Fernando Sánchez-Zubieta

PROCALCITONIN AND C-REACTIVE PROTEIN SERUM LEVELS AS MARKERS OF INFECTION IN A PEDIATRIC POPULATION WITH FEBRILE NEUTROPENIA AND CANCER

Background: Procalcitonin and C-reactive-protein are inflammatory markers for sepsis. The authors evaluated their sensitivity and specificity in pediatric patients with cancer and febrile neutropenia. Procedure: Serum procalcitonin and C-reactive-protein were evaluated. Patients (n = 54) were divided into 2 groups, with severe infection (n = 18) or without documented infection (n = 36). Results: Procalcitonin and C-reactive protein were significantly higher in the high-risk group. Procalcitonin displayed 72.2% sensitivity and 80.5% specificity. C-reactive-protein had a sensitivity of 77.7% and specificity of 77.2%. Conclusions: Procalcitonin is an accurate predictor of bacterial infection in neutropenic children, while C-reactive-protein may be a better screening test in emergency settings.

Improved treatment results in Mexican children with acute myeloid leukemia using a Medical Research Council (MRC)-acute myeloid leukemia 10 modified protocol

We analysed the results of three protocols from 1990 to 2005. Protocol I (1990–1996) consisted of a 2 year VAPA regime. Protocol II (1996–2003) on 1 year daunorubicin/cytarabine alternating with etoposide/cytarabine. Protocol III (2003–2005) on six cycles MRC AML 10 modified. Patients with de novo acute myeloid leukemia 0 to 18 years were included. Demographic and clinical characteristics were analysed. Patients with >100,000 leukocytes, M4 or M5 and primary CNS disease were considered high risk. We compared remission rate, overall and event-free survival. Descriptive statistics, chi square, Kaplan-Meier and long rank tests were used. One hundred forty-five patients were included, 46 in Protocol I; 60 in II and 39 in III. There were no differences in characteristics between groups, except for more low risk patients in Protocol II (61%vs. 43% and 41%. (p = 0.05). Remission rate for Protocol I was 52%, for II 50% and for III 92% (p = 0.0001). Relapse was 18, 30 and 35, respectively (p = 0.141). Five-year event-free survival was 17.9% ± 6.6%, 15.5% ± 4.1% and 43.5% ± 4.1% (s.e) (p = 0.0002). Five-year overall survival was 19.5% ± 8%, 17.2% ± 5.9% and 51.2% ± 4.1% (s.e) (p = 0.0002). The results were superior in the MRC-10 derived protocol.

Hepatoblastoma in a patient with Goldenhar syndrome born to a diabetic mother

J. Román Corona-Rivera,* Fernando Sánchez-Zubieta, Noemı́ Silva-Padilla, Oscar González-Ramella, Eloy López-Marure, Ezequiel Vélez-Gómez, Jaime Orozco-Pérez, Verónica Soto-Chávez, and Alfredo Corona-Rivera Servicio de Genética Pediátrica, Hospital Civil de Guadalajara ‘‘Dr. Juan I. Menchaca’’, Hospital-Escuela, Guadalajara, México Servicio de Oncologı́a y Hematologı́a Pediátrica, Hospital Civil de Guadalajara ‘‘Dr. Juan I. Menchaca’’, Hospital-Escuela, Guadalajara, México Servicio de Radiologı́a Pediátrica, Hospital Civil de Guadalajara ‘‘Dr. Juan I. Menchaca’’, Hospital-Escuela, Guadalajara, México División de Patologı́a, Hospital Civil de Guadalajara ‘‘Dr. Juan I. Menchaca’’, Hospital-Escuela, Guadalajara, México Servicio de Cirugı́a Oncológica, División de Pediatrı́a, Hospital Civil de Guadalajara ‘‘Fray Antonio Alcalde’’, Guadalajara, México Instituto de Genética Humana ‘‘Dr. Enrique Corona Rivera’’, Centro Universitario de Ciencias de la Salud, Universidad de Guadalajara, Guadalajara, México

Pediatric donor cell leukemia after allogeneic hematopoietic stem cell transplantation in AML patient from related donor

Here we present a male patient with acute myeloid leukemia (AML) initially diagnosed as M5 and with karyotype 46,XY. After induction therapy, he underwent a HLA-matched allogeneic hematopoietic stem cell transplantation, and six years later he relapsed as AML M1 with an abnormal karyotype //47,XX,+10[2]/47,XX,+11[3]/48,XX,+10,+11[2]/46,XX[13]. Based on this, we tested the possibility of donor cell origin by FISH and molecular STR analysis. We found no evidence of Y chromosome presence by FISH and STR analysis consistent with the success of the allogeneic hematopoietic stem cell transplantation from the female donor. FISH studies confirmed trisomies and no evidence of MLL translocation either p53 or ATM deletion. Additionally 28 fusion common leukemia transcripts were evaluated by multiplex reverse transcriptase-polymerase chain reaction assay and were not rearranged. STR analysis showed a complete donor chimerism. Thus, donor cell leukemia (DCL) was concluded, being essential the use of cytological and molecular approaches. Pediatric DCL is uncommon, our patient seems to be the sixth case and additionally it presented a late donor cell leukemia appearance. Different extrinsic and intrinsic mechanisms have been considered to explain this uncommon finding as well as the implications to the patient.

Variantes fenotípicas menores en pacientes con leucemia linfoblástica aguda del occidente de México

INTRODUCTION Acute lymphoblastic leukemia (ALL) has been associated with an excess of minor phenotypic variants (MPV), including common variants and minor anomalies, indicative of an altered phenogenesis. The objective of the study was to determine the association between MPV and ALL. PATIENTS AND METHODS In a hospital based case-control study, we studied 120 children with ALL (including standard and high risk) and 120 healthy children as a control group, matched for age and sex, seen in the Hospital Civil de Guadalajara Dr. Juan I. Menchaca (Guadalajara, Mexico). In both groups, 28 anthropometric measurements were made, as well as a systematic search for 405 MPV, through a physical examination. Adjusted odds ratio was estimated (aOR) with its intervening variables by logistic regression. The confidence interval was 95% (95%CI). RESULTS Anthropometric signs associated with ALL were: long upper segment (aOR= 2.19, 95%CI: 1.01-4.76), broad jaw (aOR= 2.62, 95%CI: 1.29-5.30), narrow ears (aOR= 6.22, 95%CI: 2.60-14.85), and increase in internipple distance (aOR= 2.53, 95%CI: 1.07-5.98). The hypoplasia mesofacial, broad forehead, small nose, short columella, narrow ears, telethelia, Sydney crease (SC), Greek type feet and café-au-lait spots (CALS), had a 3 to 17 times higher frequency in children with ALL. By number, an association was found from ≥4 MPV (aOR= 2.14, 95%CI: 1.25-3.66, P=.004). CONCLUSIONS From ≥4 MPV, an association was found with ALL, suggesting prenatal factors in phenogenesis and leukemogenesis. CALS and SC were confirmed as MPV in children with ALL.

A translocation t(5;15)(q15;q11-13) infant case with acute lymphoblastic leukemia and literature review: prognosis implications.

Infant acute lymphoblastic leukemia (ALL) represents poor prognosis despite intensive chemotherapy. Rearrangements of chromosome 11q23 are not observed in 34% of the cases. Infant ALL patients with t(5;15)(p15;q11-13) are rare and sporadic. In large series of infant ALL studies, 6 patients have been reported. We present a new case of an infant ALL patient with t(5;15)(p15;q11-13), and a literature review. Considering the data provided by our case and previous reports, we reinforce that this chromosomal abnormality is characteristic of ALL patients under 12 months of age sharing break point in 5p15 and 15q11-13 and strengthen the existence of an infant ALL subgroup characterized by pre-B L1 ALL, CD10-positive, complete remission (100%), and event-free survival (71%), with a relatively good prognosis and clearly less severe than the 11q23 rearrangement cases. This abnormality can be considered a recurrent abnormality on this nosologic group.

Estrategias de afrontamiento del equipo multiprofesional ante la muerte del paciente pediátrico con cáncer

The aim of the study was to determine how the multidisciplinary team faces the death of cancer patients through coping strategies. For this, the Spanish version of the Coping Strategies Inventory was applied to 66 health professionals in the Department of Pediatric Hematology- Oncology of a tertiary hospital. The team of participants was composed of the medical staff, nursing, social work, psychology, nutrition and cleaning employees. Statistical analysis was performed through SPSS version 20 program. Variables were compared using T tests and ANOVA. Results are discussed and we find that the strategy that was most employed is Problem solving, which is present in all participants, while self-criticism was a less common strategy. We conclude that at the death of the patient, the multidisciplinary team in the first instance uses problem solving strategy evenly, pro and independently of the profession; while differences in the use of coping strategies are related to socio-demographic variables of participant as age, marital status, schedule and job seniority.