Hyperhemolysis Syndrome Complicating Pregnancy in Homozygous δβ-Thalassemia

Mechery, Joseph; Abidogun, Kehinde; Crosfill, Fiona; Jip, James

doi:10.3109/03630269.2011.649150

Cited by 7 publications

(13 citation statements)

References 5 publications

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“…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]. Anti-Jka causes over one-third of DHTRs [8] may be severe.…”

Section: Discussionmentioning

confidence: 99%

“…IVIG and steroids [8], avoiding further blood transfusion [9], plasma-to-RBC replacement [11] are different treatment of the HS. Hemolysis was a sever condition in our patient.…”

Section: Discussionmentioning

confidence: 99%

“…His Hb level was increased to 8.3 g/dL during 14 days. Mechery et al suggested that splenectomy could resolve the crisis of hyperhemolysis in a child with beta-thalassemia also [9].…”

Section: Discussionmentioning

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.

show abstract

Section: Discussionmentioning

confidence: 99%

“…IVIG and steroids [8], avoiding further blood transfusion [9], plasma-to-RBC replacement [11] are different treatment of the HS. Hemolysis was a sever condition in our patient.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

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

Moghaddam¹,

Anaraki²,

Shaiegan³

et al. 2015

J Hematol

View full text Add to dashboard Cite

show abstract

“…She had tachycardia (heart rate 110 beats/min). At the time of admission hemoglobin (Hb) was 2.2g/dl with red cell count 0.99x10 6 Her blood group types as O; rhesus antigen CcDee (R1r) and Mi antigen were positive. Glucose 6 phosphate dehydrogenase (G6PD) enzyme level was normal.…”

Section: Case Presentationmentioning

confidence: 99%

“…Only a slight jaundice with 2cm splenomegaly was noticed on physical examination. Hb was maintained at 5.9g/dl with red cell count 2.69x10 6 …”

Section: Outcome and Follow-upmentioning

confidence: 99%