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Thalassemia in pregnancy
The document discusses thalassemia in pregnancy according to 2014 guidelines from the Royal College of Obstetricians and Gynaecologists (RCOG). It defines thalassemia as a quantitative disorder of globin chain production affecting either the alpha or beta globin chain. It describes the epidemiology, genetics, types (alpha and beta thalassemia), complications, and management both outside and during pregnancy. The RCOG guidelines provide recommendations for preconception care, antenatal care, intrapartum care, postpartum care, booking appointments, and schedule of antenatal appointments for women with thalassemia.
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Thalassemia in pregnancy
- 1. Thalassemia in pregnancy (RCOG Green-top 2014guidelines) Iqra Yasin Resident, Obs & Gyne Unit I SIMS/Services Hospital, Lahore
- 2. Outline – Etymology – Definition –Epidemiology – Genetics – α thalassemia – β thalassemia – RCOG Green top guidelines # 66
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- 4. Definition – Quantitative disorderof globin chain production that either affect α or β globin chain
- 5. Epidemiology – Common inMediterranean countries – Incidence – UK 1:10,000 – Cyprus 1: 7 – Pakistan (no definite data available: estimated 5000 – 9000 babies born with thalassemia per year with carrier rate 5 – 7 % in general population)
- 6. Genetics – Autosomal recessivepattern of inheritance – α chain – 2 pairs of genes (Total 4; 1 pair from each parent) – Chromosome 16 – β chain – 2 genes (1 from each parent) – Chromosome 11
- 7. Autosomal recessive patternof inheritance
- 8. α thalassemia – Causedby when 1-4 α gene deletion
- 9. α thalassemia – αthalassemia trait – No/mild anemia in pregnancy – No abnormal Hb found – Not detected by Hb electrophoresis – HbH disease – Chronic hemolytic anemia – Moderate anemia (Hypochromia, marked microcytosis) – 5-30 % HbH Hb in peripheral blood (detected by Hb electrophoresis) – HbH inclusion bodies in red cells (golf ball cells –supravital staining) – Can transmit as α thalassemia trait in children
- 10. α thalassemia – αthalassemia major – No α chain – No HbA, HbA2 and HbF – Accumulation of Fetal ϒ4 ( Hb barts*) – Severe anemia failure of O2 delivery to tissue Cardiac failure & abnormal organogenesis Hydrops fetalis + Polyhydroamnios + Placentomegaly – Serious Obstetric complications (Pre-eclampsia, difficult delivery due to large fetus and placenta) *This variant of hemoglobin is so called as it was discovered at St. Bartholomew's Hospital in London, also called St. Barts.
- 11. α thalassemia -management
- 12. β thalassemia – Causedby defective β gene. – Severity of disease depend upon nature of mutation and presence of mutation in 1 or both alleles – 2 types of mutation – Β+ reduced function – Βo absent function β thalassemia major = Mediterranean anemia or Cooley anemia Thomas Benton Cooley (1871 – 1945) American pediatrician and hematologist
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- 18. β thalassemia PERIPHERAL SMEAR Targetcells red cells with central staining with precipitated haemoglobin Basophilic Stripping (Punctate Basophilia) accumulation of ribosomes in periphery of red cell Howell Jolly Bodies basophilic nuclear remnants (clusters of DNA) in circulating erythrocytes. William Henry Howell (1860- 1945) American Physiologists Justin Marie Jolly (1870 – 1953) French hematologist and histologist
- 19. β thalassemia -Mnemonic
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- 22. β thalassemia -complications
- 26. β thalassemia -management
- 27. β thalassemia -management
- 28. Thalassemia vs Irondeficiency anemia
- 29. Management – outside pregnancy –Blood transfusion – Iron chelating therapy – Folic acid supplements – Bone marrow stem cell transplant – Possible future options – Gene therapy – Trigger to stimulate HbF production (hydrocyurea)
- 31. RCOG 2014 Green-top guidelines –Preconception care – Antenatal care – Intrapartum care – Postpartum care – Booking appointments – Schedule of antenatal appointments
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