Acute myeloid leukaemia (AML) is a disorder characterized by a clonal proliferation derived from primitive haematopoietic stem cells or progenitor cells. Abnormal differentiation of myeloid cells results in a high level of immature malignant cells and fewer differentiated red blood cells, platelets and white blood cells. The disease occurs at all ages, but predominantly occurs in older people (>60 years of age). AML typically presents with a rapid onset of symptoms that are attributable to bone marrow failure and may be fatal within weeks or months when left untreated. The genomic landscape of AML has been determined and genetic instability is infrequent with a relatively small number of driver mutations. Mutations in genes involved in epigenetic regulation are common and are early events in leukaemogenesis. The subclassification of AML has been dependent on the morphology and cytogenetics of blood and bone marrow cells, but specific mutational analysis is now being incorporated. Improvements in treatment in younger patients over the past 35 years has largely been due to dose escalation and better supportive care. Allogeneic haematopoietic stem cell transplantation may be used to consolidate remission in those patients who are deemed to be at high risk of relapse. A plethora of new agents - including those targeted at specific biochemical pathways and immunotherapeutic approaches - are now in trial based on improved understanding of disease pathophysiology. These advances provide good grounds for optimism, although mortality remains high especially in older patients.

pdf/acute-myeloid-leukaemia-ude1qpwnsn.pdf

Acute myeloid leukaemia.

Neurofibromatosis type 2

The diagnostic evaluation and multidisciplinary management of neurofibromatosis 1 and neurofibromatosis 2

ObjectiveEarly studies suggest that bevacizumab treatment can result in tumor shrinkage and hearing improvement for some patients with neurofibromatosis type 2 (NF2). The aim of this study was to report extended follow-up in a larger cohort of similarly treated patients.Study DesignRetrospective stu

/pdf/bevacizumab-for-progressive-vestibular-schwannoma-in-2646442q0m.pdf

Bevacizumab for progressive vestibular schwannoma in neurofibromatosis type 2: a retrospective review of 31 patients.

Objective(s) To evaluate the clinical effectiveness and cost-effectiveness of a range of diagnostic strategies for investigating patients with unilateral hearing loss and/or tinnitus, with a view to confirming or eliminating a diagnosis of acoustic neuroma, and to describe the natural history of acoustic neuroma. Data sources Major electronic databases were searched from January 1980 to August 2008. Review methods Selected studies were assessed and subjected to data extraction and quality assessment using standard methods. Results Studies comparing auditory brainstem response (ABR) with magnetic resonance (MR) imaging were highly heterogeneous. ABR has high sensitivity compared with MR imaging for acoustic neuromas greater than 1 cm in size but not for smaller neuromas. The sensitivities of T2-weighted (T2W) and T2-star-weighted (T2*W) imaging strategies compared with gadolinium-enhanced T1-weighted (GdT1W) MR imaging (gold standard) were high and relatively homogeneous. The specificity of T2W and T2*W studies ranged from 90% to 100% and from 86% to 99% respectively. The review of cost-effectiveness showed that GdT1W MR imaging immediately or in conjunction with ABR appears to be more cost-effective than 'traditional' protocols; ABR/GdT1W MR imaging protocols were more cost-effective than going directly to GdT1W MR imaging. Non-contrast-enhanced MR imaging was found to be a more cost-effective test for acoustic neuroma than GdT1W MR imaging. The incidence of acoustic neuroma has increased over the last 30 years, with the median age at diagnosis remaining at 55 years. Most patients present with insidious symptoms of unilateral hearing impairment, tinnitus and/or vertigo. The pattern and rate of growth of acoustic neuroma are highly variable and currently unpredictable. At least 50% of tumours do not grow, at least for some years after diagnosis. Some studies have found large initial size to be a determinant of later growth, with the opposite also being reported. The mean growth rate for all tumours varies between 1 and 2 mm/year, with a rate of 2-4 mm/year for only those that grow; however, there are cases with significant regression (5%) or exceptional growth (which may exceed 18 mm/year). Conclusions The majority of the evidence reviewed was poorly reported and there is therefore an inherent risk of bias. Given the recent improvement in resolution and reduction in cost of MR imaging, ABR can no longer be considered appropriate as the primary test used to screen for acoustic neuroma. T2W or T2*W sequences enable accurate evaluation of the VIIIth and VIIth cranial nerves within the cerebellopontine angle and internal auditory canal as well as evaluation of the cochlea and labyrinth, and inclusion of GdT1W sequences is unlikely to contribute information that would alter patient management in the screening population. The quality of the imaging chain and experience of the reporting radiologist are key factors determining the efficacy of a non-contrast screening strategy. Based on a cost-effectiveness model developed to reflect UK practice it was concluded that a diagnostic algorithm that deploys non-contrast MR imaging as an initial imaging screen in the investigation of acoustic neuroma is less costly than and likely to be as effective as available contrast MR imaging.

https://njl-admin.nihr.ac.uk/document/download/2001731

The role of magnetic resonance imaging in the identification of suspected acoustic neuroma: a systematic review of clinical and cost effectiveness and natural history.

Objective Describe the changes in hearing ability and progression of disease over time in subjects with neurofibromatosis Type 2 enrolled in a multicenter natural history study of vestibular schwannomas in neurofibromatosis Type 2. Study design Retrospective clinical study. Setting International neurofibromatosis Type 2 tertiary care centers. Patients Study participants had a clinical diagnosis of neurofibromatosis Type 2, at least one untreated vestibular schwannoma, and were at least 5 years old. Sixty-three subjects (108 ears) with audiology data at either short-term follow-up (7 mo-2 yr) or long-term follow-up (3-5 yr) after diagnosis were examined in this study. Main outcome measures Changes in four-frequency pure-tone average and speech discrimination score before any treatment intervention for both follow-up intervals. Results Within 2 years of the diagnosis of neurofibromatosis Type 2, 27% of the ears experienced a significant loss in pure-tone average relative to diagnosis, and 73% of the ears experienced no significant change in hearing. Conclusion Newly diagnosed neurofibromatosis Type 2 patients who do not require immediate treatment of both vestibular schwannomas are likely to have stable hearing in the unoperated ear(s) for approximately 1 to 2 years.

Hearing changes after diagnosis in neurofibromatosis type 2.

Context Successful outcome of complex surgical techniques depend on the surgical team's level of experience. However, unless benefit is demonstrated in the presence of cost savings, insurance companies are reluctant to approve care from highly experienced hospitals and surgical teams. Objective To determine the relationship between the number of acoustic neuroma surgeries performed at California hospitals with surgical outcome and hospital stay cost. Design Acoustic neuroma surgery information was extracted from the California hospital discharge database and was grouped according to the number of surgeries performed at each hospital. Setting A database review of California hospitals. Patients Persons (n=1,213) undergoing acoustic neuroma surgery at a California hospital between 1996 and 1998. Main outcome measures Outcome measures included discharge status (routine vs. not routine), medical procedures indicating surgical complications, total cost of hospitalization, and average cost per hospitalization day. Odds ratios were used to compare the hospital volume groups on likelihood of a routine surgical outcome. Results The frequency of routine surgical outcome increased as hospital acoustic neuroma surgical volume increased, ranging from 68% among patients in the lowest volume group to 97% in the highest hospital volume group. Patients in the highest hospital volume group were 15 times more likely to have a routine surgical outcome than patients in the lowest hospital volume group. Patients in the second highest volume group were 5 times more likely to have a routine surgical outcome than in the lowest volume group. The two highest volume hospital groups had lower total charges and average charges per day than the two lower volume hospital groups. Conclusions Acoustic neuroma surgeries at higher volume hospitals had higher frequency of routine discharge, lower frequency of apparent complications, and lower average cost of hospitalization.

Acoustic neuroma surgical cost and outcome by hospital volume in California

Differentiation of primary human vestibular nerve schwannomas (VS) caused by mutations of the NF2 gene was evaluated by examining the expression patterns of genes that are specifically expressed in different stages of Schwann cell lineage. In schwannoma cells that are not in contact with an axon, the expression levels of the major myelin sheath proteins, such as protein zero glycoprotein (P0), myelin basic protein (MBP), and peripheral myelin protein 22 (PMP22), were greatly reduced. However, high expression levels of nerve growth factor receptor (NGFR), neural cell adhesion molecule (N-CAM), and cell adhesion molecule L1 (L1) were observed. In addition, expression of transcription factors Krox20, Krox24, and SCIP/Oct6 was also detected in the tumor cells. These results suggest that loss of the NF2 gene was responsible for the transformation of the Schwann cells into a neoplastic stage that has a similar genetic profile to the pro-myelinating stage. Finally, the primary human vestibular schwannoma cells failed to be regulated and redifferentiated by a regenerating axon, when the human tumors were transplanted into sciatic nerve of nude rat. These results suggest that the NF2 gene might be involved in the differentiation of Schwann cells.

Immunohistochemistry study of human vestibular nerve schwannoma differentiation.

In a large matched population-based case-control study of acute myelogenous leukemia (AML), we did not find incident AML in Los Angeles County (1987-1994) to be associated with previous exposure to electric blankets, electrically heated waterbeds, or occupations with presumed high exposure to electromagnetic fields.

Adult onset acute myelogenous leukemia and electromagnetic fields in Los Angeles County: bed-heating and occupational exposures.

CSNS Young Neurosurgeon Award: acoustic neuroma surgical cost and outcome by hospital volume.

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