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Biological functions

Biological functions. transport albumin transferin ceruloplasmin haptoglobin oncotic pressure regulation coagulation immunity. Hypoproteinemia. with hypoalbuminemia impairment of liver function protein loss changes in ECF without hypoalbuminemia severe immunoglobulin deficiency.

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Biological functions

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  1. Biological functions • transport • albumin • transferin • ceruloplasmin • haptoglobin • oncotic pressure regulation • coagulation • immunity

  2. Hypoproteinemia • with hypoalbuminemia • impairment of liver function • protein loss • changes in ECF • without hypoalbuminemia • severe immunoglobulin deficiency

  3. Hyperproteinemia • hypergammaglobulinemia • polyclonal • chronic inflammation • chronic liver disease • autoimmune diseases • monoclonal • multiple myeloma • Waldenstrom`s macroglobulinemia • heavy-chains disease • dehydration

  4. Methods of separation • SPE - serum protein electrophoresis • IEP – immunoelectrophoresis • IFE – immunofixation electophoresis

  5. Immunoelecctrophoresis (IEP)

  6. Immunofixation (IFE)

  7. Albumin (migrates to the anode) -1 globulins • -1 protease inhibitor (a-1 antitrypsin) • *-1 glycoprotein (* orosomucoid) •  fetoprotein (if present) • high density lipoprotein (HDL) -2 globulins • -2 macroglobulin • antithrombin III • ceruloplasmin • haptoglobin (this is usually the predominant component) Beta globulins • beta and pre-beta lipoproteins (LDL and VLDL) • C3 • C-reactive protein • hemoglobin (free) • plasminogen • transferrin (*"principal component of the beta1 subdivision") Gamma globulins • Immunoglobulins

  8. Acute phase response (APR) • positive APR • negative APR

  9. beta - gamma (IgA) junction

  10. Multiple myeloma • B cell proliferation • monoclonal protein, Bence-Jones proteinuria • anemia, leukopenia, low platelet count • hypercalcemia • „myeloma kidney” • increased viscosity •  TP, ESR • SPE, IEP, quantitating serum Ig

  11. Heavy chain disease • lymphocytic cell proliferation • only heavy chain production

  12. Benign monoclonal gammopathy • "monoclonal gammapathy of uncertain significance" • "MGUS” • paraprotein < 2.0 gm/dL, Bence-Jones protein (rarely present) < 60 mg/L

  13. Benign monoclonal gammopathy

  14. Case 1 • A 66-year-old man presented with sharp, constant, low back pain, dating from a fall from a ladder 6 weeks earlier. On direct questioning, he did admit to vague malaise for over 6 months. On examination, he was in considerable pain but otherwise seemed fairly fit. • He was mildly anaemic but had no lymphadenopathy and no fever. There were no signs of bruising, no finger clubbing, no hepatosplenomegaly and no abdominal masses

  15. Case 1 • On investigation, his haemoglobin was low (102g/l) due to fewer red cells but his white-cell count was normal (6.2 x 109/l). He had a normal differential white-cell count and a normal platelet count but his ESR was 98mm/h. • Total serum proteins were raised at 98g/l (NR 65-75g/l)

  16. Case 1 • His serum albumin, creatinine and urea were normal. • He had a raised serum calcium level (3.2mmol/l) but a normal alkaline phosphatase. • Serum protein electrophoresis revealed a monoclonal band in the gamma region, with considerable immunosuppression of the rest of this region. • The band was typed by immunoelectrophoresis and shown to be IgG of kappa type.

  17. Quantitation of serum immunoglobulins showed a raised IgG of 67g/l (NR 7.2-19.0g/l), a low IgA of 0.3g/l (NR 0.8-5.0g/l), and a low IgM of 0.2g/l (NR 0.5-2.0g/l). • Electrophoretic examination of concentrated urine showed a monoclonal band in the beta region. On immunoelectrophoresis, this band was composed of free kappa light chains. • X-rays of his back showed a small, punched-out lesion in the second lumbar vertebra

  18. Case • Bone marrow examination showed an increased number of atypical plasma cells; these constituted 45% of the nucleated cells found on the film. This man showed the features required for a diagnosis of multiple myeloma

  19. Case • A 49-year-old woman presented with a 6-month history of vague aches and pains in her chest. On examination, she was overweight but had no abnormal physical signs.

  20. Case 2 • Her haemoglobin was 136g/l with a white-cell count of 6.7 x 109/l and a normal differential. • Her ESR was 34mm/h. • Tests of thyroid function were normal.

  21. Case 2 • However, protein electrophoresis showed a small paraprotein band in the gamma region; this band was an IgG of lambda type. • Her serum IgG was raised at 20.1g/l (NR 7.2-19.0g/l), • with an IgA of 1.9g/l (NR 0.8-5.0g/l) and an IgM of 3.0g/l (NR 0.5-3.0g/l). • electrophoresis of concentrated urine showed no proteinuria. The paraprotein measured 10g/l by densitometry. • A bone marrow examination showed only 12% plasma cells.

  22. Case 2 • the absence of • osteolytic lesions, • monoclonal free light chains in the urine • normal serum IgA and IgM levels, • these findings supported a diagnosis of benign monoclonal gammopathy, also known as a monoclonal gammopathy of unknown significance (MGUS) • This woman has been followed at 6-monthly intervals for 3 years with no change in the paraprotein level, and the urine remains free of monoclonal light chains. She will continue to be seen at yearly intervals.

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