Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A patient with retroperitoneal and axillary lymphadenopathy and splenomegaly was demonstrated histologically to have the hyaline vascular type of giant lymph node hyperplasia, with plasma cell infiltrates in each region. The abdominal lesions were not surgically resectable and did not respond to radiotherapy. The clinical findings included polyclonal gammopathy, high cold agglutinin titers, neuropathy, and bilateral papilledema. All of these abnormalities have persisted three years since the initial diagnosis.
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PMID:Multicentric giant lymph node hyperplasia. 61 17

A 39-year-old woman presented with polyneuropathy, hepatomegaly, splenomegaly, endocrinopathy, monoclonal protein and skin changes, several of the many clinical features of the recently described POEMS syndrome. In addition, she had a Castleman's disease (angiofollicular lymph node hyperplasia). In this case ascites was a main presenting feature. Thus, the POEMS syndrome must be added to the list of rare causes of ascites. Electron microscopy of the liver showed perisinusoidal fibrosis.
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PMID:[Unusual cause of ascites: the POEMS syndrome]. 222 42

The multicentric variant of Castleman's disease (MCCD) is associated with a rapidly progressive and fatal course. The case described herein manifested unique clinical and histological features. Initial presentation as isolated splenomegaly was subsequently followed by widespread organ involvement, including lymph nodes and bone marrow. In spite of this, the patient had a very benign course of her disease. The case serves to expand even further the already wide clinical spectrum of Castleman's disease.
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PMID:A benign course of multicentric Castleman's disease with involvement of the spleen and bone marrow. 369 64

Two homosexual men with the acquired immunodeficiency syndrome (AIDS) who developed a multicentric variant of angiofollicular lymph node hyperplasia (AFLNH) (Castleman's disease) and Kaposi's sarcoma are reported. Both had diffuse adenopathy, splenomegaly, and a systemic inflammatory state. Both had an absolute increase in Leu 1+ lymphocytes, which was associated with markedly decreased Leu 3+ lymphocytes, markedly increased Leu-2+ lymphocytes, and a very low Leu 3/2 ratio. The lymphocytes of both patients had a normal blastogenic response to PHA. The lymphocytes of patient 1 had a poor response to autologous or allogenic cells in the mixed lymphocyte culture reaction. AFLNH represents another lymphoreticular complication of AIDS. Given the interrelationships between AFLNH, the development of Kaposi's sarcoma, and the aggressive clinical course seen in our two patients and those in the literature, the aggressive use of lymph node biopsy may be an important prognostic tool for the patient with the acquired immunodeficiency syndrome.
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PMID:Multicentric angiofollicular lymph node hyperplasia (Castleman's disease) followed by Kaposi's sarcoma in two homosexual males with the acquired immunodeficiency syndrome (AIDS). 387 3

Fifteen patients (11 males, four females; median age 57) manifested a disease characterized by (1) the histopathologic features of Castleman's disease, plasma cell type, in lymph node biopsies; (2) predominantly lymphadenopathic disease, involving multiple, preferentially peripheral nodal groups; (3) varied manifestations of multisystemic involvement (such as constitutional symptoms; splenomegaly and hypergammaglobulinemia; elevated ESR, anemia, and thrombocytopenia; hepatomegaly and altered liver function tests (LFTs); signs of renal disease); and (4) idiopathic nature. Two main patterns of evolution were recognized: persistent, with sustained clinical manifestations, and episodic, with recurrent exacerbations and remissions. Seventy-three percent of patients had infectious complications, and 27% developed malignancies. Complete remissions were obtained occasionally with antineoplastic agents and with splenectomy but not with glucocorticosteroids alone. The median survival time is 30 months; 60% of patients have died. Median follow-up in the six surviving patients is 97+ months. A review of 50 cases in the literature revealed similar clinical and laboratory features. Despite some similarities with autoimmune diseases, the main features of this process seem to best fit a hyperplastic-dysplastic lymphoid disorder in a setting of immunoregulatory deficit.
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PMID:A systemic lymphoproliferative disorder with morphologic features of Castleman's disease: clinical findings and clinicopathologic correlations in 15 patients. 403 67

A 21-month-old boy presented with a papular rash, lymphoadenopathy, and splenomegaly. He developed symmetric polyarthritis, fever, and progressive glomerulonephritis. Serologies for viral agents including HIV were negative. Antinuclear antibody was transiently positive, but no anti-DNA antibodies were present. CH50 and serum C3 values were low. Biopsies of skin, kidney, bone marrow, and lymph node were obtained. There was a perivascular and periadnexal lymphocytic infiltrate in the skin, with a normal epidermis. Renal biopsy showed proliferative mesangial glomerulonephritis. Bone marrow showed an increased number of plasma cells. Lymph node showed histologic changes described in multicentric Castleman's disease including marked follicular hyperplasia, vascular proliferation, and interfollicular expansion with numerous plasma cells. IL-6 mRNA was demonstrated in cells in the marginal zone and interfollicular regions of the node by in situ hybridization. Likewise, the serum IL-6 level was elevated during a clinical exacerbation of the patient's nephritis. These data suggest an underlying lymphoproliferative disorder, such as Castleman's disease, with overproduction of IL-6 resulting in systemic features of the disease, including glomerulonephritis.
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PMID:Increased interleukin-6 (IL-6) production in a young child with clinical and pathologic features of multicentric Castleman's disease. 788 66

Castleman's disease (CD) is a enigmatic lymphoid disease of unknown etiology which rarely manifest itself as an isolated pelvic mass. We report a case of pelvic Castleman's disease masquerading as a uterine myoma. The patient presented symptoms related to compression of adjacent structures, splenomegaly and abdominal lymphadenopathy, the laboratory data revealed positive Epstein-Barr virus serology, elevated beta 2-microglobulin level and presence of antinuclear antibodies. The intraabdominal involvement, histological patterns and clinical forms of this condition are reviewed. Likewise etiopathogenic, radiologic and therapeutic aspects related with this entity are discussed. We suggest that pelvic Castleman's disease should be included in the differential diagnosis of females presenting a pelvic mass containing calcifications.
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PMID:[Pelvic Castleman's disease: apropos of a case]. 875 12

Castleman's disease is a lymphoproliferative disorder thought to be related to deregulated production of IL-6. We have previously shown that mice lacking the trans-acting factor C/EBP beta, a transcriptional regulator of IL-6 and a mediator of IL-6 intracellular signaling, develop a pathology nearly identical to multicentric Castleman's disease, together with increasingly high levels of circulating IL-6. We describe here how the simultaneous inactivation of both IL-6 and C/EBP beta genes prevents the development of pathological traits of Castleman's disease observed in C/EBP beta-deficient mice. Histological and phenotypic analysis of lymph nodes and spleen of double mutant mice did not show either the lymphoadenopathy and splenomegaly or the abnormal expansion of myeloid, B and plasma cell compartments observed in C/EBP beta-/- mice, while B cell development, although delayed, was normal. Our data demonstrate that IL-6 is essential for the development of multicentric Castleman's disease in C/EBP beta-/- mice.
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PMID:Inactivation of the IL-6 gene prevents development of multicentric Castleman's disease in C/EBP beta-deficient mice. 887 30

Multicentric Castleman's disease (MCD), also called multicentric angiofollicular lymphoid hyperplasia, is a systemic lymphoproliferative disorder causing fever, lymphadenopathy and splenomegaly. Recently, Kaposi's sarcoma-associated herpesvirus/human herpesvirus 8 (KSHV/HHV-8) DNA sequences have been detected in cases of MCD. We examined HHV-8 DNA sequences in the peripheral blood mononuclear cells (PBMCs) of two HIV-negative patients with MCD and in PBMCs and the lymph node of a HIV-negative patient with localized Castleman's disease (LCD) by the polymerase chain reaction. The novel sequences were detected in all DNA samples. Furthermore, the sequences were detected in only the CD19+ B-lymphocyte fraction of the patient with LCD as previously reported. However, the sequences were detected in CD19+ B-lymphocyte and CD2+ T-lymphocyte fractions of two patients with MCD. These results suggest that HHV-8 has tropisms for both B lymphocytes and T lymphocytes in Castleman's disease.
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PMID:Tropism of human herpesvirus 8 for peripheral blood lymphocytes in patients with Castleman's disease. 943 23

A 62 year-old man had fever, splenomegaly, systemic lymphadenopathy and palpable petechiae. Laboratory studies showed leukocytosis (10,200/microliters) with plasma cells (23%) and polyclonal gammopathy. Southern blot analysis of peripheral blood mononuclear cells showed no rearrangement of immunoglobulin genes, indicating polyclonal plasmacytosis in peripheral blood. A diagnosis of Castleman's disease was made by the cervical lymph node biopsy, which showed onion-skin structures in the germinal centers and plasmacytosis in the interfollicular region. A skin biopsy of the purpura lesion disclosed leukocytoclastic vasculitis. This is an unusual case of Castleman's disease presenting plasmacytosis in peripheral blood and leukocytoclastic vasculitis.
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PMID:[Polyclonal plasmacytosis in peripheral blood and leukocytoclastic vasculitis in Castleman's disease]. 957 45


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