The heavy chain diseases (HCDs) are rare B-cell malignancies that are distinguished by the production of a monoclonal immunoglobulin heavy chain (HC) without an associated light chain by the malignant B-cells. There are three types of HCD defined by the class of immunoglobulin (Ig) HC produced: IgA (alpha-HCD), IgG (gamma-HCD), and IgM (mu-HCD). Alpha-HCD is the most common and occurs most commonly as intestinal malabsorption in a young adult from a country bordering the Mediterranean Sea. Treatment consists of antibiotics and improved nutrition and hygiene. Surgery is occasionally required for patients with bulky masses at risk for bowel perforation. If there is no response to antibiotics or if aggressive non-Hodgkin's lymphoma (NHL) is diagnosed, the patient should be treated with chemotherapy. Gamma- and mu-HCD are rare and essentially are found in patients with a B-cell NHL that produces an abnormal Ig heavy chain. These patients occasionally may be diagnosed with a monoclonal gammopathy of undetermined significance (MGUS). Patients with MGUS with NHL should be administered chemotherapy. Screening the serum and urine of patients with lymphoplasmacytoid NHL would likely identify more patients with gamma- or mu-HCD.
"Alpha heavy chain disease is a rare disorder also known as Mediterranean lymphoma or immunoproliferative small intestine disease . It is a rare malignancy that shows a distinct geographic epidemiology with most cases reported from northern Africa, Israel, and the Middle East in patients of low socioeconomic status and poor nutrition and who live in areas with poor hygiene  . The disorder typically presents in patients < 30 y of age with symptoms that include intestinal malabsorption, diarrhea, weight loss, and abdominal pain . "
[Show abstract][Hide abstract] ABSTRACT: Fibrinogen present in serum specimens can interfere with the interpretation of serum protein electrophoresis. We report here the unexpected precipitation of fibrinogen by an IgA antiserum used in immunofixation electrophoresis.
Immunofixation electrophoresis of plasma combined with ethanol precipitation, serial dilution of plasma, and fibrinogen adsorption of the antiserum were used to investigate the apparent immunoreactivity of a commercial source of IgA antiserum to fibrinogen.
Fibrinogen immunoreactivity by IgA antiserum was observed at fibrinogen concentrations > or =0.93 g/l. Ethanol precipitation of plasma removed fibrinogen and prevented the immunofixation of the protein by the IgA antiserum. Adsorption of the IgA antiserum with human fibrinogen removed its ability to precipitate fibrinogen, demonstrating cross-reactivity between the IgA antiserum and fibrinogen.
Commercial sources of antiserum used for immunofixation electrophoresis may contain antibodies with specificity towards proteins typically absent from serum such as fibrinogen and can produce clinically misleading results.
[Show abstract][Hide abstract] ABSTRACT: Introduction of spectrophotometric detection of serum indices and decision rules to be carried out manually increased the detection rate of relevant hemolysis 6.04- fold. After introduction of the algorithm in the LIS, the detection rate for relevant serum interference was 69.37- fold higher than in the first 1-year period. For icteric samples, a large increase in reported interference was observed: whereas the occurrence of icterus in the sample was not reported at all in the first period, during the second 1-year period, 0.05% of specimens were reported as significantly icteric, causing deviations in the assay results. This number was increased 10-fold (0.54%) in the third period. Interestingly, only one case of lipemia was reported in the first period and none in the second period. Appar- ently, automated spectrophotometric detection together with written instructions in the second 1-year period did not lead to adequate reporting to the clinician. In the third year, 1012-fold more cases of clinically relevant lipemia were reported than in the first year. It should be pointed out here that the evaluation performed and the observed quality improvement depend on the validity of the auto- mated procedure. Summarizing, we believe that this algorithm can be used as a blueprint for processing of test results in general. Every test result is now evaluated automatically in real time against predetermined tolerance limits for the extent of interfering substances, and the algorithm is designed as an almost technician-independent method. We encourage cooperation in this project, and copies of the MISPL algorithm and tolerance tables can be obtained free of charge from the corresponding author.
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