Measurement of macrophage marker in hyperhaemolytic transfusion reaction: a case report.
BACKGROUND
Hyperhaemolytic transfusion reaction (HHTR) has been well described in patients with sickle cell disease (SCD). It is characterised by a decrease in haemoglobin concentration to levels below those before transfusion and a fall in the absolute reticulocyte count. As red blood cells (RBC) alloantibodies are typically not detected in post-transfusion samples in acute forms of HHTR, we have previously proposed that both the transfused and autologous RBCs cells (HbSS/reticulocytes) are destroyed by activated macrophages.


METHODS
We report a patient with SCD who presented with vaso-occlusive sickle cell crisis and developed a severe HHTR attributable to anti-Fy3. In addition to the usual supportive measures, the patient was treated with intravenous immunoglobulin (IVIG) and steroids. Serum ferritin levels were measured as an aspecific marker of macrophage activation.


RESULTS
Steroids and IVIG were effective in managing HHTR. Ferritin levels were high at the time of haemolysis, (>10000 µg L(-1)) whereas recovery and cessation of haemolysis correlated with a decrease in ferritin levels.


CONCLUSIONS
Serum ferritin values >10,000 µg L(-1) are considered pathognomic for conditions characterised by abnormal macrophage activation. In our case, serum ferritin levels correlate well with the disease activity and clinical response. This further supports our previous proposal that the activated macrophages play an important role in HHTR. Serum ferritin is a nonspecific marker of inflammation. A rapid specific bio-marker to measure the activity of macrophages in SCD in HHTR is desirable, and this area warrants further investigation.
Department of Red Cell Immunology (RCI), NHS-Blood and Transplant, Tooting Centre, London, UK. nay.win@nhsbt.nhs.uk
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D000328:Adult; D000755:Anemia, Sickle Cell / Q000097:blood / Q000276:immunology / Q000628:therapy*; D015415:Biomarkers / Q000097:blood; D004375:Duffy Blood-Group System / Q000097:blood / Q000276:immunology; D017707:Erythrocyte Transfusion / Q000009:adverse effects*; D005293:Ferritins / Q000097:blood* / Q000276:immunology; D006461:Hemolysis; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage; D007155:Immunologic Factors / Q000008:administration & dosage; D007518:Isoantibodies / Q000097:blood / Q000276:immunology; D008262:Macrophage Activation; D008264:Macrophages / Q000276:immunology / Q000378:metabolism*; D008297:Male; D011956:Receptors, Cell Surface / Q000097:blood / Q000276:immunology
C473745:ACKR1 protein, human; D015415:Biomarkers; D004375:Duffy Blood-Group System; D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors; D007518:Isoantibodies; D011956:Receptors, Cell Surface; D005293:Ferritins