Hemophagocytic lymphohistiocytosis mimicking surgical symptoms and complications: lessons learned from four cases.

OBJECTIVE: Hemophagocytic lymphohistiocytosis (HLH) is a severe immunological disorder that leads to a massive inflammatory reaction that may prove rapidly fatal. We show that HLH may present by masquerading as surgical disease or as a postoperative complication leading to delays in diagnosis and treatment. STUDY DESIGN: A case series of four children with acute surgical presentation and prolonged unexplained postoperative sepsis, who were diagnosed with HLH. RESULTS: Four children with different clinical presentations (1. neonatal abdominal distension, 2. ileostomy closure and Hirschsprung's disease, 3. iatrogenic sigmoid perforation and Crohn's disease, and 4. streptococcal toxic shock syndrome with primary peritonitis) were diagnosed with HLH at our regional pediatric surgical centre in the last two years. All developed signs of prolonged postoperative sepsis with hepatosplenomegaly and pancytopenia, requiring intensive care support. In the absence of explanation for their symptoms and deteriorating clinical condition, a total of six 'negative' exploratory laparotomies were performed. Eventually, HLH was diagnosed with bone marrow aspiration after an average of 23 days (range 17-40), following the finding of significantly elevated ferritin (up to 293150 ng/ml) and triglyceride levels. All children improved with initiation of high-dose steroid treatment followed by etoposide and cyclosporin. CONCLUSION: HLH may rarely present with symptoms and signs of surgical disease or complicate post-operative recovery. This diagnosis should be considered in children with unexplained prolonged fever, hepatosplenomegaly and pancytopenia, especially if associated with high ferritin levels. HLH can prove rapidly fatal without appropriate treatment.

Early UK experience in the use of clofarabine in the treatment of relapsed and refractory paediatric acute lymphoblastic leukaemia.

Clofarabine is a second-generation purine nucleoside analogue, which has shown promising activity in relapsed and refractory paediatric acute lymphoblastic leukaemia (ALL). This report summarizes the early United Kingdom experience of clofarabine for the treatment of paediatric ALL in 23 patients, outside of the context of a clinical trial. Our results demonstrated that clofarabine-based chemotherapy regimes were effective and well-tolerated in this heavily pre-treated group, with an overall response rate of 67% when used in combination regimes. Responses were seen in both B and T cell disease and in patients with adverse cytogenetics.

Effects of early atipamezole reversal of medetomidine-ketamine anesthesia in mice.

Rodents are often anesthetized by using ketamine and medetomidine, with reversal by atipamezole. Methods vary for times of administration of the atipamezole, and literature is lacking regarding appropriate reversal time. We investigated the recovery of mice reversed with atipamezole 10 min (early) or 40 min (late) after induction of anesthesia. Time to regain pinch-reflex or righting reflex did not differ between the 2 reversal points, but time to walking was significantly greater in mice that underwent early reversal with atipamezole. This delay was not mitigated by administration of atropine as part of the anesthetic regimen. Inclusion of acetylpromazine in the anesthetic regimen shortened the time needed to reach a surgical plane of anesthesia but also prolonged recovery times as determined by righting reflex and time to walking.

Reducing blood loss in synostosis surgery: the Liverpool experience.

Correction of craniosynostosis represents much of the workload in the pediatric designated UK craniofacial units. We reviewed recent operations as part of an ongoing unit audit cycle with outcome determined as blood use and complications or readmissions within 6 weeks of surgery. A pro forma was designed, and information from a chart search was collated on a Microsoft Excel (Microsoft, Seattle, WA) spreadsheet. Thirty-four patients were treated between March 2005 and December 2006, including 13 who underwent scaphocephaly correction (SC) and 21 who underwent fronto-orbital advancement and remodeling (FOAR). In the FOAR group, 13 patients received continuous autologous transfusion, and 8 patients did not. Continuous autologous transfusion system was not used in any of the patients who underwent SC. The results showed that allogeneic transfusion rates were 46 +/- 36% red cell volume (RCV) in FOAR when a cell saver was used and 50 +/- 21% RCV when it was not. There was no significant difference between each group; however, 25% of patients received no transfusion in the cell saver group, whereas all patients undergoing FOAR were transfused in the non-cell saver group. Transfusion in SC was calculated as 26 +/- 25% RCV, and 33% of patients underwent SC without transfusion. In conclusion, we show that significant reductions in blood transfusion are possible in correction of craniosynostosis both with and without the use of cell savers by application of a simple protocol. Our findings support the recommendation that all of these cases be carried out in multidisciplinary units where high patient throughput allows both maintenance of skills and completion of audit cycles.

Chickenpox in varicella IgG positive patients: experience of a regional paediatric oncology centre.

Aciclovir prophylaxis was previously given to all immunocompromised patients treated by our unit, following contact with varicella zoster. In 2003, we changed practice according to National Guidelines, giving prophylaxis only to patients without serum varicella zoster immunoglobulin G antibody (VZ IgG) at diagnosis of their malignancy. Since then we have seen nine patients with acute lymphoblastic leukaemia (ALL) and VZ IgG positivity at diagnosis of their malignancy develop chickenpox. Our observations question current practice for patients with ALL.

Non-myeloablative transplantation for severe congenital neutropenia.

Severe Congenital Neutropenia is a rare condition characterized by a very low neutrophil count, which pre-disposes the affected child to recurrent bacterial infections. Treatment with granulocyte colony stimulating factor (G-CSF) has dramatically improved the prognosis of these children; but in patients who have become G-CSF refractory, hematopoeitic stem cell transplant is still the only effective curative treatment. We describe a patient who was unresponsive to escalating doses of G-CSF and underwent a successful reduced intensity conditioning, matched unrelated donor allograft resulting in cure.

Factor X deficiency presenting as a pseudotumor. Case report.

The authors report their experience in successfully treating a 15-week-old child who became comatose following a spontaneous intracerebral hemorrhage. It was initially believed that a tumor in the right frontal lobe caused the hemorrhage. Coagulation studies revealed abnormal results on presentation, and the problem was only partially corrected after an infusion of fresh frozen plasma. The child underwent an emergency craniotomy in which the hematoma was evacuated, and a biopsy specimen was obtained from a firm mass at the base of the hematoma cavity. Postoperatively, the child recovered completely, and an analysis of detailed coagulation studies revealed that the child had a factor X deficiency. Histological analysis of the biopsy specimen revealed normal brain tissue with hemorrhagic infiltration. Subsequently, the child achieved normal developmental milestones. A diagnosis of congenital bleeding disorder should be considered in children with spontaneous intracerebral hemorrhage, even in those with no prior episode of extracerebral spontaneous hemorrhage.