Secondary Neoplasms in Children with Hodgkin's Lymphoma Receiving C-MOPP and Radiotherapy: Presentation of Four Cases.

Patients who survive Hodgkin lymphoma (HL) are at increased risk of secondary neoplasms (SNs). A wide variety of SNs have been reported, including leukemias, non-Hodgkin's lymphomas, and solid tumors, specifically breast and thyroid cancers. Herein we report subsequent neoplasms in four patients with HL receiving chemoradiotherapy.

Long-term follow-up and treatment of congenital alveolar proteinosis.

Background: Clinical presentation, diagnosis, management and outcome of molecularly defined congenital pulmonary alveolar proteinosis (PAP) due to mutations in the GM-CSF receptor are not well known.

Case Presentation: A 2 1/2 years old girl was diagnosed as having alveolar proteinosis. Whole lung lavages were performed with a new catheter balloon technique, feasible in small sized airways.

Respiratory disease in Niemann-Pick type C2 is caused by pulmonary alveolar proteinosis.

Niemann-Pick diseases are hereditary neurovisceral lysosomal lipid storage disorders, of which the rare type C2 almost uniformly presents with respiratory distress in early infancy. In the patient presented here, the NPC2 exon 4 frameshift mutation c.408_409delAA caused reduced NPC2 protein levels in serum and lung lavage fluid and the synthesis of an aberrant, larger sized protein of around 28 kDa.

De novo osteogenic sarcoma of mastoid bone.

The most common primary malignant tumor of the bone is osteosarcoma. Primary involvement of the craniofacial bones in osteosarcoma is relatively rare. The mandible and the maxillae are the most commonly affected bones of the head.

The clinical value of flow cytometric DNA content analysis in patients with soft tissue sarcomas.

Purpose. The purpose of this study was to evaluate: (1) the correlation between grade and ploidy or S-phase fraction (SPF), (2) the prognostic value of DNA flow cytometric study in soft tissue sarcomas.Patients /Methods.

Cathepsin H and napsin A are active in the alveoli and increased in alveolar proteinosis.

Pulmonary alveolar proteinosis (PAP) is a group of rare diseases with disturbed homeostasis of alveolar surfactant. While 90% of the primary adult forms are caused by granulocyte-macrophage colony-stimulating factor autoantibodies, the underlying cause of the juvenile form remains unknown. In order to distinguish primary from secondary effects in the pathogenesis of these two forms, the present authors studied the surfactant protein processing proteases napsin A and cathepsin H.

High-dose ifosfamide with hematopoietic growth factor support in advanced bone and soft tissue sarcomas.

Aim: Ifosfamide and doxorubicin are the most effective agents in the treatment of sarcomas, although their contributions to survival are usually limited. High-dose ifosfamide can be used as a salvage treatment in patients with recurrent or advanced sarcoma. We evaluated efficacy and toxicity of high-dose ifosfamide in the patients with recurrent or advanced sarcoma in this study.

Post-transfusion acute graft versus host disease in a 17-year-old girl with a malignant mesenchymal tumor--report of a case.

Post-transfusion graft-versus-host disease (PT-GVHD) is seldom, but it has a high mortality rate, exceeding 90 percent. There is no standard treatment for PT-GVHD and irradiation of blood and its components before transfusion is broadly accepted for the prevention of PT-GVHD. In this report we present a case (a 17-year-old female) of PT GVHD, who died in spite of the use of all available therapeutic options.

Trace Element Analyses (Zinc and Selenium) in Pediatric Malignant Lymphomas.

Ninetysix untreated patients with Malignant lymphoma's, 81 Hodgkin's disease and 15 Burkitt's lymphoma were studied for zinc (Zn) and selenium (Se) status. Plasma and hair Zn, and Se levels were measured by atomic absorption spectrophotometry. Chronic Zn and Se deficiencies (low plasma and low hair Zn and Se levels together) were found to be associated with Malignant lymphoma's in Turkish children.

Characteristics of "Early Type-I Pattern" (0-6 years) Hodgkin's Disease in Turkish Children.

The clinico-epidemiologic characteristics of 54 children with HD in 0-6 years of age group were retrospectively analyzed. This group represented 27% of 200 HD cases observed in our center and was named as early type-I pattern HD. The association of EBV with HD was also shown by serologic and immunohistochemical methods (LMP1) in these very young Turkish patients.

Prospective randomized comparison of morning versus night daily single subcutaneous administration of granulocyte-macrophage-colony stimulating factor in patients with soft tissue or bone sarcoma.

Background: Hematopoietic growth factors (HGFs) have been used to reduce the neutropenic complications of cytotoxic chemotherapy so that higher doses may be given. The authors have previously shown that endogenous serum granulocyte-colony stimulating factor (G-CSF) and granulocyte-macrophage-colony stimulating factor (GM-CSF) levels at night (p.m.

Hodgkin's disease in 82 Turkish children diagnosed over a 10-year period: epidemiological, clinical, and histopathologic features and prognosis with prolonged chemotherapy.

In this study, 82 Turkish children with Hodgkin's disease (HD) between 1 and 14 years of age and diagnosed over a 10-year period were evaluated retrospectively. More than half of the patients (54%) presented with advanced stages of HD. Mixed cellularity (MC) was the most frequent (56.

Burkitt's lymphoma in 63 Turkish children diagnosed over a 10 year period.

Sixty-three Turkish children with Burkitt's lymphoma (BL) diagnosed over a 10-year period in a single institution were retrospectively analyzed. Burkitt's lymphoma included 41.7% of non-Hodgkin's lymphomas and 17.

Granulocyte-macrophage colony stimulating factor (rh-GM-CSF) in the treatment of chemotherapy-induced neutropenia.

Neutropenic pediatric patients with solid tumors and malignant lymphomas were treated with recombinant granulocyte-macrophage colony stimulating factor (rh-GM-CSF). Eleven patients, including seven lympho-reticular malignancies, two Ewing's sarcoma and one patient in each group with the diagnosis of nasopharyngeal rhabdomyosarcoma, malignant mesenchymal tumor, entered the study. Six were females and five were males, the mean age was 10.

Late effects of chemoradiotherapy in pediatric Hodgkin's disease.

We treated 137 Turkish children with biopsy-proven Hodgkin's disease, followed up between the years 1964 and 1989. Most patients were treated and were in advanced stage with histological subtype of mixed cellularity (67.5%).

Burkitt's lymphoma in Turkish children: clinical, viral [EBV] and molecular studies.

Eighty-one Turkish children with Burkitt's lymphoma (BL) were observed during a period of 24 years (1968-1992). The diagnosis was established histologically according to WHO criteria. BL represented 48.

Orbito-ocular granulocytic sarcoma (OOGS) and acute myeloblastic leukemia (AML) with duplication of Philadelphia chromosome.

A 6-year-old Turkish boy with bilateral orbito-ocular granulocytic sarcoma and AML is described. Cytogenetic studies on peripheral blood disclosed an abnormal hyperdiploid population with a double Ph chromosome. Despite intensive chemotherapy, he achieved only partial remission.

T-lymphocyte sub-populations in orbito-ocular granulocytic sarcoma (OOGS) and acute myelocytic leukemia (AML): a preliminary study.

The aetiology of GS remains obscure and a little is known about the immune competence of these patients. Interestingly, all children with OOGS were from low 'socio-economic status' and showed diminished delayed hypersensitivity reactions and reduced T cell counts (E-R) in our previous observation. We present herewith a preliminary data on evaluation of T cell sub-populations determined by monoclonal antibodies (CD3, CD4, CD8 and CD16 cells) in 10 patients with OOGS and AML prior to treatment.

[Thermal expansion of dental alloys and porcelains].

Thermal behaviour of metal and porcelain against heat is an important factor in metal-porcelain bonding. For this reason thermal compatibility and the thermal expansion of metal and porcelain are some of the important subjects to be studied carefully. In this study, by means of dilatometric measurements, we determined the thermal expansion coefficients of metal alloy (Ivotect P) and porcelain (ITS-HTE, Ivoclar) which we use in routine practice.

[Evaluation of stresses in different cingulum rest seats].

Both lingual and incisal rest seats are used on anterior teeth. Lingual rest seats are preferred since they are more acceptable esthetically and located closer to the centre of rotation of the tooth. Lingual rest seats can be prepared directly on the abutment teeth as grooves, shoulders or may require restorations such as crowns, inlays and prefabricated rest seats to provide proper contour.

Aggressive Kaposi's sarcoma in children: a case report.

A three-year-old-boy with generalized Kaposi's sarcoma (KS) is presented. The child died of progressive pulmonary insufficiency on the eighteenth day of the course of his illness, the tenth hospital day. On postmortem examination diffuse KS infiltration was observed in the respiratory and gastrointestinal tracts, lymph nodes, liver, spleen and thymus.

[Effect of different thermal properties and different pontic frame designs to breaking strength of metal-porcelain bridges].

Bonding of two different materials, like porcelain and metal, is affected by many factors during the firing process. Thermal compatibility is one of the prerequisites in metal-porcelain bonding. Plus, the geometric differences are also accepted as an effect in thermal compatibility.