Systemic Amyloidosis in a Patient With Familial Mediterranean Fever and Hodgkin Lymphoma: A Case Report


Demir F., BAHADIR A., AYDIN MUNGAN S., ÇOBANOĞLU Ü., KALYONCU M.

JOURNAL OF PEDIATRIC HEMATOLOGY ONCOLOGY, vol.42, no.3, pp.234-237, 2020 (Peer-Reviewed Journal) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 42 Issue: 3
  • Publication Date: 2020
  • Doi Number: 10.1097/mph.0000000000001504
  • Journal Name: JOURNAL OF PEDIATRIC HEMATOLOGY ONCOLOGY
  • Journal Indexes: Science Citation Index Expanded, Scopus, CAB Abstracts, EMBASE, MEDLINE
  • Page Numbers: pp.234-237
  • Keywords: familial Mediterranean fever, Hodgkin lymphoma, MEFV, systemic amyloidosis, COLCHICINE, TURKEY, FMF

Abstract

Systemic amyloidosis is a clinical manifestation of the accumulation of amyloid fibrils in tissues because of persistent acute phase elevation and chronic inflammation. Its most common causes are inflammatory diseases and malignancies. Here, we present a 12-year-old girl diagnosed with systemic amyloidosis and Hodgkin lymphoma (HL) who was also previously diagnosed with familial Mediterranean fever (FMF). Despite colchicine treatment for FMF, the patient had a persistent elevation of acute phase reactants and AA-type amyloid deposits were observed in a kidney biopsy. Anakinra, an interleukin-1 antagonist, was added to the treatment. Shortly after the diagnosis of amyloidosis, mediastinal lymphadenopathy was recognized, and she was also diagnosed with HL. A chemotherapy protocol of doxorubicin, bleomycin, vinblastine, and dacarbazine was initiated. After 6 cycles of the chemotherapy and 8 months of the anakinra treatment, no recurrence or residual malignancy was observed and proteinuria was decreased. To the authors' knowledge, this is the first reported case of systemic amyloidosis in the literature associated with both FMF and HL.