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. 2019 Apr;104(4):e127-e130.
doi: 10.3324/haematol.2018.205682. Epub 2018 Oct 18.

Low serum haptoglobin and blood films suggest intravascular hemolysis contributes to severe anemia in hereditary hemorrhagic telangiectasia

Lieze Thielemans  1 , D Mark Layton  2 , Claire L Shovlin  3
Affiliations

Low serum haptoglobin and blood films suggest intravascular hemolysis contributes to severe anemia in hereditary hemorrhagic telangiectasia

Lieze Thielemans et al. Haematologica. 2019 Apr.
No abstract available

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Figures

Figure 1.
Figure 1.
Non iron-deficient red cell and iron indices in HHT patients. (A) Mean corpuscular volume (MCV) in 1409 datasets of 486 HHT patients, by hemoglobin. Red values indicate normal to high values in the 221 datasets (where hemoglobin <110g/L). Note the number of datasets ranged from 1-37 (median 3) per patient. (B) Anemia and iron indices in 86 consecutively reviewed HHT patients using iron- data indicate a single dataset per patient. All iron users were categorised by whether or not they used intravenous (iv) iron, or purely oral iron tablets. Upper graph: hemoglobin, lower graph: ferritin. Boxplot boxes indicate median (line) and interquartile range, error bars represent two standard deviations. Normal ranges indicated by grey bars.
Figure 2.
Figure 2.
Hemolytic markers in HHT patients. (A) Serial measurements over 17 months of a typical severely anemic HHT patient in the cohort of 27. Hemoglobin (blue symbols) in relation to iron infusions (yellow lines), red cell transfusions (red lines), and one-week course of prednisolone 30mg od (for asthma, green). Three serum haptoglobin measurements were below the normal range (Online Supplementary Figure S1). (B) Serum haptoglobin in 27 selected HHT patients, categorised by values above and below the lower limit of normal. Error bars demonstrate median and interquartile range. Shaded area represents normal range. C) Manual blood films reported in 11 of the 27 selected HHT patients: symbols demonstrate mean and 95% confidence intervals. D) Mean corpuscular volume (MCV) in HHT patients without recent transfusions, categorised by haptoglobin values above and below the lower limit of normal. Symbols demonstrate median and interquartile range. Note in B-D, data represent a single dataset per patient, and D presents data from the subset of those in Figure 2B who had not received a blood transfusion in the previous month. Data not shown for one patient who had previously been treated for a bone marrow pathology (though inclusion did not materially alter the analyses).
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