Phase I study of tandem high-dose chemotherapy with autologous peripheral blood stem cell rescue for children with recurrent brain tumors: a Pediatric Blood and MarrowTransplant Consortium study.

Background: High-dose chemotherapy with autologous stem cell rescue (HDC/SCR) has produced responses and prolonged survival for some children with recurrent brain tumors, but is associated with considerable morbidity and mortality. A Phase I trial of two cycles of HDC/SCR for recurrent brain tumors in children was performed to determine the maximum tolerated doses for a novel regimen.

Procedures: Two cycles of HDC/SCR were given.

A pilot study of addition of amifostine to melphalan, carboplatin, etoposide, and cyclophosphamide with autologous hematopoietic stem cell transplantation in pediatric solid tumors-A pediatric blood and marrow transplant consortium study.

Limited information is available regarding the use of amifostine in pediatric hematopoietic stem cell transplant (HSCT) patients. Melphalan, carboplatin, etoposide +/- cyclophosphamide is a commonly used preparatory regimen in pediatric solid tumor HSCT. Therefore, we decided to determine the feasibility of the addition of amifostine (750 mg/m b.

Sickle cell cerebrovascular disease: usual and unusual findings on MR imaging and MR angiography.

This paper demonstrates usual and unusual findings on MR imaging (MRI) including fluid-attenuated inversion recovery (FLAIR), diffusion- and perfusion-weighted image and MR angiography (MRA) in 30 patients with sickle cell cerebrovascular disease. This paper also illustrates the underlying pathophysiology of sickle cell cerebrovascular disease by using the cases with usual and unusual MR findings, including long-time follow-up cases during transfusion therapy. MRI and MRA are useful in defining the basis for stroke and assessing the effects of chronic transfusion therapy.

Bacillus cereus central line infection in an immunocompetent child with hemophilia.

Bacillus species are increasingly recognized as pathogens in immunocompromised patients. The authors report a case of Bacillus cereus infection of a central line in an immunocompetent patient with hemophilia, which required line removal for complete cure.

Neuronal stem cells biology and plasticity.

Recent studies have suggested that stem cells are able to cross primordial tissue barriers. Their ability to respond to unrelated microenvironmental signals strongly suggest that they have greater potential than previously imagined especially for their future clinical use for the regeneration of tissues or even perhaps organ systems. In particular there is an intriguing reciprocal relationship between the hematopoietic and neuronal stem cell systems.

Sentinel nodal assessment in patients with carcinoma of the lung.

Background: Assessment of sentinel nodes to predict metastases in a regional nodal basin is valuable for staging patients with melanoma and breast carcinoma. This study tested whether injection of isosulfan blue and technetium-99 could identify mediastinal sentinel nodes in patients with lung carcinoma and determine whether sentinel node histology predicts distal nodal metastases.

Methods: Isosulfan blue and technetium-99 were injected into the tumor and pulmonary resection performed.

Palliative uses of methylphenidate in patients with cancer: a review.

Purpose: Cancer is, unfortunately, often a terminal disease. The goal of therapy for many patients with cancer is palliation of symptoms common at the end of life, including pain, depression, and cognitive dysfunction. Methylphenidate is a psychostimulant most commonly used in the treatment of attention deficit hyperactivity disorder.

Air embolism after intravenous injection of contrast material.

Venous air embolism, though considered rare, occurs more frequently than it is recognized. We report the case of a 54-year-old man who was incidentally found to have air in the left brachiocephalic vein and right ventricle after contrast-enhanced computed tomography (CT). Air embolism has been noted to occur after contrast-enhanced CT examination in up to 23% of patients.

Acute lymphoblastic leukemia with the (8;14)(q24;q32) translocation and FAB L3 morphology associated with a B-precursor immunophenotype: the Pediatric Oncology Group experience.

Five pediatric patients are described with acute lymphoblastic leukemia (ALL) who at presentation had clinical findings suggestive of B cell ALL and lymphoblasts with FAB L3 morphology and the characteristic t(8;14)(q24;q32). However, the leukemia cells of all five patients failed to express surface immunoglobulin (sIg) and kappa or lambda light chains. Based on initial immunophenotyping results consistent with B-precursor ALL, four of these cases were initially treated with conventional ALL chemotherapy.

Hurler syndrome: II. Outcome of HLA-genotypically identical sibling and HLA-haploidentical related donor bone marrow transplantation in fifty-four children. The Storage Disease Collaborative Study Group.

Untreated patients with Hurler syndrome (MPSIH) experience progressive neurologic deterioration and early death. Allogeneic bone marrow transplantation (BMT) ameliorates or halts this course. The Storage Disease Collaborative Study Group was formed to evaluate the effectiveness and toxicity of BMT.

Factor V Leiden is not responsible for stroke in patients with sickling disorders and is uncommon in African Americans with sickle cell disease.

Cerebrovascular accidents in patients with sickle cell anemia are among the most devastating complications of the disease. It has recently been demonstrated that some patients have a hypercoagulable state on the basis of the presence of an abnormal factor V molecule, factor V Leiden. We undertook this study to evaluate the presence of factor V Leiden in sickle cell patients with stroke.

Refractory T cell acute lymphoblastic leukemia with unusual karyotype and interesting immunophenotype.

Karyotype, immunophenotype, and molecular studies are important in the evaluation of Acute Lymphocytic Leukemia as these data provide diagnostic as well as prognostic information. We present a case of acute lymphoblastic leukemia with unusual cytogenetics, 45,XY,i(7q),der(9)t(3;9)(q12;p22),del(12)(p12), :der(18)t(3;18)(p14;q22),-3. This karyotype is hypodiploid, showing loss of chromosome 3, a very rare occurrence.

Decreased hematopoietic accessory cell function following bone marrow transplantation.

Hematologic engraftment following bone marrow transplantation requires not only pluripotent stem cells but also functioning accessory cells whose trophic factors support the proliferation and differentiation of stem cells and progenitors to mature blood cells. To better understand the regulation of hematopoiesis following transplantation, we studied hemopoietic accessory cell function in bone marrow transplant recipients 3 weeks to 10 months following transplantation. In general, hematopoietic accessory cell function was decreased following bone marrow transplantation.

Retarded recovery of functional T cell frequencies in T cell-depleted bone marrow transplant recipients.

We have studied the effect of removing donor T cells by treatment with the monoclonal antibody Leu-1 and complement before marrow transplantation on the regeneration of functionally competent T lymphocytes in the blood at selected times after transplant. Using sensitive limiting-dilution methods that allow us to enumerate helper, cytotoxic, and proliferating T lymphocyte precursors, we report that regeneration of a functional T cell compartment is more severely impaired for the first 180 days after transplantation in those patients given T cell-depleted bone marrow than in recipients of untreated marrow. After this first 6 months, however, patients given T cell-depleted bone marrow had blood T cell frequencies comparable to those observed in patients given untreated marrow.

Functional assessment of T cell depletion from bone marrow prior to therapeutic transplantation using limiting dilution culture methods.

We evaluate the usefulness of limiting dilution culture methods in assessing the extent of T lymphocyte depletion from bone marrow inocula, prior to transplant, using either ex vivo antibody/complement-mediated depletion or immunotoxin treatment. Complement-mediated depletion using anti-Leu-1 antibody was shown to result in a consistent decline of 99%-99.9% in the frequencies of T cells able to proliferate in mitogen-stimulated, interleukin-2 (IL-2)-supplemented cultures.

Long-lasting deficit of functional T cell precursors in human bone marrow transplant recipients revealed by limiting dilution methods.

We have applied limiting dilution methods suitable for the estimation of mitogen-reactive helper (pHTL) and cytotoxic (pCTL) T cell frequencies to the analysis of immune function in patients 1 mo to 6 yr after allogeneic bone marrow transplantation (BMT). Although the majority of these patients have regained normal levels of Leu-3+ (helper) and Leu-2+ (killer/suppressor) cells by 6 to 12 mo after BMT as assessed by cytofluorimetry, the fraction of these cells that can function in limiting dilution cultures is substantially below normal levels in nearly all patients. Although some BMT patients eventually recover normal frequencies of pCTL and pHTL, values typically remain greatly depressed even in patients transplanted as many as 4 to 6 yr previously.

Stages of T cell activation: continued antigen dependence of IL 2-producing cells after IL 2 receptor expression.

Alloantigen stimulation leads, within 48 to 72 hr, to the expression of IL 2 receptors (IL 2R) on the surface of most of the helper T cells with specificity for the stimulating antigens. The IL 2R-bearing cells, separated by flow cytometry from 3-day human or mouse mixed lymphocyte cultures, were found by limiting dilution methods to be enriched 10- to 20-fold (compared to IL 2R- cells) for antigen-specific helper T cells detected by IL 2 production. Although these cells have been activated to an IL 2R+ stage, most of them are unable to produce detectable IL 2 unless they are cultured together with the original, activating antigen.