PubMed · 15470225
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Joel G Schwab, Herbert T Abelson. 2004-10-07. Hemoglobin C disease.. https://doi.org/10.1056/nejm200410073511520
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Monoclonal gammopathies of undetermined significance constitute a body of asymptomatic diseases characterized by a selective increase of a single serum immunoglobulin due to an only dysregulated clone of B lymphocytes. They occur in up to 10% of persons over 80 years of age. The diagnosis is suggested in presence of both M component in serum <30 g/L and bone marrow plasma cells <10% by the exclusion of multiple myeloma or lymphoproliferative disorders. In most MGUS, however, populations of normal and myelomatous plasma cells coexist in a stable ratio. The overall relative risk for progression is 7 times the expected rate, depending from the disease and the type of M component.
Diffuse pulmonary hemorrhage denotes a diffuse bleeding into the alveoli as a result of severe damage of the alveolocapillary membrane. Autoimmune diseases, toxic injury and hemodynamic changes are the most frequent causes. A 45 year old male patient presented with diffuse alveolar hemorrhage. An Immunoglobulin A (IgA) paraprotein secreting myeloma was found to be the underlying cause. Immunohistochemistry revealed dense pericapillary and perivascular deposits of IgA, indicating a paraprotein mediated damage of the alveolocapillary membrane. The predominantly vascular pattern of damage was regarded as the most likely cause of the pulmonary hypertension in this patient. The diffuse pulmonary bleeding stopped after initiation of treatment consisting of vincristine, adriamycine and dexamethasone.
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