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Maria Stella Graziani

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Martina Zaninotto

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Ferruccio Ceriotti
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Giuseppe Agosta

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Chiara Riva
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ISSN print: 0393 – 0564
ISSN digital: 0392- 7091

BC: Articoli scritti da M. Martelloni

Disfibrinogenemia indotta da una catena leggera libera kappa delle immunoglobuline
Ig-free light chains induced dysfibrinogenemia
<p>Despite several pathological conditions are associated with free&nbsp;light chains (FLC) deposition in human tissues, only few cases of human diseases caused by the specific binding&nbsp;activity of monoclonal FLC are described. A 65-year old male patient, with highly abnormal functional coagulation&nbsp;tests and undetectable functional fibrinogen was admitted to the Hematological Clinic of the University Hospital of&nbsp;Pisa. The same tests were within the reference intervals one year before. After excluding a number of causes for&nbsp;abnormal coagulation tests, we focused on potential causes of acquired dysfibrinogenemia. Due to the presence of&nbsp;abnormal values of FLC, we performed an immunofixation: while serum did not show any detectable monoclonal&nbsp;band, the immunofixation of a plasma sample revealed the presence of monoclonal FLC of kappa type co-migrating&nbsp;with fibrinogen. The serum kappa FLC concentrations were much lower than plasma levels, suggesting that the&nbsp;majority of these FLC were bound to fibrinogen, remaining associated to fibrin after clotting. Bone marrow biopsy&nbsp;showed 4% monoclonal plasma cells producing kappa light chains. The patient was diagnosed as affected by a FLC&nbsp;MGUS. After two courses of dexamethasone, the plasma concentration of kappa FLC decreased substantially and&nbsp;most of the coagulation tests normalized. The nature of the interaction between fibrinogen and kappa FLC is currently&nbsp;under investigation to elucidate the mechanism able to inhibit fibrinogen polymerization.</p>
Biochimica Clinica ; 39(5) e16-e18
Casi clinici - Case report