Hemophagocytic lymphohistiocytosis in a child with human immunodeficiency virus infection - a case report.

INTRODUCTION: Hemophagocytic lymphohistiocytosis (HLH) is a rare condition and furthermore human immunodeficiency virus (HIV)-associated HLH is rarely reported in the literature. The most frequent presentation of secondary HLH is in association with infections, malignancies or drugs. In HIV-positive patients, the viral infection itself or the antiretroviral therapy (ART) could trigger HLH. CASE PRESENTATION: A 14-year-old boy was admitted for persistent diarrhea, severe weight loss and chest burns. Laboratory tests revealed important neutropenia, which led to HIV infection diagnosis. ART in combination was started associated with granulocyte-colony stimulating factor. Viral copies declined after six weeks of treatment, but the cluster of differentiation 4 (CD4) T-lymphocytes and neutrophil counts remained very low. Infections and malignancies were ruled out. The bone marrow aspirate revealed hemophagocytosis which in association with fever, bicytopenia, hypofibrinogenemia and hypertriglyceridemia established HLH diagnosis. Cortisone therapy and intravenous immunoglobulins were added. Due to lack of response, HLH-2004 protocol was initiated in collaboration with pediatric hematologist. In the first six months of treatment, CD4 T-lymphocytes and neutrophil count remained low and then they showed significant increase simultaneously. During treatment, the patient developed spontaneous severe back pain. Magnetic resonance imaging showed vertebral compression, osteomalacia and a thoracic vertebral fracture. CONCLUSIONS: Having ruled out the usual associated infections and malignancies, this is a case of HIV-associated with HLH. In this case, only the ART and immunomodulatory therapy were not enough and failed to work. Systemic immunosuppression also worked only after a prolonged course which led to an unfortunate complication: a vertebral fracture.

Endometrial stromal sarcoma in a 27-year-old woman. Case report and literature review.

Endometrial stromal tumors are very rare, representing approximately 0.2% of uterine malignancies, having an incidence of one to two from a million of women. The diagnosis cannot be established by imaging, it is histopathological only, often necessitate supplementary immunohistochemistry tests. We report the case of a 27-year-old woman who had an initial diagnosis, in another hospital, of uterine adenomyoma, established by dilatation and uterine curettage and then by subsequently histopathological exam. This diagnosis led to an initial non-oncological surgery, with interannexial total hysterectomy. The establishment of the final histopathological diagnosis of stromal endometrial sarcoma has led to a serious reassessment of the case. Making a review of the literature, we found very few cases of endometrial stromal sarcoma in young women less than 30 years old and we have not identified any clear strategy of treatment. However, from precautionary and considering that may be at risk, even with very few cases reported, the distance metastases can be present, sometimes at large intervals of time, we decided, for oncological safety, reintervention after one month. At the second surgery, it was practiced bilateral salpingo-ovarectomy, cardinal ligaments excision, partial omentectomy, bilateral pelvic lymphadenectomy extended lumbo-aortic and interaortico-cava, sampling biopsy from the inguinal femoral adenopathy and re-excision of the vaginal vault. The evolution was favorable, the patient being follow-up together with the oncologist specialist.

Role of serum VEGFA, TIMP2, MMP2 and MMP9 in monitoring response to adjuvant radiochemotherapy in patients with primary cervical cancer--results of a companion protocol of the randomized NOGGO-AGO phase III clinical trial.

AIM: The aim of the current study was to analyze the type of variations in expression profiles of matrix metalloproteinase 2 (MMP2), matrix metalloproteinase 9 (MMP9), tissue inhibitor of metalloproteinase 2 (TIMP2), and vascular endothelial growth factor A (VEGFA) before and after radiochemotherapy in patients with locally advanced FIGO stage Ib-IIb cervical cancer. We analyzed the role of these biomarkers in monitoring response to treatment. PATIENTS AND METHODS: Serum from 72 patients with cervical cancer treated within a phase III trial with either simultaneous radiochemotherapy (S-RC) with cisplatin, or systemic paclitaxel and carboplatin followed by percutaneous radiation (PC-R) was analyzed by ELISA. Sera were obtained during surgery and after the end of adjuvant treatment. RESULTS: The median age at time of diagnosis was 46 years (range=30-71 years). The most common histological types were squamous cell (73.6%) and adenocarcinoma (25%). Thirty-five (48.6%) patients underwent surgery followed by S-RC and 37 (51.4%) patients were treated with surgery followed by PC-R. Five patients developed recurrence within six months after radiochemotherapy. VEGFA levels were significantly higher before and after adjuvant treatment in patients who developed early recurrence (p=0.001). An increase of more than 500 pg/ml VEGFA and a decrease of more than 9% of the pre-therapeutic value of TIMP2 were significantly associated with a higher risk of early recurrence (RR=8.5, 95% CI=1.8-39.8 and RR=11.0, 95% CI=2.5-48.2, respectively). TIMP2 expression and risk score for early relapse (which is calculated using values of VEGFA and TIMP2) were independent prognostic factors for overall survival (p=0.043, HR=0.96, 95% CI=0.93-0.99 and p=0.002, HR=1.09, 95% CI=1.03-1.15, respectively). CONCLUSION: Our results indicate a predictive value of VEGFA and TIMP2 in monitoring cervical cancer patients undergoing radiochemotherapy.