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PubMed · 730728

Primary systemic amyloidosis presenting as scleroderma: (a case report).

C J Mistry·U L Wagholikar·A K Deshpande·S V Chitale
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AmyloidosisDiagnosis, DifferentialHumansMaleMiddle AgedScleroderma, SystemicCase ReportsJournal Article

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C J Mistry, U L Wagholikar, A K Deshpande, S V Chitale. 1978. Primary systemic amyloidosis presenting as scleroderma: (a case report).. https://pubmed.ncbi.nlm.nih.gov/730728/

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PubMed1992

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.

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