Increased immature T-cells detected by flow cytometry in post chemotherapeutic patients with acute myeloid leukemia, a case report and small series study.

Detection of minimal/measurable residual disease (MRD) in bone marrow specimens by flow cytometry is widely used in patients with T cell acute lymphoblastic leukemia (T-ALL). It plays a central role in guiding treatment and assessing prognosis. However, the occurrence of a normal physiologic reactive immature T-cell population in treated bone marrow is unknown. To investigate this, we examined 14 post chemotherapeutic bone marrow specimens with a T-ALL MRD flow cytometry panel. This included 9 acute myeloid leukemia (AML) and 5 T-ALL cases. Immature T-cells are defined as surface CD3 negative cells that coexpress cytoplasmic CD3 (cyCD3) and terminal deoxynucleotidyl transferase (TdT), or as cells that express CD34 with coexpression of multiple T-cell markers. Immature T-cells were present in 1 of 9 AML cases (11%), between day 20-31 post chemotherapy. Follow-up of this patient who had 4.00% cyCD3+ TdT+ immature T-cells, showed the population gradually decreased to 0.50% at day 31, 0.15% at day 46, and was undetectable (0.00%) at day 116. This population remained undetectable at the most current follow-up on day 147. This pilot study shows that a low level of cyCD3+ TdT+ immature T-cells may be present in post chemotherapeutic regenerating bone marrow and can be detectable by flow cytometry. Thus, extra caution should be taken when interpreting T-ALL MRD results, especially between days 20-31 post chemotherapy.

Molecular profiling identifies prognostic subgroups of pediatric glioblastoma and shows increased YB-1 expression in tumors.

PURPOSE: Pediatric glioblastoma (pGBM) is a rare, but devastating brain tumor. In contrast to GBM in adults (aGBM), little is known about the mechanisms underlying its development. Our aim is to gain insight into the molecular pathways of pGBM. MATERIALS AND METHODS: Thirty-two pGBM and seven aGBM samples were investigated using biochemical and transcriptional profiling. Ras and Akt pathway activation was assessed through the phosphorylation of downstream effectors, and gene expression profiles were generated using the University Health Network Human 19K cDNA arrays. Results were validated using real-time polymerase chain reaction and immunohistochemistry and compared with existing data sets on aGBM. RESULTS: There are at least two subsets of pGBM. One subset, associated with Ras and Akt pathway activation, has very poor prognosis and exhibits increased expression of genes related to proliferation and to a neural stem-cell phenotype, similar to findings in aggressive aGBM. This subset was still molecularly distinguishable from aGBM after unsupervised and supervised analysis of expression profiles. A second subset, with better prognosis, is not associated with activation of Akt and Ras pathways, may originate from astroglial progenitors, and does not express gene signatures and markers shown to be associated with long-term survival in aGBM. Both subsets of pGBM show overexpression of Y-box-protein-1 that may help drive oncogenesis in this tumor. CONCLUSION: Our work, the first study of gene expression profiles in pGBM, provides valuable insight into active pathways and targets in a cancer with minimal survival, and suggests that these tumors cannot be understood exclusively through studies of aGBM.

Common behavioral dilemmas of the school-aged child.

PCPs are typically the first professionals to come into contact with children who have behavioral or developmental problems. The most common behavioral concerns that PCPs encounter in the pediatric primary care setting include noncompliance, temper tantrums, and problems with eating and sleeping routines. To adequately detect these types of problems, it essential that PCPs incorporate behavioral assessment procedures (eg, clinical interviews, behavior rating scales, ABC recording form) into the consultation process. Behavioral assessment procedures are useful in identifying the environmental events (ie, antecedents and consequences) that may be maintaining the problem behavior. Data that are collected from behavioral assessment can be used to develop a tentative hypothesis on functions of the behavioral problem. By identifying the potential function of the behavior, more effective behavioral management strategies can be developed. An existing model of behavioral consultation can be used by PCPs during office visits to more effectively identify and manage many of the common and less severe behavioral problems.

Psychological assessment and treatment of somatization: adolescents with medically unexplained neurologic symptoms.

Adolescent patients who report physical symptoms that are unexplained by physical disease or pathophysiologic processes are prevalent in health care settings. Physical symptoms with no notable physical pathology are often referred to as medically unexplained symptoms (MUS). Common MUS found in adolescent populations include headaches, abdominal pain, back pain, fatigue, dizziness, numbness and tingling sensations in the limbs, and gastrointestinal symptoms. The most important diagnostic concern is the exclusion of neurologic and other general medical conditions. Failure to diagnose real physical pathology appropriately can have serious, deleterious consequences. However, it is also important for physicians to address psychological and other psychosocial factors that may play a role in the etiology or maintenance of MUS. The onus often falls on the primary care physician to screen for such problems and to make cost-effective and appropriate referrals. This article reviews some alternative treatment guidelines for physicians to assist in the assessment, intervention, and referral process for adolescent patients with MUS. The advantages of integrating psychological screening practices into the evaluation process and present recommendations regarding the management of such patients are discussed.

Prion disease resembling frontotemporal dementia and parkinsonism linked to chromosome 17

OBJECTIVE: To compare the clinical features of a familial prion disease with those of frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP-17). BACKGROUND: Prion diseases are not usually considered in the differential diagnosis of FTDP-17, since familial Creutzfeldt-Jakob disease (CJD), the most common inherited prion disease, often manifests as a rapidly progressive dementia. Conversely, FTDP-17 usually has an insidious onset in the fifth decade, with abnormal behavior and parkinsonian features. METHOD: We present the clinical features of 12 patients from a family with CJD associated with a point mutation at codon 183 of the prion protein gene. RESULTS: The mean age at onset was 44.0 Ý 3.7; the duration of the symptoms until death ranged from two to nine years. Behavioral disturbances were the predominant presenting symptoms. Nine patients were first seen by psychiatrists. Eight patients manifested parkinsonian signs. CONCLUSION: These clinical features bear a considerable resemblance to those described in FTDP-17