Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Kaposi's sarcoma-associated herpesvirus (KSHV), which was originally detected in Kaposi's sarcoma, also has been found in primary effusion lymphomas (PELs) and some cases of multicentric Castleman's disease. We describe two transplant recipients who developed Kaposi's sarcoma and a spectrum of non-neoplastic lymphoproliferative disorders that show pronounced plasmacytic and plasmacytoid features. The first patient had recurrent pleural effusions and Castleman's disease-like changes in lymph nodes. The second patient had systemic lymphadenopathy and hepatosplenomegaly secondary to diffuse infiltration by polyclonal plasma cells and plasmacytoid B lymphocytes that clinically mimicked Epstein-Barr virus (EBV)-associated posttransplant lymphoproliferative disease. In both cases, KSHV DNA was detected by polymerase chain reaction and Southern blotting, and KSHV vIL-6 protein expression was identified in affected tissues by immunohistochemical localization. In contrast, no evidence of KSHV coinfection was detected in any of 31 EBV-related posttransplant lymphoproliferative disorders or 112 non-PEL lymphomas tested. The pathologic findings in these two patients were not representative of malignancy by morphologic, immunophenotypic, or molecular criteria. This study underscores the marked propensity for hematolymphoid proliferations associated with KSHV infections to show plasmacytic features. Additionally, this study describes use of an antibody reactive against KSHV vIL-6 that can readily detect a subpopulation of KSHV-infected hematopoietic cells.
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PMID:Posttransplantation plasmacytic proliferations related to Kaposi's sarcoma-associated herpesvirus. 1089 31

A previously healthy 18-year-old boy presented with daily spiking fever, polyarthritis, and evanescent skin rashes, as well as hepatomegaly and Raynaud's phenomena for 2 months. He was initially diagnosed with adult-onset Still's disease (AOSD). During the period of follow-up, intermittent fever and migratory polyarthritis persisted and an insidiously growing mass over the right axillary region was noted 1 year after the diagnosis of AOSD. Excisional biopsy of the mass revealed a group of lymph nodes with histological features of the hyaline vascular type of Castleman's disease. The patient's symptoms disappeared soon after excision of the lymph nodes. evanescent rash, lymphadenopathy, hepatosplenomegaly and serositis [5]. A clinical picture compatible with the diagnosis of AOSD has not been described in the localised hyaline vascular type of Castleman's disease. We report such a case in an 18-year-old male patient who presented prolonged fever and polyarthritis with an initial diagnosis of AOSD. The diagnosis of hyaline vascular type of Castleman's disease was made 1 year later, when the patient developed an insidiously growing mass over the right axilla.
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PMID:Localised hyaline vascular type of Castleman's disease mimicking adult-onset Still's disease. 1063 75

Castleman disease (CD; angiofollicular lymphoid hyperplasia) is a heterogeneous group of lymphoproliferative disorders of uncertain cause. Three histologic variants (hyaline vascular, plasma cell, and mixed) and two clinical types (localized and multicentric) of CD have been described. We report 5 cases of CD treated in our institute and review the literature about the management of this relatively rare disorder. Localized and multicentric CD may be different clinical disorders with overlapping histologic features. Localized disease generally presented with a single enlarged lymph node or widening of the mediastinum, whereas multicentric disease is a systemic lymphoproliferative disorder characterized by lymphadenopathy, hepatosplenomegaly, constitutional symptoms, anemia, hypoalbuminemia, and hypergammaglobulinemia. Unlike the localized type, for which surgical excision is curative regardless of the histologic type, multicentric disease often necessitates aggressive systemic therapy and portends a poorer outcome.
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PMID:The heterogeneity of Castleman disease: report of five cases and review of the literature. 1106 58

POEMS syndrome is characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal protein and skin changes. This polyneuropathy belongs to the chronic inflammatory demyelinating polyradiculoneuropathies (CIDP) group. Sporadic reports have emphasized the clinical association between CIDP and central nervous system (CNS) demyelination, but as far as we know not in the context of POEMS syndrome. We report the case of a 67 years old patient who developed POEMS syndrome associated to Castleman's disease, characterized by demyelinating polyradiculoneuropathy, hepatosplenomegaly, mediastinal and supraclavicular adenopathies and monoclonal IgG lambda gammapathy. Cranial magnetic resonance imaging disclosed diffuse alteration of the cerebral white matter highly suggestive of demyelination. CNS demyelination can be another feature of POEMS syndrome. Cranial MRI should be performed in patients with POEMS syndrome in order to verify this new feature.
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PMID:[POEMS Syndrome with central and peripheric nervous system demyelination]. 1461 50

A 52-year-old Japanese man presented with fever spikes, generalized fatigue, anorexia, and anasarca. The patient was referred for the evaluation of fever of unknown origin in association with swelling of cervical, axillary, and inguinal lymph nodes. He also manifested nephrotic syndrome, acute renal failure, hepatosplenomegaly, massive pleural effusion, ascites, disseminated intravascular coagulation, and hypergammaglobulinemia. C-reactive protein was positive and plasma vascular endothelial cell-derived growth factor (VEGF) and serum interleukin-6 levels were markedly elevated. Lymph node biopsy results showed that findings were compatible with Castleman's disease of hyaline vascular type associated with interfollicular plasmacytosis. In conjunction with the clinical findings, a diagnosis of multicentric Castleman's disease was made. The patient underwent renal biopsy because of nephrotic syndrome, and the results showed proliferation of mesangial cells, lobulation of glomeruli, and tram track pattern of the capillary wall without immune complex deposition. Electron microscopy showed widening of the subendothelial space. No electron-dense deposits were present in both mesangial and subendothelial regions. Pathologic features were compatible with glomerular microangiopathy and membranoproliferative glomerulonephritis-like lesions. With corticosteroid therapy, systemic symptoms disappeared; both VEGF and interleukin-6 levels were normalized, and he went into complete remission of nephrotic syndrome. In this article, the role VEGF plays in the pathogenesis of nephrotic syndrome and glomerular microangiopathy is discussed.
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PMID:Multicentric Castleman's disease associated with glomerular microangiopathy and MPGN-like lesion: does vascular endothelial cell-derived growth factor play causative or protective roles in renal injury? 1471 66

Castleman's disease is a rare lymphoproliferative disorder of unknown aetiology. The presentation is varied, diagnosis is difficult, and optimum management is still unknown. We report our experience with a case of Castleman's disease in a 34-year old woman who presented with pallor, hepatosplenomegaly, and a right iliac fossa mass that was 5 cm in diameter. this was initially diagnosed as a soft tissue sarcoma and preoperative tumour embolization was planned before excision. Mesenteric arteriogram revealed that the feeder arteries arose from the superior mesenteric artery and embolization was aborted for fear of causing bowel ischaemia. On laparotomy, lymphoid enlargement was found between the leaves of the jejunal mesentery. The tumour was relatively avascular and the overlying mesenteric vessels contributed to teh duplex ultrasound and computerized tomography appearance of hypervascularity. The tumour with the mesentery and the overlying segment of jejunum was excised completely. Histopathology confirmed Castleman's disease. The purpose of this report is to present this rare case that caused a diagnostic dilemma and to review the management of this disorder.
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PMID:Localized Castleman's disease presenting as a vascular right iliac fossa mass. 1471 17

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

Several lymphoproliferative disorders may be interpreted as multicentric Castleman's disease (MCD) clinicopathologically. These include HIV infection, autoimmune-disease-associated lymphadenopathy, idiopathic plasmacytic lymphadenopathy with polyclonal hyperimmunoglobulinemia, "idiopathic MCD", POEMS syndrome (polyneuropathy, anasarca, organomegaly, endocrinopathy, M-proteins, and skin lesions), and non-Hodgkin's lymphomas. Among these, idiopathic MCD appears to be relatively rare. We report on the clinicopathologic and immunohistologic findings of five cases of idiopathic MCD and discuss the problems regarding their differential diagnosis. Some of the characteristic clinical findings of POEMS syndrome, including hepatosplenomegaly, skin change, endocrine abnormalities and anasarca, were present in all five cases. However, during the course of disease, minimal diagnostic criteria for POEMS syndrome, i.e., monoclonal plasma cell proliferation and sensory motor neuropathys, were absent in all five cases. The serum interleukin-6 level and the vascular endothelial growth factor level were found to be elevated in two of the cases examined. Various autoantibodies were detected in three cases. However, none of them fulfilled the diagnostic criteria for any of the definite autoimmune-disease. Histologically, three lesions exhibited a mixed type of Castleman's disease, and two exhibited the hyaline-vascular type. The majority of the germinal centers were of the hyaline-vascular or epithelioid germinal type, with a few hyperplastic germinal centers. The interfollicular area was characterized by prominent vascularity. Moderate to large sheets of plasma cells were observed in three mixed type cases. The polytypic nature of B-lymphocytes was demonstrated by immunohistochemistry and polymerase chain reaction. Immunohistochemical study demonstrated that the majority of germinal centers exhibited a tight/concentric pattern of FDC network. Few CD57-positive T-cells were observed in the hyaline-vascular and epithelioid follicles. The lack of CD57-positive T-cells appears to be related to the formation of abnormal germinal centers in the MCD.
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PMID:Idiopathic multicentric Castleman's disease. A clinicopathologic and immunohistochemical study of five cases. 1599 40

Castleman's disease is a rare disorder of unknown etiology and different clinical manifestations, occurring in young adults (usually prior to age 30 years). In this case report we present one such case who was a 16 years old male with abdominal pain, weight loss, cervical lymph adenopathy and hepatosplenomegaly. Lymph node biopsy revealed hyaline-vascular type of Castleman's disease. He was put on chemotherapy but was lost to follow-up.
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PMID:Castleman's disease -- a rare disease of lymph nodes. 1644 98

The Castleman Disease, also known as angiofollicular lymph node hyperplasia is a rare disorder that results in abnormal proliferation of B-lymphocytes and plasma cells in lymphoid organs. It was first described as a pathologic entity in 1954 and later defined by Castleman et al. in 1956. The Castleman's disease is characterised by lymph node hyperplasia with vascular proliferation and endothelial hyperplasia. On the basis of histologic criteria it has been divided into hyaline-vascular and plasma cell variant. The hyaline-vascular type (90 % of cases), generally benign, occurs in younger patients with a localised mediastinal mass - surgical excision is curative. The plasma cell variant (10 % of cases), localised or multicentric, is generally aggressive, and occurs in older patients with generalised lymphadenopathy, hepatosplenomegaly, systemic symptoms, anaemia, elevated inflammatory indices, and a polyclonal rise in immunoglobulins. The prognosis is poor despite the Chemotherapy. Surgery is not indicated. We present two cases of unicentric Castleman Tumor of hyaline-vascular type. The patients were cured by surgical resection.
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PMID:[The angiofollicular lymph node hyperplasia Castleman--casuistic and review of the literature]. 1658 3


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