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1/146. lymphomatoid granulomatosis following autologous stem cell transplantation.

    lymphomatoid granulomatosis (LYG) is a rare angio-destructive lymphoproliferative disorder (LPD) of uncertain etiology, with prominent pulmonary involvement. Recent studies indicate that LYG is an Epstein-Barr virus (EBV)-associated B cell LPD with large numbers of background reactive T lymphocytes (T cell-rich B cell lymphoma). Although the disease frequently, but not exclusively, occurs in various immunodeficiency states, it has not been reported in association with the transient immunosuppression following autologous bone marrow/peripheral stem cell transplantation (ABM/PSCT). We describe a patient who developed lymphomatoid granulomatosis of the lung approximately 2 weeks after high-dose chemotherapy and autologous peripheral stem cell transplantation for multiple myeloma. Although molecular studies showed no evidence of EBV genome in the biopsy material, the serologic profile with high IgM titers was suggestive of primary EBV infection. Complete radiologic remission occurred following reconstitution of the patient's immune response after a 2-week course of ganciclovir treatment. Despite the apparently low frequency of LPD (both LYG and EBV-associated post-transplant lymphoma) in the ABMT setting, we believe that it should be considered in the differential diagnosis of patients whose clinical course following ABMT is complicated by fevers, in the absence of an identifiable infectious process.
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2/146. 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.
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3/146. Pulmonary lymphomatoid granulomatosis in a 4 year old.

    The authors report a pulmonary lymphomatoid granulomatosis (LG) in a 4-year-old girl. The clinicopathologic and radiological features of this rare entity are discussed with special emphasis on differential diagnosis, including a brief literature review. LG is an aggressive multiorgan disease that primarily affects the adult lung. There are no specific presumptive clinical and laboratory findings, including tumor markers and imaging techniques, that distinguish LG from other pulmonary nodular lesions. The most important diagnostic aid is to bear this entity in mind when a child presents with pulmonary nodule associated with intractable long-lasting symptoms. Open lung biopsy and total excision is mandatory for the appropriate diagnosis and treatment.
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4/146. 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.
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5/146. 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.
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6/146. 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.
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7/146. 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.
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8/146. lymphomatoid granulomatosis in a boy with long-term follow-up.

    lymphomatoid granulomatosis is an angiocentric lymphoreticular proliferative disease. Some patients go on to develop frank neoplasia. The authors describe a patient who presented at the age of 12 years with pulmonary nodules and hepatosplenomegaly and was followed up for 6 years, and who subsequently developed lymphoma after cessation of therapy.
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9/146. 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.
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keywords = granulomatosis
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10/146. A pediatric case of lymphomatoid granulomatosis with onset after completion of chemotherapy for acute myeloid leukemia.

    In this case report, we present a pediatric case of lymphomatoid granulomatosis (LG) with onset just after the completion of chemotherapy for childhood acute myeloid leukemia (AML). After the completion of maintenance therapy, the patient was admitted to our clinic with a complaint of cough. Radiologic examinations revealed nodular lesions in lungs, liver, and kidney. His bone marrow was in remission. The histopathologic examination of the open lung biopsy was consistent with LG. He received only one cycle of cyclophosphamide and high-dose methyl prednisolone treatment and continued to receive interferon (IFN) alpha-2b therapy for 18 months. This treatment regimen resulted in an excellent response. In conclusion, LG may occur after the treatment of pediatric AML as a rare complication and IFN alpha-2b may be an effective treatment choice in these patients.
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