951 results match your criteria Lymphomatoid Granulomatosis


Pulmonary lymphomatoid granulomatosis: An uncommon disease but not to be forgotten-a single centre experience.

Respirol Case Rep 2021 Jul 26;9(7):e00789. Epub 2021 May 26.

Department of Respiratory & Sleep Medicine St George Hospital Kogarah NSW Australia.

Pulmonary lymphomatoid granulomatosis (PLG) is a rare multisystem Epstein-Barr virus (EBV)-associated lymphoproliferative disorder. Exact incidence is unknown and, with its variable clinical presentation, making an accurate diagnosis of PLG can be difficult. We present two distinct cases at our tertiary centre that underline PLG's non-specific clinical presentations. Read More

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Primary Central Nervous System Lymphomatoid Granulomatosis Presenting as Diffuse Corpus Callosum Lesions.

Neurol India 2021 Mar-Apr;69(2):497-499

Department of Neurology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, No. 324 JingWu Road, 250021 Jinan, Shandong, P.R. China.

Lymphomatoid granulomatosis (LYG) is a rare lymphoproliferative disease characterized by angiocentric and angiodestructive infiltrate. It primarily affects the lung and sometimes may also affect the central nervous system (CNS), skin, kidney, liver, etc., but the involvement of lymph nodes and/or bone marrow is extremely rare, and if present, other diagnoses are usually considered. Read More

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A case of central nervous system lesion pathologically characterized by angiocentric, T-cell-rich lymphoid cell infiltrates: a case report and literature review.

Brain Tumor Pathol 2021 Mar 30. Epub 2021 Mar 30.

Department of Neurosurgery, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-Ku, Tokyo, Japan.

Lymphomatoid granulomatosis (LYG) is a rare lymphoproliferative disease with angiocentric and angiodestructive infiltrates, and by definition, Epstein-Barr virus (EBV)-associated B-cell malignancy. It most frequently involves the lung, and in some cases, the lesions are confined to the central nervous system (isolated CNS-LYG). However, it remains a controversial disease in terms of pathophysiology, especially in those confined to the CNS. Read More

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Lymphomatoid granulomatosis in one patient with newly diagnosed HIV infection and Kaposi's sarcoma: a case report and literature review.

J Hematop 2021 Mar 20:1-5. Epub 2021 Mar 20.

Department of Pathology, Instituto Nacional de Cancerología, México City, México.

Lymphomatoid granulomatosis is a very rare B cell lymphoproliferative disease associated with Epstein-Barr virus infection. It is related to states of immunosuppression and affects the lung in more than 90% of cases, forcing the clinician to establish a differential diagnosis with other diseases such as infections, Wegener's granulomatosis, lymphoma, or lung metastases. There is no standard treatment for this disease. Read More

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EBV Positive Lymphomatoid Granulomatosis Following Dental Extractions.

Ear Nose Throat J 2021 Feb 26:145561321997536. Epub 2021 Feb 26.

22629Saint Vincent Health Center, Erie, PA, USA.

Epstein-Barr virus (EBV) associated lymphoproliferative disorders includes a diverse group of diagnoses, encompassing both B-cell and T-cell lineages. With EBV mucocutaneous ulcers becoming a World health Organization diagnosis in 2018, introduction of the disease entity will be beneficial to the practicing otolaryngologist. We are reporting a case of a 69-year-old male with history of rheumatoid arthritis on methotrexate, recently undergoing dental extractions, who then developed multiple oral ulcerations and bony erosions of his palate and alveolar ridge. Read More

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February 2021

Chronic lymphocytic infiltration with pontine perivascular enhancement responsive to steroids (CLIPPERS) and its association with Epstein-Barr Virus (EBV)-related lymphomatoid granulomatosis: a case report.

BMC Neurol 2021 Feb 18;21(1):80. Epub 2021 Feb 18.

Department of Neurology, Northern Health, 185 Cooper Street, Epping, Victoria, 3076, Australia.

Background: Chronic lymphocytic infiltration with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a neuro-inflammatory syndrome first described in 2010. It has a relationship with lymphoproliferative disorders that has not been fully elucidated. This case represents an unusual progression of CLIPPERS to Epstein-Barr Virus (EBV)-related lymphomatoid granulomatosis (LYG). Read More

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February 2021

Angioinvasive lymphoma (lymphomatoid granulomatosis) in a cat, with cutaneous and ocular metastasis.

J Vet Diagn Invest 2021 Mar 10;33(2):340-344. Epub 2021 Feb 10.

Kansas State Veterinary Diagnostic Laboratory, College of Veterinary Medicine, Kansas State University, Manhattan, KS.

Lymphomatoid granulomatosis (LYG) is a rare variant of an angioinvasive T-cell lymphoproliferative disorder that primarily affects the lungs, with common sites of metastasis including the skin and subcutis. In humans, it is a B-cell lymphoproliferative disorder associated with Epstein-Barr virus infection. Our case is a 7-y-old, spayed female, domestic longhair cat that decompensated and was euthanized following an initial diagnosis of angioinvasive lymphoma from a skin biopsy. Read More

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Primary Pulmonary B-Cell Lymphoma: A Review and Update.

Cancers (Basel) 2021 Jan 22;13(3). Epub 2021 Jan 22.

Pathology Unit, Azienda Ospedaliera S. Maria di Terni, University of Perugia, 05100 Terni, Italy.

Primary pulmonary B-cell lymphomas (PP-BCLs) comprise a group of extranodal non-Hodgkin lymphomas of B-cell origin, which primarily affect the lung without evidence of extrapulmonary disease at the time of diagnosis and up to 3 months afterwards. Primary lymphoid proliferations of the lung are most often of B-cell lineage, and include three major entities with different clinical, morphological, and molecular features: primary pulmonary marginal zone lymphoma of mucosa-associated lymphoid tissue (PP-MZL, or MALT lymphoma), primary pulmonary diffuse large B cell lymphoma (PP-DLBCL), and lymphomatoid granulomatosis (LYG). Less common entities include primary effusion B-cell lymphoma (PEL) and intravascular large B cell lymphoma (IVLBCL). Read More

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January 2021

A case of resected pulmonary lymphomatoid granulomatosis.

Respir Med Case Rep 2021 28;32:101327. Epub 2020 Dec 28.

Department of Pathology, Hakodate National Hospital, 0418512, Hakodate, Hokkaido, Japan.

Lymphomatoid granulomatosis (LYG) is a rare Epstein-Barr virus-associated B-cell lymphoproliferative disorder and was incorporated into the WHO classification of Tumours of the Lung, Pleura, Thymus and Heart in 2015. LYG is known to be associated with the host's immune function, and can be caused by some immunosuppressive agents, including methotrexate. A woman in her sixties with an 18-year history of methotrexate treatment for rheumatoid arthritis visited our hospital after detection of an abnormal chest shadow on her radiograph. Read More

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December 2020

Primary central nervous system lymphomatoid granulomatosis: a case report.

Neurol Sci 2021 04 20;42(4):1587-1590. Epub 2020 Oct 20.

Department of Neurology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, No. 324 JingWu Road, Jinan, 250021, Shandong, People's Republic of China.

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Primary lymphomatoid granulomatosis of the central nervous system: A diagnostic challenge.

Neurologia (Engl Ed) 2020 Oct 16. Epub 2020 Oct 16.

Servicio de Anatomía Patológica, Hospital Universitario de Cruces, Barakaldo, España.

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October 2020

Primary Central Nervous System Lymphomatoid Granulomatosis: Systemic Review.

Front Neurol 2020 11;11:901. Epub 2020 Sep 11.

Department of Neurology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan, China.

Lymphomatoid granulomatosis (LYG) is an infrequent lymphoproliferative disease that typically involves the lungs, but may also affect the central nervous system (CNS). Isolated CNS involvement is very rare, and its clinicopathological features have not been fully elucidated. Here, we systematically reviewed the English literature through PubMed to collect all relevant case reports and small case series with pathologically confirmed primary CNS-LYG. Read More

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September 2020

A Case Report of a Female Patient With Hodgkin Lymphoma Localized in the Central Nervous System and With Concomitant Pulmonary Lymphomatoid Granulomatosis.

Front Neurol 2020 8;11:963. Epub 2020 Sep 8.

Chair and Department of Haematooncology and Bone Marrow Transplantation, Medical University of Lublin, Lublin, Poland.

The involvement of the central nervous system (CNS) in Hodgkin lymphoma (HL) has been rarely reported, especially in its primary isolated form. Herein, we present a case of a 33-year-old woman, who received immunosuppressive treatment due to ulcerative colitis (at the beginning azathioprine and sulfasalazine, changed to mesalazine), with repetitive episodes of loss of consciousness for a few weeks and with no other symptoms. Magnetic resonance imaging scans of the head revealed a tumor in the lateral part of the left temporal lobe and in the cerebellum. Read More

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September 2020

A rare case of severe right heart failure due to pulmonary artery lymphomatoid granulomatosis.

ESC Heart Fail 2020 Sep 10. Epub 2020 Sep 10.

The Department of Cardiology, Gansu Provincial Hospital, Lanzhou, 730000, China.

Lymphomatoid granulomatosis is a rare, vascular-centric, and vessel-destroying lymphoproliferative disease that hardly involves the pulmonary arteries. Herein, we report a case with severe right heart failure and pulmonary arterial stenosis caused by pulmonary artery lymphomatoid granulomatosis. This case was diagnosed by percutaneous transluminal pulmonary artery biopsy and was effectively treated with stent implantation and steroid administration. Read More

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September 2020

Use of rituximab in lymphomatoid granulomatosis with isolated central nervous system involvement.

BMJ Case Rep 2020 Sep 7;13(9). Epub 2020 Sep 7.

Houston Methodist Cancer Center, Houston, Texas, USA.

A 33-year-old woman presented to the emergency room with severe headaches. A CT scan of the head revealed two brain lesions with associated vasogenic oedema. Diagnostic resection of one of the lesions followed by pathological analysis revealed grade III lymphomatoid granulomatosis (LYG). Read More

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September 2020

Lymphomatoid granulomatosis: a mimicker of systemic inflammatory rheumatological disease.

Scand J Rheumatol 2021 Mar 16;50(2):165-166. Epub 2020 Jul 16.

Clinical Immunology Unit, Stavanger University Hospital, Stavanger, Norway.

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Neuroimages and Neuropathology of a Stroke-Like Cerebral Lymphomatoid Granulomatosis.

Can J Neurol Sci 2021 01 6;48(1):114-115. Epub 2020 Jul 6.

Unit of Neurology, Neurophysiology, Neurobiology, Department of Medicine, Università Campus Bio-Medico di Roma, via Álvaro del Portillo 21, 00128Rome, Italy.

A 70-year-old man presented to the Emergency Department reporting the acute onset of non-fluent aphasia, hyposthenia, and hemi-anesthesia of the right body. Brain computerized tomography revealed a subcortical hypodense lesion in the middle cerebral artery territory. Neck ultrasounds of internal and external carotid arteries and of the vertebral arteries showed a focal moderate stenosis of the left internal carotid artery due to a soft atheromasic plaque. Read More

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January 2021

Mimickers of pulmonary lymphoma.

Semin Diagn Pathol 2020 Nov 9;37(6):283-295. Epub 2020 Jun 9.

Department of Pathology, City of Hope, Duarte, CA, USA. Electronic address:

There are multiple entities that involve the lung that have radiographic, clinical, and morphologic overlaps with pulmonary lymphoma. In this review, we will discuss these entities in detail and provide relevant updates. Read More

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November 2020

Extranodal NK/T cell lymphoma and lymphomatoid granulomatosis in a patient with chronic lymphocytic leukaemia: Case report for a new perspective on Richter syndrome.

Medicine (Baltimore) 2020 May;99(19):e20106

Department of Pathology.

Rationale: Richter syndrome (RS) defines the transformation of chronic lymphocytic leukemia (CLL) into a more aggressive lymphoma. Although the term RS is most often reserved for transformation of CLL into diffuse large B-cell lymphoma (DLBCL), and less frequently Hodgkin lymphoma , the list of cases with more variable presentations in the literature is growing.

Patient Concerns: A 71-year-old Caucasian man initially consulted an otolaryngologist for a 1-year history of nasal congestion. Read More

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Pulmonary Recurrence of Lymphomatoid Granulomatosis Diagnosed on F-18 FDG PET/CT.

Indian J Nucl Med 2020 Apr-Jun;35(2):167-169. Epub 2020 Mar 12.

Department of Nuclear Medicine, Post Graduate Institute of Medical Education and Research, Chandigarh, India.

Lymphomatoid granulomatosis (LYG) is a rare, extranodal B-cell lymphoproliferative disorder. The disease commonly presents with nonspecific symptoms and imaging features, making the diagnosis and therapeutic response assessment difficult. While histopathology is the mainstay of diagnosis, different imaging modalities such as computed tomography (CT), magnetic resonance imaging, or F18-fluorodeoxyglucose positron emission tomography/computed tomography (F-18 FDG PET/CT) can help in identifying the different organs involved. Read More

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Pathobiology and treatment of lymphomatoid granulomatosis, a rare EBV-driven disorder.

Blood 2020 04;135(16):1344-1352

Lymphoid Malignancies Branch, Center for Cancer Research and.

Lymphomatoid granulomatosis (LYG) is a rare Epstein-Barr virus (EBV)-driven B-cell lymphoproliferative disease (LPD). This disease is hypothesized to result from defective immune surveillance of EBV, with most patients showing evidence of immune dysfunction, despite no known primary immunodeficiency. Pathologically, LYG is graded by the number and density of EBV+ atypical B cells, and other characteristic findings include an angioinvasive/angiodestructive reactive T-cell infiltrate and various degrees of necrosis. Read More

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Pulmonary Lymphomatoid Granulomatosis With Hemophagocytic Lymphohistiocytosis as the Initial Manifestation.

Front Oncol 2020 29;10:34. Epub 2020 Jan 29.

Department of Hematology, Xijing Hospital, Fourth Military Medical University, Xi'an, China.

Lymphomatoid granulomatosis (LYG) is an extremely rare angio-centric and angio-destructive B-cell lymphoproliferative disease. Driven by Epstein-Barr virus (EBV), LYG predominantly involves the bilateral lungs. Commonly presenting as multiple nodules in the lung, pulmonary LYG can masquerade as various infectious diseases, vasculitis, lung cancer, or other metastatic neoplasm. Read More

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January 2020

[A Case of Primary Central Nervous System Lymphomatoid Granulomatosis that was Completely Ameliorated by Corticosteroid Treatment].

Brain Nerve 2020 Feb;72(2):159-165

Department of Neurosurgery, Kindai University School of Medicine.

Lymphomatoid granulomatosis (LYG) is an angiocentric, angiodestructive lymphoreticular proliferative disease that usually affects the lungs but it has been speculated to also effect the central nervous system (CNS). However, unique primary LYG of the CNS has rarely been reported in the literature. Herein, we describe a clinical case of a 37-year-old female patient with grade 1 primary CNS-LYG having a good prognosis owing to corticosteroid treatment. Read More

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February 2020

A 4-Year-Old Boy With Prolonged Cough and Fever.

J Pediatric Infect Dis Soc 2020 Feb;9(1):92-95

Division of Infectious Diseases, Department of Pediatrics, Stony Brook Children's Hospital, Stony Brook, New York, USA.

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February 2020

Isolated lymphomatoid granulomatosis of the central nervous system: A case report and literature review.

Neuropathology 2019 Dec 20;39(6):479-488. Epub 2019 Nov 20.

Department of Neurology, Chinese PLA General Hospital, Beijing, China.

Lymphomatoid granulomatosis (LYG) is an angiocentric and angiodestructive lymphoproliferative disease which can involve multiple organs of the body and is most common in the lungs. Its pathological features are proliferation of large atypical B-cells related to Epstein-Barr virus, T-cell infiltration and tissue necrosis. This disease is rare, and LYG which uniquely involves the central nervous system (CNS) is extremely rare. Read More

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December 2019

Multiple Neurologic Deficits and Cognitive Decline in a Young Woman.

Ann Indian Acad Neurol 2019 Oct-Dec;22(4):506-512. Epub 2019 Oct 25.

Department of Neurology, All India Institute of Medical Sciences, New Delhi, India.

We present the clinicopathologic conference of a 34-year-old lady with history of facial palsy 14 years ago who developed new deficits of mononeuritis multiplex, maculopapular rash, pancytopenia, splenomegaly, lung involvement and cognitive decline rapidly over three years. Investigations revealed pancytopenia, reversal of albumin globulin ratio, mediastinal adenopathy, ANA positivity, low C3 levels with the CSF being inflammatory and MRI showing extensive hemorrhagic lesions with mass effect. She had a rapidly progressive fatal course over three years with the disease being undiagnosed. Read More

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October 2019

Diffuse large B-cell lymphoma variants: an update.

Pathology 2020 Jan 15;52(1):53-67. Epub 2019 Nov 15.

Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA. Electronic address:

Diffuse large B-cell lymphoma (DLBCL) is the most common type of lymphoma, representing approximately one-third of all cases worldwide. In the World Health Organization (WHO) classification of lymphomas, most cases of DLBCL are designated as not otherwise specified (NOS). About 20% of cases, however, are designated as specific variants of DLBCL. Read More

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January 2020

Traumatic Ulcerative Granuloma with Stromal Eosinophilia: CD30 analysis and clonality for T cell receptor gene re-arrangement.

Acta Histochem 2019 Nov 29;121(8):151450. Epub 2019 Oct 29.

Institute of Pathology, Tel-Aviv Sourasky Medical Center, Tel-Aviv, Israel; Department of Oral Pathology and Oral Medicine, Goldschleger School of Dental Medicine, Tel-Aviv University, Tel Aviv, Israel; Sourasky Faculty of Medicine, Tel-Aviv University, Israel. Electronic address:

Introduction: Traumatic Ulcerative Granuloma with Stromal Eosinophilia (TUGSE) is a rare oral ulcerated lesion of uncertain etiology, showing eosinophil-rich granulation tissue, with occasional large atypical CD30 positive mononuclear cells. It had been suggested that it may represent an oral counterpart of cutaneous lymphomatoid papulosis, with a potential to evolve into CD30 + T cell lymphoma OBJECTIVES: To compare TUGSE and non-specific oral ulcers (NSU) clinically, histopathologically and by clonality analysis for T-cell receptor re-arrangement, aiming to determine whether TGUSE with atypical cells is a lymphomatous premalignant condition, and whether therapeutic approach should be radical or conservative.

Materials And Methods: Retrospective archival analysis included 17 TUGSE and 8 NSU cases. Read More

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November 2019

Magnesium Restores Activity to Peripheral Blood Cells in a Patient With Functionally Impaired Interleukin-2-Inducible T Cell Kinase.

Front Immunol 2019 27;10:2000. Epub 2019 Aug 27.

Laboratory of Infectious Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, United States.

Interleukin-2-inducible T cell kinase (ITK) is critical for T cell signaling and cytotoxicity, and control of Epstein-Barr virus (EBV). We identified a patient with a novel homozygous missense mutation (D540N) in a highly conserved residue in the kinase domain of ITK who presented with EBV-positive lymphomatoid granulomatosis. She was treated with interferon and chemotherapy and her disease went into remission; however, she has persistent elevation of EBV DNA in the blood, low CD4 T cells, low NK cells, and nearly absent iNKT cells. Read More

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October 2020

An atypical case of a pulmonary mass in an immunocompromised patient.

Acta Clin Belg 2020 Oct 17;75(5):370-374. Epub 2019 Aug 17.

Department of Pulmonology, University Hospitals Leuven , Leuven, Belgium.

Objectives: Pulmonary lymphomatoid granulomatosis (PLG) is a rare angiocentric and angiodestructive EBV-associated lymphoproliferative disorder which almost always affects the lungs. PLG is more commonly diagnosed in patients with immunodeficiency and is associated with Epstein-Barr virus (EBV). 'Drug induced PLG' or 'iatrogenic immunodeficiency-associated lymphoproliferative disorder' is a special form of PLG described in patient with inflammatory bowel diseases treated with Azathioprine. Read More

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October 2020