Factor VII Deficiency in Systemic Primary Amyloidosis: A Rare Case

Abstract

Introduction: Isolated and combined factor deficiencies are known to occur in systemic primary amyloidosis. The most common factor deficiency known in these cases is isolated factor X deficiency. Other factor deficiencies are relatively less frequent. Isolated factor VII deficiency occurs very rarely in cases of systemic primary amyloidosis. Case report: A 58-year-old male patient previously presenting to another health center with complaints of generalized edema, fatigue, and itching had proteinuria and then he was diagnosed with systemic primary amyloidosis after the renal biopsy for proteinuria etiology. The patient's laboratory tests showed prolongation of prothrombin time and factor VII deficiency. The patient responded well to the treatment for primary amyloidosis and factor VII deficiency. Discussion: In cases of systemic primary amyloidosis, if the etiology of prolonged prothrombin time involves no liver disease, warfarin use, or malabsorption, physicians should always keep in mind rare factor deficiencies such as factor VII deficiency, along with common factor deficiencies.

Acquired Isolated Factor VII Deficiency in Plasma Cell Dyscrasias: A Brief Presentation of Two Plasma-Cell-Leukemia-Related Cases and Review of Literature

“…Seven cases of acquired isolated FVII deficiency associated with plasma cell disorders (MM/PCL and/or AL amyloidosis) are reported in the literature [ 12 , 13 , 14 , 15 , 16 ], including the two personal cases here described, and one patient with combined FVII and FX deficiency [ 17 ]. The analysis also includes a patient taken from our case records with AL amyloidosis and isolated PT prolongation, whose abnormality resolved immediately after splenectomy for splenic rupture, but who was not specifically tested for FVII activity.…”

“…FVII deficiency in plasma cell dyscrasias does not appear to be related to the presence and amount of serum and urine monoclonal protein. This condition has been described in one case of light chain MM with no intact monoclonal immunoglobulin production [ 16 ] and in one patient with amyloidosis detected in tissue biopsy samples with no monoclonal band on serum and urine electrophoresis [ 15 ]. Elezovic et al report treatment with plasmapheresis in an attempt to decrease paraprotein concentration in a patient with MM and amyloidosis and life-threatening bleeding, with very limited response in terms of rise in FVII and X levels.…”

“…Treatment options for acquired FVII deficiency in plasma cell disorders are summarized in Table 3. Parenteral vitamin K [15,17] Ineffective. The mechanism of acquired FVII deficiency in plasma cell disorders is largely independent of hepatic synthesis…”