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Systemic Amyloidosis in a Patient With Familial Mediterranean Fever and Hodgkin Lymphoma

A Case Report

Demir, Ferhat MD*; Bahadir, Ayşenur MD; Mungan, Sevdegül MD; Çobanoğlu, Ümit MD; Kalyoncu, Mukaddes MD*

Journal of Pediatric Hematology/Oncology: May 13, 2019 - Volume Publish Ahead of Print - Issue - p
doi: 10.1097/MPH.0000000000001504
Clinical and Laboratory Observations: PDF Only

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.

Departments of *Pediatric Rheumatology

Pediatric Hematology and Oncology

Pathology, Faculty of Medicine, Karadeniz Technical University, Trabzon, Turkey

The authors declare no conflict of interest.

Reprints: Ferhat Demir, MD, Department of Pediatric Rheumatology, Faculty of Medicine, Karadeniz Technical University, Trabzon 61187, Turkey (e-mail:

Received August 5, 2018

Accepted April 1, 2019

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