Hyperhaemolysis in a patient with HbH disease

Abstract

Dear Sir, Hyperhaemolysis is most frequently described in patients with sickle cell disease, although there are cases of this event occurring in patients with thalassaemia and myelofibrosis (Grainger et al., 2001;Treleaven & Win 2004). We report a case of hyperhaemolysis in a patient with Haemoglobin H (HbH) disease, and outline our institutions approach to the management of hyperhaemolysis.A 65-year-old lady with HbH disease and baseline haemoglobin (Hb) of 85 g L −1 , presented to hospital with breathlessness which was felt to be secondary to exacerbation of her asthma. Her Hb at the time of admission was 74 g L −1 , in view of her hypoxia she was transferred to the intensive care unit where she was treated with antibiotics and transfused 4 units of red cells. Iron studies pre-transfusion revealed a serum iron of 2·5 μmol L −1 (11-29 μmol L −1 ) and transferrin saturation of 8%. She made a good recovery and was discharged home, at this time her Hb was 115 g L −1 .She presented to hospital 12 days later with general lethargy, fever, right upper quadrant discomfort and dark urine. The Hb was 77 g L −1 , bilirubin 266 (0-21 μmol L −1 ), C-reactive protein CRP 125 (0-4 mg L −1 ). She was diagnosed with cholecystitis and commenced on antibiotics. In less than 24 hours her Hb had dropped to 51 g L −1 . At this stage haematology was contacted and the provisional working diagnosis was a delayed haemolytic transfusion reaction (DHTR).Lactate dehydrogenase (LDH) was >2000 IU L −1 (240-480 IU L −1 ), reticulocyte count 68 × 10 9 L −1 (10-100 × 10 9 ) and haptoglobin was <0·08 g/L (0.3-2.0 g/L). The direct antiglobulin test (DAT) was moderately positive against IgG 3+ and anti C3d 2+. Pre-transfusion red cell immunology detected the presence of a historical anti E, post-transfusion anti-Jkb and anti-Lua were detected by indirect antiglobulin test (IAT). The eluate revealed anti-Jkb and anti-Lua by IAT. On review of the pre-transfusion units, two of four packed red cell units were phenotyped and shown to be Jkb+ve and the remaining two were Lua+ve.The Hb continued to drop and was 44 g L −1 the following day. The patient became increasingly reticulocytopenic with a reticulocyte count of 13 × 10 9 L −1 and it was felt that the DHTR had triggered hyperhaemolysis with a drop in Hb below pre-transfusion baseline.

Hyperhemolysis in Patients With Hemoglobinopathies: A Single-Center Experience and Review of the Literature

“…Bone marrow aspirates carried out in a number of cases of hyperhemolysis have clearly showed increased erythroid activity, including one of our own patients with HbH [30]. This supports the role of peripheral consumption (due to macrophage activation) in reticulocytopenia.…”

Hyperhaemolysis Syndrome: A Review of Cases

“…However, there is growing evidence that hyperhaemolysis is not restricted only to patients with haemoglobinapathies [16]. Hyperhaemolysis subtype of Delayed Haemolytic Transfusion Reaction (DHTR) [18]. It is commonly present in patients with underlying sickle cell disease, but it can also be seen in patients with other underlying haemoglobinapathies.…”

“…It is well-known that sickle cell RBCs are more susceptible to lysis. If laboratory fi ndings suggest the absence of alloantibodies, it was suspected that the development of platelet antibodies resulting from the increase in the number of red blood cells during transfusion could lead to the lytic reaction [18]. Besides that, some proportion of it was noted to be due to antibodies to human leukocyte antigen.…”

“…can also be given on case-to-case basis. For example, in case of reduction vitamin B12 level, folic acid can be prescribed [18]. Additional blood transfusion should be avoided at all costs and if indicated, should be commenced and covered by steroids and IVIG.…”

Hyperhaemolysis in a pregnant patient with HbH disease

“…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, ). The increasing incidence of hyperhaemolysis now makes this condition reportable via the UK Serious Hazards of Transfusion scheme (Serious Hazards of Blood Transfusion (SHOT), ), and this potentially fatal complication results in challenging management issues in those affected.…”