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Intravascular lymphoma CNS

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
Shinichi Kan
With a typical case, imaging diagnosis of central nervous system malignant lymphoma is not difficult. High density on non contrast CT, periventricular location, homogenous contrast enhancement, iso- to hypointensity to gray matter on T(2) weighted MR imaging and high intensity on diffusion weighted MR imaging are characteristic findings. Hemorrhage is rare. When a patient is immunocompromised, irregular ring enhancement is noted on enhanced study. Intravascular lymphomatois is a rare type of lymphoma. A variety of imaging findings are reported...
August 2014: Brain and Nerve, Shinkei Kenkyū No Shinpo
Michito Namekawa
Malignant lymphoma can affect the central nervous system (CNS) in three different ways: as a consequence (relapse or invasion) of systemic lymphoma, as a primary CNS lymphoma (PCNSL) without systemic involvement, and through intravascular lymphomatosis (IVL). It is essential to distinguish PCNSL from the others, since the therapeutic strategy for treating this disease differs. FDG-PET/CT fusion imagery is a powerful tool for detecting systemic lesions. If a marked elevation of lactate dehydrogenase and the soluble IL-2 receptor suggests IVL, a random skin biopsy can permit a differential diagnosis...
August 2014: Brain and Nerve, Shinkei Kenkyū No Shinpo
Yuka Kanazawa, Noriko Hagiwara, Ryu Matsuo, Shuji Arakawa, Tetsuro Ago, Takanari Kitazono
A 60-year-old man was admitted to our hospital complaining of fever, headache and vertigo. Neurological examination on admission showed mild ataxic gait. Brain magnetic resonance imaging showed linear high intensity in the left parietal lobe on diffusion-weighted imaging (DWI) and laboratory data revealed elevated serum lactate dehydrogenase and soluble interleukin-2 receptor. Although intravascular lymphoma was suspected from these findings, bone marrow and skin biopsies were negative. Two months later, he presented with sensory disturbance of the left upper limb, and new lesions in the right frontal and bilateral parietal lobes were detected on DWI...
2014: Rinshō Shinkeigaku, Clinical Neurology
Ekokobe Fonkem, Edwin Lok, David Robison, Shiva Gautam, Eric T Wong
Intravascular lymphomatosis (IVL) is a rare and clinically devastating form of extranodal B-cell non-Hodgkin's lymphoma. We performed a comprehensive analysis of the literature on IVL's published between 1959 and 2011 and evaluated the natural history as well as identified prognostic and predictive factors in patients. Nonparametric two-tailed Mann-Whitney U-test and Mantel-Cox log rank test were used to evaluate the survival intervals and prognostic factors. Multivariate analysis of variance (MANOVA) and chi-squared statistics were carried out to examine treatment-related predictive factors...
August 2014: Cancer Medicine
Yusuke Takamine, Natsumi Ikumi, Hajime Onoe, Misa Hayase, Yousuke Nagasawa, Masashi Sakagami, Kaita Sugiyama, Suguru Nakagawa, Yoshito Uchino, Hiromichi Takahashi, Yukio Hirabayashi, Takamasa Nozaki, Katsuhiro Miura, Hirotake Inomata, Noriyoshi Iriyama, Hidetaka Shiraiwa, Sumiko Kobayashi, Noboru Kitamura, Yoshihiro Hatta, Yoshihiro Matsukawa, Masami Takei
We report a 68-years-old woman with systemic sclerosis and interstitial pneumonia (IP). She had developed subacute progressively encephalopathy and dementia while treated with oral cyclophosphamide and prednisolone. She admitted to our hospital because of syncope. Laboratory tests indicated slight elevated cerebrospinal fluid protein, and levels of serum C-reactive protein (CRP), levels of soluble IL-2 receptor was normal. But, magnetic resonance imaging (MRI) of the brain showed multiple infarct-like lesions mainly in the white matter, which mimics progressive multiple leukoencephalopathy (PML)...
2014: Nihon Rinshō Men'eki Gakkai Kaishi, Japanese Journal of Clinical Immunology
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