Malignancy and chemotherapy induced haemophagocytic lymphohistiocytosis in children and adolescents-a single centre experience of 20 years.

Haemophagocytic lymphohistiocytosis (HLH) is a possibly life-threatening syndrome of immune dysregulation and can be divided into primary (hereditary) and secondary forms (including malignancy-associated HLH (M-HLH)). We retrospectively analysed epidemiological, clinical, virological and laboratory data from patients with M-HLH treated at our department between 1995 and 2014. Out of 1.

Successful Unrelated Stem Cell Transplantation in an Infant With Congenital Acute Myelogenous Leukemia FAB M5 Showing Massive Cutaneous Infiltrations--A Challenging Multidisciplinary Approach.

The multidisciplinary management of a male neonate presenting with congenital acute myelogenous leukemia of monoblastic phenotype is reported using conventional chemotherapy, high dose conditioning, and matched unrelated donor stem cell transplantation. These therapies were combined to add a graft versus leukemia effect to the treatment. Although chimerism studies showed a decrease of donor white blood cells, T-cells remained stable of allogeneic origin.

Sirolimus for the treatment of children with various complicated vascular anomalies.

Unlabelled: Vascular anomalies include a heterogeneous group of disorders that are categorized as vascular tumors or vascular malformations. Treatment options include resection, embolization, laser therapy, and sclerotherapy or medical treatment such as propranolol, steroids, interferon, and cytostatic chemotherapy. Mammalian target of rapamycin seems to play a key role in the signal pathway of angiogenesis and subsequently in the development of vascular anomalies.

Unrelated CD3/CD19-depleted peripheral stem cell transplantation for Hurler syndrome.

For patients with mucopolysaccharidosis type IH (MPS1-H; Hurler syndrome), early allogeneic hematopoietic stem cell transplantation (HSCT) is the treatment of choice. One boy and one girl aged 20.5 and 22 months, respectively, with MPS1-H received a conditioning regimen consisting of thiotepa, fludarabine, treosulfan, and ATG.

Amphotericin B transfer to CSF following intravenous administration of liposomal amphotericin B.

Objectives: Although amphotericin B (AmB) and its lipid formulations are used for the treatment of fungal infections of the CNS, the kinetics of AmB in the CSF after intravenous administration of liposomal amphotericin B (LAmB) are not well characterized.

Patients And Methods: From 14 paediatric haemato-oncological patients (aged 0.4-19.

Short-term intravenous fish-oil emulsions in pediatric oncologic patients--effect on liver parameters.

Pediatric oncologic patients often need parenteral nutrition (PN) during chemotherapy. Long-term use of soybean-based lipid emulsions is associated with progressive liver disease and cholestasis, whereas fish-oil based emulsions have anticholestatic effects. We studied the potentially hepato-protective effects of short-term use of SMOF lipids in children undergoing chemotherapy.

Paley's multiplier method does not accurately predict adult height in children with bone sarcoma.

Background: The majority of patients with osteosarcoma and Ewing's sarcoma are diagnosed before skeletal maturity. Paley's multiplier is used for height prediction in healthy children, and has been suggested as a method to make growth predictions for children with osteosarcoma and Ewing's sarcoma when considering limb salvage options. To our knowledge, no evaluation of this method in this particular patient group has been performed, but a temporary growth deficit has been observed in children undergoing chemotherapy.

Age- and gender-related differences in teicoplanin levels in paediatric patients.

Objectives: Teicoplanin is a glycopeptide antibiotic active against Gram-positive bacteria, including methicillin-resistant staphylococci. While teicoplanin trough levels (TTLs) >10 mg/L are commonly considered appropriate, levels >20 mg/L are aimed for in the treatment of severe infections. Due to toxicity, it is recommended to avoid levels >60 mg/L.

Add-on-therapy with bevacizumab in children and adolescents with poor prognosis non-CNS solid tumors.

Bevacizumab is increasingly being used in adult patients with cancer and children with central nervous system (CNS) tumors. Little, however, is known about the efficacy, risks, and benefits of bevacizumab administration in non-CNS tumors of childhood. The aim of the present study was to report on bevacizumab administered as add-on-therapy for poor prognosis non-CNS solid tumors of childhood and adolescence, including a prospective evaluation of side effects of bevacizumab.

Bone marrow necrosis in a girl with Hodgkin's disease.

Bone marrow necrosis (BMN) is a rare finding in children with malignancy occurring most commonly in children with acute lymphoblastic leukemia. This article describes the first case of a girl who developed BMN during treatment for Hodgkin's disease. During the second cycle of chemotherapy, she experienced sudden profound bone pain in the lumbosacral region associated with elevated levels of lactate dehydrogenase (LDH), fibrin degradation products (D-Dimer), and alkaline phosphatase as well as pancytopenia and leukoerythroblastosis.

Alternative donor HSCT in refractory acquired aplastic anemia - prevention of graft rejection and graft versus host disease by immunoablative conditioning and graft manipulation.

Early alternative donor HSCT is a potentially curative therapeutic option for patients with AAA not responding to IST. Seven patients (median age at diagnosis, 11 yr) with refractory AAA without a MSD underwent HSCT from matched unrelated (n = 6) or haploidentical (n = 1) donors. Conditioning regimens included CY (n = 7), muromonab-CD3/ATG (n = 7), TT (n = 6), FLU (n = 5), and TLI (n = 2).

The spectrum of parvovirus b19 infection in a pediatric hemato-oncologic ward.

Of 1059 children, 35 children with various hemato-oncologic diseases were diagnosed with parvovirus B19 infection. The clinical spectrum included 11 immunocompromised patients presenting with prolonged pancytopenia, 7 patients with delayed hematologic recovery after stem cell transplantation, 5 patients with parvovirus B19 as possible cause of severe aplastic anemia or myelodysplastic syndrome, and 12 children with hemolytic anemia and transient aplastic crisis.

Survival and late effects in children with stage 4 neuroblastoma.

Background: Treatment of metastatic neuroblastoma (NB) demands aggressive oncological therapy, which may cause long-term sequelae in survivors. The aim of this retrospective single center study is to give an overview of survival in children with stage 4 NB and to describe the spectrum of late effects seen in survivors.

Procedure: Medical records of 31 patients with stage 4 NB treated between 1984 and 2009, who were included in a follow-up programme, were reviewed for information on tumor, treatment and late effects.

Pheochromocytoma in a 2.75-year-old-girl with a germline von Hippel-Lindau mutation Q164R.

Pheochromocytomas are rare tumors of the adrenal gland occurring sporadically or as part of familial cancer syndromes. Here we report on the case of a pheochromocytoma due to the germline missense mutation c.491A>G (Q164R) in exon 3 of the von Hippel-Lindau gene in a girl as young as 2.

Severe epstein-barr virus encephalitis with hemophagocytic syndrome: rapid clearance of virus following allogeneic hematopoietic stem cell transplantation from a seropositive donor.

Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis with encephalitis is a life-threatening condition that requires treatment with immunochemotherapy; refractory patients are eligible for allogeneic hematopoietic stem cell transplantation. We report on an adolescent female who failed to respond to induction immunochemotherapy and was salvaged by allogeneic hematopoietic stem cell transplantation from her human leukocyte antigen-identical, Epstein-Barr virus-seropositive brother leading to rapid clearance of Epstein-Barr virus from blood and cerebrospinal fluid.

Peripheral blood stem cell mobilization with pegfilgrastim compared to filgrastim in children and young adults with malignancies.

Background: Pegfilgrastim, the long acting agent of rh-GCSF, has been shown to be as effective as Filgrastim in children undergoing cytotoxic chemotherapy by reducing the duration of neutropenia. Recent studies in adults have also shown that Pegfilgrastim is effective to mobilize CD34+ stem cells, resulting in earlier peripheral stem cell collections (PSCC). The aim of the study was to compare the efficacy of Pegfilgrastim with Filgrastim for CD34+ stem cell mobilization in children.

Medulloblastoma in a child with down syndrome: long-term remission with multimodality treatment.

A 4(3/4)-year-old male with Down syndrome (DS) presented with unsteady gait and fatigue. Neuroimaging revealed a cerebellar mass with concomitant obstructive hydrocephalus and additional metastatic lesions. He was successfully treated and is still in complete remission 5 years from diagnosis.

Intracranial reparative giant cell granuloma secondary to cholesteatoma in a 15-year-old girl.

A 15-year-old girl presented with pain in the right ear and acute onset of total peripheral facial nerve palsy. Imaging studies revealed an intracranial mass of the right temporal bone causing temporal lobe displacement. A first biopsy led to the diagnosis of intracranial giant cell reparative granuloma (GCRG), a rare benign tumor of the bone or soft tissue that can show expansive growth.

Management of brain abscesses in children treated for acute lymphoblastic leukemia.

Brain abscesses in children with leukemia or other malignancies are rare and potentially fatal. We report on four children who developed brain abscesses during treatment for acute lymphoblastic leukemia (ALL). All patients received multimodal broad-spectrum antibiotic therapy and liposomal amphotericin-B in combination with hyperbaric oxygen.

A scoring system to quantify late effects in children after treatment for medulloblastoma/ependymoma and its correlation with quality of life and neurocognitive functioning.

Background: The aim of this study was to quantify the severity of late effects by a simple numerical score (late effects severity score, LESS) in patients who received radiochemotherapy for medulloblastoma or ependymoma. The LESS was correlated with neurocognitive and quality of life (QoL) outcomes.

Patients And Methods: The LESS was calculated by assigning 0, 1, or 2 points within each of four different categories (neurology, endocrine, visual/auditory, others).

Effective management of pulmonary aspergillosis invading the thoracic spine in a child with high risk ALL requiring allogeneic bone marrow transplantation.

Due to unacceptably high mortality, invasive fungal infections (IFI) have long been considered a contraindication against allogeneic stem cell transplantation. Despite severe immunosuppression an 11-year-old girl requiring allogeneic bone marrow transplant (BMT) for relapsed acute lymphoblastic leukemia was cured of a concurrent invasive pulmonary aspergillosis. Treatment comprised combinations of liposomal amphotericin B, caspofungin and voriconazole with donor granulocyte transfusions.

Neuroophthalmological side effects following intrathecal administration of liposomal cytarabine for central nervous system prophylaxis in three adolescents with acute myeloid leukaemia.

Three adolescents with central nervous system (CNS) negative acute myeloid leukaemia (AML) refused cranial irradiation for CNS prophylaxis. Instead, these patients received four doses of 50 mg of intrathecal (IT) liposomal cytarabine on day 1, 15, 43 and 71 of maintenance therapy. Corticosteroids were given to prevent chemical arachnoiditis.