Hemoglobin thresholds and triggers for blood transfusions have changed over the years moving from a higher to a lower level. This review article summarizes the current evidence of transfusion thresholds in the hospitalized as well as in the outpatient setting and particularly in myelodysplasia. Fatigue is the main reported symptom in this group of patients and current clinical trials are looking for a more liberal approach of red cell transfusion and the effect on quality of life as opposed to the restrictive strategy used in the critical care setting. Practical considerations, the cost effectiveness of this strategy in addition to the possible complications, and the use of quality of life questionnaires have also been reviewed.
A 59-year-old man, with a background of multiply relapsed myeloma, presented with a 3-week history of confusion, short-term memory impairment and behavioural changes. CT head showed bilateral white matter changes and numerous, large lytic lesions of the skull vault. MRI brain revealed multiple areas of hyperintensity on T2-weighted sequences which did not enhance (many of which showed diffusion restriction) unexpectedly bringing progressive multifocal leukoencephalopathy (PML) into the differential. Initial cerebrospinal fluid studies were largely unremarkable, aside from a mildly elevated protein; cultures were negative. PCR for the John Cunningham (JC) virus was positive. Considering the patient’s medical history and rapidily progressive symptoms, a palliative approach was adopted, with the patient dying 14 days later. We present this case as an example of PML in a patient with multiple myeloma, highlighting the need to consider this diagnosis in an enlarging population of heavily treated, severely immunocompromised, patients.
IntroductionHH is a common inherited disorder of iron metabolism. We reviewed risk factors for cirrhosis, screening tests, rates of end organ damage and the outcome of patients treated with venesection.MethodsA retrospective analysis of 167 patients with HH in the Royal Infirmary of Edinburgh attending the venesection service in 2013 and 2014.Results118 males and 49 females were identified with a mean age of 56 (range 19–83). 79 (47%) had end-organ involvement at presentation: 18% cirrhosis, 17% endocrine, 10% arthropathy, 1% cardiac, 1% skin. The most common endocrine problems were diabetes and hypogonadotropic hypogonadism. Arthropathy was generalised and mostly involved the small joints. 50% had deranged liver function tests (LFTs) at diagnosis. With therapeutic venesection, LFTs improved in 93% whereas arthropathy improved in only 31%.Of the 30 patients with cirrhosis, 26 had serum ferritin (SF) >1000 μg/L at presentation, 25 were C282Y homozygotes, 3 cases progressed to hepatocellular carcinoma, 22 had other risk factors for liver disease [2 Hepatitis C, 13 alcohol and 7 non-alcoholic fatty liver disease (NAFLD)]. Serum Hyaluronic acid (HA), an indicator of hepatic fibrosis, was measured in 73 patients: a normal level reliably excluded cirrhosis [negative predictive value 97.8% (CI 88.7 to 99.9%)].After completion of the therapeutic phase (SF < 100 μg/L), subsequent venesection requirements were variable: 9 patients did not require venesection for more than 1 year.With a median follow-up of 6 years, no uncomplicated cases at presentation venesected per protocol developed end organ damage.ConclusionIt is important to identify patients with cirrhosis, particularly if other risk factors for liver disease are present. HA is a useful screening test for cirrhosis in this setting. Protocol venesection is highly successful in preventing end-organ damage: uncomplicated patients at presentation would be suitable for a virtual clinic to lessen the burden on out-patient services.Disclosure of InterestNone Declared
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