Uterine amyloidosis in menopause

Abstract

A 78 year old woman was admitted to our hospital because of postmenopausal bleeding. Cervical dilatation and uterine curettage appeared to be impossible due to a very narrow vaginal introitus after an anterior colporrhaphy. Transrectal ultrasonography revealed highly reflective scattering densities of a punctate nature (Fig. 1). As bleeding persisted and endometrial pathology could not be excluded, an abdominal hysterectomy was performed. During a difficult operation a crumbly irregular uterus, bleeding on every touch, was removed accompanied by bilateral salpingo-oophorectomy. Total blood loss during the procedure amounted to 4 L. Apart from blood transfusion the post-operative course was uneventful.The surgical specimen consisted of a 88 g uterus with frayed and crumbly ovaries and fallopian tubes. Histologically, the endometrium was atrophic, no hyperplasia or carcinoma was found (endometrial thickness < I mm). However, massive deposits of amorphous, eosinophilic substance were found in the myometrial stroma with encroachment on smooth muscle cells and local atrophy, and in vessel walls of the uterine corpus and fallopian tubes (Fig. 2). The left ovary was normal. The right ovary could not be identified. Some deposits had elicited a foreign body giant cell reaction and metaplastic bone formation. Congo red stains imparted a pinkish colour to the deposits and showed green birefringence with cross-polarised light consistent with amyloid. The amyloid deposits were found to be resistant to pretreatment with potassium permanganate. Immunostaining with antibodies to serum amyloid A protein, prealbumin and p 2 microglobulin were negative. These observations are consistent with immunoglobulin light chain-derived (AL) amyloidosis. Subsequent extensive haematological analysis did not reveal a plasma cell dyscrasia or any other B-cell neoplasm. Repeated urinanalyses for Bence Jones proteins were negative and serum immunoelectrophoresis and quantitative immunoglobulins findings were normal. Thor-

Menorrhagia due to uterine amyloidosis in familial Mediterranean fever: case-based review

“…We believe that platelet dysfunction along with uterine amyloid deposition (amyloid angiopathy) was the major risk factor for bleeding in our case. Jongen et al claim that uterine amyloidosis, despite infrequent, must be included in the differential diagnosis of postmenopausal bleeding [18]. Nevertheless, our patient was of premenopausal age.…”

“…Two of the AL amyloidosis patients had multiple myeloma and two had primary amyloidosis. Moreover, localized primary amyloidosis formerly reported in one patient was exclusively localized in the uterus [18]. (Causes of uterine amyloidosis according to the literature are summarized in Table 2.)…”

“…According to Copeland et al, amyloid infiltration of corpus uteri compromises uterine contractions, eventually promoting prolonged bleeding events during menstrual periods [11]. Jongen et al also proposed that amyloidosis-related bleeding diathesis is most probably caused by increased fragility and impaired contractility of the vasculature (amyloid angiopathy) due to subendothelial amyloid build-up [18]. Although our patient had hypersplenic thrombocytopenia (with an onset of menometrorrhagia while PLT within normal limits), platelet dysfunction was considered due to uraemia and systemic amyloidosis.…”