Hyperhemolysis Syndrome without Underlying Hematologic Disease

Abstract

Introduction. Hyperhemolysis is characterized by a life-threatening hemolytic transfusion reaction, with hemoglobin (Hb) and hematocrit (Hct) dropping markedly lower than before transfusion. This phenomenon, commonly described in sickle cell disease, is a rare occurrence in patients without hemoglobinopathies. Case Report. A 55-year-old male presented to the hospital after a motorcycle crash and received 10 units of cross-matched blood for active bleeding. The patient was blood group O, with a negative antibody screen. Ten days later, he represented complaining of dyspnea and was found to have a hematocrit of 12%. The direct antiglobulin test was positive for anti-immunoglobin G and complement. Indirect antiglobulin test was positive for anti-Jka alloantibodies. The presence of Jka antigen was revealed in one unit of previously transfused blood; patient's RBCs were negative for the Jka antigen. Laboratory data demonstrated findings consistent with DHTR, as well as reticulopenia and elevated ferritin levels. He continued to show signs of active hemolysis, requiring a total of 4 subsequent units of pRBCs. Each transfusion precipitated a drop in Hb and Hct to levels lower than before transfusion; once transfusions were held, the patient slowly recovered. Discussion. Hyperhemolysis in the setting of a DHTR can occur in patients without hematologic disease.

Hyperhemolysis Syndrome in a Patient With B-Thalassemia Due to an Anti-Jka Alloantibody

“…Eberly et al [3] recently reported a case with hyperhemolysis without hematologic disease, that her laboratory tests results were as the same as our patient results. IVIG and steroids [8], avoiding further blood transfusion [9], plasma-to-RBC replacement [11] are different treatment of the HS.…”

“…Santos et al [5] described two acute (with negative DAT) and chronic (with positive DAT) forms of HS. Transfusions in a hyperhemolytic episode are able to accelerate hemolysis [3], which was seen in our case. Jka negative blood product (compatible blood) could not increase Hb level in our patient, and his positive auto control result directed that autologous RBC destruction may occur.…”

“…It is suggested that antibodies reacting with foreign antigens on transfused cells can cause activation of complement, leading to a phenomenon known as "bystander hemolysis" [3]. Our patient RBC was negative for Jka but had anti-Jka antibodies with a DAT 4+ result for IgG and 3+ result for C3b reflecting RBCs sensitization.…”

“…2015;4(3):210-213 (HS). Alloantibody production occurs in DTHR that destroys transfused RBCs; delayed hyperhemolysis is suggested to be a subset of DTHR with destruction of both donor and host RBCs [3]. HS first was known in patients with sickle cell anemia [4,5], and it is rarely reported in thalassemia.…”

“…This phenomenon is described in beta-thalassemia also and rarely in patients without hemoglobinopathies [3]. The exact mechanism is not well known but it involves destruction of both transfused donor cells and recipients' red blood cells (RBCs) [4,5] via alloimmunization.…”