Granulomatosis linfomatoide. Presentación de un caso / Lymphomatoid granulomatosis. Case Presentation

RESUMEN Se presenta el caso de un paciente masculino de 21 años, quien el 30 de diciembre de 2017 comenzó con aumento de volumen, rubor y calor en región lateral derecha del cuello, posterior a la presencia de un forúnculo en región occipital del cuero cabelludo. Se inició tratamiento con antibiótico, pero aparecieron lesiones eritematopapulosas en tronco y extremidades que se interpretaron como una reacción adversa al medicamento. Comenzó con fiebre mantenida de 380C, decaimiento marcado, pérdida de peso, hepatomegalia y adenopatías pequeñas y no dolorosas en regiones inguinales y axilares, por lo que fue ingresado el 14 de enero de 2018 en el Hospital Gustavo Aldereguía Lima, de Cienfuegos y se impuso tratamiento con antibiótico ante un perfil hematológico que indicaba sepsis asociada. Se realizó aspiración con aguja fina que resultó negativa y se hicieron coordinaciones para biopsia. El 15 de enero empeoró el estado clínico del paciente con fiebre elevada, desorientación, marcada excitación, toma de conciencia e insuficiencia respiratoria, por lo que falleció el día 16. La autopsia reveló como causa básica de muerte la granulomatosis linfomatoide. Por ser esta una entidad infrecuente, desconocida por gran cantidad de clínicos y cuyo tratamiento y pronóstico no está definido, se decidió la presentación del caso.

ABSTRACT The case of a 21-year-old male patient is presented, who on December 30, 2017 began with increased volume, flushing and heat in the right lateral region of the neck, after the presence of a boil in the occipital region of the scalp. Treatment with antibiotic was started, but erythe-matopapular lesions appeared on the trunk and extremities that were interpreted as a medication adverse reaction. He started with a maintained fever of 38 0C, malaise, weight loss, hepatomegaly and small non-painful adenopathies in the inguinal and axillary regions, so he was admitted on January 14, 2018 at the Gustavo Aldereguía Lima Hospital in Cienfuegos and was treated with antibiotic before a hematological profile which indicated associated sepsis. A fine-needle aspiration was performed, which was negative and arrangement for biopsy was made. On January 15, the clinical condition of the patient worsened with high fever, disorientation, marked excitement, awareness and respiratory insufficiency, and he died on the 16th. Autopsy revealed lymphomatoid granulomatosis as the main cause of death.

Primary subcutaneous lymphomatous granuloma: a case report and review of literature / 临床与实验病理学杂志

Purpose To investigate the clinicopathological features of primary subcutaneous lymphomatoid granulomatosis (LYG). Methods A case of primary subcutaneous LYG was observed by analysis of the clinical, histological features, immunophenotype and molecular pathology with review of the related literature. Results The male patient, 78-year-old, inadvertently found a mass of right axillary for more than 10 days. The boundary of the mass was clear, it seemed to have a capsule, the cut surface was grayish yellow and grayish red, the texture was medium. A large amount of coagulative necrosis was observed in the center of the mass under microscope. The peripheral area showed a morphological change of panniculitis, accompanied by pleomorphic lymphoid infiltration, showed central and vascular destructive infiltration, pathological mitosis was occasionally observed. Immunophenotyping showed that atypical large lymphoid cells expressed CD45 RB, CD20, CD30, while CD3, CD15, CD56, TIA-1, Granzyme B, ALK, CD21, Langerin and CD1 a, S-100 and CK (AE1/AE3) were negative. The proliferation index of Ki-67 ranged from 50％ to 60％. EBER in situ hybridization showed that positive cells were> 20/HPF.Neither acid fast staining nor TB-DNA testing supported tuberculosis. Molecular pathology found clonal Ig K gene rearrangement, TCRB + TCRG gene rearrangement showed the absence of monoclonal proliferating T cell population. Conclusion The primary subcutaneous LYG is a rare tumor. which can be diagnosed by combination of morphology, immunophenotype and molecular pathology.

Lymphomatoid granulomatosis: A case series from South India

Context: Lymphomatoid granulomatosis (LYG) is a rare B-lymphoproliferative disorder characterised by an angiocentric and angiodestructive pattern along with Epstein - Barr virus (EBV) association. It is one of the diagnostic challenges in lymphoma pathology. Deregulation of EBV immune surveillance is one of the narrated hypotheses in the literature. Extrapulmonary manifestations are rare with LYG. Morphological grading is done based on the number of EBV-positive B cells, which is useful to strategize treatment protocol. Aims: We report here a series of nine cases of LYG to discuss the clinical, histological, and immunohistochemistry findings. Settings and Design: This is the first case series from India in published literature. Subjects and Methods: We reviewed cases of LYG diagnosed at our center for the past 11 years (2006-2016). A total of nine cases were included in this study. Histomorphology was studied in conjunction with immunohistochemistry and clinical details. Cases without classical morphology and negative for EBV immunostain were excluded from the study. Results: There were nine patients in our study (7 males and 2 female; M:F ratio 3.5:1). The age of these patients ranged from 4 years to 57 years (mean age: 30 years). The most common site involved was the lung (4, 44%), followed by the skin (2, 22%), central nervous system (2, 22%) and lymph node (1, 11%). One patient had primary immunodeficiency. Another patient had undergone renal transplant 11 years before the development of the lesion. Angiocentricity and angioinvasion were appreciated in all nine cases (9/9) with necrosis in four cases (44%) and ill-defined histiocytic aggregates in three cases (33%). The histological features were as follows: Grade 1(4 cases, 44%), Grade 2(2 cases, 22%), and Grade 3(3 cases, 33%). Conclusion: LYG is a rare EBV driven angiodestructive disease with predominantly lung involvement as well as isolated extrapulmonary sites as seen in our study. It is often progressive and ultimately fatal in the absence of appropriate treatment. Grading of the lesion helps to initiate the appropriate treatment of choice.

Progress of lymphomatoid granulomatosis / 白血病·淋巴瘤

Lymphomatoid granulomatosis (LG) is a kind of Epstein-Barr virus (EBV)-positive B-cell lymphoproliferative disease. Pathologically, it is characterized by angioinvasion and large EBV-positive atypical B cells in an inflammatory background with numerous T cells. Clinically, it mainly involves the bilateral lungs in the mid and lower lung fields, extra-pulmonary sites such as central nervous system, skin have also been reported. There are still no uniform treatment protocols about the disease, and pathological grading is a main reference. Overall, the prognosis is poor. Because of the rarity, lack of typical symptoms and special imaging characteristics, LG was often misdiagnosed. The article summarizes the diagnosis, treatment and prospects of LG to improve the clinician's understanding of the disease.

Low Grade Pulmonary Lymphomatoid Granulomatosis with an Endobronchial Mass / 결핵및호흡기질환

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.

A case of lymphomatoid granulomatosis with fever, pulmonary nodules, joint pain and superficial lymphadenopathy / 中华老年医学杂志

A 69-year-old man with fever,pulmonary nodules,joint pain and superficial lymphadenopathy was admitted to our hospital.The patient had a history of ten year hypertension.She smoked a pack of cigarettes daily for forty years and quitted for fifteen years.Family history of coronary heart disease,diabetes,cancer or other diseases was negative.Chest CT showed a nodule in the left lung lower lobe.Percutaneous lung biopsy revealed a large number of atypical B cell proliferation and infiltration which involved the vessel wall.The atypical B-cell phenotype and genotype was EBERs (+),CD20 (+),CD30 (+),CD15 (-).The patient was diagnosed as pulmonary lymphomatoid granulomatosis (LYG),an angiodestructive and angioinvasive lymphoproliferative disorder which is an Epstein-Barr virus associated B cell disorder with reactive T lymphocytes.The patient received six courses of chemotherapy.In this rare case,misdiagnosis of LYG often occurred due to the complex clinical presentation and non-specific imaging.Percutaneous or open lung biopsy is the main choice in the diagnosis of LYG.

Lymphomatoid Granulomatosis with Spinal Involvement after Childhood Acute Lymphoblastic Leukemia

Lymphomatoid granulomatosis (LYG) is a lymphoproliferative disease involving the lungs most frequently; however, it may also involve the kidneys, skin and especially the central nervous system. Unique initial presentation of spinal involvement is extremely rare and epidural lesion of thoracic spine has not been reported. The prognosis for LYG has been reported to be poor, and there currently exists no satisfactory established treatment protocol. The purpose of this study is to report a case of successful treatment with surgery and rituximab combination therapy in thoracic spinal LYG.

Lymphomatoid Granulomatosis: CT and FDG-PET Findings

OBJECTIVE: Lymphomatoid 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 METHODS: Between 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. RESULTS: Pulmonary 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. CONCLUSION: Pulmonary 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.

Granulomatose linfomatóide-relato de caso / Lymphomatoid granulomatosis -case report

Objetivo: descrever um caso com manifestaçõs clínicas e achados histopatológicos de Granulomatose Linromatóide (GL), uma rara doença linfoproliferativa das células B, angiocêntrica e angiodestrutiva associada ao vírus Epstein-Barr (EBV). A apresentação clínica mais comum é no pulmão, podendo acometer qualquer órgão, sendo os achados extra-pulmonares as principais manifestações nestes casos. Relato do caso: mulher de 65, com lesão cutânea vulvar e que evoluiu com quadro neurológico associado a lesão expansiva envolvendo parênquima cerebelar, sem alterações pulmonares. Considerações Finais: pelo seu prognóstico e capacidade de ser confundida com outras vasculites a GL impõe-se como um importante diagnóstico diferencial dessas doenças.

lntroduction: this report describe a case of Lymphomatoid Granulomatosis (LG,s a rare linfoproliferative disorder of B cell showing angiocentric growth and destructive behaviour caused by Epstain-Barr virus (EBV). The predominant clinical presentations are confined to the pumonary system, however ; extra-pumonary manifestations can sometimes be the main feature of the dieseses. Method: here we report a 65 year old female, with a skin vulvar lesion, and another in cerebelus, without pulmonary disease. Conclusion: the LG can mimics systemic vasculits and is a diagnostic challenge.

Central Nervous System Lymphomatoid Granulomatosis Presenting with Parkinsonism

Lymphomatoid granulomatosis (LG) is a potentially malignant lymphoproliferative disorder. The lung is the most common involved site, followed by the skin and nervous system. However, LG of the central nervous system presenting with Parkinsonism is very rare. We report a patient with LG who presented with parkinsonian features such as bilateral rigidity, bradykinesia, and agitation. Brain magnetic resonance imaging showed multifocal punctuate enhanced lesions in both supra- and infratentorial areas. Steroid pulse therapy resulted in a dramatical improvement in the symptoms and MRI abnormalities.

A case report of lymphomatoid granulomatosis and review of the literature / 北京大学学报(医学版)

To explore the clinical manifestation of lymphomatoid granulomatosis (LG) and the early diagnosis. Retrospective analyses of the clinical features of LG and pathology features by open lung bio-psies were conducted. The patient was a 42-year-old female with irregular fever,and her chest X-ray and computed tomography showed nodules with cavity and pleural effusion. Open lung biopsy proved LG. LG is seldom seen in clinic. Open lung biopsy is very important for pathology diagnosis. Early diagnosis and treatment are the key to improving survival in these patients. The therapeutic effect is good.

The radiographic findings of lymphoproliferative disorders of the lung / 中华放射学杂志

Objective To study the radiographic findings of lymphoproliferative disorders of the lung. Methods Twenty five patients with lymphoproliferative disorders of the lung were examined by X ray film, tomography, and CT. Results Multiple and mediastinal lymphadenopathy were observed in 2 patients with pulmonary pseudolymphoma. Multiple nodules or masses were observed in 4 patients with pulmonary lymphomatoid granulomatosis. Hilar and mediastinal lymphadenopathy was observed in each patient with angioimmunoblastic lymphadenophathy, 2 patients had multiple nodules or masses, 8 patients had single or multiple patchy infiltrations, 10 had diffuse interstitial infiltrations. 3 patients with Castlemen′ disease had a mass in the mediastinum, and another patient had mediastinal lymphadenopathy. Conclusion Radiographic findings of lymphoproliferative disorders of the lung are varied, and the final diagnosis relies on pathology.

Clinical and pathological features of lymphomatoid granulomatosis:report of one case / 临床神经病学杂志

Objective To investigate the clinical and pathological features of lymphomatoid granulomatosis(LG).Methods The data of clinic and the results of pathological study of brain biopsy which stained by immunohistochemical method were reviewed in one case with LG.Results The patient was presented with headache,dizzy,slurred speech and myasthenia of limbs.MRI showed abnormal signals on bilateral cerebral white matter,pons,cerebellum and cervical-thoracic spinal cord.Gd-enhanced imaging showed punctiform enhancement.The protein and IgG in cerebrospinal fluid elevated slightly.Brain biopsy showed angiocentric,angiodestructive granuloma formation combined with infiltration of multiple cells.The infiltrated cells confirmed immunohistochemically were lymphocytes and mononuclear phagocytes.In situ hybridization technique showed EBV positive.The status of this patient improved after radiotherapy and glucocorticosteroid treatment.Conclusion LG lacks characteristic features in clinic,laboratory and radiological examinations,so the pathological examination is important for diagnosis of this disease.

A Case of Pulomonary Lymphomatold Granulomatosis / 대한내과학회지

Lymphomatoid granulomatosis was described by Liebow as a peculiar angiocentric and angiodestructive lymphoreticular proliferative granulomatous disease. Although the lung is most frequently involved, lymphomatoid granulomatosis is also commonly found in the upper respiratory tract, skin, kidneys and central nervous system. Pulmonary infiltrates are histologically polymorphous and show variable degree of cytologic atypic in the lymphoid cells. Infrequent involvement of the bone marrow, spleen and peripheral lymph nodes initially supported the distinction of lymphomatoid granulomatasis from lymphoma. We experienced a rare case of 26-year-old female diagnosed as lymphomatoid granulomatosis by per cutaneous needle aspiration biopsy and transbronchial lung biopsy with symptoms of fever, cough, sputum and right chest pain. After 4th chemotherapy it showed partial remission and chemotherapy is going on.

Lymphomatoid granulomatosis with isolated involvement of the brain: case report

Lymphomatoid granulomatosis usually presents as a primary lung affliction with secondary metastatic spread to the central nervous system(CNS), and its initial manifestation purely as a CNS disease is rare. A 57-year-old man with histologically proven lymphomatoid granulomatosis of the brain as the sole manifestation of the disease is presented.

Primary Lymphomatoid Granulomatosis in the Frontal Lobe: Case Report

We report a case of cerebral lymphomatoid granulomatosis in a 50-year-old man. Lymphomatoid granulomatosis is an unusual condition which usually presents as a pulmonary manifestation, which occasionally affects the brain, and causes focal inflammatory lesions. Primary cerebral lymphomatoid granulomatosis is very rare. We describe a case of lymphomatoid granulomatosis in the left frontal lobe without pulmonary involvement.