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K Klopfenstein

Publications and source records attributed to K Klopfenstein.

6 recordsLinked to original sources

Inflammatory myofibroblastic tumor following hematopoietic stem cell transplantation: report of two pediatric cases.

Inflammatory myofibroblastic tumors are benign neoplasms histologically composed of lymphocytes, histiocytes, macrophages, foam cells, and plasma cells among a spindle-shaped stroma. Their etiology and potential for metastatic spread is controversial. Numerous predisposing factors have been suggested, including preceding infections, radiotherapy, and local trauma. We present two cases of pseudotumors that developed in children following hematopoietic stem cell transplantation. These are the first cases after hematopoietic transplant reported in the literature. As these neoplasms are difficult to diagnose and are often confused with highly aggressive tumors, our cases demonstrate that a high index of suspicion for such lesions must be maintained when evaluating masses in post transplant patients.

Child↗

Mobilization of peripheral blood stem cells in children using G-CSF after carboplatin containing myelosuppressive chemotherapy.

PURPOSE: Peripheral blood stem cell (PBSC) apheresis provides an alternative to autologous marrow harvest as a source of hematologic stem cells for transplantation in children with solid tumors. PATIENTS AND METHODS: Eight children with metastatic or recurrent solid tumors underwent 27 apheresis procedures. Recovery from myelosuppressive chemotherapy occurred without continuous daily growth factor support prior to mobilization. Granulocyte colony stimulating factor (G-CSF) at 16 microgs/kg/day was used to increase stem cells in the peripheral circulation. CD 34 positive cells, mononuclear cells (MNC), and CFU-GM were measured in the apheresis products. Prior chemotherapy was examined as a clinical factor that affected PBSC yield. RESULTS: A significant correlation was found between CD 34+/kg and CFU-GM/kg of the products (r = 0.758, P < 0.001). Patients receiving cumulative doses of carboplatin over 1,600 mg/m2 produced adequate MNC (1 x 10(8)/kg) but yielded significantly less CD 34+ cells or CFU-GM than those patients receiving less carboplatin. Prior doses of etoposide and ifosfamide did not effect PBSC yield. CONCLUSIONS: The mobilization technique was well tolerated, and the products obtained produced trilineage engraftment in the patients that underwent peripheral blood stem cell transplantation. Peripheral blood stem cell apheresis in children can be optimized by selection of appropriate candidates and mobilization with G-CSF after an absence of hematopoietic growth factor support.

Adolescent↗

Pulmonary function is compromised in children with mediastinal lymphoma.

PURPOSE: Lymphomas account for nearly 20% of the malignancies in childhood and the majority of patients with Hodgkin's Disease (HD) and non-Hodgkin's lymphoma (NHL) have radiological evidence of mediastinal involvement at presentation. Children with mediastinal tumors are at risk for the development of lethal airway obstruction during general anesthesia. This study quantitates the degree of the airway obstruction and the functional significance of tracheal compression in a cohort of 51 children with HD and NHL. RESULTS: Thirty patients with HD (mean age, 14.6 years) and 21 with NHL (mean age, 9.2 years) were included in this study. Twenty-five children (49%) had respiratory symptoms at the time of presentation. Respiratory complaints were much more common in children with NHL (76%) when compared with those with HD (30%). Pulmonary function was also significantly worse in the NHL patients who had a mean upright forced vital capacity (FVC) of 66 +/- 21%. The comparable value for the children with HD was 85 +/- 15% (P = .031). Patients with respiratory symptoms at presentation had both obstructive and restrictive deficits of pulmonary function. Their mean upright forced expiratory volume in 1 second (FEV1) was 69 +/- 22% and the FVC was 69 +/- 18%. Children with large mediastinal masses also had significantly decreased pulmonary function compared with those with small tumors. The upright FEV1 for these two groups was 72 +/- 18% versus 98 +/- 15% (P = .016). Their FVC values were 68 +/- 20% and 91 +/- 17%, respectively (P = .049). Mean tracheal compression was measured at 44% in the children with large tumors versus 27% for those with small lesions (P = .048). CONCLUSION: Children with mediastinal lymphomas have both obstructive and restrictive deficits on pulmonary function testing. Pulmonary function is significantly decreased in patients with NHL, children who present with respiratory symptoms, and those with very large mediastinal masses (mediastinal mass ratio > 45%). The extent of tracheal compression correlates with the size of the mediastinal mass.

Adolescent↗

Chromosome analyses in a rhabdoid tumor of the brain.

A malignant rhabdoid tumor of the brain from a 19-month-old child was studied. Two related clones, 46,XX,-8,+der(8)t(8;22)(p11;q?12)x2,-22,del(22)(q12q?13) and 46,XX-8,+der(8)t(8;22) (p11;q?12) x2,-22,r(22) were found after chromosome analyses of primary and recurrent tumor, and multiple nude mouse passages of the tumor. Breakpoints were studied using FISH.

Animals↗