31/97. Waldenstrom's macroglobulinaemia presenting as isolated epistaxis: a common complaint but a rare cause.A patient with isolated severe epistaxis and non-specific history or examination findings was shown to have anaemia, raised erythrocyte sedimentation rate (ESR), renal impairment and raised total protein with hypercoagulable blood. Waldenstrom's macroglobulinaemia was diagnosed after serum electrophoresis and bone marrow studies. Urgent plasma exchange and chemotherapy were undertaken to good effect. This case was a diagnostic dilemma, as the presentation of this common complaint did not initially suggest the rare underlying cause.- - - - - - - - - - ranking = 1keywords = blood (Clic here for more details about this article) |
32/97. t(1;14) and t(11;18) in the differential diagnosis of Waldenstrom's macroglobulinemia.Waldenstrom's macroglobulinemia is caused by several B-cell proliferative disorders including lymphoplasmacytic lymphoma, marginal zone B-cell lymphoma, b-cell chronic lymphocytic leukemia and multiple myeloma. Differential diagnosis between lymphoplasmacytic lymphoma and extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue is particularly difficult as there is a considerable overlap in histological presentation. We report a case of Waldenstrom's macroglobulinemia with involvement of the peripheral blood, bone marrow and stomach. serum chemistry revealed an IgM of 5.4 g/dl, but Bence-Jones protein in urine was negative. Abnormal lymphoid cells were detected in both blood and the bone marrow. flow cytometry of the bone marrow aspirate showed that majority of cells were CD20( ), CD38( ), expressing immunoglobulin lambda light chain, but CD5(-) and CD10(-). Gastric biopsies revealed infiltration of the gastric mucosa by small lymphoid cells showing plasmacytoid differentiation and occasional Dutcher bodies. Lymphoepithelial lesions and helicobacter pylori were not seen. Thus, the differential diagnosis between lymphoplasmacytic lymphoma and mucosa-associated lymphoid tissue lymphoma was raised. To resolve this, we performed BCL10 immunohistochemistry and reverse transcriptional polymerase chain reaction (RT-PCR) for the API2-MALT1 fusion transcript of t(11;18)(q21;q21). Both bone marrow and gastric biopsies showed strong BCL10 nuclear staining, similar to that seen in t(1;14)(p22;q32) positive mucosa-associated lymphoid tissue lymphoma, but absence of the API2-MALT1 fusion transcript. To further ascertain whether the detection of t(1;14)(p22;q32) and t(11;18)(q21;q21) can be reliably used for the differential diagnosis between lymphoplasmacytic lymphoma and mucosa-associated lymphoid tissue lymphoma, we screened for these translocations by BCL10 immunohistochemistry in 58 lymphoplasmacytic lymphomas and RT-PCR for t(11;18)(q21;q21) in 40 lymphoplasmacytic lymphomas, respectively. None of the lymphoplasmacytic lymphomas studied harbored these translocations. Thus, detection of t(1;14)(p22;q32) and t(11;18)(q21;q21) is useful in the differential diagnosis between lymphoplasmacytic lymphoma and mucosa-associated lymphoid tissue lymphoma.- - - - - - - - - - ranking = 2keywords = blood (Clic here for more details about this article) |
33/97. Hyperviscosity syndrome with pulmonary involvement.A 44-year-old male presented with dizziness, blurring of vision, unproductive cough, dyspnoea, heaviness of head, fever and hepatosplenomegaly along with reticulonodular infiltration in chest x-ray. His serum showed monoclonal IgM gammopathy. Peripheral blood smear, bone marrow examination and serum protein electrophoresis suggested it was a case of Waldenstrom's macroglobulinaemia. Chest x-ray and CT scan of thorax were suggestive of pulmonary involvement in the form of interstitial lung disease.- - - - - - - - - - ranking = 128343.37619824keywords = viscosity, blood (Clic here for more details about this article) |
34/97. Protein precipitation as a possible important pitfall in the clinical chemistry analysis of blood samples containing monoclonal immunoglobulins: 2 case reports and a review of the literature.Two case reports are presented, both illustrating an analytical interference caused by monoclonal immunoglobulins. Falsely low results were obtained in the routine analysis of glucose, CRP and HDL-cholesterol. When analysing samples containing paraproteins, various problems can be encountered in the clinical laboratory: next to the antibody effect, pseudohyponatraemia, hyperviscosity, cryoglobulinaemia and gel formation have to be taken into account. In our two cases the interference was caused by paraprotein precipitation, causing an increased turbidity and an apparent increase of light absorbance at every wavelength due to light scattering, including the wavelengths used in the clinical chemistry assays. We review the literature on this sometimes overlooked interference in photometric/turbidimetric assays. This reaction is based on the insolubility of these proteins in specific physico-chemical circumstances in which many variables are involved, among others: pH and ionic strength, presence of preservatives and surfactants in the assays, pI and other specific properties of the monoclonal immunoglobulins. The complexity of the problem makes predicting or preventing this probably infrequent interference usually impossible. This artifact can cause both false positive and false negative results in multiple parameters (e.g. bilirubin, creatinine, iron, urea, uric acid), the most frequently reported analyte being phosphate. The Sia water test (Sia euglobulin precipitation test) can provide a first clue to a paraprotein aggregation; confirmation can be obtained by observing the time/ absorbance curves of the analysis, performing the test manually or setting up a serial dilution of the sample. The problem can be solved by avoiding the presence of the proteins in the assay, performing the analysis using an alternative method or diluting out the interference. Both laboratorians and clinicians should be aware of interferences in the clinical laboratory since the clinical consequences could be important.- - - - - - - - - - ranking = 32089.594049559keywords = viscosity, blood (Clic here for more details about this article) |
35/97. 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 = 64172.188099118keywords = viscosity, blood (Clic here for more details about this article) |
36/97. Amyloid arthropathy and waldenstrom macroglobulinemia.Amyloid arthropathy occurs chiefly as a manifestation of beta(2) microglobulin amyloidosis in patients receiving chronic hemodialysis. AL amyloidosis complicating multiple myeloma is a less common cause. Amyloid arthropathy is exceedingly rare in patients with waldenstrom macroglobulinemia. We report a case characterized by lymphoplasmocytoid malignancy with monoclonal IgM production and amyloid arthropathy manifesting as bilateral symmetric polyarthritis. A synovial membrane biopsy established the diagnosis. Chemotherapy was effective in alleviating the joint manifestations. Joint symptoms in patients with monoclonal gammopathies, including those characterized by IgM secretion, should suggest amyloid arthropathy. Treatment of the blood disease may improve the joint symptoms.- - - - - - - - - - ranking = 1keywords = blood (Clic here for more details about this article) |
37/97. Hyperosmolar hyperglycemic nonketotic coma in Waldenstrom's macroglobulinemia associated with type II diabetes and complicated by pulmonary tuberculosis.In this paper we describe a successfully treated case of hyperosmolar hyperglycemic non ketotic coma (HHNC). The HHNC was observed in a patient affected by Waldenstrom's macroglobulinemia, associated with type II diabetes and complicated by pulmonary tuberculosis. Hyperosmolar hyperglycemic nonketotic coma is a clinical condition with a high mortality rate associated with a severe increase in blood viscosity. This increase in blood viscosity justifies several clinical manifestations of the HHNC. We believe that an increase in the blood viscosity produced by the simultaneous presence of the single diseases mentioned above may have encouraged the development of hyperosmolar coma in the case reported.- - - - - - - - - - ranking = 96259.782148677keywords = viscosity, blood (Clic here for more details about this article) |
38/97. Waldenstrom's macroglobulinemia presenting with spinal cord compression: a case report.Waldenstrom's macroglobulinemia (WM) is a rare lymphoplasmacytic lymphoma characterized by a wide range of clinical presentations related to direct tumor infiltration and the production of IgM. Most commonly it presents with cytopenia, hepatosplenomegaly, lymphadenopathy, constitutional symptoms, and hyperviscosity syndrome. We report a case of WM in an 81-year-old man who initially presented with severe back pain. The patient had no peripheral lymphadenopathy or hepatosplenomegaly and his peripheral blood smear was normal. MRI of the spine revealed an epidural mass causing spinal cord compression at T9. Surgical decompression was performed and pathological analysis of the mass revealed a lymphoproliferative B-cell process. The diagnosis of WM was established after cytomorphologic and immunohistochemical analysis of the patient's bone marrow revealed the presence of a lymphoid/lymphoplasmacytoid-like bone marrow infiltrate along with an elevated serum IgM level. The patient responded both clinically and serologically to local radiotherapy. This case is unusual because the patient lacked all common clinical features of WM. This is the first reported case of epidural spinal cord compression as the initial manifestation of WM, adding to the spectrum of clinical presentations seen in this disease.- - - - - - - - - - ranking = 32086.594049559keywords = viscosity, blood (Clic here for more details about this article) |
39/97. IgM multiple myeloma: report of four cases and review of the literature.The differential diagnosis between multiple myeloma (MM) and Waldenstrom's macroglobulinemia (WM) is generally well defined. Consistent with a diagnosis of MM is the presence of a non-IgM monoclonal gammopathy associated to multiple osteolytic lesions and plasma cell infiltration of the bone marrow. Characteristic of WM is the presence of an IgM monoclonal gammopathy associated to lymphoadenopathy, hepatosplenomegaly, anemia and hyperviscosity syndrome in the presence of a monoclonal lymphoplasmacytoid proliferation in the bone marrow. Nonetheless, few cases of IgM myeloma have been reported that display clinico-pathologic features intermediate between MM and WM. Here, this study describes four of 317 (1.2%) patients with an IgM monoclonal gammopathy in whom the morphologic and clinical features were consistent with a diagnosis of IgM myeloma.- - - - - - - - - - ranking = 32085.594049559keywords = viscosity (Clic here for more details about this article) |
40/97. A new serum lipoprotein associated erythrocyte antigen which reacts with a monoclonal IgM. The stored human red blood cell SHRBC antigen.A monoclonal IgM (IgM) was found to react with stored human red blood cells (SHRBC) and human low density lipoprotein (LDL). IgM Re Fab fragment was reactive. SHRBC antigen was present, in a hidden state, in all fresh human ABO erythrocytes studies; it was not present in fresh or stored animal RBC. Cross reactivity of SHRBC and LDL antigens against IgM Re and IgM Re Fab was demonstrated. In contrast with SHRBC antigen, LDL antigen has no species specificity and was also found in rabbit and rat LDL. Furthermore, cross-reacting soluble inhibitors were also found in human saliva, and in a small molecule fraction issued from human and rabbit serum. The IgM Re also reacted with human and animal glomerular membrane. These findings are suggestive of two closely related but not identical antigens: a human specific SHRBC antigen and a LDL-associated, SHRBC-like antigen, which is a secreted, non-human specific substance. The former is part of the human RBC structure, the latter is a soluble molecule which may be found in saliva and in serum where it may be free or bound to LDL; it also binds to glomerular membranes.- - - - - - - - - - ranking = 5keywords = blood (Clic here for more details about this article) |
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