1/35. Exclusive CNS involvement by lymphomatoid granulomatosis in a 12-year-old boy: a case report.BACKGROUND: lymphomatoid granulomatosis [LYG] is an angiocentric, angiodestructive disease at the higher grade end of the spectrum of angiocentric immunoproliferative lesions. It primarily involves the lungs, but it may also involve several extrapulmonary sites including the central nervous system (CNS), skin, and kidneys. CASE DESCRIPTION: Clinical presentation, radiology and treatment of LYG in a 12-year-old male child with multiple intracranial extraaxial lesions is described. A 12-year-old boy presented with sudden onset of left focal motor seizures with associated history of headache and vomiting. Computerized tomographic scan of the brain suggested high-density, bilateral, parietal extraaxial lesions. On magnetic resonance imaging, the lesions were iso- to hyperintense on T1-weighted images and hyperintense on T2-weighted images. The lesions were excised in two stages and histopathological examination confirmed the diagnosis. CONCLUSION: LYG seldom involves the CNS exclusively. The present case demonstrates exclusive CNS involvement by LYG in a young boy.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system, brain (Clic here for more details about this article) |
2/35. radiotherapy to control CNS lymphomatoid granulomatosis: a case report and review of the literature.lymphomatoid granulomatosis (LG) is an uncommon but potentially fatal disease. The disease primarily involves the lungs; however, skin, renal, and central nervous system (CNS) are seen in varying proportions. Neurological involvement occurs in one third of the patients, and confers a worse prognosis. The use of radiotherapy to treat CNS involvement in LG has not been well studied. We report a case of a 33-year-old man with multiple CNS lesions treated successfully with radiotherapy and review 6 other cases in the literature using similar treatment. These cases support the use of radiotherapy for CNS involvement in LG.- - - - - - - - - - ranking = 0.97474357390079keywords = central nervous system, nervous system (Clic here for more details about this article) |
3/35. lymphomatoid granulomatosis in a 4-year-old boy.lymphomatoid granulomatosis is a necrotizing angiocentric and angiodestructive infiltrative process involving primarily the lung, skin, central nervous system, and kidney. The incidence is highest in middle-aged men and is rare in children. We report a case of lymphomatoid granulomatosis involving both skin and lung in a 4-year-old boy. The disease progressed to peripheral T-cell lymphoma, which was unusual in light of recent evidence suggesting a B-cell origin in the majority of cases.- - - - - - - - - - ranking = 0.97474357390079keywords = central nervous system, nervous system (Clic here for more details about this article) |
4/35. hiv-2 infection with cerebral toxoplasmosis and lymphomatoid granulomatosis.A Nigerian man had acute onset of headache and vertigo due to a cerebellar mass. A brain biopsy of the mass revealed toxoplasmosis despite repeated negative hiv-1 serology. The presence of an opportunistic infection and his country of origin raised the suspicion for hiv-2; this was confirmed by positive hiv-2 serology. Despite his preliminary pathological diagnosis, results of physiological magnetic resonance imaging (MRI) (perfusion MRI and proton magnetic resonance spectroscopy) were not typical for toxoplasmosis. The lesion showed a biochemical and perfusion pattern that was intermediate for infectious and neoplastic processes. Further neuropathology confirmed a secondary diagnosis of lymphomatoid granulomatosis.- - - - - - - - - - ranking = 0.025256426099206keywords = brain (Clic here for more details about this article) |
5/35. lymphomatoid granulomatosis after childhood acute lymphoblastic leukemia: report of effective therapy.lymphomatoid granulomatosis, a rare condition in children, affects the lungs primarily but may have significant extrapulmonary manifestations, especially in the central nervous system. We report a case of lymphomatoid granulomatosis with onset after the completion of chemotherapy for childhood acute lymphoblastic leukemia. Two months after treatment ended, the 7-year-old girl developed splenomegaly, cervical adenopathy, and bilateral interstitial pulmonary infiltrates. She improved on cefotaxime but experienced a seizure 1 month later. A computed tomography scan of the head was normal, but her pulmonary infiltrates had become nodular. A computed tomography-guided biopsy of 1 of the nodules revealed cellular interstitial pneumonitis. One month later, she had persistent pulmonary infiltrates, marked splenomegaly, and new seizures. Magnetic resonance imaging of the head revealed cerebral nodules. itraconazole was begun, and the pulmonary infiltrates resolved. Five months after her initial symptoms, she developed tonic pupil and a decreased level of consciousness. dexamethasone was initiated. Needle biopsies of the brain were carried out, yielding the diagnosis of severe chronic inflammatory changes focally consistent with granuloma. The child redeveloped splenomegaly and fever, and then suffered an acute decompensation with hypoxemia, tachypnea, splenomegaly, and cardiac gallop. Open-lung biopsy revealed lymphomatoid granulomatosis. Lymphoma-directed therapy was initiated, and the patient had complete resolution of pulmonary and cerebral nodules 5 months later. No intrathecal chemotherapy was administered, and radiation therapy was not necessary. Neuropsychological testing obtained after completion of therapy revealed an improvement in attention, coordination, and fine motor speed over time. She is now in good health and attending school.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system, brain (Clic here for more details about this article) |
6/35. Autologous hematopoietic stem cell transplantation for refractory lymphomatoid granulomatosis.lymphomatoid granulomatosis (LG) is a rare lymphoproliferative disorder. There is no standard therapy for refractory patient. Here we present the case of a patient with LG of the lung and the brain who was refractory to polychemotherapy. An autologous hematopoietic stem cell transplantation was done and the patient achieved a complete remission. This represents the first case of high-dose chemotherapy with hematopoietic stem cell support in this disease.- - - - - - - - - - ranking = 0.025256426099206keywords = brain (Clic here for more details about this article) |
7/35. Isolated EBV lymphoproliferative disease in a child with wiskott-aldrich syndrome manifesting as cutaneous lymphomatoid granulomatosis and responsive to anti-CD20 immunotherapy.patients with primary immunodeficiencies such as the wiskott-aldrich syndrome (WAS) are prone to develop Epstein-Barr virus (EBV) related lymphoproliferative disorders (LPDs). EBV LPD is most frequently seen in patients receiving immunosuppressive treatment after organ transplantation (post-transplant lymphoproliferative disorder), but can also arise in the primary immunodeficiencies. Typically, EBV LPD presents as a diffuse systemic disease with lymphadenopathy and organ involvement. A rare angiocentric and angiodestructive form of EBV associated B cell LPD, lymphomatoid granulomatosis (LyG), has also been reported in association with WAS. LyG most commonly involves the lung, but can also be seen in brain, kidney, liver, and skin. This report describes the case of a 16 year old boy with WAS who presented with an isolated non-healing ulcerating skin lesion. biopsy revealed an EBV related LPD with the histological features of LyG. This cutaneous lesion responded dramatically to treatment with specific anti-CD20 immunotherapy and the patient remains clinically free of LPD at 18 months.- - - - - - - - - - ranking = 0.025256426099206keywords = brain (Clic here for more details about this article) |
8/35. A case of lymphomatoid granulomatosis/angiocentric immunoproliferative lesion with long clinical course and diffuse brain involvement.lymphomatoid granulomatosis (LYG)/angiocentric immunoproliferative lesions (AIL) consist of angiocentric and angiodestructive lymphoreticular proliferation predominantly involving the lungs and other extranodal sites, such as the central nervous system (CNS). This clinical entity is considered as a B cell process related to Epstein-Barr virus (EBV) infection and EBV positive diffuse large B-cell lymphoma. The CNS is involved in 20% of cases of LYG, but initial involvement is rare. In cases without pulmonary symptoms, diagnosis may be difficult. We report a rare case involving initial progression of CNS symptoms followed by a pulmonary abnormality.A 14-year-old girl suffered from high fever, ataxic gait and paraparesis. MRI revealed diffuse T2 high signals with multiple gadolinium enhancements in the cerebellum, brain stem and cerebral white matter. Her symptoms briefly improved after steroid therapy, but ataxia gradually progressed. dyspnea due to pulmonary interstitial involvement appeared when she was 18 years old. Steroid therapy proved effective for respiratory symptoms. At 20 years old she suffered from disseminated intravascular coagulopathy (DIC) and hemophagocytic syndrome (HPS) with respiratory symptoms and repeated seizures. Her symptoms improved after the administration of cyclophosphamide. Mild hemiparesis and gait disturbance appeared when she was 22 years old. MRI revealed new lesions at the basal ganglia and subcortical white matter, brain atrophy and diffuse T2 high intensity of cerebral white matter. cyclophosphamide was effective and there has been no recurrence of symptoms in the last 5 years. We reviewed the non-tumorous LYG/AIL involving the CNS, and discussed the clinical features, MRI imaging and diagnosis of the LYG/AIL.- - - - - - - - - - ranking = 1.126282130496keywords = central nervous system, nervous system, brain (Clic here for more details about this article) |
9/35. magnetic resonance imaging of lymphomatoid granulomatosis: punctate and linear enhancement preceding hemorrhage.lymphomatoid granulomatosis (LG) is an angiocentric and angiodestructive lymphoproliferative disease that mainly involves the lung. Thirty percent of patients with LG have neurological symptoms. We report serial changes in MRI of a patient with LG of the brain. Postcontrast MRI demonstrated multiple punctate and linear areas that preceded hemorrhage, as indicated by hypointensity with surrounding hyperintensity on subsequent T2-weighted images. The diagnostic value of these lesions observed with contrast MR images is discussed. We consider that enhancing areas correspond to walls of small vessels affected by LG.- - - - - - - - - - ranking = 0.025256426099206keywords = brain (Clic here for more details about this article) |
10/35. Successful treatment of systemic and central nervous system lymphomatoid granulomatosis with rituximab.lymphomatoid granulomatosis (LYG) is a rare lymphoproliferative disorder with a mortality rate approaching 60% in the first year. The median survival is 14 months from the time of diagnosis. Although a variety of chemotherapeutic regimens have been utilized, there is no standard treatment. Studies have shown that in most cases the malignant cells are B-cells, which induce massive infiltration of reactive t-lymphocytes in the background. The disease is therefore considered as a T-cell rich B-cell lymphoproliferative disorder. We report a case of LYG with pulmonary, hepatic, central and peripheral nervous system involvement that was successfully treated with the anti-CD20 (B-cell) monoclonal antibody, Rituximab.- - - - - - - - - - ranking = 4.1502694813934keywords = central nervous system, nervous system (Clic here for more details about this article) |
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