Hyperhemolysis syndrome in sickle cell disease: case report (recurrent episode) and literature review

Abstract

This is the second reported case of recurrent HS in a child. Recent studies have shown that the adhesion molecules expressed on RBC erythroid precursor cells and reticulocytes can interact with macrophages and can cause hemolysis. Because RBC alloantibodies and HLA antibodies were not identified in this case, it is believed that the patient's cells and transfused cells were destroyed by macrophages either by direct contact lysis or by erythrophagocytosis. The possible mechanism of IVIG and steroids on suppression of macrophages resulting in cessation of hemolysis is discussed. Our case illustrates the danger of recurrent HS and the difficulty of balancing this against the need for transfusions in patients presented with severe hemolysis.

Fatal Delayed Hemolytic Transfusion Reaction and Hyperhemolysiswithout Detectable Alloantibodies or Autoantibodies in a Patient withSickle Cell Disease: A Case Report and Literature Review

“…In such cases, intravenous immunoglobulin (IVIG) and Erythropoietin (EPO) should be administered concurrently [7]. For severe cases, EPO-Eculizumab should be considered [3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20]. In cases where additional transfusions are necessary, EPO-Rituximab may prove successful [3].…”

“…DHTR typically occurs in patients 5-15 days after transfusion and is often associated with a vaso-occlusive crisis characterized by the following symptoms: anemia, dark (cola-colored) urine, elevated indirect bilirubin, and increased levels of lactate dehydrogenase (LDH) [2,3]. It remains unknown why some cases of DHTR directly involve or lead to hyperhemolysis syndrome (HS), causing the destruction of both donor and recipient red blood cells, brisk intravascular hemolysis, and very low levels of total hemoglobin (Hb) (below pretransfusion levels) [4][5][6]. HS typically causes a fall in absolute reticulocyte count (a decrease from the baseline concentration) during hemolysis, and a rise in Hb and reticulocyte counts often accompanies recovery [2,5].…”

“…It remains unknown why some cases of DHTR directly involve or lead to hyperhemolysis syndrome (HS), causing the destruction of both donor and recipient red blood cells, brisk intravascular hemolysis, and very low levels of total hemoglobin (Hb) (below pretransfusion levels) [4][5][6]. HS typically causes a fall in absolute reticulocyte count (a decrease from the baseline concentration) during hemolysis, and a rise in Hb and reticulocyte counts often accompanies recovery [2,5]. Delayed reactions typically result from a secondary immune response in a previously transfused or pregnant patient who has developed an alloantibody, and such reactions can be life-threatening [7,8].…”

The five key things you need to know to manage adult patients with sickle cell disease

“…42 The mechanism of hyperhemolysis is unclear; autoimmune bystander hemolysis, suppression of erythropoiesis, and phosphatidylserine exposure on the surface of red cells leading to physiologic clearance by macrophages have all been implicated. 42,43 Iron overload is another complication associated with transfusion. The prevalence of this complication has not been extensively studied in people with SCD.…”

Hyperhemolysis with Hemoglobin H Disease

“…10 The acute form usually occurs less than seven days after receiving the blood transfusion. The DAT is usually negative and no red cell alloantibodies are identified in the patient's serum.…”