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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Lymphadenopathy is a common clinical finding and is frequently benign. Warning signs suggestive of a malignant etiology include lymph nodes >2 cm in size, supraclavicular location, and generalized lymphadenopathy associated with hepatosplenomegaly or systemic symptoms. A metastatic solid tumor is always in the differential diagnosis of localized lymphadenopathy, particularly in older individuals. In the case of more generalized lymphadenopathy, in addition to the more common lymphomas, benign etiologies as well as benign and atypical lymphoproliferative disorders need to be considered. Benign etiologies of lymphadenopathy can include infections, autoimmune disorders, drug hypersensitivity reactions, sarcoidosis, and amyloidosis. Rare but benign lymphoproliferative disorders include Kikuchi's disease, Rosai-Dorfman disease, and progressive transformation of germinal centers. Atypical lymphoproliferative disorders that bear close surveillance for evolution to a more aggressive malignancy include Castleman's disease, lymphomatoid granulomatosis, and lymphomatoid papulosis. Previously considered in this category but now classified as a true lymphoma is angioimmunoblastic lymphadenopathy with dysproteinemia. Physicians need to be aware of all of these disorders when evaluating suspicious lymphadenopathy, while also considering the more common lymphomas and leukemias.
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PMID:Clinical mimics of lymphoma. 1695 99

In this atypical case of sarcoidosis with an unusual combination of clinical and laboratory findings, a 32-year-old male presented with a 3-month history of thoracic pain complicated with dyspnea. Laboratory tests, chest radiography, and CT scans of the chest and abdomen revealed eosinophilia of pleural effusion and blood, pleural thickening, hepatosplenomegaly, and bronchiolitis obliterans. In cases such as this, in which pleural fluid eosinophilia is accompanied by peripheral eosinophilia and splenohepatomegaly, underlying malignancies such as Hodgkin lymphoma should be ruled out. A biopsy of the mediastinal lymph nodes suggested noncaseating epithelioid granulomas, characteristic of sarcoidosis. The patient underwent prednisolone therapy for 1 year and is doing well 2 years after initial diagnosis.
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PMID:Eosinophilic pleural effusion, peripheral eosinophilia, pleural thickening, and hepatosplenomegaly in sarcoidosis. 1644 Sep 25

We proceeded to an extensive etiologic search in a young women with a hepatosplenomegaly and a chronic persistent fever. We discuss the differential diagnosis of this situation with a final diagnosis of sarcoidosis.
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PMID:[A 25-year-old woman with fever and hepatosplenomegaly: diagnostic approach]. 1688 26

Sarcoidosis is a granulomatous disease of unknown etiology and is only rarely seen in infants and children. We present the case of a 9-year-old boy who developed sarcoidosis with multi-organ involvement 9 years after cardiac transplantation for Shone complex. The patient was on immunosuppressive therapy with tacrolimus and mycophenolate mofetil. He presented with severe respiratory distress due to marked mediastinal lymphadenopathy and bilateral pulmonary infiltrates in association with fatigue, low-grade fever, hepatosplenomegaly and generalized lymphadenopathy. Lymph node histology showed non-caseating epitheloid cell granulomas and giant cells. Initialization of therapy with prednisolone resulted in prompt clinical recovery and resolution of all symptoms except for the development of mild pulmonary fibrosis. Tapering of the steroids led to recurrence of mediastinal lymphadenopathy 5 months after the initial disease, which responded to an increase in steroid dose. The clinical course, the medical management, and the possible role of immunosuppression in the etiology of the disease are discussed.
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PMID:Systemic sarcoidosis after cardiac transplantation in a 9-year-old child. 1704 40

Sarcoidosis is a multisystem disease characterized by noncaseating granulomas in the affected organs, including skin, heart, nervous system, and joints. Diagnosis of sarcoidosis is generally based upon a compatible history, demonstration of granulomas in at least two different organs, negative staining and culture for acid fast bacilli, absence of occupational or domestic exposure to toxins, and lack of drug-induced disease. Involvement of the hollow organs is rare. Rather than being due to sarcoidosis, some reported mucosal lesions may simply have incidental granulomas. Extrinsic compression from lymphadenopathy can occur throughout the gastrointestinal tract. The stomach, particularly the antrum, is the most common extrahepatic organ to be involved, while the small bowel is the least common. Liver involvement frequently occurs and ranges from asymptomatic incidental granulomas to portal hypertension from granulomas in the portal triad, usually with relatively preserved liver function. CT scans show hepatosplenomegaly and adenopathy, followed in frequency by focal low-attenuation lesions of the liver and spleen. Ascites is usually a transudate from right heart failure (because of pulmonary hypertension) or portal hypertension (because of biliary cirrhosis). Rarely, an exudative ascites may occur from studding of the peritoneum with nodules. Pancreatic involvement presents as a mass, usually in the head or a diffusely firm, nodular organ. Corticosteroids should be instituted when organ function is threatened, usually lungs, eyes, and central nervous system. Their role in the treatment of hepatic sarcoidosis is unclear. The overall prognosis is good although most patients will have some permanent organ impairment. Cardiac and pulmonary diseases are the main causes of death.
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PMID:Gastrointestinal and hepatic manifestations of sarcoidosis. 1936 62

A 62-year-old man had had renal dysfunction and hepatosplenomegaly since 2000. In 2006, he complained of general fatigue and hemodialysis therapy was initiated because his renal function had deteriorated worse. In May 2007, he was admitted to our hospital because his general fatigue took a turn for the worse. He also had hypoxia. A chest radiograph showed no abnormal shadows. A chest computed tomography showed ground glass opacities in both lower lobes slightly. However, 67Ga-citrate scintigraph showed marked accumulation of 67Ga-citrate in the lungs, liver, spleen and kidneys. Transbronchial lung biopsy (TBLB) and bone marrow biopsy showed noncaseating epithelioid cell granulomas, and anti-PAB antibody positive cells were detected in epithelioid cell granulomas in the TBLB specimens. Therefore we diagnosed sarcoidosis. Although we could not perform renal or liver biopsy, we assumed that he had renal and liver sarcoidosis. After oral corticosteroid therapy, his symptoms and image findings improved. We report a rare case of sarcoidosis with hypoxia showing slight ground glass opacities
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PMID:[A case of sarcoidosis with hypoxia showing slight ground glass opacities on chest CT]. 1906 63

Sarcoidosis is a systemic disorder with prominent cutaneous component. Skin lesions are of diverse morphology, of which few are specific for the disease. We describe a 30-year-old woman with polymorphic skin lesions including papules, plaques, and nodules, as well as uncommon variants like eyelid papules, palmar and digital nodules, tattoo sarcoid, as well as scar sarcoid. The patient also had stage II pulmonary sarcoidosis, and articular as well as reticulo-endothelial system involvement manifested by enlarged mediastinal and abdominal lymph nodes and hepatosplenomegaly. The presentation of polymorphic skin lesions with involvement of multiple extra-cutaneous systems is uncommon in a single patient.
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PMID:Mortimer's Malady revisited: a case of polymorphic cutaneous and systemic sarcoidosis. 2065 47

Sarcoidosis is a multisystemic disease with cutaneous lesions present in about one fourth of patients. Cutaneous lesions may be specific or nonspecific based on the presence or the absence of sarcoidal granulomas. Subcutaneos sarcoidosis is the less frequent of the specific cutaneous lesions of sarcoidosis. We report here 2 new cases and review 83 cases reported in literature of subcutaneous sarcoidosis. Subcutaneous sarcoidosis present usually with asymptomatic firm nodules covered by normal-appearing skin, mostly on the forearms and legs. Diagnosis may require a high index of suspicion. In the vast majority of patients, subcutaneous nodules were the manifestation that allowed the diagnosis of systemic sarcoidosis. There is a strong association between subcutaneous sarcoidosis and bilateral hilar lymphadenopathy (72.7%). About 15% of patients have in order of frequency uveitis, parotitis, arthritis, mucositis, dactylitis, neurological and renal involvement, hepatosplenomegaly.
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PMID:Subcutaneous sarcoidosis: report of two cases and review of the literature. 2146 50

Gaucher's disease is not commonly considered in the differential diagnosis of adult patients with hepatosplenomegaly and increased serum ACE. A 19-year-old girl presented with recurrent epigastric and left hypochondrial pain over a period of 9 years, associated with episodes of nausea and diarrhoea. She was extensively investigated and found to have splenomegaly and raised serum ACE. A screen for haematological disorders was negative. She reported an insect bite during an overseas holiday preceding her symptoms. She was therefore also screened for infectious causes of hepatosplenomegaly but without success. Later on in life, she reported joint pain and discomfort. Sarcoidosis was thought to be the putative cause on more than one occasion. However, the presence of splenomegaly and her relatively young age, led the rheumatologist to the correct diagnosis.
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PMID:An ACE diagnosis. 2341 80

A 39-year-old male reported fevers, weight loss, watery loose stools, and decreased visual acuity in his right eye over the prior five years. He was pancytopenic, had an elevated American council on exercise level, total bilirubin, and alkaline phosphatase. Computed tomography revealed massive hepatosplenomegaly and emphysematous lung changes. Liver biopsy showed non caseating granulomas. The patient was diagnosed with extrapulmonary sarcoidosis and was treated with prednisone. The patient symptomatically improved but 5 mo later presented with abdominal pain caused by perforation of the cecum. He underwent a cecectomy and pathology revealed pneumatosis cystoides intestinalis. This represents the first reported association between pneumatosis cystoides intestinalis and sarcoidosis. The etiology of pneumatosis cystoides intestinalis in this case was likely multifactorial and involved both effects of the corticosteroids as well as the advanced nature of the gastrointestinal sarcoidosis. Furthermore this case has the unique features of emphysematous lung changes and pancytopenia which are uncommon with sarcoidosis.
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PMID:Gastrointestinal sarcoidosis associated with pneumatosis cystoides intestinalis. 2346 42


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