1/10. Pulmonary cystic disorder related to light chain deposition disease.light chain deposition disease (LCDD) is a rare disorder that very uncommonly affects the lung. We report three cases of severe cystic pulmonary LCDD leading to lung transplantation. Such a presentation has never been previously reported. The three patients present with a progressive obstructive pulmonary pattern associated with numerous cysts diffusely distributed in both lungs. The disease was histologically characterized by non-amyloid amorphous deposits in the alveolar walls, the small airways and the vessels. It was associated with emphysematous-like changes and small airway dilation. Monotypic kappa light chain fixation was demonstrated on the abnormal deposits and along the basement membranes. Electron microscopy revealed coarsely granular electron-dense deposits in the same localizations. Mild extrapulmonary deposits were found in salivary glands in one patient. No immunoproliferative disorder was identified. We conclude that LCDD may primarily affect the lung, present as a pulmonary cystic disorder, and lead to severe respiratory insufficiency.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/10. Uncommon type of meningiomas with conspicuous plasmo-lymphocytic infiltration. An immunohistochemical and histochemical study.Two cases of meningioma revealing conspicuous plasmo-lymphocytic tissue and hyalinized fibrous tissue components are reported. Histopathological examination of the plasmo-lymphocytic infiltration was performed. Both lesions showed polyclonality of plasma cells as revealed by positive reactions for IgG and paraimmunoglobulin kappa- and lambda-light chains, and amyloid infiltration into the fibrous stroma and blood vessel walls. The histochemical and immunohistochemical characteristics of the lesion in relation to its etiology are briefly discussed.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/10. Angiomatoid malignant fibrous histiocytoma with extensive lymphadenopathy simulating Castleman's disease.We report the association in a 10-year-old boy of an angiomatoid malignant fibrous histiocytoma (AMFH) of the left thigh with ipsilateral inguinal, pelvic and extensive retroperitoneal lymphadenopathy, and severe systemic manifestations. These include growth retardation, fever, severe anemia, hypergammaglobinemia, and hypoalbuminemia. At ultrastructural level the tumor was characterized by an abundance of myofibroblasts, occasional histiocytes, and small vessels with marked reduplication of the basal lamina. Biopsies of the inguinal and abdominal lymph nodes showed follicular hyperplasia and massive plasmacytosis indistinguishable from Castleman's disease (giant lymph node hyperplasia) of plasma cell type. The radical surgical excision of the primary tumor in the thigh resulted in the disappearance of the abdominal lymphadenopathy and a marked reduction in size of the pelvic lymph nodes with marked decrease of the gammaglobulins, thus proving that the nodal lesions were the expression of a reactive process to the tumor rather than a coincidental independent lymphoproliferative disorder. Retroperitoneal and pelvic node dissection was performed 1 year after the radical excision of the thigh tumor because of persistent pelvic lymphadenopathy and failure of serum immunoglobulins M and A to return to normal level, with a recent peak of IgA to twofolds that of normal value. Metastatic AMFH was found in the three pelvic nodes. One month postoperatively IgA returned to near normal level whereas IgM remained slightly elevated.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/10. light chain cardiomyopathy associated with small-vessel disease.Intramyocardial small-vessel disease associated with systemic light chain deposition is a rare condition that may occur in patients with monoclonal plasma cell proliferation. This article describes a 39-year-old woman who had experienced several episodes of subendocardial myocardial infarction and was found to have plasma cell dyscrasia. Endomyocardial biopsy revealed kappa light chain deposits along the sarcolemmal and vascular basement membranes, the latter of which resulted in vascular occlusion and myocardial infarction. Postmortem examination showed polyvisceral deposition of kappa light chains. This rare complication of plasma cell proliferative process has a poor prognosis.- - - - - - - - - - ranking = 5keywords = vessel (Clic here for more details about this article) |
5/10. Chronic progressive sensory ataxic neuropathy with polyclonal gammopathy and disseminated focal perivascular cellular infiltrations.One autopsied case of chronic progressive sensory-ataxic neuropathy with polyclonal elevation of serum and CSF IgG and IgA and without malignancy is reported. A marked loss of large myelinated fibers was universal in both the central and peripheral rami of primary sensory neurons. Fiber loss showed a multifocal patchy pattern in the proximal nerve trunks. The posterior root ganglion cell bodies were moderately atrophic and loss of large cells was observed. Unmyelinated axons were well preserved. The ventral spinal roots, ventral spinal horn cells, and muscles showed minimal involvement. There were focal perivascular mononuclear inflammatory cells without necrotizing vasculitis around the endoneurial and epineurial vessels. Similar perivascular cellular invasions were observed in the visceral organs, occasionally forming germinal follicle centers. This case suggested that this neuropathy has a unique background with a possible immune-mediated basis.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
6/10. association of systemic light-chain deposition disease and amyloidosis: a report of three patients with renal involvement.Three patients with renal involvement, plasma cell dyscrasia and systemic light chain deposition are reported in whom well characterized amyloid deposits were also found in the vessel walls. This association, not yet reported, is probably more frequent than believed and still brings nearer these two manifestations of monoclonal light chain deposition. Whether or not the finding of amyloid deposits during systemic light chain deposition is a separate entity and modifies the prognosis remains to be answered.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
7/10. Primary cardiovascular amyloidosis with benign monoclonal gammopathy.A 54-year-old woman is reported whose primary amyloidosis was diagnosed at autopsy. Amyloid deposits were found in the myocardium, the striated muscles, the smooth muscle layers of the gut and the wall of the blood vessels. The deposits showed resistance to induced proteolysis. A large number of mature plasma cells was demonstrated in the bone marrow, and immunocytochemical studies revealed a considerable increase in the proportion of plasma cells which were positive for kappa light chains of immunoglobulins, indicating a monoclonal gammopathy. This view was strongly supported by the unexpected finding that amyloid deposits were positive for kappa light chains. The relationship between the kappa positive reaction of amyloid and its resistance to induced proteolysis are discussed.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
8/10. Ocular pathology in light chain deposition disease.light-chain deposition disease (LCDD), a rare form of monoclonal gammopathy, is characterized by deposits of amorphous light-chain material, mainly in the kidneys but also in various other organs. Here we present the first report of a light-, electron microscopic and immunohistochemical study of the globes of a patient suffering from LCDD secondary to multiple myeloma. Massive deposits of kappa light chains similar to those typically present in the kidneys were found beneath the basement membrane of the ciliary pigment epithelium, on vessels of the ciliary body, within the collagenous zones of Bruch's membrane, and in the innermost part of the choroid. The choriocapillaris in the macular area was partly obstructed by these deposits, and an exudative retinal detachment was present. Whether this detachment was the consequence of disturbed circulation of the choriocapillaris remains speculative.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
9/10. Proteinaceous (angiocentric sclerosing) lymphadenopathy: a polyclonal systemic, nonamyloid deposition disorder.Proteinaceous lymphadenopathy with hypergammaglobulinemia (PLWH) is an exceedingly rare disease of unknown etiology. Described primarily as a pathologic entity, relatively little is known about its clinical manifestations or its response to therapy. The disease is often referred to and treated as an unusual form of plasma cell dyscrasia or light chain deposition disease. We have recently encountered a young patient with PLWH who presented with generalized lymphadenopathy, marked liver function abnormalities, hypocomplementemia, cryoglobulinemia, decreased T4/T8 ratio, and ophthalmopathy. Contrary to the notion that PLWH is a clonal disorder, we found no evidence of clonality in this patient. The most characteristic finding in this and in another patient, previously seen at our institution, was marked angiocentric hyaline sclerosis of the small and mid-sized blood vessels of involved lymph nodes and organs. Based on these findings, we propose the term angiocentric sclerosing lymphadenopathy, which more accurately defines this clinicopathologic entity that appears to be distinct from light chain deposition disease and other plasma cell dyscrasias.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
10/10. sjogren's syndrome plasma cell panniculitis and hidradenitis.We present a 42-year-old woman with primary sjogren's syndrome and a polyclonal gammopathy who presented with pretibial petechiae, purpura, and tender indurated plaques. The indurated plaques revealed a lobular plasma cell panniculitis, and thus sjogren's syndrome should be added to the short list of collagen vascular diseases that can present as plasma cell panniculitis. Her biopsies also demonstrated intense perieccrine plasma cell infiltrates that may account for sjogren's syndrome-associated hypohidrosis. We also observed occasional vascular occlusion of vessels with an amorphous, eosinophilic material possibly related to her hypergammaglobulinemic purpura.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
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