Turkish community-based palliative care model: a unique design.

An organized palliative care system was lacking in Turkey before 2010. One of the pillars of Turkish Cancer Control Programme is palliative care. The Pallia-Turk project in this respect has been implemented by the Ministry since 2010. The project is unique since it is population based and organized at the primary level. This means, the whole population (>70 million) will have the quickest and easiest way for access to palliative care. This manuscript briefly summarizes the situation before the project and updates what has been done in last 2 years with the project.

Evaluation of low-to-moderate arsenic exposure, metabolism and skin lesions in a Turkish rural population exposed through drinking water.

BACKGROUND: There is no human data regarding the exposure, metabolism and potential health effects of arsenic (As) contamination in drinking water in the Central Anatolian region of Turkey. METHODS: Residents in ten villages with drinking water of total As (T-As) level >50 µg L-1 and 10-50 µg L-1 were selected as an exposed group (n = 420) and <10 µg L-1 as an unexposed group (n = 185). Time-weighted average-As (TWA-As) intake was calculated from T-As analysis of drinking water samples. Concentrations of T-As in urine and hair samples, urinary As species [i.e., As(III), As(V), MMA(V) and DMA(V], and some micronutrients in serum samples of residents of the study area were determined. Primary and secondary methylation indices (PMI and SMI, respectively) were assessed from urinary As species concentrations and the presence of skin lesion was examined. RESULTS: TWA-As intake was found as 75 µg L-1 in the exposed group. Urinary and hair T-As and urinary As species concentrations were significantly higher in the exposed group (P < 0.05). The PMI and SMI values revealed that methylation capacities of the residents were efficient and that there was no saturation in As metabolism. No significant increase was observed in the frequency of skin lesions (hyperpigmentation, hypopigmentation, keratosis) of the exposed group (P > 0.05). Only frequency of keratosis either at the hand or foot was higher in individuals with hair As concentration >1 µg g-1 (P < 0.05). CONCLUSIONS: Individuals living in the study area were chronically exposed to low-to-moderate As due to geological contamination in drinking water. No significant increase was observed in the frequency of skin lesions. Because of the controversy surrounding the health risks of low-to-moderate As exposure, it is critical to initiate long-term follow-up studies on health effects in this region.

Medical and Surgical Care of Patients With Mesothelioma and Their Relatives Carrying Germline BAP1 Mutations.

The most common malignancies that develop in carriers of BAP1 germline mutations include diffuse malignant mesothelioma, uveal and cutaneous melanoma, renal cell carcinoma, and less frequently, breast cancer, several types of skin carcinomas, and other tumor types. Mesotheliomas in these patients are significantly less aggressive, and patients require a multidisciplinary approach that involves genetic counseling, medical genetics, pathology, surgical, medical, and radiation oncology expertise. Some BAP1 carriers have asymptomatic mesothelioma that can be followed by close clinical observation without apparent adverse outcomes: they may survive many years without therapy. Others may grow aggressively but very often respond to therapy. Detecting BAP1 germline mutations has, therefore, substantial medical, social, and economic impact. Close monitoring of these patients and their relatives is expected to result in prolonged life expectancy, improved quality of life, and being cost-effective. The co-authors of this paper are those who have published the vast majority of cases of mesothelioma occurring in patients carrying inactivating germline BAP1 mutations and who have studied the families affected by the BAP1 cancer syndrome for many years. This paper reports our experience. It is intended to be a source of information for all physicians who care for patients carrying germline BAP1 mutations. We discuss the clinical presentation, diagnostic and treatment challenges, and our recommendations of how to best care for these patients and their family members, including the potential economic and psychosocial impact.

Accumulation of heavy metals with water quality parameters in Kizilirmak River Basin (Delice River) in Turkey.

Kizilirmak River has been used as Ankara's drinking water source for approximately 1.5 years. Therefore, this region's water, sediment, and fish samples are measured for detecting the heavy metals. This is important for the current situation as well as the future in terms of potential impact. The amount of heavy metals in drinking water should be within the limited values; otherwise, the accumulation of heavy metals will cause many problems to living organisms. Especially high levels of arsenic, cadmium, nickel, mercury, etc. are very dangerous to freshwater ecosystems as for human if the water is being use as drinking water. In this study, water, sediment samples, muscle, and gills of three fish species (Capoeta tinca, Capoeta capoeta, Leuciscus cephalus) were analyzed for the presence of heavy metals such as (Al, Fe, As, Cd, Ni, Mn, Se, Si) to determine present accumulation levels and possible toxic effect. The accumulation pattern of heavy metals in the water, sediment, and fish tissue follows the sequence: Si>Fe>Al>Mn>As>Ni>Se>Cd, Fe>Al>Mn>Ni>As>Se>Cd, and Fe>Al>Mn>As>Ni>Si>Cd. In addition, the detected concentrations of heavy metals in the Kizilirmak and Delice Rivers are compared with other heavy metal studies in the other main rivers and lakes in Turkey.

Excessive naked megakaryocyte nuclei in myelodysplastic syndrome mimicking idiopathic thrombocytopenic purpura: a complicated pre- and post-transplantation course.

A boy 3 years 7 months old with thrombocytopenia and history of intracranial hemorrhage who underwent bone marrow transplantation is presented. He was refractory to steroids, immunoglobulin G, vincristine, azathioprine, cyclosporine A, interleukin-11, chemotherapy, and splenectomy. Idiopathic thrombocytopenic purpura was excluded by light /electron microscopic and flow cytometric findings; the diagnosis of refractory cytopenia, a subgroup of pediatric myelodysplastic syndrome, was made. Naked megakaryocyte nuclei were 55.38 +/- 28.2% vs. 31.67 +/- 23.22% of all megakaryocytes in the patient and the control group of 9 patients with idiopathic thrombocytopenic purpura, respectively (p = .016). The posttransplatation course was complicated by delayed platelet engraftment, bronchiolitis obliterans associated with pneumocystis carinii pneumonia, which resolved completely.

Successful treatment with gemtuzumab ozogamicin monotherapy in a pediatric patient with resistant relapse of acute myeloid leukemia.

There are few therapeutic options in relapsed or refractory acute myeloid leukemia patients. CD33 antigen is expressed on approximately 90% of myeloblasts, and gemtuzumab ozogamicin, as a monoclonal antibody directed against the CD33 surface antigen, may be a good target for these patients. Herein, we present a 15-year-old acute myeloid leukemia patient who was resistant at relapse and could achieve remission with gemtuzumab ozogamicin at a total dose of 9 mg/m2, divided into three doses and delivered to hematopoietic stem-cell transplantation; however, the patient relapsed in a short time without application of transplantation.

The absence of peripheral blood blasts at diagnosis may predict CNS involvement or CNS relapse in pediatric acute lymphoblastic leukemia patients.

A high tumor burden at the time of diagnosis of childhood acute lymphoblastic leukemia has an unfavorable outcome. Peripheral white blood cell count is commonly used to reflect the leukemic burden and is used as one of the most important factors during determination of the risk-based treatment. However, peripheral blood blast count may not always reflect the tumor burden if leukocytes are not in blast nature. In the present study, we observed no central nervous system involvement at the time of diagnosis in patients with no peripheral blood blasts at the beginning, and furthermore, none of the patients with no peripheral blasts at the diagnosis had central nervous system relapse.

The prognostic impact of myeloid antigen expression in pediatric acute lymphoblastic leukemia patients.

The incidence of mixed-lineage leukemias in the pediatric age group was previously reported as 13.8% for myeloid antigen-positive ALL and 11.1% for lymphoid antigen-positive acute myeloid leukemia (AML). Recent studies showed that extensive chemotherapy protocols overcome the risk of myeloid lineage. Our study also supports most of the previous data and we postulate that myeloid antigen expression in pediatric ALL cases has insignificant effect on clinical presentation, relapse rates and survival. Importantly, 54% of myeloid antigen-expressing ALL patients received high-risk treatment protocols for some other reasons and this may also have contributed to similar outcome in these patients to that observed in myeloid antigen-negative ALL patients.

The neurologic complications in pediatric acute lymphoblastic leukemia patients excluding leukemic infiltration.

This study presents retrospective analyses of 20 acute lymphoblastic leukemia (ALL) patients who developed neurologic complications (except leukemic infiltration). These subjects represent 9.9% of 203 ALL patients aged 16 years or younger followed in our hospital between March 1991 and January 2003. Fourteen male and six female patients, whose ages ranged between 6 and 168 months, developed 24 episodes of neurologic complications after the diagnosis of ALL. The most common complication was meningitis, which developed in six (25%) episodes, and two thirds of the patients who had meningitis were evaluated to be iatrogenic. Cerebral infarct and venous thrombosis were detected in five (21%) of the episodes. In two (8%) episodes progressive cerebral dysfunction developed after radiotherapy. The remaining 11 (45%) episodes are due to varying types of complications. Interestingly, one patient had abundant histiocytes exhibiting hemophagocytosis in the cerebrospinal fluid (CSF) examination and this patient was subsequently diagnosed with fungal meningoencephalitis, by further investigation. Six (30%) patients died; epilepsia developed in five (25%) patients in the follow-up period and the remaining nine (45%) are healthy. By close follow-up and effective treatment of thrombosis and, especially, of infections including iatrogenic meningitis in developing countries, the morbidities and mortalities of these complications can be decreased.

Children with acute myeloblastic leukemia presenting with extramedullary infiltration: the effects of high-dose steroid treatment.

To evaluate whether children with acute myeloblastic leukemia (AML) presenting with extramedullary infiltration (EMI) have different clinical, morphologic features and prognosis from children without EMI, a 127 consecutive previously untreated children with AML were entered in this study. Fifty-one children (40%) had EMI at diagnosis and 27% of these showed multiple site involvement. Twenty-seven of 127 children (21%) presented myeloid tumors. No age related differences in the incidence of EMI was noted. However, analysis of clinical and biological features at diagnosis showed that WBC count > or =50 x 10(9) l(-1), hepatosplenomegaly >5 cm, FAB AML-M4 and AML-M5 subtypes and CD13, CD14 expression of bone marrow (BM) leukemic cells (>20%) were more frequent in children with EMI. Two consecutive treatment protocols were used. In both protocols remission was achieved with combined high-dose methylprednisolone (HDMP) as a differentiating and apoptosis inducing agent with mild cytotoxic chemotherapy (low-dose cytosine arabinoside (LD Ara-C), weekly mitoxantrone and Ara-C or 6-thioguanine). Administration of short-course (4-7 days) HDMP (20-30 mg/kg per day) alone resulted in a remarkable decrease in peripheral blood, BM blasts and in the size of EMI in responding patients. In both protocols, remission rate in patients with EMI was 71 and 80%, which was lower than that of the patients without EMI (87 and 89%). This may be attributed to the higher frequency of unfavorable features in children with EMI. However, in patients who presented with myeloblastoma and treated with a more intensive post-remission therapy (AML-94), the 4-year disease-free survival (DFS) and event-free survival (EFS) rates were not found to be significantly different from children who had no EMI (P>0.05). Whereas, the outcome of children who presented with gingival infiltration did not improve. In further studies, the prognostic significance of different localisation of EMI and the effect of addition of HDMP to cytotoxic chemotherapy should be explored in larger series.

Acute lymphoblastic leukemia in infants.

Between January 1978 and August 1999, 29 infants with newly diagnosed acute lymphoblastic leukemia (ALL) were treated on three consecutive protocols. Eighteen patients with infant ALL diagnosed between 1978-1991 were included in Group 1. In this group, treatment comprised a two- or three-drug induction with prednisolone, vincristine and L-asparaginase, and maintenance therapy consisted of weekly oral administration of mercaptopurine and methotrexate for three years. Group 2: Between 1991 and 1999, 11 infants with ALL were treated by St. Jude Total Therapy XI (n=4) and XIII (n=7) protocols with minor modification. Three years' event-free survival (+/-SE) was 14 +/- 9% in group 1. In group 2, this rate was 25 +/- 22% in Total XI and 57 +/- 19% in Total XIII. Outcome for infants on protocols Total Therapy XI and XIII improved compared with that of group 1.