Hyperhemolysis in a patient with β-thalassemia major

Morawakage, R Lakmali; Perera, Bjc; Dias, Paula; Wijewardana, SK

doi:10.4103/0973-6247.45259

Cited by 12 publications

(16 citation statements)

References 13 publications

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“…They discuss that IgA may lyse RBCs while common serologic tests are negative. They explain that ADCC may occur with low level of antibodies that were not detectable by serologic tests [4].…”

Section: Discussionmentioning

confidence: 99%

“…In the absence of HLA antibodies and RBC alloantibodies, it is suggested that macrophages are involved in RBCs destruction [10]. Morawakage et al (2009) reported a case of HS in a child with thalassemia with 2+ DAT for C3b but they could not find any antibody in the patient's serum. They discuss that IgA may lyse RBCs while common serologic tests are negative.…”

Section: Discussionmentioning

confidence: 99%

“…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. Several theories are suggested to explain the hemolytic destruction of RBCs like: macrophages hyperactivation, defects in complement regulation, HLA antigen-antibody reactions, a bystander hemolysis and suppression of erythropoiesis [2,9,10].…”

Section: Discussionmentioning

confidence: 99%

“…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. Azarkeivan et al [6] reported that alloimmunization rate in 441 thalassemic patients was 11.3%.…”

Section: Introductionmentioning

confidence: 99%

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

Moghaddam¹,

Anaraki²,

Shaiegan³

et al. 2015

J Hematol

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Hyperhemolysis syndrome (HS) is a delayed type of transfusion reactions (DTHRs). These may occur in patients with hemoglobinopathies due to destruction of red blood cells (RBCs). A 17-year-old boy with B-thalassemia and a history of multiple transfusions, after several RBCs transfusions developed hemolysis and hemoglobinuria with a decreased hemoglobin (Hb) level in the absence of bleeding that continued with splenomegaly. The antibody screen test (LISS AHG) and direct anti-globulin test (DAT) were performed. An 11-cell antibody identification panel (IBTO home-made) was used. Acid elution procedure was done using a commercial kit. The patient RBC phenotyping was attempted by treating the cells, following a chloroquine treatment procedure. Laboratory findings showed that his blood group was "A" Rh positive. His RBC phenotyping was negative for Jka antigen. The LISS antibody screening test result was negative with a weakly positive auto control result but DAT was positive for both C3d and IgG. The antibody identification test showed the presence of anti-Jka alloantibody and also anti-Jka was identified with eluate reacting stronger with homozygous cells (Jka+Jka+). The patient received steroid and IVIG as the main treatment and followed by receiving compatible blood units but hemolysis was not resolved and his Hb level was not increased, finally he was splenectomized. The patient's Hb level and clinical symptoms improved after the splenectomy, showing that the spleen may have a role in the destruction of the recipient and the donor blood cells.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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

Moghaddam¹,

Anaraki²,

Shaiegan³

et al. 2015

J Hematol

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“…Of note reticulocytopenia is also present, in contrast to other types of hemolytic reactions, where reticulocytosis is usually notable 4. HHTR most commonly occurs in patients with sickle cell disease,2,3,5 while occasionally it has been reported in patients with thalassemia4,6,7 and in individuals without underlying hematologic diseases 8,9…”

Section: Introductionmentioning

confidence: 99%

Hyperhemolytic Syndrome complicating a Delayed Hemolytic Transfusion Reaction due to anti-P1 alloimmunization, in a pregnant woman with HbO-Arabβ-thalassemia

Bezirgiannidou

Christoforidou

Kontekaki

et al. 2016

Mediterr J Hematol Infect Dis

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BackgroundHyperhemolytic Syndrome or Hyperhemolytic Transfusion Reaction (HHTR), a life-threatening subset of Delayed Hemolytic Transfusion Reaction (DHTR) is characterized by destruction of both transfused and autologous erythrocytes evidenced by a fall in post transfusion hemoglobin below the pre-transfusion level.Case reportWe describe a case of DHTR due to anti-P1 alloimmunization manifesting with hyperhemolysis in a 30-year-old Greek Pomak woman with thalassemia intermedia (HbO-Arab/β-thalassemia), during the11th week of her first gestation. She was successfully managed with avoidance of further transfusions and administration of IVIG and corticosteroids.ConclusionA high index of suspicion for HHTR is of vital importance among clinicians especially since optimal methods for its prevention and treatment remain yet to be defined. Early recognition of HHTR leading to prompt cessation of additional transfusions and initiation of immunosuppressive treatment can be life-saving, especially in clinical settings where limited therapeutic options are available, such as in pregnancy.

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Post‐transfusion hyperhemolysis syndrome in a patient with beta thalassemia major

Kasinathan

Sathar

2021

Clinical Case Reports

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Hyperhemolysis in a patient with β-thalassemia major

Cited by 12 publications

References 13 publications

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

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

Hyperhemolytic Syndrome complicating a Delayed Hemolytic Transfusion Reaction due to anti-P1 alloimmunization, in a pregnant woman with HbO-Arabβ-thalassemia

Post‐transfusion hyperhemolysis syndrome in a patient with beta thalassemia major

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