We investigated 52 of 457 patients with congenital factor deficiencies with 57 episodes of intracranial haemorrhage (ICH) between 1998 and 2007. There were 38 severe haemophiliacs, 6 with factor XIII deficiency, 5 with factor X deficiency, 2 factor V-deficient patients, and 1 with type 3 von Willebrand disease (VWD). The median age was 8 years (range 1 month-22 years). Most patients were below 15 years of age (86.5%). All patients with factor X deficiency were between 1 and 5 months of age. ICH was the primary bleeding episode leading to detection of factor deficiency in 19.2% (five patients with severe haemophilia and all patients with factor X deficiency). Trauma caused bleeding in 66%. None of the patients with factor X deficiency had history of prior trauma. Surgery was performed in five patients with subdural haematomas, all of whom survived. Conservative factor replacement with 100% correction for 3 days followed by 50-60% correction for 7 days was possible in 60% patients. Seizures requiring prolonged therapy were noted in eight patients. Death was recorded in 15 patients (29%). Inadequate therapy in the form of delay or insufficient replacement was noted in 7/15 deaths. ICH was seen in 11.3% of all patients with coagulation factor deficiencies. Factor X deficiency presented with ICH at an earlier age. Inadequate replacement therapy including delayed treatment caused nearly 50% of all deaths. Most patients can be managed satisfactorily with adequate replacement therapy alone, with surgery being reserved for those with worsening neurological conditions.

Key Points Adults with SCD have an increased incidence of VTE, but similar data in children are lacking. In this 7-year, multicenter retrospective study, 1.7% of children with SCD developed VTE.

Ketamine hydrochloride is a safe and rapid-acting non-opioid, lipid soluble anaesthetic with a short elimination halflife that is used for medical and veterinary purposes. It produces a state of "dissociative anaesthesia", probably from action on N-methyl-D-aspartate (NMDA) receptors. The psychotropic effects of ketamine range from dissociation and depersonalization to psychotic experiences and include a sensation of feeling light, body distortion, absence of time sense, novel experiences of cosmic oneness and out-of-body experiences. Abuse of ketamine has been reported, the typical abuser being an individual who uses multiple drugs and has some contact with medical agencies. This case demonstrates the effects of large doses of ketamine in a person with polysubstance abuse. The case also highlights development of significant tolerance to ketamine without prominent withdrawal symptoms. Caution in use of ketamine is reiterated in light of its abuse liability.

Histiocytic sarcoma (HS) is a rare malignancy of tissue histiocytes with a dismal prognosis. We report a 4-year-old male who developed HS during maintenance chemotherapy for precursor B-cell acute lymphoblastic leukemia (pre-B ALL). Both tumors showed identical clonal immunoglobulin and T-cell receptor gene re-arrangement patterns, as well as homozygous deletion of the CDKN2A gene encoding p16(INK4A). These data suggest a clonal relationship between the two neoplasms despite their distinct lineages. Since CDKN2A deletion predisposes to development of HS in experimental models, the cytogenetic features of the patient's pre-B ALL may have predisposed to this change in lineage.