title: Fatal Delayed Haemolytic Transfusion Reaction and Hyperhaemolysis Syndrome in a Pregnant Woman with Sickle Cell Anaemia.
abstract: Clinical manifestations of sickle cell disease (SCD) arise from the tendency of the sickle haemoglobin to polymerize and deform red blood cells into the characteristic sickle shape. Sickle cell crisis is a devastating complication that may occur in patients with SCD. If not managed properly permanent organ damage and even death may be the final outcome. A case of a 32-year-old Nigerian lady, Gravida 1 Para 0 in her first trimester, with SCD who developed signs and symptoms of delayed haemolytic transfusion reaction after receiving packed red cell transfusion is demonstrated. Multiple red cell alloantibodies were detected in the patient's plasma; anti-Fy a, anti-Jk b and anti-E. The patient miscarriaged and succumbed to complications of hyperhaemolysis with delayed haemolytic transfusion reaction, acute chest syndrome and renal failure. There is an urgent need for mandatory red cell antibody screen and identification especially in high-risk cases. Prevention of alloimmunization by supplying phenotype-specific red cells is also required.
authors.affiliation: Faculty of Medicine and Health Sciences, Universiti Sains Islam Malaysia, Level 13 Menara B, Persiaran MPAJ, Jalan Pandan Utama, 55100 Kuala Lumpur, Malaysia.
authors.forename: Asral Wirda Ahmad
authors.initials: AW
authors.lastname: Asnawi
authors.affiliation: Department of Haematology, Hospital Ampang, Jalan Mewah Utara, Pandan Mewah, 68000 Ampang, Selangor Malaysia.
authors.forename: Jameela
authors.initials: J
authors.lastname: Sathar
authors.affiliation: Department of Pathology, Hospital Ampang, Jalan Mewah Utara, Pandan Mewah, 68000 Ampang, Selangor Malaysia.
authors.forename: Rashidah
authors.initials: R
authors.lastname: Mohamed
authors.affiliation: Department of Pathology, Hospital Ampang, Jalan Mewah Utara, Pandan Mewah, 68000 Ampang, Selangor Malaysia.
authors.forename: Rohayu
authors.initials: R
authors.lastname: Deraman
authors.affiliation: Department of Haematology, Hospital Ampang, Jalan Mewah Utara, Pandan Mewah, 68000 Ampang, Selangor Malaysia.
authors.forename: Sri
authors.initials: S
authors.lastname: Kumaran
authors.affiliation: Department of Haematology, Hospital Ampang, Jalan Mewah Utara, Pandan Mewah, 68000 Ampang, Selangor Malaysia.
authors.forename: Shahada Sobah Abd
authors.initials: SS
authors.lastname: Hamid
authors.affiliation: Department of Haematology, Hospital Ampang, Jalan Mewah Utara, Pandan Mewah, 68000 Ampang, Selangor Malaysia.
authors.forename: Muhd Zanapiah
authors.initials: MZ
authors.lastname: Zakaria
keywords: Delayed haemolysis; Hyperhaemolysis; Sickle cell disease; Sickling; Transfusion reaction