V. Beckers MD

Successful treatment of immunoglobulin light chain amyloidosis with associated factor X deficiency

SUMMARY

Bleeding diathesis in light chain amyloidosis may be due to an acquired coagulation factor deficiency, most commonly factor X deficiency. This report describes the case of an elderly myeloma patient with associated light chain amyloidosis, nephrotic syndrome and factor X deficiency. Treatment with nine cycles of subcutaneous bortezomib + melphalan + prednisone resulted in a complete haematological remission with resolution of the nephrotic syndrome and normalisation of coagulation tests. This case report highlights the importance of performing coagulation screening tests in light chain amyloidosis. In addition, it illustrates that bortezomib-based regimens can induce rapid and complete haematological response with long-term correction of factor X levels.

(BELG J HEMATOL 2017;8(7):272–5)

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