1/13. Waldenstrom's macroglobulinaemia presenting as reticulate purpura and bullae in a patient with hepatitis b virus infection.Cutaneous manifestations of Waldenstrom's macroglobulinaemia (WM) include purpura, ulcers, urticaria, leukocytoclastic vasculitis, and immunobullous dermatoses. No association has been reported previously of WM and hepatitis b virus (HBV) infection. A 40-year-old female HBV carrier was admitted to hospital because of generalized oedema, oliguria, haematuria, hypertension, fever and blood-tinged sputum. Cutaneous manifestations included generalized petechiae, palpable purpura mainly on the legs, multiple necrotic ulcerations and gangrenous changes on the toes, and necrotic, giant confluent reticulate purpura on the trunk surmounted by several tense bullae. Laboratory investigations revealed monoclonal gammopathy of IgM kappa type (6.7 g/L), membranoproliferative glomerulonephritis associated with HBV infection, Bence Jones proteinuria, and an increased number of abnormal plasmacytoid cells in the bone marrow. Pathologic examination demonstrated immune complex-mediated vasculitis with deposits of IgM in the walls of dermal vessels and secondary subepidermal bulla formation. HBV infection may have caused WM or modified the clinical course in this fatal case.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/13. Initial increase in the cryoglobulin level after rituximab therapy for type II cryoglobulinemia secondary to waldenstrom macroglobulinemia does not indicate failure of response.Type II (mixed) cryoglobulinemia is a systemic vasculitis that may affect many organs, including the skin, leading to ulcerations secondary to immune complex deposition. We report a patient who presented with a large necrotic ulcerative lesion on the right ankle secondary to type II cryoglobulinemic vasculitis due to waldenstrom macroglobulinemia that was resistant to multiple modalities of therapy. amputation to prevent further necrosis and infections was almost performed. Treatment with rituximab was initiated and the patient had a dramatic response. This case report highlights the effectiveness and prompt response to rituximab when used in patients with small vessel vasculitis and skin ulceration. We describe a rise in the cryoglobulin level that may occur initially after rituximab therapy.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/13. Cutaneous infiltration with waldenstrom macroglobulinemia.A 59-year-old Japanese man was diagnosed as Waldenstrom macroglobulinema. With impaired general performance and a 2-year history of pruritic eruptions that were initially confined to the forearms, but later involved the face, limbs, and trunk. A skin biopsy that was performed on the forehead showed infiltration with abnormal cells of the dermis around the central vessels. PCR analysis of the skin biopsy showed immunoglobulin heavy chain rearrangement. As the rearrangement band occurred at the same site as that of the bone marrow aspiration, skin infiltration with abnormal cells was proven.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/13. Bilateral central retinal vein occlusion in Waldenstrom's macroglobulinemia.Waldenstrom's macroglobulinemia is a rare progressive neoplastic plasma cell disorder. Hyperviscosity is a major component in primary macroglobulinemia due to the excessive production of IgM paraprotein, a tumor by-product. The ocular manifestations of hyperviscosity can include congestion of the conjunctival vessels, dilation, segmentation and tortuosity of retinal vessels, retinal hemorrhages and serous retinal detachment. This case of bilateral central retinal vein occlusion represents an uncommon complication of Waldenstrom's macroglobulinemia.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
5/13. Mid-peripheral hemorrhages secondary to Waldenstrom's macroglobulinemia.Waldenstrom's macroglobulinemia is a progressive neoplastic syndrome of the reticuloendothelial system and is characterized by the presence of monoclonal IgM paraproteins. The ocular manifestations of macroglobulinemia can include mid-peripheral hemorrhages, sludging of blood in the conjunctival vessels, blurred disc margins, exudates in the fundus and serous retinal detachment. Waldenstrom's macroglobulinemia and its ocular complications are herein presented.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
6/13. Primary macroglobulinemia presenting as multiple ulcers of the legs.Multiple ulcers developed on the lower legs of a 67-year-old man suffering from macroglobulinemia. Histological and direct immunofluorescence studies on biopsy specimens taken from the lesions revealed dilation of capillaries, deposition of large quantities of IgM in the vessels, and deposition of C3 in the vessel walls. A marked increase in blood Clq binding immune complexes was also noted. These findings suggest immune complex-induced ulceration. The fact that IgM-type cryoglobulins were positive implies that cryoglobulins derived from macroglobulins may play an important causative role in ulceration.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
7/13. Leukoencephalopathy in Waldenstrom's macroglobulinemia. Immunohistochemical and electron microscopic observations.The clinical and pathologic features of a case of Waldenstrom's macroglobulinemia with leukoencephalopathy are reported. Multiple cerebral foci of demyelination, accompanied to a lesser extent by axonal degeneration, were associated with perivascular infiltrates of plasmacytoid lymphocytes and with permeation of the white matter by macroglobulins. Immunohistochemical studies demonstrated a predominance of IgM kappa within the blood plasma, in cerebral blood vessel walls, in the foci of demyelination, and within perivascular histiocytes. Electron microscopy disclosed the presence, in macrophages and pericytes, of membrane-bound cytoplasmic inclusions consisting of tubular arrays, suggestive of cryoglobulin deposits. We hypothesize that the high serum levels of macroglobulins accompanied by lymphoplasmocytic infiltrates may, either by way of viscosity-related ischemia, or by a direct toxic effect, have caused abnormal vascular permeability, infiltration of the cerebral parenchyma by paraproteins, and, ultimately, focal degeneration of the white matter.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
8/13. Immunofluorescent studies of the eye in Waldenstrom's macroglobulinemia.The eyes of a 53-year-old woman with K light chain macroglobulinemia were studied histopathologically. Retinal hemorrhages were observed on gross examination. Microscopic examination disclosed eosinophilic, PAS-positive deposits in cystoid spaces within the outer plexiform layer, in the inner retina, and about photoreceptors. Immunofluorescent studies showed that the deposits contained IgM. Aneurysmal dilations of small retina vessels were demonstrated as well.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
9/13. Deposits of paraprotein in small vessels as a cause of skin ulcers in Waldenstrom's macroglobulinemia.A 71-year-old man presented with epistaxis and large ulcers on arms and legs. A monoclonal IgM-kappa gammopathy and high levels of cryoglobulins were found. histology of the affected skin showed deposits of paraprotein in the small vessels, causing luminal obstruction. This in turn caused ischemic skin lesions, which were successfully treated with cortisone (fluocortolone 100 mg/day every second day) and cyclophosphamide (100 mg/day).- - - - - - - - - - ranking = 5keywords = vessel (Clic here for more details about this article) |
10/13. Cardiac paraprotein associated with Waldenstrom's macroglobulinemia: a case report.A case of myocardial paraprotein deposition in a patient with Waldenstrom's macroglobulinemia is presented. Routine light microscopy revealed diffuse widening of interstitial regions by pale eosinophilic material that had a grayish cast on trichrome stains and was congo red negative. Immunofluorescence showed strong immunoglobulin m and kappa light chain staining around blood vessels but predominantly around myocytes. Ultrastructural examination revealed massive deposition of flocculent electron-dense material around myocytes and occasionally around blood vessels.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
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