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Intravascular lymphoma CNS

Asangi R Kumarapeli, Murat Gokden
Central nervous system (CNS) hematologic malignancies, whether primary or secondary, are uncommon and their clinical presentations vary. Intravascular lymphoma (IVL) is a very rare, aggressive systemic malignancy that is often difficult to diagnose and susceptible to early CNS involvement. Blast crisis in myeloid leukemias can cause widespread neoplastic emboli. Here, we report two adult patients (72 years and 22 years of age) who presented with altered mental status followed by rapid decline in their conditions leading to death...
March 25, 2018: Diagnostic Cytopathology
Yutaro Suzuki, Hiroaki Tanaka, Kennichiro Suyama, Hidetoshi Mochida, Yoshio Suzuki
Intravascular large B-cell lymphoma (IVL) is a rare type of extranodal diffuse large B-cell lymphoma (DLBCL), which often infiltrates the central nervous system (CNS) during the clinical course. Cerebral hemorrhage in patients with CNS lymphoma at presentation is rare. Herein, we describe a case of secondary CNS lymphoma with intratumoral hemorrhage, which was suggested as IVL from autopsy findings. A 76-year-old Japanese man with a history of treatment for B-cell non-Hodgkin's lymphoma was transferred to our hospital in an ambulance for generalized convulsions...
November 2017: Journal of Clinical Medicine Research
Bo Luo, Jia-Mei Chen, Jie Liu, Wen-He Li, Yu-Xiang Shi, Pan Zeng, Yong-Hui Xie, Hong-Feng Zhang
BACKGROUND: Intravascular large B-cell lymphoma (IVLBCL) is a subtype of diffuse large B-cell lymphoma (DLBCL) that is rare and highly aggressive and that may progressively involve many organs. CNS (central nervous system), BM (bone marrow) and skin are the most common systems involved. To date, only 2 cases of IVLBCL involving the thyroid have been reported. CASE PRESENTATION: Here, we report a case of IVLBCL involving the thyroid and accompanied by bilateral nodular goiter...
August 25, 2017: Diagnostic Pathology
Damir Nizamutdinov, Nitesh P Patel, Jason H Huang, Ekokobe Fonkem
Purpose of review The purpose of this review was to discuss therapeutic manipulations and effective current interventions available to treat intravascular lymphoma in the central nervous system. Recent findings Patients experienced resolution and remission of disease for 14 months and up to 2 years after eight cycles of R-CHOP and four courses of intrathecal therapy with MTX, cytarabine, and prednisolone. Intravascular use of unfractionated heparin during therapy may contribute to better outcome. Summary Series of therapeutic avenues were analyzed and compared...
August 23, 2017: Current Treatment Options in Neurology
F M Brett, D Chen, T Loftus, Y Langan, S Looby, S Hutchinson
BACKGROUND: In patients presenting with rapidly progressive dementia, prion disease may enter the differential diagnosis. The commonest malignancies masquerading as prion disease are primary CNS lymphoma and intravascular large B-cell lymphoma, both rare and difficult to diagnose without brain biopsy. CASE PRESENTATION: This 82-year-old lady with a past history of hypertension, presented with rapidly progressive cognitive impairment and ataxia. The possibility of sCJD was raised...
July 19, 2017: Irish Journal of Medical Science
Kate Poropatich, Dave Dittmann, Yi-Hua Chen, Kirtee Raparia, Kristy Wolniak, Juehua Gao
BACKGROUND: Intravascular large B-cell lymphoma (IVLBCL) is a rare type of extranodal lymphoma with growth mainly in the lumina of vessels. We studied a small series of IVLBCL and focused on its central nervous system (CNS) involvement. METHODS: Searching the medical records of Northwestern Memorial Hospital, we identified five cases of IVLBCL from January 2007 to January 2015. Clinical information, hematoxylin and eosin stained histologic slides and immunohistochemistry studies were reviewed for all cases...
May 2017: Journal of Pathology and Translational Medicine
Tara L Sharma, Gabrielle A Yeaney, Payam Soltanzadeh, Yuebing Li, Claudiu V Cotta
Intravascular T-cell lymphomas are rare, poorly characterized lesions. We discuss the clinical, radiologic and especially the laboratory characteristics of a lesion which presented in a 62-year-old woman with a history of progressive CNS abnormalities. Throughout the course of the disease, radiologic findings consisted mainly of multifocal mixed areas of ischemia and vasogenic edema involving cortical and subcortical regions. A brain biopsy identified an abnormal T-cell population confined to lumens of vessels...
March 20, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
Daulath Singh, Devika Kapuria, Suparna Nanua, Rakesh Gaur
Intravascular large B-cell lymphoma is an extremely rare extranodal lymphoma that proliferates in the lumen of the blood vessels while sparing the organ parenchyma. It usually presents with CNS and skin involvement. A 65-year-old Caucasian female presented with fevers and chills of 3-4 months' duration. Bone marrow biopsy done 3 months prior showed no significant myelodysplasia or lymphoid aggregates. The patient later died due to multiorgan failure. A bone marrow biopsy showed 20-30% CD5+ B cells consistent with infiltrative large B-cell lymphoma...
2016: Case Reports in Hematology
Naoto Tomita
Diffuse large B-cell lymphoma (DLBCL) accounts for 30-40% of all lymphoma subtypes. More than half of DLBCLs are estimated to arise from extranodal sites, and are called extranodal DLBCLs. Generally, the outcomes of extranodal DLBCL are not different from those of nodal DLBCL. To our knowledge, this report is the first to discuss the concept and the cell of origin of extranodal DLBCL, with the significance of the presence/absence of each involvement site in determining the outcome. Second, the evidence of clinical manifestation and outcomes of several extranodal DLBCLs requiring treatments other than R-CHOP, which is the current standard therapy for this malignancy, are discussed...
2016: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
Gelsomina Mansueto, Anna Di Vito, Clara Belluomo, Patrizia Murino, Valentina Natella, Caterina Camastra, Ivan Presta, Natalia Malara, Gaetano de Rosa, Giuseppe Donato, Chiara Mignogna
Intravascular large B cell lymphoma (IVLBCL) is a rare extranodal non-Hodgkin lymphoma characterized by proliferation of malignant cells within the lumen of small vessels, with a predilection for the CNS and the skin. IVLBCL clinical course is highly aggressive, clinical signs and symptoms are not specific and may consist of neurological and cognitive impairment, fever of unknown origin and cutaneous lesions, lacking of a typical neuroimaging pattern. For all these reasons the diagnosis is commonly missed and the exitus is frequent, therefore post mortem evaluation is necessary to clarify the clinical history...
October 2016: Neuropathology: Official Journal of the Japanese Society of Neuropathology
Ekokobe Fonkem, Samantha Dayawansa, Edana Stroberg, Edwin Lok, Paul C Bricker, Batool Kirmani, Eric T Wong, Jason H Huang
BACKGROUND: Patients with intravascular lymphoma (IVL) frequently have neurological signs and symptoms. Prompt diagnosis and treatment is therefore crucial for their survival. However, the spectrum of neurological presentations and their respective frequencies have not been adequately characterized. Our aim is to document the spectrum of clinical symptoms and their respective frequencies and to create a clinical framework for the prompt diagnosis of IVL. METHODS: A comprehensive meta-analysis of 654 cases of IVL published between 1957 and 2012 was performed to provide better insight into the neurological presentations of this disease...
January 16, 2016: BMC Neurology
Kazuyuki Shimada
Intravascular large B-cell lymphoma (IVLBCL) is a rare distinct disease entity of the extranodal large B-cell lymphoma type, characterized by the selective growth of tumor cells in the lumina of small vessels of various organs. Lack of remarkable lymphadenopathy and non-specific clinical abnormalities including fever of unknown origin and lactate dehydrogenase elevation generally make timely and accurate diagnosis difficult. Recent diagnostic advances in the detection of this disease using FDG-PET/CT and random skin biopsies are expected to increase the diagnostic yield...
August 2015: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
Pavan Bhargava, Fazeel Siddiqui, Brajesh Aggarwal, Brian E Moore, Rodger J Elble
INTRODUCTION: Angiotropic lymphoma is a rare large B-cell lymphoma involving the intravascular compartment without local tissue or vessel wall infiltration. CASE REPORT: A 48-year-old male presented with 8 months of progressive paraparesis and bowel and bladder incontinence. Initial cerebrospinal fluid analysis showed high protein, lymphocytic pleocytosis, high IgG synthesis, but no oligoclonal bands. Brain imaging at presentation was unrevealing. Electromyography revealed evidence of bilateral lumbosacral radiculoplexopathy [R>L]...
July 2015: Neurologist
Mizuki Watanabe, Daisuke Kaji, Meiko Hashimoto-Maeda, Go Yamamoto, Yuki Asano-Mori, Naoyuki Uchida, Shuichi Taniguchi, Koji Izutsu
A 52-year-old female presented with stroke-like symptoms after high-dose methotrexate (HDMTX) therapy and MTX intrathecal injection (IT-MTX) as central nervous system (CNS) prophylaxis for intravascular large B-cell lymphoma (IVLBCL). She had been diagnosed as having IVLBCL without CNS involvement 5 months earlier and had received 6 courses of R-CHOP and 2 courses of HDMTX combined with IT-MTX. She experienced acute-onset right hemiparesis involving the face and arm, along with dysarthria, 7 days after the second HDMTX infusion...
March 2015: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
Bimal Patel, Geeta Chacko, Sheila Nair, Jamie Anandan, Ari G Chacko, Vedantam Rajshekhar, Mazda Turel
BACKGROUND: Primary central nervous system lymphomas (PCNSL) constitute a rare group of extranodal non-Hodgkin's lymphomas (NHLs). AIM: To study the clinical and immunophenotypic profile of patients with a PCNSL who presented between the years 2000 and 2013 in a tertiary care center in South India. MATERIALS AND METHODS: This was a retrospective study. Demographic and clinical data were obtained from the clinical case records. INCLUSION CRITERIA: Cases of PCNSL involving brain...
January 2015: Neurology India
Takeshi Sawada, Yasushi Omuro, Takeshi Kobayashi, Tunekazu Hishima, Fumiaki Koizumi, Yusuke Kanemasa, Tatsu Shimoyama, Eisaku Sasaki, Yoshiharu Maeda
This report describes a patient with intravascular large B-cell lymphoma (IVLBCL) with central nervous system involvement at the time of diagnosis who achieved complete remission for over 5 years in response to therapy. The patient, a 71 year-old woman, was previously healthy with the exception of taking verapamil for paroxysmal supraventricular tachycardia. She had presented with pyrexia and gradually progressive anemia. Brain magnetic resonance imaging revealed an infarct-like lesion in the pons, although no paralysis was observed...
2014: OncoTargets and Therapy
Sanda Alexandrescu, James P Orengo, Shahed Toossi, Arie Perry, Patrick Treseler, Christopher Hess, Marta Margeta
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...
April 2015: Neuropathology: Official Journal of the Japanese Society of Neuropathology
Sujata Chaturvedi, Ishita Pant, Suman Kushwaha, Deepak Kumar Jha
Intravascular lymphoma (IVL) is a rare extra nodal variant of non Hodgkin's lymphoma characterised by neoplastic lymphoid cells growing inside the lumina of medium and small vessels. IVL limited to the central nervous system (CNS) is an extremely rare condition as IVL is usually found with systemic lesions. Most cases of IVL are not diagnosed until post mortem because of variable clinical presentation and non-specific laboratory findings. Even if diagnosed early the disease is clinically aggressive and usually fatal, even with early detection and treatment...
2014: BMJ Case Reports
Yong Hyun Jang, Seok-Jong Lee, Yoon Hyuk Choi, Weon Ju Lee, Do Won Kim, Jeongshik Kim, Tae In Park, Yee Soo Chae
Intravascular lymphoma (IVL) is a rare disorder characterized by the presence of large neoplastic lymphoid cells restricted to the lumens of small vessels with a predilection for the skin and the central nervous system. While the vast majority of cases involving IVL are of B-cell lineage, the disease rarely affects the T-cell, the histiocytes, and the natural killer cells. We report a case of intravascular T-cell lymphoma (IVTL) associated with Epstein-Barr virus (EBV). A 23-year-old healthy woman presented with tender indurated erythematous patches with overlying telangiectasia on her right breast, abdomen, both the upper and the lower extremities and the back for 3 months...
August 2014: Annals of Dermatology
Shigeru Koyano, Shunta Hashiguchi, Fumiaki Tanaka
Intravascular lymphoma (IVL) is a rare form of malignant lymphoma characterized by the selective growth of lymphoma cells within the lumina of vessels, without the involvement of adjacent parenchymal tissue. IVL is predominantly of B-cell lineage, but cases of T-cell or natural killer cell lineage have been described occasionally, predominantly involving the skin. IVL usually affects elderly patients with a poor performance status, elevated serum lactic dehydrogenase levels, anemia, and B symptoms. The clinical presentation varies in different geographical areas, particularly between patients diagnosed in Europe and Asia...
August 2014: Brain and Nerve, Shinkei Kenkyū No Shinpo
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