Pancytopenia Due to Vitamin B12 and Folic Acid Deficiency—A Case Report

peer reviewedWe report a case of severe pancytopenia in a 15-year-old patient due to a severe deficiency in vitamin B12 and folic acid, probably of nutritional origin. The clinical and biological course was favorable after vitamin supplementation. With this case, we discuss the diagnostic approach of pancytopenia with megaloblastic anemia in children and adolescents, as well as the mechanisms involved in vitamin B12 and B9 deficiency. Hypovitaminosis B12 is known in its severe form but its diagnosis is often made difficult by insidious signs and symptoms. Traditional intramuscular replacement therapy has now proven to be effective orally. The clinical manifestations of folic acid deficiency are relatively similar to those of vitamin B12 deficiency, reflecting their intricate co-enzymatic functions. Its supplementation is administered orally

Angiogenèse dans la leucémie aiguë lymphoblastique de l enfant (étude des facteurs angiogéniques urinaires sur 116 cas)

Les études actuelles sur les LAL de l enfant cherchent à isoler de nouveaux facteurs pronostiques permettant de fournir les traitements les plus adaptés à chaque LAL. Par ailleurs, il est maintenant admis que les LAL sont dépendantes de l angiogenèse. La littérature témoigne d une grande variabilité dans l interprétation des dosages de facteurs angiogéniques. Il serait intéressant de pouvoir attribuer des valeurs pronostiques précises aux principaux facteurs pro-angiogéniques retrouvés dans les LAL de l enfant. L objectif principal de ce travail était de vérifier le mauvais pronostic associé à des dosages urinaires élevés de VEGF et bas de bFGF au diagnostic de LAL. Les taux urinaires ont été comparés aux critères pronostiques et à l évolution des patients. Des corrélations ont été recherchées avec les caractères cliniques et biologiques des patients. Un des derniers objectifs était d établir des sous-populations cibles pour un éventuel traitement anti-angiogénique. Entre 1998 et 2010, des dosages urinaires de VEGF et bFGF ont été réalisés chez 116 enfants au diagnostic de LAL. Parmi eux, 101 patients étaient suivis pour une LAL de type B, et 15 suivis pour une LAL de type T. Le groupe témoin se composait de 21 enfants en bonne santé. Les résultats ne correspondent pas tous à ceux obtenus dans les études précédentes. Le taux de VEGF apparaît plus bas en cas de LAL T par rapport à celui des témoins (p < à 0,001). Le taux de VEGF est significativement plus élevé en cas de LAL B d évolution défavorable comparé aux LAL B d évolution favorable (p à 0,03), notamment en cas de mort directement liée à la maladie. Le taux de bFGF est plus élevé en cas de LAL B avec translocation t(9;22) par rapport à celui de la population globale des LAL B (p à 0,03). Une corrélation positive est décrite entre l élimination urinaire de VEGF et celle du bFGF. L élimination urinaire de VEGF est inversement corrélée au taux d hémoglobine dans les LAL T. Notre étude confirme le mauvais pronostic associé à un taux élevé de VEGF au diagnostic de LAL B. Comme déjà évoqué par certains auteurs, l angiogenèse semble être impliquée différemment selon l immunophénotype et la cytogénétique de la LAL. Les sous-populations susceptibles de bénéficier d un traitement anti-angiogénique seraient les enfants atteints de LAL B présentant au diagnostic un taux élevé de VEGF et/ou une translocation t(9;22). Il est toutefois nécessaire de réaliser des études multicentriques pour élargir les sous groupes de LAL B avec chromosome Philadelphie et de LAL T, afin de mieux cibler les populations pouvant bénéficier réellement d un traitement anti-angiogénique.ROUEN-BU Médecine-Pharmacie (765402102) / SudocSudocFranceF

Pediatric refractory acute immune thrombocytopenic purpura secondary to asymptomatic SARS-CoV2.

editorial reviewedAcute immune thrombocytopenic purpura (ITP) has been revealed as an uncommon complication of COVID-19 in children. Severe bleeding may occur but is rarely life threatening. Management is based on the severity of bleeding symptoms and the degree of thrombocytopenia. We report the case of a 7-year-old girl with severe acute ITP secondary to a COVID-19 infection -without any respiratory symptoms. The initial clinical examination showed a large bulging mediodorsal hematoma, purpuric lesions, and posterior pharyngeal hemorrhage. The patient was monitored in a pediatric intensive care unit. Initial medical management consisted of intravenous immunoglobulins and systemic steroids. Despite this treatment, bleeding and thrombocytopenia worsened, and secondary macroscopic haematuria occurred, requiring 6-hourly platelet transfusions and increased steroid doses to obtain sufficient hemostasis. This case presents a rare and severe acute pediatric ITP secondary to asymptomatic SARS-COV2 which was refractory to initial management and opens the discussion to second line therapeutic interventions

Cervicothoracic neuroblastomas : benefits of transmanubrial osteomuscular-sparing approach

peer reviewedWe report the case of a child suffering from a neonatal cervicomediastinal neuroblastoma encasing the left subclavian artery and the left vertebral artery. There is only a few pediatric tumors extending from the neck to the upper part of the thorax. Because of the complex vascular and neurological anatomy of this area, the surgical excision of these cervicothoracic neuroblastomas is a real challenge. It is why, when we decided to propose a surgical management, we used the Transmanubrial Osteomuscular-Sparing Approach (TOSA), of which technique and benefits will be explained in this article

Langerhans Cell Histiocytosis With Vertebral Involvement Diagnosed and Treated Over the Last 15 Years in a Single Canadian Pediatric Academic Institution.

We report 11 children with vertebral lesion of Langerhans cell histiocytosis (LCH) diagnosed and treated between 2000 and 2015. Vertebral lesions were usually present at LCH diagnosis. No child developed neurological symptoms. Among 29 vertebral lesions, only 2 were unstable. Chemotherapy was used in all children but 3. A LCH recurrence was observed in 6 patients, involving vertebrae in 4 cases. All children were disease-free at their last follow-up. Sequelae were more often radiologic than clinical. Since potential recurrences and incomplete bone regeneration exist, discussion about optimal treatment and long-term follow-up of vertebral lesions are essential

Gray Zone Lymphoma Arising in the Neck of a Teenager With a Germline Mutation in TP53.

Gray zone lymphoma is an aggressive disease for which appropriate management is still debated. We report a 15-year-old girl with a cervical mass, an enlarged ipsilateral tonsil, and anemia. Both sites showed hypermetabolism on F18-FG positron emission tomography/CT. Surgical resection was diagnostic of Epstein-Barr virus-negative gray zone lymphoma cervical and tonsillar involvement. No abnormality was found in cytogenetic analysis on tumor cells. However, exome sequencing in peripheral blood DNA revealed a germline mutation in TP53. Complete response was achieved after surgery and 6 cycles of rituximab with dose-adjusted etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin regimen

The rise of targeted therapies in pediatric oncology

peer reviewe

What follow-up after pediatric cancer ? The SALTO consultation experience

peer reviewedDuring the past 50 years, the mortality due to childhood cancers decreased dramatically thanks to improvements in supportive care and the use of multimodal approaches. In this context, the long-term follow up after childhood cancer has become a main concern for pediatric oncologists. The SALTO programme was developed in 2012 at the CHR Citadelle in Liège in order to organize a multidisciplinary long-term follow-up for the patients previously treated in our department for a childhood cancer. The aim of the present study was to review, for the most frequent childhood cancers, the long-term sequellae and the second cancers developed by the patients participating to the SALTO programme in order to define the follow-up needed. Our data confirm the importance of a multidisciplinary long-term follow-up, based on the treatments received and following international guidelines

High Risk of Anal and Rectal Cancer in Patients With Anal and/or Perianal Crohn’s Disease

International audienceBackground & AimsLittle is known about the magnitude of the risk of anal and rectal cancer in patients with anal and/or perineal Crohn’s disease. We aimed to assess the risk of anal and rectal cancer in patients with Crohn’s perianal disease followed up in the Cancers Et Surrisque Associé aux Maladies Inflammatoires Intestinales En France (CESAME) cohort.MethodsWe collected data from 19,486 patients with inflammatory bowel disease (IBD) enrolled in the observational CESAME study in France, from May 2004 through June 2005; 14.9% of participants had past or current anal and/or perianal Crohn’s disease. Subjects were followed up for a median time of 35 months (interquartile range, 29–40 mo). To identify risk factors for anal cancer in the total CESAME population, we performed a case-control study in which participants were matched for age and sex.ResultsAmong the total IBD population, 8 patients developed anal cancer and 14 patients developed rectal cancer. In the subgroup of 2911 patients with past or current anal and/or perianal Crohn’s lesions at cohort entry, 2 developed anal squamous-cell carcinoma, 3 developed perianal fistula–related adenocarcinoma, and 6 developed rectal cancer. The corresponding incidence rates were 0.26 per 1000 patient-years for anal squamous-cell carcinoma, 0.38 per 1000 patient-years for perianal fistula–related adenocarcinoma, and 0.77 per 1000 patient-years for rectal cancer. Among the 16,575 patients with ulcerative colitis or Crohn’s disease without anal or perianal lesions, the incidence rate of anal cancer was 0.08 per 1000 patient-years and of rectal cancer was 0.21 per 1000 patient-years. Among factors tested by univariate conditional regression (IBD subtype, disease duration, exposure to immune-suppressive therapy, presence of past or current anal and/or perianal lesions), the presence of past or current anal and/or perianal lesions at cohort entry was the only factor significantly associated with development of anal cancer (odds ratio, 11.2; 95% CI, 1.18-551.51; P = .03).ConclusionsIn an analysis of data from the CESAME cohort in France, patients with anal and/or perianal Crohn’s disease have a high risk of anal cancer, including perianal fistula–related cancer, and a high risk of rectal cancer