1/20. November 1998--70 year old woman with SLE, paraproteinemia and polyneuropathy.A 70 year old woman developed a sensory-motor polyneuropathy 12 years after almost continuous treatment with hydrochloroquine for SLE. sural nerve biopsy disclosed abundant lipid inclusions in schwann cells and vessels against a background of axonal degeneration. After cessation of therapy the patient improved moderately. This case underscores the usefulness of nerve biopsy in the study of management of patients with peripheral neuropathy associated with systemic illnesses.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/20. spleen localization of light chain deposition disease associated with sea blue histiocytosis, revealed by spontaneous rupture.Splenic involvement by a light chain deposition disease (LCDD) associated with sea-blue histiocytosis occurred in a 55-year-old man presenting with LCDD of the kidney without myeloma. Lambda light chain deposits were demonstrated by immunohistochemistry in vessel walls and along the ring fibres of the red pulp sinuses. Accumulation of sea blue histiocytes in the cords was also present. Stiffness of the walls of the red pulp sinuses resulting from light chain deposits may have induced accumulation and destruction of circulating blood cells. Lipid catabolism with production of ceroids may have resulted in lipidic histiocytosis with a sea blue histiocyte pattern.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/20. Acute unilateral corneal immunoprotein deposition in IgM monoclonal gammopathy.A healthy 43-year-old officer of a merchant ship at sea developed pain, redness, and photophobia in his right eye. During the next 2 weeks, he noted the presence of a band of opacity spreading from his temporal limbus toward his central cornea. His episcleral vessels were engorged in a distribution contiguous with the peripheral, sectorial, fleck-like corneal opacities. The opacity had progressed during topical and systemic antibiotic therapy, but halted with use of topical corticosteroids. Systemic evaluation showed mild IgM monoclonal gammopathy. Transmission electron microscopy of a corneal biopsy specimen revealed electron-dense fibrils identified as immunoprotein. To our knowledge, this is the first report of a case of acute unilateral deposition of corneal immunoprotein in a patient with monoclonal gammopathy. Clinicians should begin with a broad differential diagnosis when evaluating patients with corneal opacity.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/20. Immunotactoid-like endoneurial deposits in a patient with monoclonal gammopathy of undetermined significance and neuropathy.An 85-year-old man with a 2-year history of progressive lower limb weakness and paresthesia was found to have an IgG kappa monoclonal gammopathy of undetermined significance (mgus). Clinical and electrophysiological studies revealed a severe distal bilateral symmetrical polyneuropathy. A sural nerve biopsy showed extensive nerve fibre loss with the deposition of large amounts of amorphous material throughout the endoneurium. Electron microscopy showed the deposits to be composed of microtubular structures which were located diffusely throughout the endoneurium. The deposits were also located within the lumina of the vasa nervorum, some of which were undergoing disintegration and rupture with release of the proteinaceous material into the endoneurium. The regions of the nerve in which they appeared most numerous showed more severe nerve fibre damage than other areas. These microtubular structures were also observed in disintegrating vessels and adjacent endoneurium. On immunohistochemistry they stained with antibody to IgG. Identical deposits were found in the dermis in which there was a leucocytoclastic vasculitis. Located in linear arrays within the axons of myelinated and unmyelinated fibres were highly organised tubular structures resembling immunotactoids. Identification of immunotactoid-like structures within the nerve is unique and may be another mechanism by which monoclonal proteins can induce nerve fibre injury.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
5/20. A case of type I cryoglobulinemia associated with a monoclonal gammopathy of undetermined significance (MGUS).A 79-year-old Japanese male who had been suffering from renal insufficiency of unknown causes visited us with reddish papules and purpura on both forearms of a month's duration. A skin biopsy was performed from the right forearm, and the histopathology showed an obstruction of superficial blood vessels with eosinophilic amorphous materials suggesting cryoglobulins. Although further investigations revealed the presence of monoclonal immunoglobulin (Ig) G-kappa type I cryoglobulin, a bone marrow biopsy demonstrated that there was 7.6% proliferation of plasmacytoid cells, and the serum level of Ig G was less than 3 g/dl. The diagnosis was type I cryoglobulinemia associated with monoclonal gammopathy of undetermined significance as the possible cause of chronic renal failure. Therefore, purpuric changes on extremities of the elderly, especially those complaining of renal failure should, be taken seriously and not regarded simply as an age-related phenomenon.- - - - - - - - - - ranking = 18.94131926309keywords = blood vessel, vessel (Clic here for more details about this article) |
6/20. Diffuse dermal angiomatosis: A previously undescribed pattern of immunoglobulin and complement deposits in two cases.Two cases of diffuse dermal angiomatosis are reported in middle-aged women. This rare disease of unknown origin is characterized by increased dermal angiomatosis and ulceration. The clinical and histologic presentations of the presently reported lesions were typical for this disorder. endothelial cells exhibited a normal immunophenotype. The perivascular basement membranes showed a distribution of collagen alpha chains typical for blood vessels, but not for lymphatics. immunohistochemistry revealed other undescribed features. At the site of the clinical lesions, linear and granular deposits of immunoglobulins A and M, and complement were found around the vessels and at the dermal-epidermal junction. The same deposits were also found restricted to the dermal-epidermal junction in the peripheral clinically intact skin. No serological signs of auto-immune disorder were detected in one patient. A monoclonal gammopathy was disclosed in the other patient. A pattern of immunoreactant deposits similar to that disclosed in the two patients was not found in the control specimens, and has not been described so far in other types of vascular hyperplasia and neoplasia. A pathogenic role of these deposits is unsettled and should be further explored.- - - - - - - - - - ranking = 19.94131926309keywords = blood vessel, vessel (Clic here for more details about this article) |
7/20. Urticarial vasculitis associated with a monoclonal IgM gammopathy: Schnitzler's syndrome.We describe two cases of Schnitzler's syndrome presenting with urticarial vasculitis, in which there was a monoclonal IgM of kappa light-chain isotype. skin histology showed a perivascular leucocytic infiltrate and leucocytoclasis. One patient had an abnormal complement profile and positive rheumatoid factor while the other had antibodies to unidentified soluble nuclear antigens, anti-cytoplasmic antibodies and circulating immune complexes. In addition, in the first case deposits of IgM, C3 and, to a lesser degree, of IgG were observed in the walls of small blood vessels.- - - - - - - - - - ranking = 18.94131926309keywords = blood vessel, vessel (Clic here for more details about this article) |
8/20. Polyclonal plasma cell proliferation with systemic capillary hemangiomatosis, endocrine disturbance, and peripheral neuropathy.An autopsy case of so-called plasma cell dyscrasia (PCD) of a 37-year-old woman was reported. This case showed typical clinical symptoms of PCD, except for no abnormality in serum immunoglobulins. Besides the typical histological findings often observed with PCD, the autopsy revealed an additional finding of systemic angiomatous proliferation of small blood vessels throughout the body, especially in the skin, kidneys, brain, various lymph nodes, and soft tissue. A peculiar glomerular lesion in this case, similar to membranoproliferative glomerulonephritis, was likely to be a result of proliferation of glomerular capillaries. In addition, this case showed histological evidence of retroperitoneal fibrosis and osteosclerosis. These findings were also considered to be due to the vascular abnormality. Although the etiology of the vascular lesions is obscure, it is emphasized that the abnormality is probably an important cause of the clinical symptoms seen in PCD.- - - - - - - - - - ranking = 18.94131926309keywords = blood vessel, vessel (Clic here for more details about this article) |
9/20. Immune complex hyperlipidemia induced by an apolipoprotein-reactive immunoglobulin a paraprotein from a patient with multiple myeloma. Characterization of this immunoglobulin.An antibodylike paraprotein has been isolated from a patient with multiple myeloma and autoimmune hyperlipoproteinemia. The paraprotein bound to apolipoprotein B (apo B)-containing lipoproteins that formed macromolecular aggregates, and globules thought to be aggregated complexes of lipoproteins and reactive immunoglobulins were observed circulating within the retinal blood vessels of this patient. This binding specificity permitted purification of the paraprotein from both the agglutinated immune complexes and from the plasma. The protein is an IgA, kappa-immunoglobulin which exists primarily in a polymeric state. Capillary immunoprecipitation demonstrated reactivity with very low density lipoproteins (VLDL) and low density proteins (LDL), but not with high density lipoproteins (HDL). Delipidated apo B and apo E, but not apo A or apo C, formed precipitates with this immunoglobulin. In using a radioimmunoassay format, the affinity of the immunoglobulin was greatest for VLDL and decreases sequentially for intermediate density lipoproteins and LDL. No binding occurred with a dispersion of LDL lipids or with HDL. Deglycosylation did not change the binding to LDL. The apolipoproteins b and E bound with similar affinity, but no binding occurred with apo A-I or apo A-II. Weak binding appeared to occur with apo C. This paraprotein immunoprecipitated apo B-containing lipoproteins from all classes of vertebrates tested. Displacement of the lipids of LDL by Triton X-100 resulted in the formation of an apo B-Triton complex which, however, did not bind to the immunoglobulin; apparently the binding site on apo B was lost. Upon enzymatic digestion with the IgA-specific protease from streptococcus sanguis the immunoglobulin was cleaved into Fc and Fab fragments, and the binding of LDL occurred only with the latter, consistent with the behavior of an immunoglobulin. The immunoreactivity of this paraprotein with apo B and apo E raises the interesting possibility that it may be binding to a site on these apolipoproteins which is reactive with the apo B, E receptor of the plasma membrane, a site which is conserved throughout the vertebrate phylum.- - - - - - - - - - ranking = 18.94131926309keywords = blood vessel, vessel (Clic here for more details about this article) |
10/20. 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) |
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