1/7. Immunoblastic sarcoma following Waldenstrom's macroglobulinemia.Immunoblastic sarcoma has been observed in association with, or subsequent to, chronic immune stimulation, connective tissue disorders, and immunoblastic lymphadenopathy. A case of Waldenstrom's macroglobulinemia progressinng after a few years into immunoblastic sarcoma is reported. splenectomy led to disappearance of hemolytic anemia and of pulmonary infiltrates, as well as to marked reduction of macroglobulins. Although immunoblastic sarcoma usually terminates fatally within two or three months, complete remission was induced by combination chemotherapy. It is speculated that Waldenstrom's macroglobulinemia, immunoblastic lymphadenopathy, and immunoblastic sarcoma are related disorders reflecting a clonal nature of immunoblastic lymphoma cells and the plasmacytic cells.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/7. Bilateral, persistent serous macular detachments with Waldenstrom's macroglobulinemia.PURPOSE: Waldenstrom's macroglobulinemia is a rare, malignant lymphoplasmacytic disorder characterized by the monoclonal proliferation of immunoglobulin type M (IgM) producing b-lymphocytes. Ocular manifestations of Waldenstrom's macroglobulinemia have been described in association with the conjunctiva, cornea, uvea, retina, and periocular adnexa. Only rarely have macular findings, particularly serous macular detachments, been described in the presence of monoclonal hypergammaglobulinemias. The majority of reports to date have documented resolution of these serous macular detachments after treatment with blood plasmapheresis. This report presents a case of bilateral, persistent serous macular detachments in the presence of Waldenstrom's macroglobulinemia. CASE REPORT: A 53-year-old black man presented with gradual bilateral reduction in visual acuity over the last 2 months. He revealed having been diagnosed with Waldenstrom's macroglobulinemia approximately 1 month before presentation. He also reported having undergone multiple plasmapheresis treatments in conjunction with systemic chemotherapy over the same period. Funduscopic and fluorescein angiographic examination revealed bilateral, serous macular detachments in the presence of mild venous stasis retinopathy secondary to serum hyperviscosity. Subsequent funduscopic evaluations and serial optical coherence tomography readings confirmed the persistence of the serous detachments despite multiple plasmapheresis treatments. CONCLUSIONS: Although the funduscopic manifestations of excess serum immunoglobulins are typically the sequelae of circulatory stasis, the serum hyperviscosity induced in Waldenstrom's macroglobulinemia has been shown on rare occasions to induce serous macular detachments. Physical elimination of the excess macroglobulins through plasmapheresis is quite effective in resolving the neurosensory detachments induced by Waldenstrom's macroglobulinemia. Only a fraction of neurosensory detachments resulting from Waldenstrom's macroglobulinemia persist despite multiple plasmapheresis treatments. Novel angiographic evidence of retinal pigment epithelium atrophy beneath the area corresponding to the serous detachment provides a plausible explanation for unresponsive nature of this presentation. Identification of this rare variant of the classic angiographically silent macula known to occur with Waldenstrom's macroglobulinemia can aid the clinician in determining a patient's potential response to plasmapheresis and their final visual prognosis.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
3/7. Mononeuritis multiplex in Waldenstrom's macroglobulinemia.We describe a patient with mononeuritis multiplex who had cranial nerve involvement in association with Waldenstrom's macroglobulinemia. sural nerve biopsy showed abundant endoneurial granulofibrillary deposits related to microangiopathic changes. These changes were distributed in a multifocal pattern among the fascicles. immunohistochemistry demonstrated the IgM nature of the deposit located in the endoneurial interstitium.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
4/7. Abnormal liver-spleen scan associated with hyperviscosity syndrome.A patient with hyperviscosity syndrome was noted to have an abnormal liver radionuclide scan. Ultrasound and normalization of the liver scan after plasmapheresis confirmed the non-neoplastic nature of the patchy labeling defects. This case suggests a new cause for labeling abnormalities on liver radionuclide scans in patients with a syndrome where liver biopsies would be unusually hazardous. Possible mechanisms for this scintigraphic pattern in the hyperviscosity syndrome are briefly discussed.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
5/7. Acquired von Willebrand's syndrome in the course of Waldenstrom's disease.Acquired von Willebrand's syndrome with a regressive evolution is described in a 66 year old man with Waldenstrom's disease. An inhibitor electively directed against ristocetin cofactor activity has been demonstrated, active in vitro after incubation at 37 degrees C. serum fractionation showed that the inhibitor was independent of the monoclonal IgM and subsequent purification that it was IgG in nature. The results permit its classification as an auto-antibody.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
6/7. Multiple ocular impairment in a patient affected by Waldenstrom's macroglobulinaemia.BACKGROUND: We studied a bilateral tumefaction of the lacrimal gland in a female patient. METHOD: Ocular and general clinical examinations were carried out. RESULT: Computerized tomography (CT) of the cranial orbit showed a tumefaction of solid density in the lacrimal gland. Histological examination of material removed by needle aspiration revealed the presence of elements of a lymphoplasmacytoid nature. fluorescein angiography showed dilatation of the veins, intraretinal flame haemorrhages and small ischaemic areas. Chest CT showed an increase in the size of the middle and upper mediastinal lymph nodes, and examination of a specimen of bone marrow from the chest revealed the presence of small lymphocytes with a plasmacytoid tendency. CONCLUSION: On the basis of the findings, we diagnosed Waldenstrom's disease with rare multiple ocular impairment (lacrimal gland and retina) in an early stage.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
7/7. Examination of cutaneous macroglobulinosis by immunoelectron microscopy.Cutaneous macroglobulinosis is a rare cutaneous manifestation of Waldenstrom's disease. Lesions are though to result from accumulation of macroglobulin in the dermis and are therefore called IgM storage papules. Ultrastructural findings in the previously reported cases were contradictory and the nature of the deposits was not established by electron microscopy. The purpose of this study was to analyse such deposits by the use of immunoelectron microscopy. A 60-year-old woman had multiple erythematous papules for 1 year. The histopathological changes consisted of plasmocytic infiltration of the dermis and eosinophilic deposits. The skin changes and other investigations led to a diagnosis of Waldenstrom's disease. Samples from normal and diseased skin were analysed by electron microscopy and by immunogold labelling with anti-IgM antibodies, after Lowicryl K4M embedding. An extracellular electron dense granular and filamentous material was observed in the mid- and upper dermis, between and within the collagen bundles. No periodicity was noted and no deposits were seen at the dermoepidermal junction. Immunoelectron microscopy showed a positive labelling located only on these deposits, in both normal-appearing skin and in lesions. In this patient, immunoelectron microscopy clearly demonstrated the presence of large amounts of IgM in the dermis, which were found in the lesions of cutaneous macroglobulinosis and in normal skin. These results suggest that the IgM storage papules result from a greater density of deposits rather than a site-specific accumulation.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |