Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0042384 (vasculitis)
 20,525 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two cases of hepatic follicular dendritic cell (FDC) tumor are described. Both patients were female, aged 57 and 51 years. They presented with epigastralgia or abdominal fullness and weight loss. The first patient refused surgical resection. She developed progressive polyclonal gammopathy and then bilateral purpura over the legs. Skin biopsy revealed leukocytoclastic vasculitis with granular vascular deposits of IgA and C3. The second patient had marked peripheral blood and tissue eosinophilia. The histological diagnosis was confirmed by positive staining for CD21 and CD23. The stromal lymphocytes were predominantly composed of CD3(+)and CD8(+) cells. In situ hybridization for EBER showed a positive nuclear signal in tumor cells but not in inflammatory cells. Polymerase chain reaction amplification for Exon 3 of the latent membrane protein-1 (LMP-1) gene showed a characteristic 30-bp deletion between nucleotides 168282 and 168253, corresponding to the B95-8 sequence. The unique clinicopathological features of our cases have not been reported for FDC tumors before. The clinical significance of the 30-bp deletion in Exon 3 of the LMP-1 gene in FDC tumor of the liver warrants further investigation.
 ...
PMID:Follicular dendritic cell tumor of the liver: a clinicopathologic and Epstein-Barr virus study of two cases. 1130 53

A 50-year-old man presented with an asymptomatic, 1.5 x 1.5 cm, dark-brown noduloplaque with a rubbery consistency (Fig. 1) on the lateral aspect of the left lower leg of uncertain duration. His general condition was healthy, and he did not recall any trauma or insect bite at this site. No similar skin lesions were found elsewhere and no lymphadenopathy was observed. The lesion revealed a nonencapsulated, but well-circumscribed, deep dermal nodule with several lymphoid aggregates and germinal center-like structures within the tumor and also at the periphery, when examined microscopically at scanning power (Fig. 2a). The epidermis showed no remarkable changes, except for basal hyperpigmentation. At higher power, a mixed inflammatory infiltrate composed of histiocytes, foamy histiocytes (Fig. 2b), lymphocytes, and abundant plasma cells (Fig. 2c) with Russell bodies was revealed. The stroma contained mainly hyalinized and sclerotic collagen fibers (Fig. 2d). Prominent venules were noted, especially in the sclerotic areas, and some were surrounded by dense collagen fibers. No vasculitis or emperipolesis was found. No foreign materials were observed by polarization microscopy, and no organisms could be identified by periodic acid-Schiff (PAS), Grocott methenamine silver (GMS), Giemsa, Gram, acid-fast, or fite stains. The results of testing for infection by Epstein-Barr virus (EBV) (latent membrane protein 1, LMP-1) were negative. No spindle cells were found in the lesion. Immunohistochemical studies demonstrated mature plasma cells stained with CD138, and polyclonality was confirmed by the expression of both kappa and lambda light chains. The germinal center-like lymphoid aggregates were found to be B cells, which reacted positively with CD20. Scarce S100-positive cells and even rarer CD1a-positive cells were detected. Test results for smooth muscle actin (SMA) and anaplastic lymphoma kinase (ALK) were negative. Abundant CD68+ macrophages were observed within the lesion (Fig. 3a), and about 50-75% of the inflammatory cells were found to express cyclooxygenase-2 (COX-2) (Fig. 3b). The patient's condition was diagnosed as cutaneous plasma cell granuloma (CPCG). One year after excision, no evidence of recurrence was observed.
 ...
PMID:Cutaneous plasma cell granuloma: report of a case with novel histologic and immunohistochemical findings. 1933 29