Neoadjuvant chemotherapy with taxotere-epirubicin-5-fluorouracil (TEF) in local-regionally advanced breast cancer: a preliminary report

AIMS AND BACKGROUND: Sixty-three patients with local-regionally advanced breast cancer were treated with neoadjuvant chemotherapy consisting of docetaxel (Taxotere), epirubicin, and 5-fluorouracil (TEF). METHODS AND STUDY DESIGN: Preoperatively, patients received four cycles of Taxotere (80 mg/m2), epirubicin (60 mg/m2), and 5-fluorouracil (500 mg/m2), repeated every 21 days. Following completion of four cycles of chemotherapy, appropriate surgery was performed. After the surgery, patients received one cycle of the TEF chemotherapy regimen; following chemotherapy, radiotherapy was applied, and at the end two more cycles of TEF chemotherapy regimen were given. RESULTS: Sixty-three patients with locally advanced breast cancer were treated. Three patients were excluded from the study before the evaluation of response. Median age of the patients was 50 years (range, 25-77). Twenty-seven and 33 patients were premenopausal and postmenopausal, respectively. Thirty-nine patients were in stage IIIA and 21 in stage IIIB. Complete and partial responses were observed in 15 (25%) and 42 (70%) of the patients following four cycles of preoperative TEF chemotherapy regimen, respectively. Overall response was 95%, and primary lesion progressed only in 3 (5%) patients. The mean disease-free survival was 15.9 +/- 6.8 (range, 3.5-28) months and the mean overall survival was 18.6 +/- 7.2 (range, 5-30) months. The most frequent side effects were nausea-vomiting, mucositis, alopecia and leukopenia. CONCLUSIONS: TEF therapy is a treatment with a high overall response rate and toxicities similar to other taxotere combinations. A longer follow-up of patients is necessary for the determination of disease-free survival and overall survival.     

The effect of scleral buckling surgery on corneal astigmatism, corneal thickness, and anterior chamber depth

PURPOSE: To evaluate the effect of scleral buckling surgery on corneal topography, corneal thickness, and anterior chamber depth. METHODS: Thirty-two eyes of 32 patients who underwent encircling buckling surgery for rhegmatogenous retinal detachment were included in the study. Fellow eyes comprised the control group. Orbscan II topography system was used to determine the changes in corneal topography, corneal thickness, and anterior chamber depth before surgery and 1 week, 1 month, and 3 months after surgery. Statistical analyses used in the study were independent t test, paired t test, one way analysis of variance, and correlation analyses. RESULTS: Postoperatively, mean central corneal astigmatism at 1 week, 1 month, and 3 months was 4.3 +/- 2.0 diopters (D), 3.3 +/- 1.6 D, and 3.1 +/- 1.0 D, respectively. The change between 1 week and 3 months was statistically significant. Central cornea was thickened at the first postoperative week. The thickness gradually decreased to the preoperative levels at 3 months. Anterior chamber depth decreased, and the anterior chamber remained shallow after the surgery. The comparison between preoperative (2.94 +/- 0.6 mm) and postoperative anterior chamber depth was statistically significant at 1 week (2.57 +/- 0.7 mm), 1 month (2.83 +/- 0.7 mm), and 3 months (2.73 +/- 0.6 mm). CONCLUSIONS: Corneal changes after scleral buckling surgery were mild to moderate and the cornea almost resumed its preoperative parameters in 3 months' time. However, the anterior chamber remained shallow during 3 months of follow-up.     

Multicenter retrospective analysis regarding the clinical manifestations and treatment results in patients with hairy cell leukemia: twenty‐four year Turkish experience in cladribine therapy

In this multicenter retrospective analysis, we aimed to present clinical, laboratory and treatment results of 94 patients with Hairy cell leukemia diagnosed in 13 centers between 1990 and 2014. Sixty‐six of the patients were males and 28 were females, with a median age of 55. Splenomegaly was present in 93.5% of cases at diagnosis. The laboratory findings that came into prominence were pancytopenia with grade 3 bone marrow fibrosis. Most of the patients with an indication for treatment were treated with cladribine as first‐line treatment. Total and complete response of cladribine was 97.3% and 80.7%. The relapse rate after cladribine was 16.6%, and treatment related mortality was 2.5%. Most preferred therapy (95%) was again cladribine at second‐line, and third line with CR rate of 68.4% and 66.6%, respectively. The 28‐month median OS was 91.7% in all patients and 25‐month median OS 96% for patients who were given cladribine as first‐line therapy. In conclusion, the first multicenter retrospective Turkish study where patients with HCL were followed up for a long period has revealed demographic characteristics of patients with HCL, and confirmed that cladribine treatment might be safe and effective in a relatively large series of the Turkish study population. Copyright © 2014 John Wiley & Sons, Ltd.     

Bilateral massive choroidal hemorrhage secondary to Glanzmann's syndrome

Characteristics of intraocular bleeding and its management in association with blood dyscrasias are discussed. We present a patient with massive bilateral choroidal hemorrhage secondary to Glanzmann's syndrome. Magnetic resonance imaging and ultrasonographic findings were ordered. During the clinical course, bilateral intravitreal hemorrhage and tractional retinal detachment occurred. Left pars plana vitrectomy was performed under general anaesthesia. The procedure was unsuccessful because of intraoperative uncontrolled bleeding.     

Phase II study of gemcitabine plus paclitaxel in metastatic breast cancer patients with prior anthracycline exposure

Chemotherapy provides palliation and modest prolongation of symptom-free survival in metastatic breast cancer. Taxane containing regimens are commonly considered to be among the initials in metastatic setting due to earlier use of anthracyclines in the course of breast cancer. Therefore, we conducted this Phase II study to assess efficacy and safety of gemcitabine plus paclitaxel (GT) combination therapy in anthracycline pretreated metastatic first-line setting. Patients and Methods: The study enrolled 26 women with pathologically confirmed and measurable metastatic breast cancer who were previously treated with anthracycline but no prior chemotherapy for metastatic disease. Twenty six and twenty four patients were eligible for toxicity and efficacy evaluations respectively. Mean age was 47.3 years and median ECOG performance status was 0. Twenty patients (76.9 percent) had visceral metastases, most commonly located in liver and lung. Treatment schedule was as follows: paclitaxel 175 mg/m² was administered intravenously in 3 hours on Day 1 and gemcitabine 1000 mg/m² was administered intravenously in 30 minutes on Day 1 after paclitaxel application, and on Day 8 every 21 days. Results: Objective response rate was 41.7 percent (95 percent CI: 21.9-61.4) with 16.7 percent (95 percent CI: 1.7-31.6 percent) CR, and 25.0 percent (95 percent CI: 7.6-42.3 percent) PR. Median time to progression and overall survival were 9.6 and 14.5 months, respectively. Grade 3-4 toxicity was observed in 34.6 percent (9) patients. Treatment of two patients was discontinued due to toxicity, consisting of Grade 3 hypersensitivity reactions and Grade 4 infections in one patient each. Dose reductions due to myelotoxicity were performed in 4 (15.3 percent) patients. Hematologic toxicities were generally manageable with appropriate dose modifications and supportive care. Conclusion: Gemcitabine and paclitaxel combination regimen is effective and has manageable toxicity profile as first line metastatic setting.     

A coincidence of renal cell carcinoma and hematological malignancies

Hematological malignancies with renal cell carcinoma (RCC) are rarely seen. We reported here two cases of coincidence of RCC with multiple myeloma (MM) and Hodgkin??s disease (HD). A 69-year-old male patient with tumor that was located at the upper pole of left kidney was admitted to our clinic. Partial tumoral resection was perfomed and stage-I RCC was diagnosed after the histopathological examination of tumor. Moreover, he was diagnosed with IgG kappa stage-IIA MM as a result of bone marrow examination and serum immunofixation electrophoresis. Radiotherapy, combination chemotherapy, and autologous stem cell transplantation (ASCT) were performed. The patient is still alive who achieve a complete remission. A 53-year-old male patient suffered from cervival lymphadenopathy. He has a story of right radical nefrectomy that has been performed 4?years ago. Stage II-B lymphocyte-predominant HD was diagnosed. Combination chemotherapy was started, but relapse occurred 25?months later. ASCT was not planned due to cardiovascular problems and the patient died. Clinicans should keep in mind the coincidence of RCC with MM and HD.     

T-cell-rich B-cell lymphoma: a clinicopathologic study of 21 cases and comparison with 43 cases of diffuse large B-cell lymphoma

Clinicopathologic features of 21 patients with T-cell-rich B-cell lymphoma (TCRBCL) were reviewed and compared to 43 patients with diffuse large B-cell lymphoma (DLBCL) to determine if there were distinguishing clinical characteristics and differences in response or survival to CHOP therapy. For the diagnosis of TCRBCL, the current WHO criteria was used. In all of our cases, the majority of cells are non-neoplastic T cells and <10% large neoplastic B cells are present. The initial pathologic diagnosis was nodular lymphocyte predominant Hodgkin's lymphoma (NLPHL) in two cases. Patients with TCRBCL were significantly younger (median: 46 years) and had a significantly higher incidence of B symptoms (62%), hepatomegaly (33%) and marrow infiltration (33%) at presentation when compared to DLBCL (P<0.03). The CR rate after treatment was 48% for TCRBCL patients versus 79% for the DLBCL (P<0.003). Although the CR rates in between the two groups are significant, the difference in 3 years survival rates in each CR groups was insignificant (80% versus 77%). The overall survival time in the two groups was 17 months. Event-free survival time in TCRBCL was 12 months, compared with 17 months in the DLBCL (P>0.05). The frequency of patients with TCRBCL achieving CR was 52.6% whereas that of patients with DLBCL was 79% (P<0.003). The TCRBCL 3 years event-free survival 48% and overall survival 64% were 63 and 72% for DLBCL, respectively.     

Lack of association between MAOA-uVNTR variants and excessive daytime sleepiness

Sleep disorders are highly prevalent in the population and have dramatic health, social, and economic impacts. However, their treatments may remain symptomatic due to ignorance of molecular factors which may provide fundamental insights into the neurological bases of sleep. Excessive daytime sleepiness (EDS) is a common complaint encountered in neurological practice with significant effects both on individuals and on society. We aimed to investigate the role of monoamine oxidase A (MAOA) as a candidate gene in EDS. Epworth sleepiness scale (ESS) was applied to 221 subjects who were also genotyped for MAOA upstream variable number of tandem repeats (MAOA-uVNTR). Patient group displayed higher ESS values (mean 12.67) when compared with the control group (mean 6.38). However, MAOA-uVNTR genotypes did not show a significant association with ESS scores neither on women nor on men. Finally, these data suggest further replications in different populations. Moreover, the investigation of some other genes together with MAOA and/or some possible regulatory molecular mechanisms may offer a more comprehensive approach in the role of genetic factors contributing to EDS.     

Identification of three novel plasminogen (PLG) gene mutations in a series of 23 patients with low PLG activity

Inherited severe hypoplasminogenaemia is a multisystemic disorder leading to deficient extravascular fibrinolysis. As a clinical consequence wound healing capacity of mucous membranes is markedly impaired leading to ligneous conjunctivitis and several other manifestations. Here we report the molecular genetic and clinical findings on 23 new cases with severe hypoplasminogenaemia. Homozygous or compound-heterozygous mutations in the plasminogen (PLG) gene were found in 16 of 23 patients (70%), three of which were novel mutations reported here for the first time (C166Y, Y264S, IVS10-7T/G). Compared to 79 previously published cases, clinical manifestations of the current group of patients showed higher percentages of ligneous periodontitis, congenital hydrocephalus, and involvement of the female genital tract. In contrast, involvement of the gastrointestinal or urogenital tract was not observed in any of the cases. Patients originated to a large extent (61%) from Turkey and the Middle East, and showed a comparably frequent occurrence of consanguinity of affected families and a greater female to male ratio than was derived from previous reports in the literature. Individual treatment of ligneous conjunctivitis included topical plasminogen or heparin eye drops, topical or systemic fresh frozen plasma, and surgical removal of ligneous pseudomembranes, mostly with modest or transient efficacy. In conclusion, the present study underscores the broad range of clinical manifestations in PLG-deficient patients with a trend to regional differences. Transmission of genetic and clinical data to the recently established Plasminogen Deficiency Registry should help to determine the prevalence of the disease and to develop more efficient treatment strategies.