Hyperhaemolysis in a pregnant patient with HbH disease

Abstract

Dear Sir,Hyperhaemolysis is a well-recognised transfusion complication typically described in sickle cell disease (SCD), but it is rarely reported in Haemoglobin H (HbH) disease (Danaee et al., 2014). The increasing incidence of hyperhaemolysis now makes this condition reportable via the UK Serious Hazards of Transfusion scheme (Serious Hazards of Blood Transfusion (SHOT), 2015), and this potentially fatal complication results in challenging management issues in those affected. We present a case of hyperhaemolysis in a pregnant patient with HbH disease and discuss our management strategies and clinical outcomes.A 33-year-old female patient, G4P3, with HbH disease, AB RhD+, with baseline haemoglobin (Hb) of 85 g L −1 , ferritin 57 ng mL −1 (15-300 ng mL −1 ) and bilirubin 16 moL L −1 , presented to the antenatal clinic. Her previous three pregnancies were uncomplicated and did not require red blood cell transfusions. Fourteen weeks into her fourth pregnancy, the patient developed shortness of breath and lethargy, and Hb dropped to 63 g L −1 , with a bilirubin of 18 moL L −1 . This Hb fall was attributed to the increased physiological demands of pregnancy and plasma volume expansion, which has been described in HbH disease and pregnancy (Chui et al., 2003). She was transfused 2 units of red cells (one A RhD+ and the other AB RhD+) in our haematology day unit without complications. She presented to the Emergency Department 3 days later with ongoing shortness of breath and a repeat Hb of 60 g L −1 , bilirubin 49 moL L −1 , haptoglobin <0·08 g L −1 , lactate dehydrogenase (LDH) 1355 IU L −1 and reticulocyte count of 81 × 10 9 L (normal range 100-400 ×10 9 ). On further questioning, she reported subjective fevers and dark-coloured urine observed at home. On examination, her abdomen was tender, but there was no evidence of hepatosplenomegaly. A urine dipstick showed 3+ of blood and no other abnormalities. A full septic screen, including blood cultures and urine microscopy, did not identify an infective cause for her presentation, nor had any prescribed or over-the-counter medication been recently started. The patient was managed conservatively with observation and monitoring of her blood counts. Ten days later, due to anaemic symptoms, the patient was transfused an additional 2 units (one A RhD+ and the other A RhD-), with a pre-transfusion Hb of 61 g L −1 . Post-transfusion, the patient was closely monitored on the inpatient ward, and her Hb continued to decline, with a nadir of Hb 48 g L −1 noted 5 days after her last red cell transfusion. A peripheral blood film showed spherocytes, and direct antiglobulin test (DAT) was negative. There were no alloantibodies detected on

Pregnancy in Thalassemia

“…Vaeusorn et al (1988)72 reported pre-eclampsia in 18% and congestive heart failure in 9% of 34 pregnancies among 29 Thai women, while in Italy only 5.5% had pre-eclampsia and none had heart failure 2. Aiken et al (2018)73 have recently reported a case of hyperhemolysis in a pregnant patient with Hb H disease who was treated with intravenous methylprednisolone for three days, and 0.4 g kg–1 intravenous immunoglobulins for five days. After six days of post-treatment, the patient’s hemoglobin gradually recovered to her pre-transfusion level of 6.8 g/dL.…”

Immunohaematology: the core of laboratory transfusion practice

“…Aiken et al . describe a case of hyperhaemolysis in pregnancy where the inherited haemolytic anaemia secondary to HbH disease and transfusion appeared to provide a stimulus for the development of hyperhaemolysis (Aiken et al, ). The case clearly illustrates the typical features of the hyperhaemolysis syndrome, namely, a fall in Hb to lower than pre‐transfused levels, a reticulocytopenia and hyperferritinaemia, often without alloantibodies or a positive DAT.…”