Recurrent, isolated factor X deficiency in myeloma: repeated normalization of factor X levels after cytostatic chemotherapy followed by late treatment failure associated with the development of systemic amyloidosis.
We describe the case of a 64-year-old woman with isolated severe factor X deficiency associated with kappa light chain myeloma. At the time of diagnosis there was no evidence for amyloidosis. Complete remission (CR) of myeloma as well as normalization of factor X levels were achieved after cytostatic chemotherapy. Subsequently, factor X deficiency recurred twice without any evidence for relapse of myeloma. The first time factor X normalized again following cytostatic treatment, the second time, however, factor X deficiency was refractory to chemotherapy. Finally, relapse of myeloma became evident associated with rapidly progressing, systemic amyloidosis, which was fatal within a few months. Initially, factor X infusion studies showed a normal recovery, but when amyloidosis became overt the recovery decreased to 0%. We assume that factor X deficiency was due to a binding of factor X to kappa light chains associated with the proliferation of the malignant myeloma cell clone.
Schwarzinger
I
I
1st Medical Department, University of Vienna, Austria.
Stain-Kos
M
M
Bettelheim
P
P
Pabinger
I
I
Kyrle
P
P
Kalhs
P
P
Kapiotis
S
S
Jäger
U
U
Lechner
K
K
D000686:Amyloidosis / Q000209:etiology*; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D001780:Blood Coagulation Tests; D005170:Factor X / Q000008:administration & dosage / Q000378:metabolism* / Q000493:pharmacokinetics; D005171:Factor X Deficiency / Q000150:complications / Q000188:drug therapy*; D005260:Female; D006207:Half-Life; D006801:Humans; D007262:Infusions, Intravenous; D008875:Middle Aged; D009101:Multiple Myeloma / Q000097:blood / Q000150:complications / Q000188:drug therapy*; D009364:Neoplasm Recurrence, Local / Q000150:complications; D012008:Recurrence; D012074:Remission Induction / Q000379:methods; D013997:Time Factors
D000970:Antineoplastic Agents; D005170:Factor X