11/87. Successful therapy of crystalcryoglobulinemia: a case report.Successful therapy for a case of multiple myeloma with a spontaneously crystallizing cryoglobulin of the IgG2-kappa light chain variety was achieved, using both continuous-flow cell centrifugation plasmapheresis to rapidly lower the M component and combination chemotherapy with phenylalanine mustard, prednisone, procarbazine, and vincristine to control the myeloma process. This resulted in resolution of incapacitating large and small necrotic cutaneous ulcerations of the extremities. Physicochemical studies of the crystalcryoprotein demonstrated that cryoprecipitation was rapid and accompanied by the formation of needle-shaped crystals, yet was completely reversible at 37 degrees C. Cryocrit determinations varied depending upon relative centrifugal forces and temperature and did not always relate linearly to the amount of abnormal protein, thus making these alone unreliable in assessing response to therapy.- - - - - - - - - - ranking = 1keywords = lower (Clic here for more details about this article) |
12/87. multiple myeloma in association with sarcoidosis.The association of sarcoidosis with hodgkin disease and non-Hodgkin lymphoma is well known. However, multiple myeloma also can occur rarely in association with sarcoidosis. We describe a patient with sarcoidosis who subsequently developed multiple myeloma. The patient was a 49-year-old woman with a 4-year history of severe, chronic, active sarcoidosis involving her lungs, lymph nodes, eyes, and bone marrow. During the initial clinical workup, a serum monoclonal paraprotein was detected and bone marrow examination revealed a slight increase in plasma cells (4%), in addition to noncaseating granulomas. Thus, the diagnoses of monoclonal gammopathy of undetermined significance and sarcoidosis were established simultaneously. She sought medical attention for her current illness when she developed low back pain and weakness of her lower extremities. serum protein electrophoresis and immunofixation revealed a monoclonal paraprotein, immunoglobulin (Ig) G kappa type, and quantification revealed an IgG level of 46.67 g/L (normal, 5.88--15.73 g/L). Bone marrow aspiration and biopsy revealed multiple myeloma and sarcoidosis. Including this patient, 11 cases of sarcoidosis and multiple myeloma have been reported to date, including 3 patients with monoclonal gammopathy of undetermined significance preceding the onset of multiple myeloma. In this case, as in most of the cases reported previously, sarcoidosis preceded the development of multiple myeloma.- - - - - - - - - - ranking = 1keywords = lower (Clic here for more details about this article) |
13/87. Meningeal relapse after double peripheral blood stem cell transplantation in IgD myeloma.A 54-year-old man diagnosed with IgD myeloma (stage IIIA) in complete remission (CR) received peripheral blood stem cell transplantation (PBSCT) twice with an interval of 4 months using high-dose melphalan 200mg/m2. However 9 months after the second PBSCT, he was readmitted because of lumbago, lower left hemiparesis, speech disturbance and left facial nerve palsy. A lumbar puncture revealed myeloma cells in the cerebrospinal fluid (CSF). The patient did not respond to any salvage chemotherapy and died of sepsis 27 months after the initial diagnosis. The findings in this patient suggest that another treatment modality including prophylactic intrathecal injection of an anti-cancer drug as well as allogeneic cell therapy is probably necessary in patients with high-risk IgD myeloma.- - - - - - - - - - ranking = 1keywords = lower (Clic here for more details about this article) |
14/87. Myeloma with xanthoderma due to an IgG lambdamonoclonal anti-flavin antibody.When yellow skin and yellow hair developed in an elderly patient with multiple myeloma, we ruled out the usual causes of such pigmentation but identified a monoclonal IgGlambda (lgGGar) with anti-flavin antibody activity. Purified IgGGar was bright yellow, and the acid-dissociated chromophore was identified as riboflavin by chromatography and absorption spectroscopy. Native IgGGar contained 1.45 moles of flavin per mole of IgG, and increased to 2 moles with addition of riboflavin to saturation. The flavin was localized to the Fab fragment and was bound to IgGGar with high affinity. IgGGar showed strongest affinities for riboflavin, flavin mononucleotide and flavin adenine dinucleotide, and lower affinities for dinitrophenyl derivatives and naphthoquinone. The demonstration of hapten bound to the circulating monoclonal immunoglobulin in this case suggests the possibility of bound but colorless haptens on other myeloma proteins as well as on normal immunoglobulins.- - - - - - - - - - ranking = 1keywords = lower (Clic here for more details about this article) |
15/87. gallium-67 demonstration of extensive soft-tissue involvement of multiple myeloma.A 46-year-old woman with multiple myeloma presented with neurological symptoms in the right upper extremity. After MR imaging of the cervical spine failed to show the cause of her symptoms, Ga-67 scintigraphy demonstrated increased uptake in multiple areas including the right supraclavicular region and bilateral lower extremities. histology of the specimen obtained from the left thigh proved soft-tissue involvement of myeloma, and extensive extramedullary involvement was diagnosed. radiotherapy to the right supraclavicular mass relieved her symptoms. Although Ga-67 scintigraphy is generally considered of limited value in multiple myeloma, this modality contributed to the development of an appropriate strategy in this patient with extensive extramedullary involvement.- - - - - - - - - - ranking = 1keywords = lower (Clic here for more details about this article) |
16/87. poems syndrome--a case report.poems syndrome is a multisystem disorder associated with polyneuropathy, organomegaly, endocrinopathy, a monoclonal protein (M-protein), and skin changes. The authors describe a patient with poems syndrome who had osteosclerotic myeloma confirmed by open bone biopsy. magnetic resonance imaging (MRI) showed discrete lesions of low signal intensity in both T1 and T2-weighted images. This patient is now being successfully treated with melphalan and prednisone with much improvement in skin thickening and sensory change in the lower extremities.- - - - - - - - - - ranking = 1keywords = lower (Clic here for more details about this article) |
17/87. multiple myeloma with tadpolelike cells and rosette formation.A 65-year-old man with lower back pain was found to have multiple myeloma. Unique findings in the marrow aspirate smears of this patient were tadpolelike plasma cells and cellular rosettes simulating those seen in neuroblastoma. This case documents yet another facet of the broad morphologic spectrum of neoplastic plasma cells in multiple myeloma.- - - - - - - - - - ranking = 1keywords = lower (Clic here for more details about this article) |
18/87. Heat insoluble cryoglobulin associated with gangrene in multiple myeloma.cryoglobulins are immunoglobulins that have tendency to precipitate in temperatures below 37 degrees C and dissolve with rewarming. Monoclonal cryoglobulins are usually associated with a distinct hematological disorder and often are asymptomatic. Heat insoluble cryoglobulin has been described with sjogren's syndrome and glomerulonephritis but, not with multiple myeloma. Severe sensitivity to cold occurs with high thermal insolubility of the cryoprotein, with dramatic symptoms when exposed to minimal lowering of the temperature. We report a case of a 49 year old man with multiple myeloma and an unusual type I cryoglobulin that caused occlusive gangrene. The cryoglobulin appeared as a milky white precipitate that was resistant to re-suspension and did not dissolve at 37 degrees C. immunoelectrophoresis of the cryoglobulin, which dissolved at 56 degrees C, showed it to be composed of a monoclonal IgG kappa protein (3.5 g/dl). Unlike most high thermal insoluble cryoglobulin, cold associated symptoms were not seen. In addition to steroids, plasmapheresis was initiated thrice a week with albumin fluid replacement. plasmapheresis caused a marked decline in cryocrit levels from 21% to less than 0.5% in 9 days after 4 procedures with resolution of the gangrene of the feet and after 6 treatments, vasculitic symptoms improved dramatically. The cryoglobulin test was negative 2 weeks after initiation of treatment. The patient was treated for the myeloma and there was no recurrence of occlusive symptoms. Proper laboratory procedure and careful examination and handling of cryoglobulinemic samples facilitate detection of unusual cryoglobulins. This is a unique report of multiple myeloma with gangrene of lower extremities that has a heat insoluble cryoglobulin.- - - - - - - - - - ranking = 2keywords = lower (Clic here for more details about this article) |
19/87. Cutaneous plasmablastic lymphoma in an hiv-positive male: an unrecognized cutaneous manifestation.Plasmablastic lymphoma (PBL) is a rare and relatively new entity originally described in hiv-infected individuals. This subset of Epstein-Barr-virus (EBV)-related non-Hodgkin lymphomas is now regarded as a distinct clinicopathological category of AIDS-associated lymphomas occurring preferentially in the oral cavity and showing a poor prognosis. We describe for the first time an EBV-associated PBL with an isolated cutaneous distribution on the lower extremities in an hiv-infected heterosexual male and point to the unique clinical, morphological and immunophenotypic characteristics of this lymphoma. The patient presented with fast growing solid and livid nodules on both legs. The large, blastic tumor cells showed the following immunophenotype: CD138 , CD45 , CD20-, CD10-, CD3-, CD30-, bcl-2-, bcl-6-, LMP-1- and EMA-. The proliferation fraction (Mib-1) was >90%. EBV association was demonstrated by in situ hybridization (EBV-encoded RNAs 1/2). Polymerase-chain-reaction-based dna analysis demonstrated a clonal IgH rearrangement in the absence of a bcl-2/IgH translocation. PBL in hiv patients may occur not only in the oral cavity, but can probably involve any other organs including the skin.- - - - - - - - - - ranking = 1keywords = lower (Clic here for more details about this article) |
20/87. Polyneuropathy with osteosclerotic myeloma--poems syndrome. A case report.A rare form of plasma cell dyscrasia characterized by associated polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes has been termed the poems syndrome. The pathophysiology is unknown; plasma cell dyscrasia is essential; secondary manifestations are unexplained. We report a 67-year-old man with a 7-month history of progressive weakness and numbness of the legs. Clinical examination revealed sensorimotor polyneuropathy, predominantly affecting the lower extremities, hepatomegaly, and skin haemangiomas. Additional investigations disclosed IgG-lambda monoclonal serum protein, endocrine abnormalities, elevated cerebrospinal fluid protein level and an osteoblastic lesion of the lumbar vertebra. biopsy of the osteosclerotic vertebra showed a marked lymphoplasmocytic infiltrate. MRI of the liver disclosed two haemangiomas; this association has not been reported previously.- - - - - - - - - - ranking = 1keywords = lower (Clic here for more details about this article) |
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