RESUMEN
A male patient in his 40s who had been unwell for months with fever of unknown origin and clinicopathological features suspicious for haemophagocytic lymphohistiocytosis presented to hospital with worsening subacute shortness of breath. CT pulmonary angiogram demonstrated ground glass changes involving all lung lobes with an apicobasal gradient. These changes, combined with long-term steroid exposure for granulomatous hepatitis without pneumocystis prophylaxis, raised concern for pneumocystis jirovecii pneumonia (PJP). A subsequent bronchoscopic lavage specimen was positive on PCR for PJP and the patient was started on appropriate therapy. Clinical and radiological changes initially improved but after completion of therapy, symptoms and radiological abnormalities returned. Retreatment with second-line treatment resulted again in initial improvement followed by relapse with acute deterioration. Further investigations for an alternate diagnosis were made, with a surgical lung biopsy performed finally revealing immunosuppression-related Epstein-Barr virus positive large B cell lymphoma with lymphomatoid granulomatosis of grade 3 pattern.
Asunto(s)
Granulomatosis Linfomatoide , Neumonía por Pneumocystis , Humanos , Granulomatosis Linfomatoide/diagnóstico , Granulomatosis Linfomatoide/tratamiento farmacológico , Masculino , Diagnóstico Diferencial , Adulto , Neumonía por Pneumocystis/diagnóstico , Neumonía por Pneumocystis/tratamiento farmacológico , Pneumocystis carinii/aislamiento & purificación , Tomografía Computarizada por Rayos X , Pulmón/diagnóstico por imagen , Pulmón/patologíaRESUMEN
Lymphomatoid granulomatosis (LYG) is a rare B cell lymphoproliferative disorder associated with Epstein-Barr virus infection. LYG diagnosis is often difficult because of non-specific and varied radiological and pathological findings. The lung is the most common organ of LYG occurrence, but extrapulmonary lesions involving the central nervous system, skin, kidneys and liver are observed. A surgical biopsy is often inevitable for LYG diagnosis.We encountered a man in his 50s who presented with progressive dyspnoea. Extrapulmonary lesions were not observed. Although he developed respiratory failure within a short period, a low dose of corticosteroid relieved his symptoms. Video-assisted thoracoscopic lung biopsy revealed grade 1 LYG. The patient was successfully treated with chemotherapy, including rituximab. Only a few cases of LYG with progressive respiratory failure are reported, and most have been diagnosed via autopsy. Our case highlights the importance of performing a surgical lung biopsy at the appropriate time to diagnose LYG.
Asunto(s)
Infecciones por Virus de Epstein-Barr , Granulomatosis Linfomatoide , Síndrome de Dificultad Respiratoria , Insuficiencia Respiratoria , Masculino , Humanos , Granulomatosis Linfomatoide/complicaciones , Granulomatosis Linfomatoide/diagnóstico , Granulomatosis Linfomatoide/tratamiento farmacológico , Infecciones por Virus de Epstein-Barr/complicaciones , Herpesvirus Humano 4 , Pulmón/diagnóstico por imagen , Pulmón/patología , Síndrome de Dificultad Respiratoria/complicaciones , Insuficiencia Respiratoria/complicacionesRESUMEN
BACKGROUND: Lymphomatoid granulomatosis (LYG) is a rare pediatric disorder driven by the Epstein-Barr virus and is considered as a part of the lymphoma spectrum. It is mostly associated with immune deficiency and patients on immunosuppressive therapy, especially with acute leukemia. It can present as a multisystemic disease, diagnosed on biopsy as atypical lymphocytes with an angiocentric pattern against a background composed of histiocytes, neutrophils, and extensive T-cell infiltration. OBSERVATION: We report 3 cases of children with Lymphomatoid granulomatosis, one with Langerhans cell histiocytosis. CONCLUSION: Combination chemotherapy was used for the treatment of Lymphomatoid granulomatosis; however, the prognosis is guarded. One of 3 patients is alive and in remission on the last follow-up visit at 15 months.
Asunto(s)
Infecciones por Virus de Epstein-Barr , Granulomatosis Linfomatoide , Humanos , Niño , Granulomatosis Linfomatoide/diagnóstico , Granulomatosis Linfomatoide/tratamiento farmacológico , Granulomatosis Linfomatoide/patología , Herpesvirus Humano 4 , Infecciones por Virus de Epstein-Barr/complicaciones , Infecciones por Virus de Epstein-Barr/patología , Pakistán , Linfocitos T/patologíaRESUMEN
A man in his 60s presented with intermittent constitutional symptoms along with waxing and waning chest radiographic abnormalities, eventually leading to a diagnosis of lymphomatoid granulomatosis (LYG). LYG is a rare, progressive Epstein-Barr virus (EBV)-driven lymphoproliferative disease associated with immune dysregulation most commonly involving the lungs. The diagnosis requires tissue biopsy; thus, the decision to pursue tissue sampling with histopathology examination in a timely manner is essential. Currently, there are no established guidelines regarding the treatment of LYG, which varies from cessation of immunosuppressants to immunochemotherapy and usually requires multidisciplinary team discussion.
Asunto(s)
Infecciones por Virus de Epstein-Barr , Granulomatosis Linfomatoide , Masculino , Humanos , Factor de Necrosis Tumoral alfa , Infecciones por Virus de Epstein-Barr/complicaciones , Infecciones por Virus de Epstein-Barr/tratamiento farmacológico , Granulomatosis Linfomatoide/inducido químicamente , Granulomatosis Linfomatoide/diagnóstico , Granulomatosis Linfomatoide/tratamiento farmacológico , Herpesvirus Humano 4 , Factores InmunológicosAsunto(s)
Granulomatosis Linfomatoide , Neoplasias Cutáneas , Humanos , Granulomatosis Linfomatoide/inducido químicamente , Granulomatosis Linfomatoide/diagnóstico , Granulomatosis Linfomatoide/tratamiento farmacológico , Inmunosupresores/efectos adversos , Neoplasias Cutáneas/inducido químicamente , Neoplasias Cutáneas/diagnóstico , Neoplasias Cutáneas/tratamiento farmacológicoRESUMEN
BACKGROUND Lymphomatoid granulomatosis (LyG) is a rare lymphoproliferative disorder associated with Epstein-Barr virus (EBV) in which there is an infection of B cells and numerous reactive T cells. The lymphoproliferative disorder progresses to organ infiltration and resultant dysfunction of affected organs. Histologically, it is characterized by a triad of polymorphic lymphoid infiltrate, angiitis, and granulomatosis. The lungs are the most commonly involved sites for lymphomatoid granulomatosis, but other sites that can be involved include the liver, skin, and central nervous system. The signs and symptoms of LyG can vary, and can produce generalized symptoms such as cough, shortness of breath, and chest tightness, but can vary depending on the location of LyG. CASE REPORT We report a case of a 60-year-old man who presented with altered mental status. Cross-sectional imaging of the brain was negative for any acute intracranial process, but a fine-needle biopsy of a retroperitoneal lymph node revealed nodular polymorphous mononuclear infiltrates containing atypical large EBV-positive B cells with positive EBER and CD30, consistent with lymphomatoid granulomatosis. The patient was started on a regimen of brentuximab/bendamustine, and instructed to follow up with Oncology on an outpatient basis. CONCLUSIONS Treatment options for lymphomatoid granulomatosis are based on the disease grading. Lymphomatoid granulomatosis can be classified by using a grading system determined by the number of EBV-positive large B cell malignant cells, along with necrosis. The most effective treatment for lymphomatoid granulomatosis is unknown, but at this time treatment protocols are based on the grade of the disease. The clinical and histological features of lymphomatoid granulomatosis are discussed in this case report.
Asunto(s)
Infecciones por Virus de Epstein-Barr , Granulomatosis Linfomatoide , Infecciones por Virus de Epstein-Barr/complicaciones , Herpesvirus Humano 4 , Humanos , Pulmón/patología , Ganglios Linfáticos/patología , Granulomatosis Linfomatoide/complicaciones , Granulomatosis Linfomatoide/diagnóstico , Granulomatosis Linfomatoide/patología , Masculino , Persona de Mediana EdadRESUMEN
CASE PRESENTATION: A 71-year-old man with history of gastroesophageal reflux disease, chronic sinusitis, arthritis, hypothyroidism, and anemia of chronic disease initially sought treatment with a recurrent left pleural effusion along with other abnormal lung findings on chest CT scan. Before his referral, he was being managed for 3 years at his local hospital for waxing and waning fevers, fatigue, productive cough, chills, and night sweats. He did not report any hemoptysis or chest pain, but reported weight loss of 13 kgs in 15 months. During those 3 years, he was treated with multiple courses of antibiotics and steroids with temporary relief of symptoms. At that time, his chronic sinusitis was suspected to be the cause of his symptoms and he underwent balloon sinuplasty. He was receiving daily sublingual immunotherapy for inhaled respiratory allergens for the previous year after showing positive test results for 17 inhaled allergens. The patient had no other known immunologic workup before our evaluation.
Asunto(s)
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Pulmón/diagnóstico por imagen , Granulomatosis Linfomatoide/diagnóstico , Anciano , Broncoscopía , Ciclofosfamida/uso terapéutico , Doxorrubicina/uso terapéutico , Empiema/fisiopatología , Infecciones por Virus de Epstein-Barr , Fiebre/fisiopatología , Humanos , Leucocitosis/fisiopatología , Pulmón/patología , Granulomatosis Linfomatoide/tratamiento farmacológico , Granulomatosis Linfomatoide/fisiopatología , Granulomatosis Linfomatoide/virología , Masculino , Prednisona/uso terapéutico , Rituximab/uso terapéutico , Tomografía Computarizada por Rayos X , Vincristina/uso terapéuticoRESUMEN
We report two patients with meningoencephalomyelitis without evidence of extra central nervous system (CNS) involvement. Brain MRI showed linear perivascular radial gadolinium enhancement patterns and spinal cord MRI showed longitudinal extensive T2-hyperintensity lesions. Pathological findings from brain biopsies were angiocentric T-cell predominant lymphoid infiltrates that lacked Epstein-Barr virus-positive atypical B cells. The patients were initially suspected to have isolated CNS-lymphomatoid granulomatosis (LYG). Thereafter, glial fibrillary acidic protein (GFAP)-immunoglobulin G were detected in their cerebrospinal fluid. This finding suggested autoimmune GFAP astrocytopathy. We speculate there is a link between isolated CNS-LYG and autoimmune GFAP astrocytopathy.
Asunto(s)
Astrocitos/patología , Enfermedades Autoinmunes del Sistema Nervioso/diagnóstico , Proteína Ácida Fibrilar de la Glía/inmunología , Granulomatosis Linfomatoide/diagnóstico , Corticoesteroides/uso terapéutico , Anciano , Especificidad de Anticuerpos , Astrocitos/inmunología , Autoanticuerpos/líquido cefalorraquídeo , Enfermedades Autoinmunes del Sistema Nervioso/líquido cefalorraquídeo , Enfermedades Autoinmunes del Sistema Nervioso/inmunología , Enfermedades Autoinmunes del Sistema Nervioso/patología , Encéfalo/diagnóstico por imagen , Encéfalo/inmunología , Encéfalo/patología , Diagnóstico Diferencial , Humanos , Inmunoglobulina G/líquido cefalorraquídeo , Inmunoglobulinas Intravenosas/uso terapéutico , Imagen por Resonancia Magnética , Masculino , Meningoencefalitis/etiología , Persona de Mediana Edad , Mielitis/etiología , Neuroimagen , Estudios Retrospectivos , Médula Espinal/diagnóstico por imagen , Médula Espinal/inmunología , Médula Espinal/patologíaRESUMEN
CASE PRESENTATION: A 67-year-old woman with a medical history significant for hypertension, hyperlipidemia, type 2 diabetes mellitus, OSA, and schizophrenia was admitted multiple times the previous 3 months for generalized abdominal pain. Her most recent admission was unique for new onset bilateral upper and lower extremity weakness with paresthesia. Pertinent review of systems included malaise, fever, cough, left lower quadrant pain without weight loss, and rash. Previous evaluation included multiple CT scans of her abdomen that revealed colonic thickening. Ensuing colonoscopy revealed chronic ulcers with cytopathic changes consistent with cytomegalovirus.
Asunto(s)
Dolor Abdominal/etiología , Encéfalo/diagnóstico por imagen , Pulmón/diagnóstico por imagen , Granulomatosis Linfomatoide/complicaciones , Parestesia/etiología , Nódulo Pulmonar Solitario/diagnóstico , Dolor Abdominal/diagnóstico , Anciano , Biopsia , Diagnóstico Diferencial , Progresión de la Enfermedad , Femenino , Humanos , Granulomatosis Linfomatoide/diagnóstico , Imagen por Resonancia Magnética/métodos , Parestesia/diagnóstico , Tomografía Computarizada por Rayos XRESUMEN
Lymphomatoid granulomatosis (LYG) is a rare Epstein-Barr virus (EBV)-driven B-cell lymphoproliferative disease affecting mainly extranodal sites such as the lung, central nervous system (CNS), skin, kidney, and liver. We report a case of low-grade LYG involving the CNS that was successfully treated with interferon alpha (IFNα). A 69-year-old woman developed necrotic erythema of the skin and was initially diagnosed with pyoderma gangrenosum based on skin biopsy. She showed a limited response to prednisolone. Approximately 6 months after the initial onset, low-grade LYG was diagnosed after detection of CNS lesions on brain biopsy. The whole blood EBV-DNA load determined by real-time polymerase chain reaction was slightly elevated. Two months into IFNα therapy, skin and CNS lesions had responded favorably and the EBV-DNA load decreased. IFNα plays an important role in treatment of LYG through its antiproliferative, immunomodulatory, and anti-EBV effects. To our knowledge, this is the first case report of successful treatment with IFNα in Japan. Further investigation is necessary to determine optimal use of IFNα for LYG.
Asunto(s)
Neoplasias del Sistema Nervioso Central/diagnóstico , Neoplasias del Sistema Nervioso Central/tratamiento farmacológico , Interferón-alfa/uso terapéutico , Granulomatosis Linfomatoide/diagnóstico , Granulomatosis Linfomatoide/tratamiento farmacológico , Anciano , Biomarcadores , Biopsia , Neoplasias del Sistema Nervioso Central/etiología , Evolución Clonal , Femenino , Humanos , Inmunohistoquímica , Interferón-alfa/administración & dosificación , Granulomatosis Linfomatoide/etiología , Imagen por Resonancia Magnética , Evaluación de Síntomas , Tomografía Computarizada por Rayos X , Resultado del TratamientoRESUMEN
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. Autopsy revealed nodules in the lungs and subcutis, and corneal thickening and cloudiness. Histologic examination of cutaneous nodules, lungs, and eye showed similar angioinvasive cellular infiltrates and pattern to that of the original skin biopsy, consistent with a diagnosis of LYG. The neoplastic cells displayed CD3-positive immunoreactivity in the skin, eye, and lung, and PCR for antigen receptor rearrangement (PARR) showed T-cell clonality in all tissues tested. This is the third case of LYG to be reported in cats and is the only case in which PARR analysis and immunophenotyping immunohistochemical staining was performed. LYG with ocular involvement has not been reported previously in cats, to our knowledge. Our case demonstrates the necessity for considering LYG when presented with a cat with respiratory signs in conjunction with subcutaneous nodules and ocular lesions.
Asunto(s)
Enfermedades de los Gatos/diagnóstico , Neoplasias del Ojo/veterinaria , Neoplasias Pulmonares/veterinaria , Granulomatosis Linfomatoide/veterinaria , Metástasis de la Neoplasia/diagnóstico , Neoplasias Cutáneas/veterinaria , Animales , Enfermedades de los Gatos/patología , Gatos , Neoplasias del Ojo/diagnóstico , Neoplasias del Ojo/secundario , Femenino , Neoplasias Pulmonares/diagnóstico , Neoplasias Pulmonares/patología , Granulomatosis Linfomatoide/diagnóstico , Granulomatosis Linfomatoide/patología , Neoplasias Cutáneas/diagnóstico , Neoplasias Cutáneas/secundarioRESUMEN
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.