A case with multiple gynecological malignancies

A patient with cervical non-Hodgkin lymphoma was treated with chemotherapy. Fourteen months after the diagnosis of the lymphoma, an endometrial adenocarcinoma was detected as a secondary malignant tumor. The patient was treated with surgery followed by radiotherapy. Approximately 7 years after the diagnosis of endometrial cancer, vaginal invasive squamous cell carcinoma was diagnosed as the third primary malignancy, and a second-line palliative radiotherapy was applied. Seven months after the last radiotherapy, postradiational sarcoma in the vagina was diagnosed. Congenital and acquired immune system disorders, viral oncogenes, and various human leukocyte antigen (HLA) types were investigated. Total blood count and lymphocyte subset analysis were performed, and CD4+ lymphopenia was detected. Serologic tests were carried out for human immunodeficiency virus, hepatitis B virus, human papillomavirus, Epstein-Barr virus, and herpes simplex virus infection. Epstein-Barr virus viral capsid antigen IgG was found positive. Low-risk human papillomavirus panel was detected by Hybrid Capture method in the cervical smear. The HLA investigation revealed HLA-A(2), HLA-A(3), HLA-B-57, HLA-B-35, HLA-B-4, HLA-B-6, HLA-DR3, HLA-DR1, HLA-DR51, HLA-DR52, HLA-DQ(6(1)), and HLA-DQ(7(3)). The patient died because of the disease

Detection of an inguinal ovary at controlled ovarian stimulation that was successfully treated by repositioning

Objective: To report a rare case of an ectopic ovary placed in the inguinal canal that was detected while performing a controlled ovarian hyperstimulation (COH). Design: Case report. Setting: A university hospital. Patient(s): A couple with primary infertility for 4 years was referred to our infertility clinic. The woman's medical history revealed a left inguinal operation at age 7. On vaginal ultrasound, only the right ovary could be seen. An infertility workup conducted for the man revealed teratospermia. The couple was subsequently admitted to the in vitro fertilization (IVF) program. While having a COH, the woman experienced a painful swelling in the inguinal area, and an ovarian image with follicular growth on the left inguinal region was observed with ultrasound. Afterward, surgery was performed, and the ectopic ovary in the left inguinal region was detected. Intervention(s): Detection of an inguinal ovary with a controlled ovarian hyperstimulation procedure and surgical repositioning of the ectopic ovary. Main Outcome Measure(s): Controlled ovarian hyperstimulation, transabdominal ultrasound, transvaginal ultrasound. Result(s): The ectopic ovary was successfully repositioned with surgery. Conclusion(s): Patients must be closely monitored while performing COH. In patients who do not have a unilateral ovary, a painful inguinal mass should alert the physician to the possible presence of an ectopic ovary in the inguinal canal. © 2006 American Society for Reproductive Medicine

Familial Mediterranean fever (FMF) in Turkey: results of a nationwide multicenter study

Familial Mediterranean fever (FMF) is an autosomal recessive disease that is prevalent among eastern Mediterranean populations, mainly non-Ashkenazi Jews, Armenians, Turks, and Arabs. Since a large proportion of all the FMF patients in the world live in Turkey, the Turkish FMF Study Group (FMF-TR) was founded to develop a patient registry database and analyze demographic, clinical, and genetic features. The cohort was composed of 2838 patients (mean age, 23.0 +/- 13.33 yr; range, 2-87 yr), with a male:female ratio of 1.2:1. There was a mean period of 6.9 +/- 7.65 years from disease onset to diagnosis; the period was about 2 years shorter for each decade since 1981. Ninety-four percent of patients were living in the central-western parts of the country; however, their familial origins (70% from the central-eastern and Black Sea regions) reflected not only the ongoing east to west migration, but also the historical roots of FMF in Turkey. Patients' clinical features included peritonitis (93.7%), fever (92.5%), arthritis (47.4%), pleuritis (31.2%), myalgia (39.6%), and erysipelas-like erythema (20.9%). Arthritis, arthralgia, myalgia, and erysipelas-like erythema were significantly more frequent (p < 0.001) among patients with disease onset before the age of 18 years. Genetic analysis of 1090 patients revealed that M694V was the most frequent mutation (51.4%), followed by M680I (14.4%) and V726A (8.6%). Patients with the M694V/M694V genotype were found to have an earlier age of onset and higher frequencies of arthritis and arthralgia compared with the other groups (both p < 0.001). In contrast to other reported studies, there was no correlation between amyloidosis and M694V homozygosity in this cohort. However, amyloidosis was still remarkably frequent in our patients (12.9%), and it was prevalent (27.8%) even among the 18 patients with a disease onset after age 40 years. Twenty-two patients (0.8%) had nonamyloid glomerular diseases. The high prevalence of vasculitides (0.9% for polyarteritis nodosa and 2.7% for Henoch-Schonlein purpura) and high frequency of pericarditis (1.4%) were striking findings in the cohort. Phenotype II cases (those patients with amyloidosis as the presenting or only manifestation of disease) were rare (0.3% or less). There was a high rate of a past diagnosis of acute rheumatic fever, which suggested a possible misdiagnosis in children with FMF presenting with recurrent arthritis. To our knowledge, this is the largest series of patients with FMF reported from 1 country. We describe the features of the disease in the Turkish population and show that amyloidosis is still a substantial problem