between patients with and without CNS involvement, but all patients with CNS involvement belonged to the high-risk group of CNS-International Prognostic Index (IPI). Thirty-one patients achieved a complete response (67%, 31/46) whereas eight patients showed disease progression and six patients died after the first or second cycle of R-CHOP. CNS-directed therapy such as high-dose methotrexate was combined with R-CHOP for patients with CNS involvement, and five patients were alive without relapse. The median overall survival of all patients was 45.0 months, and overall survival was not different according to the involvement of CNS. Conclusions The treatment outcome of patients with the Asian variant of IVLBCL is still not satisfactory. The prediction of CNS involvement based on the clinical features including CNS-IPI score might not serve to identify patients at high risk of CNS involvement, either. Thus, more effective strategies for diagnosis and treatment should be developed.

Abstract Intravascular B‐cell Lymphoma is a rare lymphoproliferative disorder with a none specific clinical presentation. The association of cutaneous telangiectasia‐like lesions and elevated inflammatory markers should be guaranteed a skin biopsy.

The diagnosis of intravascular large B-cell lymphoma (IVLBCL) is often challenging owing to its nonspecific clinical manifestations and imaging findings. Herein, we present a rare case of IVLBCL in which seizure was the initial symptom, and unilateral hemispheric hyperperfusion on arterial spin labeling (ASL) was the only abnormal finding observed on brain magnetic resonance imaging (MRI). A 68-year-old male with a history of hypertension and type 2 diabetes was transferred to the emergency room owing to the sudden onset of altered consciousness and abnormal behavior. Upon arrival, the patient was disoriented and confused, and cerebrospinal fluid analysis revealed pleocytosis and elevated protein level. Even after the administration of acyclovir and antiepileptic drugs, his consciousness remained impaired, with repeated transient right hemiparesis indicating a focal seizure. The initial and follow-up MRI scans showed no obvious abnormalities in diffusion-weighted imaging (DWI), T2-weighted imaging, or susceptibility-weighted imaging (SWI); however, ASL revealed markedly increased blood flow to the left hemisphere. Subsequently, the rapid elevation of serum lactate dehydrogenase (LDH) and soluble interleukin-2 receptor (sIL-2R) levels after admission led to the diagnosis of IVLBCL by random skin biopsy and bone marrow examination. Despite the initiation of chemotherapy, the patient developed tumor lysis syndrome and succumbed to multiple organ failure. This case underscores the importance of considering IVLBCL in adult patients with refractory seizures and highlights the potential utility of ASL on MRI for early diagnosis., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Imamura et al.)

Intravascular large B-cell lymphoma (IVLBCL) is a rare lymphoma entity with intravascular proliferation of clonal lymphocytes restricted to the lumen of small- and medium-sized blood vessels. In this case report, we describe a 70-year-old lady on maintenance hemodialysis who presented with altered sensorium and painful lesions over her inner thigh. While preliminary investigations were favoring calciphylaxis, it was the skin biopsy that showed vasculopathic lymphoid infiltrate with focal panniculitis, consistent with intravascular lymphoma. Although overall survival is poor, prompt use of intensive therapies have shown to improve the outcome. [ABSTRACT FROM AUTHOR]

Intravascular large B-cell lymphoma (IVLBCL) is a rare type of extranodal large B-cell lymphoma characterized by the selective growth of lymphoma cells within the lumina of vessels. The patient usually presents with nonspecific symptoms and a remarkable deterioration in performance status. The occurrence of synchronous IVLBCL and renal cell carcinoma (RCC) is extremely rare. A right kidney tumor was found in a 72-year-old man with a history of low back pain. The kidney was enlarged, with a tumor mass measuring 4.5 × 4 × 4 cm. Sections exhibited a RCC (clear cell type, nuclear grade I). Also an extensive tumor affecting capillaries and small veins was present, positive for CD45, CD20, BCL-2, and MUM1/IRF-4, consistent with IVLBCL. The lymphoma was circumscribed to the RCC. The final diagnosis was IVLBCL with a RCC as collision tumor. After that, with neurological findings, central nervous system compromise by lymphoma was made. The patient started a first cycle of chemotherapy, progressive deterioration of the sensorium, and positive blood cultures for Klebsiella pneumoniae and Escherichia coli. The patient died 8 days later of acute respiratory failure. No autopsy was done. IVLBCL is an aggressive and systemic disease characterized by massive proliferation of tumor cells without a known primary site. Clinical identification and histopathologic diagnosis are relevant issues in the therapeutic management of these lymphomas. Until now, only one case of IVLBCL coexisting with RCC has been reported. In this article, we report a second case of IVLBCL with RCC simultaneous, as an unusual collision tumor. [ABSTRACT FROM AUTHOR]

Background: The yield of skin biopsies in the evaluation of intravascular lymphoma (IVL) is largely unknown in Western patients. Most data supporting this test come from Asian populations, in which both prevalence and disease presentation seem to differ.Objective: To determine the yield and diagnostic properties of skin biopsy in the evaluation of IVL.Methods: We reviewed skin biopsy pathology reports of 50 patients being evaluated for IVL to calculate the diagnostic yield of this test. An additional 6 patients, who underwent skin biopsies after the diagnosis of IVL was made by other means, were included to calculate the sensitivity and specificity of our index test.Results: Skin biopsy samples were positive for 5 of the 50 patients being investigated for IVL. Sensitivity was 50% and specificity was 100%.Limitations: Only pathology reports containing IVL as an indication for the biopsy were retrieved. This might have excluded patients in whom the disease was considered but was not deemed likely enough to be listed as the indication for the test, inflating our estimative of skin biopsy yield.Conclusion: A relatively high diagnostic yield was found in the evaluation of IVL among patients with a diverse presentation in a Western hospital. [ABSTRACT FROM AUTHOR]

Pseudolymphomatous infiltrates associated with angiosarcoma are a rarely reported phenomenon. Recognition of this reactive process is critical to making an accurate diagnosis, both in diagnosing the angiosarcoma and in avoiding an incorrect diagnosis of lymphoma. Here, we present a novel histopathologic pattern, angiosarcoma with a prominently intravascular atypical lymphoid component, mimicking intravascular T‐cell lymphoma. Interestingly, serial biopsies in this case revealed a progressive increase in lymphocyte density and intravascular component over time. Despite prior reports of improved progression‐free survival and overall survival of patients with pseudolymphomatous angiosarcoma, this patient showed rapid disease progression. [ABSTRACT FROM AUTHOR]

Background: Intrathoracic involvement with lymphomas is common and manifests lymphadenopathy as well as a wide spectrum of imaging abnormalities in the lungs. Intravascular large B-cell lymphoma (IVLBCL) is a rare extranodal subtype of large B-cell lymphoma that typically involves small blood vessels and is difficult to detect.Methods: Using a computer-assisted search, we identified patients with histopathologically proven IVLBCL in the lungs at Mayo Clinic from 2001 through 2018. Medical records, imaging studies, and pathologic specimens were reviewed.Results: A total of 5 patients were diagnosed with a median age at diagnosis of 68 years (range, 44-73); 4 patients were male. The diagnosis of IVLBCL was achieved by surgical lung biopsy in 3 and at autopsy in 2. At presentation, all 5 patients had dyspnea and systemic symptoms including fever, fatigue, night sweats, and/or weight loss. Chest radiography and computed tomography (CT) failed to demonstrate the diffuse infiltrative process; positron emission tomography (PET) scan performed in 2 patients did not show fluorodeoxyglucose (FDG) uptake in the lungs. Pulmonary function tests obtained in 3 patients showed reduced diffusing capacity in all; transthoracic echocardiography yielded evidence of pulmonary hypertension in 2 of 4 patients. All 3 patients diagnosed antemortem underwent chemotherapy with 1 patient remaining alive at 4 years after diagnosis.Conclusions: IVLBCL is difficult to diagnose given variable and nonspecific clinical presentations. Microvascular disease processes such as IVLBCL should be kept in mind in cases of undiagnosed progressive dyspnea accompanied by systemic symptoms even when imaging studies are unrevealing. [ABSTRACT FROM AUTHOR]

Intravascular large cell lymphoma (IVLCL) is a rare disease characterized by proliferation of malignant lymphocytes within the small blood vessel lumens. The association of IVLCL with autoimmune hemolytic anemia (AIHA) has been described in a single case report, but the true prevalence of this co-occurrence is not known because of declining autopsy rates. Here, we report a case of a 41-year-old woman who carried a diagnosis of AIHA for 2 years, with repeated hemolytic episodes that were initially well controlled with immunomodulatory treatment. At her last presentation, the patient developed rapidly progressive neurologic symptoms and leukoencephalopathy on MRI; she died 4 weeks later with a clinical impression of thrombotic microangiopathy, a known complication of AIHA. At autopsy, the brain showed widespread platelet thrombi and intraparenchymal hemorrhages characteristic of this disorder. In addition, there was evidence of a clinically unsuspected IVLCL, most likely of B-cell lineage. This case illustrates a potential association between IVLCL and AIHA, highlights the need for broad differential diagnosis in cases with atypical disease presentation or progression, and underlines the importance of autopsy in establishing the full cause of morbidity and mortality.

We present the first report of two rare yet remarkably similar autopsy cases of Kaposi sarcoma (KS) and intravascular human herpesvirus 8 (HHV8) positive lymphoproliferative disorder in renal transplant patients. It is well established that HHV8 infection causes Kaposi sarcoma (KS). More recently, it is recognized that HHV8 is also related to several lymphoproliferative conditions. These are poorly characterized and often difficult to diagnose. In both cases described herein, the diagnoses of multifocal hepatic KS and intravascular HHV8 positive (EBV negative) systemic diffuse large B-cell lymphoma, NOS were made at autopsy. Given the findings we describe in cases with fatal outcomes, we discuss the implications of HHV8 screening in solid allograft recipients.

Abstract Background Intravascular large B-cell lymphoma (IVLBCL) is a rare subtype of extranodal diffuse large B-cell lymphoma characterized by proliferation of B cells within small vessels. Herein, we report a case of a 77-year-old man who presented with IVLBCL and massive tumor formation on the aortic wall who was previously diagnosed with sarcoidosis and focal segmental glomerulosclerosis (FSGS). To our knowledge, this is the first reported case of an IVLBCL with aortic tumor formation. Case presentation A 77-year-old ambulatory man with sarcoidosis and FSGS had neurological symptoms for nine months. The patient presented to the emergency department with sudden left leg pain, and was diagnosed with acute femoral artery occlusion. Emergency thrombectomy was performed subsequently. Pathological evaluation of the thrombi revealed that its surface was filled with large atypical B cells. Bone marrow biopsy showed infiltration of large atypical B cells within the small vessels. IVLBCL was suspected and further examination was planned, but the patient died due to sudden respiratory and cardiac arrest on hospital day twelve. Autopsy revealed intravascular tumors adherent to the aortic arch, left ventricle, and the abdominal aorta. All enlarged lymph nodes and the ventricular septum of the heart showed hyalinized lesions with granular formation consistent with sarcoidosis. The patient was diagnosed with IVLBCL with aortic tumor formation complicated with sarcoidosis and FSGS. Conclusions IVLBCL may present with tumor formation on the aortic wall. Although the cause of its affinity to the aortic wall is yet unknown, autopsy findings imply that arteriosclerosis may have contributed to the tumor formation. The literature suggests that T-cell abnormalities could possibly be the common etiology of intravascular lymphoma, sarcoidosis, and FSGS.

Livedo reticularis (LR) is a net-like cyanotic skin pattern resulting from cutaneous blood flow disturbance associated with several medical conditions. The authors present a case of a 77-year-old woman with a history of asthenia over one year, admitted to the hospital with sepsis by pneumonia. During her hospitalization, she developed a livedoid rash, and skin biopsy revealed infiltrates of atypical mononuclear cells causing capillary lumen obstruction. Intravascular large B-cell lymphoma was suspected and confirmed by bone marrow study. This case highlights the importance of being aware of different medical conditions associated with LR and the atypical presentation of a lymphoproliferative disorder.

Primary effusion lymphoma is a rare, clinically aggressive large B-cell neoplasm universally associated with human herpesvirus 8 that occurs in the setting of immune compromise. It is classically described as a lymphomatous effusion occurring within body cavities. Recently, however, solid tumor masses, and rarely an intravascular form, have been described. We report a case of a cutaneous intravascular primary effusion lymphoma occurring within ectatic vascular spaces of a Kaposi sarcoma skin lesion in a human immunodeficiency virus–positive adult. Human herpesvirus 8 immunohistochemistry was positive in the nuclei of the Kaposi sarcoma spindled cells as well as within large intravascular plasmacytoid cells. This unusual case highlights the importance of careful assessment of the nature of human herpesvirus 8–positive staining cells in an otherwise typical Kaposi sarcoma. A careful search for dual pathology in immune-compromised patients as well as the importance of histologic assessment of skin lesions in human immunodeficiency virus–positive patients is also highlighted. [ABSTRACT FROM AUTHOR]

Intralymphatic histiocytosis (ILH) is a rare skin benign condition observed in a variety of inflammatory settings. It is characterized by the presence of ectatic dermal lymphatic vessels containing aggregates of histiocytes. Associated conditions that have been identified include rheumatoid arthritis, metallic orthopedic implants, inflammatory bowel disease, and malignancies of the breast, skin, and colon. Some cases with no attributable underlying cause have been described. The pathophysiology of ILH is not well understood. It has been proposed that it may represent macrophage migration during immune activation. Herein, we present the first description of ILH observed in the healing phase of cellulitis on the skin of the breast. Awareness of this possibility is important when the diagnosis of intravascular carcinomatosis is being considered. [ABSTRACT FROM AUTHOR]

One of the rare variants of extranodal large B-cell lymphoma is intravascular large B-cell lymphoma (IVLBCL). Characteristics of IVLBCL include intraluminal selective proliferation of atypical lymphoid cells in small to medium-sized vessels. The etiologic of IVLBCL is unknown, but due to the growth pattern of this tumor, it is speculated that IVLBCL is caused by a defect in homing receptor of tumor cells. IVLBCL can involve any organ but central nervous system, lungs, and skin are the most involved sites. IVLBCL does not usually involve lymph nodes. IVLBCL mainly occurs in the middle aged to elderly population with a slight male predominance. Generally, IVLBCL is aggressive and rapidly fatal if left untreated. We here reported two cases of IVLBCL who succumbed to the disease at initial phase of treatment to emphasize the difficulty in diagnosis of IVLBCL due to its exclusive intravascular growth pattern and fulminant clinical course. [ABSTRACT FROM AUTHOR]

Intravascular lymphoma (IVL) is a rare and clinically devastating subtype of extranodal diffuse large B-cell lymphoma with a distinct presentation. Diagnostic difficulty derives from marked variability in clinical presentations and nonspecific laboratory and radiological findings, especially when central nervous system (CNS) symptoms are the only manifestation. Establishing the diagnosis premortem thus remains a major challenge. We describe a 70-year-old male with CNS IVL. He presented with acute onset of neurocognitive impairments. Diffusion-weighted magnetic resonance imaging (MRI) showed multiple high-intensity areas suggesting occlusive cerebrovascular disease due to emboli, but extensive investigations detected no embolic sources. Intracranial neoplasm was included in a differential diagnosis based on elevated serum lactate dehydrogenase and interleukin 2 receptor levels. Gadolinium-enhanced MRI or 18-fluorodeoxyglucose positron emission tomography (PET) failed to demonstrate specific findings leading to a definite diagnosis, while 11C-methionine PET (MET-PET) distinctively demonstrated an area of focally increased MET uptake in the frontal cortex, suggesting the extent of tumor infiltration. Stereotactic biopsy was conducted under MET-PET imaging guidance and immunohistological examinations confirmed the proliferation and aggregation of CD20-positive lymphoma cells within the lumina of small blood vessels. The findings of the present case first suggest that MET-PET may provide important information on the diagnosis of CNS IVL and on the selection of the optimal site for brain biopsy. Further investigation is necessary to clarify whether positive findings on MET-PET are truly specific and pathognomonic for CNS IVL.

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Twenty-eight patients were identified with large B-cell lymphoma of the uvea. Uveal involvement was iris (1 case), ciliary body (1 case), or choroidal in 14 cases. Panuveal involvement was observed in 12 cases. The clinical presentation could be categorized into uveitis (8), intraocular mass (9), neovascular glaucoma (4), and vascular disorders (4). The majority (21 cases, 77%) were diagnosed at autopsy (11) or after enucleation (10). Only 7 were diagnosed with conservative techniques. Histopathologically, 3 distinct subgroups of large B-cell lymphoma could be identified: 15 were characterized as diffuse large B-cell lymphoma, 11 as intravascular large B-cell lymphoma, and 2 as plasmablastic lymphoma. All cases had a poor prognosis, with a median survival of 14 months. Most cases (19, 67%) represented secondary uveal involvement with widespread systemic lymphoma at ophthalmic presentation. Six cases were treated with radiotherapy, most of these diagnosed before the 1990s (4). Subsequent cases (9) received systemic or local chemotherapy and adjunct radiotherapy, depending on the organs affected. Two cases were treated only with enucleation, and systemic treatment was not specified in 13 cases. Large B-cell lymphoma can rarely involve the uvea. The presenting features are nonspecific, often leading to enucleation. Effective therapy is not known. In all 3 variants, the aggressive nature and widespread involvement at ophthalmic presentation is associated with short survival. [ABSTRACT FROM AUTHOR]

Intravascular lymphoma (IVL) is a rare subtype of extranodal lymphomas that is characterized by the selective growth of neoplastic cells within the lumen of small vessels. Authors document the case of an unexpected death caused by an undiagnosed intravascular large B‐cell lymphoma with multi‐organ involvement, which had initially manifested as an infection and then as an unclarified central nervous system pathology. Histological examination showed a diffuse intravascular large B‐cell brain lymphoma with prominent cerebral involvement. The relevance of the case report reveals the importance of an autopsy of an extremely rare and threatening pathology that in most cases is diagnosed only postmortem. As a result, the role of the forensic pathologist becomes particularly important. When specifically performing an in‐depth autopsy evaluation with a specific histologic analysis, it is possible to identify the intravascular lymphoma and declare a more accurate cause of death. [ABSTRACT FROM AUTHOR]

Intravascular lymphoma (IVL) is a rare subtype of extranodal lymphomas that is characterized by the selective growth of neoplastic cells within the lumen of small vessels. Authors document the case of an unexpected death caused by an undiagnosed intravascular large B‐cell lymphoma with multi‐organ involvement, which had initially manifested as an infection and then as an unclarified central nervous system pathology. Histological examination showed a diffuse intravascular large B‐cell brain lymphoma with prominent cerebral involvement. The relevance of the case report reveals the importance of an autopsy of an extremely rare and threatening pathology that in most cases is diagnosed only postmortem. As a result, the role of the forensic pathologist becomes particularly important. When specifically performing an in‐depth autopsy evaluation with a specific histologic analysis, it is possible to identify the intravascular lymphoma and declare a more accurate cause of death. [ABSTRACT FROM AUTHOR]

Intravascular lymphoma (IVL) is a rare disease characterized by the proliferation of lymphoma cells in the lumen of the small blood vessels. Although early diagnosis of IVL is important to prolong survival of the patients, its atypical symptoms and clinical course often delay its diagnosis. More than half of the patients are diagnosed at autopsy. We report a 68-year-old man who presented with transient ideomotor apraxia and mildly elevated soluble interleukin-2 receptor levels. He was initially diagnosed with aortogenic embolic stroke. He developed rapidly progressive neurological manifestations with enlargement of brain lesions on brain computed tomography and magnetic resonance imaging and died 3 months after symptom onset. The diagnosis of IVL could not be made by random skin biopsy, but was finally made at autopsy. For the early diagnosis, sufficient random skin biopsy or brain biopsy should be planned when suspected.

Abstract Background Herpes uveitis has been previously reported to present with hyphema, but hemorrhagic hypopyon is rarely reported as a herpetic uveitis manifestation. We report a case of herpes simplex virus (HSV) presenting with hemorrhagic hypopyon, and speculate on the underlying pathophysiology with relation to an intravascular lymphoma which was subsequently diagnosed as a result. Case presentation We present a case wherein a 62-year-old Japanese rheumatoid arthritis woman, with HSV uveitis, presented with hemorrhagic hypopyon in the anterior chamber and a fever with photophobia. Patient was treated with antiviral drugs which improved the hyphema and corneal lesions, but lesions recurred 3 months later. This rare presentation of HSV induced uveitis, and its subsequent recurrence, aroused suspicion of an additional hypopyon-inducing pathology. On account of previous history of lung opacities and elevated LDH, intravascular lymphoma was eventually diagnosed via lung biopsy. She was treated for the lymphoma which also completely resolved all ocular symptoms without any recurrence as of 1.5 years later. Conclusion The exceedingly rare presentation of hemorrhagic hypopyon may have been enabled by an interaction of the HSV with the intravascular lymphoma. HSV involvement was indicated by the dendritic lesions, IgG assay, and response to anti-viral drugs. The ocular involvement of the intravascular lymphoma seems to be indicated by virtue of the anti-tumor drugs completely resolving all ocular symptoms.