Menorrhagia due to uterine amyloidosis in familial Mediterranean fever: case-based review
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Rheumatology International https://doi.org/10.1007/s00296-020-04721-2
INTERNATIONAL
CASE BASED REVIEW
Menorrhagia due to uterine amyloidosis in familial Mediterranean fever: case‑based review Döndü Üsküdar Cansu1 · Hava Üsküdar Teke2 · Deniz Arik3 · Cengiz Korkmaz1 Received: 31 August 2020 / Accepted: 1 October 2020 © Springer-Verlag GmbH Germany, part of Springer Nature 2020
Abstract Amyloidosis is described by the deposition of misfolded proteins in the tissues. Amyloidoses are classified into two as systemic and localized. Out of the systemic forms, AL (light chain) amyloidosis is the most prevalent type; however, amyloid A (AA) amyloidosis is more frequently encountered in the rheumatology practice. AA amyloidosis stands out as a major complication of familial Mediterranean fever (FMF). Splenic and renal involvement is more likely in FMF-associated systemic amyloidosis. The involvement of thyroid and adrenal glands has also been described, although infrequently. Amyloidoses have a heterogeneous plethora of clinical manifestations, with certain phenotypes associated with specific amyloid forms. Gynecological amyloidosis is a rare condition. Uterine involvement may occur in a localized fashion or may also arise as a part of systemic involvement, albeit at a lesser ratio. Several cases of uterine AL amyloidosis have been documented so far as an organ involvement in systemic AL amyloidosis. On the other hand, uterine amyloidosis associated with AA amyloidosis has been described merely in one case with rheumatoid arthritis (RA). Here, we presented a 40-year-old female patient with FMF known for 38 years who underwent splenectomy and hysterectomy due to massive splenomegaly, deep anemia, and persistent menometrorrhagia. Histological examinations of materials revealed uterine and splenic AA amyloidosis. This case report is first-of-its-kind to describe FMF-associated uterine AA amyloidosis and also provides a discussion of possible mechanisms of amyloidosis-induced uterine bleeding. Keywords Familial Mediterranean fever · Amyloidosis · Uterine hemorrhage
Introduction
* Döndü Üsküdar Cansu [email protected] Hava Üsküdar Teke [email protected] Deniz Arik [email protected] Cengiz Korkmaz [email protected] 1
Division of Rheumatology, Department of Internal Medicine, School of Medicine, Eskişehir Osmangazi University, 26480 Eskişehir, Turkey
2
Division of Hematology, Department of Internal Medicine, Eskişehir Osmangazi University, Eskişehir, Turkey
3
Division of Pathology, Eskişehir Osmangazi University, Eskişehir, Turkey
The amyloidoses are characterized by deposition of fibrillary proteins called amyloid within the tissue, resulting in multiple organ injuries. Among systemic amyloidoses, AL (light chain) remains to be the most commonly diagnosed type of amyloidosis worldwide. Other clinically significant systemic varieties are AA amyloidosis (formerly known as secondary amyloidosis), leukocyte chemotactic factor 2 amyloidosis (ALECT2), and transthyretin amyloidosis (ATTR). AA amyloido
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