A systematic approach to controlling problem bleeds in patients with severe congenital haemophilia A and high-titre inhibitors

The presence of inhibitory antibodies to clotting factors complicates the treatment of bleeding in haemophilia patients. For patients with high-titre inhibitors, bypassing agents are essential to haemostatic management. To determine optimal treatment practices, an international panel of physicians convened to develop a systematic treatment approach for problem bleeds (i.e. bleeds that are unresponsive to initial therapy with a single agent within a reasonable amount of time) in haemophilia patients with inhibitors. AIM: The goal of this panel was to develop a consensus algorithm that would aid physicians in considering a variety of treatment approaches to optimize patient care by preventing extensive therapy with inadequate treatments that may lead to suboptimal patient outcomes and unnecessary costs. METHODS: Consensus opinions were analyzed for clinical preferences at different time periods, depending on patient response to treatment. Decision-making points were defined based on the type of bleed: every 8-12 h for the first 24 h, then every 24 h thereafter for limb-threatening bleeds; every 2-4 h for 2-7 days for life-threatening bleeds. RESULTS: The resultant consensus guidelines provide a generalized methodology to guide the treatment of problem bleeds in patients with severe haemophilia A and inhibitors, and emphasize changing treatment at the first sign of an inadequate haemostatic response. The treatment algorithms apply to both paediatric and adult patients, although the differences between the two groups were reviewed. CONCLUSION: These guidelines are focused on optimising the timing of treatment decisions, which may lead to faster responses and improved outcomes.     

The Effect and Safety of the Antithrombotic Therapies in Patients with Atrial Fibrillation and CHADS2 Score 1

Anticoagulation in CHADS₂ Score 1 . Background: The revised ACC/AHA/ESC 2006 guideline recommends either aspirin or warfarin for the prevention of ischemic stroke in patients with atrial fibrillation (AF) in CHADS₂ score 1. We hypothesized that warfarin is superior to aspirin therapy for the prevention of stroke without increasing bleeding complication in AF patients with CHADS₂ score 1. Methods and Results: Among 1,502 patients (mean 62.4 ± 13.8 years old, male 65.4%) who were treated for nonvalvular AF without previous stroke, the number of patients with CHADS₂ score 1 was 422 (62.9 ± 10.7 years old, male 290 [68.7%]) and their antithrombotic therapies were as follows: warfarin (n = 143), aspirin (n = 124), other antiplatelet (n = 45), and no antithrombosis (none: n = 110). We reviewed the incidences of ischemic stroke, mortality, and bleeding complications during the follow‐up period. Results were: (1) during 22.3 ± 17.8 months of follow‐up, the incidence of ischemic stroke was significantly lower in warfarin (6 patients, 4.2%, mean international normalized ratio [INR] 2.0 ± 0.5 IU) than in aspirin (16 patients, 12.9%, P = 0.008) than none (23 patients, 20.9%, P < 0.001) without differences in all‐cause mortality. (2) The incidence of major bleeding (decrease in hemoglobin ≥2 g/dL, requiring hospitalization or red blood cell transfusion ≥2 pints) was not different between warfarin (2.1%) and aspirin (0.8%, P = NS), but minor bleeding was more common in warfarin (10.5%) than in aspirin (2.4%, P = 0.007). Conclusion: In AF patients with CHADS₂ score 1, warfarin was better to prevent ischemic stroke than aspirin without increasing the incidence of major bleeding complications. However, the incidence of minor bleeding was higher in the warfarin group than the aspirin group. (J Cardiovasc Electrophysiol, Vol. 21, pp. 501‐507, May 2010)     

Biting for attention: A case of dental discomfort manifesting in behavioural problems

There is a dearth of literature describing behaviour that expresses discomfort caused by poor dentition in patients with dementia. In this paper, we report on a patient whose behaviour only abstrusely pointed to his teeth as the source of discomfort. Although changes to his medication over a 2‐year period had little effect, eventual extraction of his caries brought about an almost immediate resolution of all antisocial behaviour. Clinicians must be mindful that poor dental care is easily meliorated but remains endemic in patients suffering from dementia.     

A case of Mollitias and Fragilitas Ossium – unusual presentation of hairy cell leukaemia followed by the diagnosis of nonsecretory myeloma

Hairy cell leukaemia (HCL) is a B‐cell malignancy with a late developmental arrest. This report describes a patient that presented with leucocytosis and splenomegaly. The abnormal leucocytes showed typical morphology and expressed CD103, CD11c, CD19 and CD20 but not CD25 by immunophenotyping. The patient failed to respond to splenectomy and then developed lytic bone lesions and pathological fractures, which progressed despite a single course of cladribine chemotherapy. Review of the pathology of the bone reamings showed nonsecretory myeloma of the same kappa‐light chain isotype. He went on to receive induction chemotherapy in preparation for an autologous stem‐cell transplant but failed to mobilize sufficient numbers of stem cells. He has had two localized relapses with bony lesions, one within 6 weeks of stopping chemotherapy for which he received localized radiotherapy and thalidomide consolidation. Sequential myeloma has been described in HCL. There is controversy whether this represents clonal evolution or a secondary malignancy. An increased rate of secondary malignancies has been reported by some, but not other, authors in long‐term survivors of HCL. This case illustrates the value of a repeat pathological review in case of unexpected complications.     

Raising airway resistance in laryngctomees incrases tissue oxygen saturation

The physiological airflow resistance from the anterior nares to the upper trachea has recently been documented. ¹This resistance is thought to be important in preventing alveolar collapse and in maintaining the optimum lung ventilation: perfusion ratio.²This resistance is lost in laryngectomees who may have a subsequent decline in lung function and arterial oxygen saturation. A stoma filter with a resistance approximating to that of the normal upper airway has been developed and laryngectomees who wear this device demonstrate a rapid increase in tissue oxygen saturation (median increase: 1.4 kPa, P= 0.0087) without significantly altering tissue carbon dioxide saturation. This finding will have a major impact on the future rehabilitation of laryngectomees and those with a temporary tracheostomy.