Pulmonary Lymphomatoid Granulomatosis Research Articles

A case of pulmonary lymphomatoid granulomatosis with difficulty in diagnosis

A case of pulmonary lymphomatoid granulomatosis with difficulty in diagnosis

47. A case of pulmonary lymphomatoid granulomatosis diagnosed on needle core biopsy

Background: Lymphomatoid granulomatosis (LYG) is a rare extranodal lymphoproliferative disorder composed of B cells positive for Epstein-Barr virus (EBV) and reactive T cells. It has a variable clinical course ranging from spontaneous remission to aggressive lymphoma with high mortality. Aims: We present a case of solitary pulmonary lymphomatoid granulomatosis in an asymptomatic, seemingly immune competent female with a distant history of breast cancer. Methods: The patient had initially presented two years earlier with radiographic evidence of multiple hepatic nodules and a single upper lobe lung lesion presumed to be disseminated metastasis; however, interval imaging showed spontaneous resolution. Two years after the initial presentation, the patient represented systemically unwell and underwent a lung needle core biopsy for a left upper lobe cavitating intensely avid lesion found on CT imaging. Results and conclusion: Histologically, the core biopsies revealed a large area of coagulative necrosis with an atypical mixed inflammatory cell infiltrate demonstrating both angiocentric and angiodestructive features. The cellular infiltrate included small lymphocytes, large atypical lymphoid cells and abundant histiocytes with vague granuloma formation. On immunohistochemistry the large atypical cells were positive for CD20 and PAX5 with in situ hybridisation confirming the presence of EBER. The patient was subsequently diagnosed with lymphomatoid granulomatosis favoured to be a grade 3 due to the numbers of atypical B-cells and extensive necrosis. In conclusion, we report a rare case of relapsing nodular lesions of the lung and liver, which after two years showed diagnostic features of lymphomatoid granulomatosis. The lung lesion was unusual being a large solitary mass. Combination chemotherapy with rituximab has led to a complete and metabolic (PET) response and remission to date.

Lymphomatoid Granulomatosis: Rare Radiologic Presentation as a Solitary Pulmonary Mass

Background: Lymphomatoid granulomatosis (LYG) is a rare Epstein-Barr virus (EBV)-driven lymphoproliferative disease. LYG is characterized by a progressive clinical course, which virtually always involves the lungs. LYG characteristically presents as bilateral pulmonary nodules. Pathologically, it is characterized by an angiocentric and angiodestructive infiltration of atypical EBV-positive B-lymphocytes admixed with reactive T-lymphocytes. We report a case of pulmonary LYG that presented as a large mass with complete occlusion of the right main stem bronchus intermedius in an 81-year-old female. Case: An 81-year-old female presented with shortness of breath to the emergency department. Inpatient imaging revealed bulky mediastinal lymphadenopathy with a right lower lobe collapse, shift of the cardiomediastinal silhouette, and a large right upper lung mass. Endobronchial ultrasound-guided biopsy (EBUS) revealed complete occlusion of right mainstem bronchus due to the right upper lung mass growth into the bronchus intermedius. Histopathological examination demonstrated clusters of large atypical EBV-positive B cells interspersed in a minimally polymorphous lymphocytic background, consistent with lymphomatoid granulomatosis, grade 3/3. Patient was treated with immunochemotherapy (cyclophosphamide, doxorubicin, vincristine, and prednisone - CHOP) and Rituximab (anti-CD20 antibody). At the patient’s most recent follow-up, 6 months later, she was in a stable condition and her respiratory symptoms have improved. Conclusion: Lymphomatoid granulomatosis is a rare disease that should be considered in the differential diagnosis of a radiographic evaluation of a solitary pulmonary lung mass. Since the radiographic impression might favor carcinoma as the top differential diagnosis, biopsy of the lesion is paramount to ensure the correct diagnosis. Lymphomatoid granulomatosis is usually treated with an immunochemotherapy regimen with CHOP, and/or interferon, and Rituximab.

Pyothorax and pulmonary lymphomatoid granulomatosis in a cat

Pyothorax and pulmonary lymphomatoid granulomatosis in a cat

Primary lymphomatoid granulomatosis in the central nervous system: A report of three cases.

Lymphomatoid granulomatosis (LYG) is a rare lymphoproliferative disorder characterized by infiltration of Epstein-Barr virus (EBV)-positive large atypical B-cells in an angiocentric fashion in a mixed inflammatory background. The histologic spectrum of LYG ranges from reactive proliferation to diffuse large B-cell lymphoma according to the number of EBV+ B-cells. It is known that virtually all patients have pulmonary involvement, whereas primary LYG of the other organs has been rarely reported. Herein, we describe three cases of primary LYG of the central nervous system (CNS) without pulmonary lesions, and this is the first collection to be reported in Korea. All of the cases revealed multifocal enhancing necrotic brain lesions masking as metastatic tumors, infection or vasculitis. These patients were successfully managed by corticosteroids and immunomodulating agents without chemotherapy against malignant lymphoma even in grade 3 LYG. We assume that primary CNS LYG might be less aggressive and more controllable than pulmonary LYG. The clinicopathologic characteristics of the cases with a special regard to the differential diagnosis and clinical courses are discussed in combination with an overview of the literature.

Low grade pulmonary lymphomatoid granulomatosis with an endobronchial mass.

Lymphomatoid granulomatosis (LYG) is an angiocentric and angiodestructive neoplastic proliferation of B and T lymphocytes commonly involving the lungs. Epstein-Barr virus is commonly detected in lesional cells. We report a case of a 54-year-old female with underlying monoclonal gammopathy of unknown significance who presented with a 4 week history of dyspnea and cough. Computed tomography scan of the chest showed multiple lung nodules as well as endobronchial narrowing causing atelectasis at the left upper lobe. Bronchoscopic findings revealed obstruction at the lingula segment due to endobronchial mass as a rare presentation. Bronchoscopic biopsy was diagnosed with LYG grade 1. After treatment, the endobronchial mass and lung lesions were completely resolved. However, the patient eventually evolved to malignant lymphoma after 1 year.

Rituximab for pulmonary lymphomatoid granulomatosis which developed as a complication of methotrexate and azathioprine therapy for rheumatoid arthritis.

We report the case of a patient with Rheumatoid Arthritis [RA] presenting with clinical-pathological and radiological features of Pulmonary Lymphomatoid Granulomatosis (PLG). This is a rare lung disorder characterized by multiple nodular lesions with lymphocytic invasion of vascular walls. We present one such case of PLG secondary to Methotrexate and Azathioprine therapy, who was successfully treated with Steroids and Rituximab. We wish to highlight the importance of lung biopsy in the diagnosis and the use of rituximab as a treatment modality for RA as well as PLG.Electronic supplementary materialThe online version of this article (doi:10.1186/2193-1801-3-751) contains supplementary material, which is available to authorized users.

Lymphomatoid granulomatosis involving the central nervous system: A case report and review of the literature.

Lymphomatoid granulomatosis (LYG) is a rare tumor with unknown etiology. Specific etiological factors for LYG are also unknown, although previous data indicates that LYG is an Epstein-Barr virus-associated B-cell proliferation associated with an exuberant T-cell reaction. According to the 2008 WHO classification, LYG is characterized by B-cell proliferation of B-lymphoma cells. Generally, treatment options for LYG are similar to those for diffuse large B-cell lymphoma. Unfortunately, LYG is a chemotherapy-resistant disease in certain patients and has a poor prognosis. The current study presents the case of a 19-year-old male patient with pulmonary LYG. The patient exhibited progressive disease following one cycle of chemotherapy with cyclophosphamide, adriamycin, vincristine and prednisone, and nodular lesions in the brain were diagnosed. Radiotherapy was delivered to the whole brain, however, this treatment did not prevent progression of the disease and the patient succumbed three months after initial presentation. An overview of the literature with regard to the etiology, clinical features, diagnosis and treatment options for LYG is also presented in the current case study.

Huge Bilaterally Cavitary and Nodulary Lung Lesions: Pulmonary Lymphomatoid Granulomatosis

SESSION TITLE: Miscellaneous Global Case Reports

Pulmonary lymphomatoid granulomatosis mimicking lung cancer

Lymphomatoid granulomatosis (LYG) of the lung is an extremely rare, Epstein-Barr virus-related lymphoproliferative disease. We report a case of pulmonary LYG that presented as a large necrotic mass.

Pulmonary Lymphomatoid Granulomatosis in Griscelli Syndrome Type 2

Griscelli syndrome type 2 (GS2) is a rare autosomal-recessive disorder associated with a RAB27A gene mutation, and clinically manifesting as hypopigmentation, disseminated chronic encephalitis, and severe immunological disorders characterized by an accelerated hematological phase, also referred to as hemophagocytic syndrome (HS), or hemophagocytic lymphohistiocytosis (HLH). The authors report the diagnosis of GS2 in an 11-year-old girl with hypopigmentation, immunodeficiency, hepatosplenomegaly, severe neurological impairments, and fatal multiorgan failure. In this patient a diagnosis of pulmonary lymphomatoid granulomatosis (LG), an Epstein-Barr virus (EBV)-related lymphoproliferative disorder, was established from radiological and histological findings. Although EBV-related malignancies are common in immunocompromised patients, this is the first report of a diagnosis of pulmonary LG in a patient with GS2.

Lymphomatoid Granulomatosis: CT and FDG-PET Findings

ObjectiveLymphomatoid granulomatosis (LG) is a rare, aggressive extranodal Epstein-Barr virus (EBV)-positive B-cell lymphoproliferative disease. The purpose of our study was to analyze the CT and fluorodeoxyglucose positron emission tomography (FDG-PET) findings of pulmonary LG.Materials and MethodsBetween 2000 and 2009, four patients with pathologically proven pulmonary LG and chest CT were identified. Two of these patients also had FDG-PET. Imaging features of LG on CT and PET were reviewed.ResultsPulmonary nodules or masses with peribronchovascular, subpleural, and lower lung zonal preponderance were present in all patients. Central low attenuation (4 of 4 patients), ground-glass halo (3 of 4 patients), and peripheral enhancement (4 of 4 patients) were observed in these nodules and masses. An air-bronchogram and cavitation were seen in three of four patients. FDG-PET scans demonstrated avid FDG uptake in the pulmonary nodules and masses.ConclusionPulmonary LG presents with nodules and masses with a lymphatic distribution, as would be expected for a lymphoproliferative disease. However, central low attenuation, ground-glass halo and peripheral enhancement of the nodules/masses are likely related to the angioinvasive nature of this disease. Peripheral enhancement and ground-glass halo, in particular, are valuable characteristic not previously reported that can help radiologists suggest the diagnosis of pulmonary LG.

Relapsed extranodal NK/T-cell lymphoma in the mandible, Nasal Type: a case report and review of the literatures

Extranodal NK/T-cell lymphoma (NTCL) is rare disease and has strong geographic prevalence. NTCL is an aggressive form of lymphoma which shows necrosis and ulceration. NTCL often shows an angiocentric and angiodestructive growth pattern . This disease is often found in extranodal sites. The nasal region is the most common involving area of nasal-type NTCL. However, nasal-type NTCL may involve the upper aerodigestive tract (UADT) to the level of the larynx, skin and subcutaneous tissue, gastrointestinal tract and testis. However, lung involvement is rare and this is the difference with pulmonary lymphomatoid granulomatosis, which has similar histological findings with nasal-type NTCL. A common complication of NTCL is a hemophagocytic syndrome and this badly affects survival in NTCL. EBV seemed to contribute to the pathogenesis of this syndrome. NTCL is highly fatal malignant neoplasm, but early pathologic diagnosis is highly difficult and has been no consensus for the treatment. The immunohistochemical findings have been major criteriar for the diagnosis and some molecular markers have been used for the evaluation of the prognosis. We recently experienced a case of Nasal-type NTCL in the mandible. Presented case might be a relapsed lesion from the neck. As the primary lesion was diagnosed as peripheral T-cell lymphoma at other hospital, the oral lesion was easily confused as a relapsed peripheral T-cell lymphoma. The case was presented with its immunohistochemical findings, treatment outcome, and review of literatures.

Successful Management with CHOP for Pulmonary Lymphomatoid Granulomatosis in a Dog

A 3-year-old, spayed female miniature dachshund was presented for vomiting and anorexia. Thoracic radiographs and CT scan revealed abnormal pulmonary opacities at bilateral caudal lobe. Cytological analysis of the pulmonary mass revealed the presence of large lymphohistiocytic cells and small lymphocytes with occasional neutrophils and plasma cells. An open lung biopsy was performed and a diagnosis of pulmonary lymphomatoid granulomatosis (LYG) was made. The dog was administered CHOP based therapy (modified UW-25), and it survived for 1,022 days after admission. Immunohistochemistry revealed pulmonary lesions consisted of many CD79a positive B cells aggregation and proliferation with prominent angiocentric pattern. This was the first case of canine pulmonary LYG managed by CHOP chemotherapy.

Primary orbital lymphomatoid granulomatosis

Lymphomatoid granulomatosis (LYG) is a rare angiocentric and angiodestructive Epstein–Barr virus (EBV)-associated B cell lymphoproliferative disorder that involves multiple organs including the lung, skin, kidney and central nervous system. Ocular...

Pulmonary Lymphomatoid Granulomatosis in a Dog: Evidence of Immunophenotypic Diversity and Relationship to Human Pulmonary Lymphomatoid Granulomatosis and Pulmonary Hodgkin's Disease

We describe a 10-month-old, intact female American Cocker Spaniel with pulmonary lymphomatoid granulomatosis (PLG). On clinical examination, this dog presented with nonproductive dry cough, serous nasal discharge, dyspnea, and lack of appetite. Radiography showed a consolidated lesion in the left cranial lung lobe. Histopathologic examination showed a mixed population of atypical lymphoid cells that had infiltrated into the pulmonary blood vessels angiocentrically. The lymphocytes were CD3 positive, consistent with a pan-T-cell phenotype. The lymphoid cells in the lesion were also positive for CD20cy and CD79a, indicative of the presence of B cells. We also observed large Reed-Sternberg-like cells that were positive for CD15 and CD30, similar to observations in human pulmonary Hodgkin's disease (PHD). In conclusion, canine PLG in this Cocker Spaniel was associated with B and T cells, which is first identified in a case of canine PLG. It was histopathologically similar to human lymphomatoid granulomatosis and immunophenotypically similar to human PHD.

Pulmonary lymphomatoid granulomatosis in a renal allograft recipient

Lymphomatoid granulomatosis (LYG) is a rare multisystemic angiocentric lymphoproliferative disease, which can masquerade as necrotic tissue. There is a paucity of reports of LYG in renal transplant recipients. Herein, we describe LYG in a 56-year-old renal allograft recipient 11 years after transplantation, on azathioprine and prednisolone maintenance immunosuppression, presenting to us with fever, weight loss, and nodular and patchy opacities in both lung fields. Initial percutaneous samples showed necrotic tissue while open biopsy revealed characteristic histopathology with evidence of Epstein-Barr virus. We have reviewed the radiological and pathological findings, and discussed clinical features, differential diagnosis, and treatment of LYG.

First cases of animal diseases published since 2000. 2. Cats1

Summary In this second article of a series of papers listing first case reports of animal diseases published since 2000, the following nine cases of cat diseases are discussed: - Congenital spongiform change in the brain stem nuclei. - Enterococcus hirae enteropathy. - Focal cerebral angiomatosis. - Glomus tumor. - Intraocular extramedullary plasmacytoma. - Lens epithelial neoplasias. - Phaeohyphomycosis due to Fonsecaea pedrosoi. - Pulmonary lymphomatoid granulomatosis. - Systemic amyloidosis in a Devon rex. After a short introduction, the bibliographical data, the abstract of the author(s) and some additional information derived from the article are given. The article will be regularly updated adding overlooked as well as new first reports.

X-linked lymphoproliferative disease: clinical, diagnostic and molecular perspective.

X-linked lymphoproliferative disease: clinical, diagnostic and molecular perspective.

Expression of cytolytic lymphocyte-associated antigens in pulmonary lymphomatoid granulomatosis.

Paraffin-embedded lung wedge biopsy specimens from 14 patients with pulmonary lymphomatoid granulomatosis (LYG) were analyzed using immunoperoxidase stains specific for T cell- and natural killer cell-associated antigens. Nine cases had a minor population of CD20+ large B-cells (B-cell LYG) amidst a background of CD3- and betaF1-immunoreactive T cells. In 8 of the 9 B-cell LYG cases, the majority of the background T lymphocytes had a cytotoxic phenotype as defined by the expression of CD8 and the cytotoxic granule proteins TIA-1 (granule membrane protein 17) and granzyme B. Five cases lacked CD20+ large cells and, instead, showed predominantly CD3+ and betaF1 + T cells (T-cell LYG). Whereas the small, medium, and large atypical lymphocytes were all positive for CD3 and betaF1 in the T-cell LYG cases, immunoreactivity for CD8, TIA-1, and granzyme B was limited to the small lymphocytes, with a distribution indistinguishable from that seen in B-cell LYG. These findings indicate that LYG is composed of a heterogeneous group of lymphoproliferative disorders that share, as unifying features, a relative paucity of neoplastic cells and a prominent reactive infiltrate rich in cytolytic lymphocytes.