Case 34 – update 3

Lots of great suggestions via #teamhaem.

Patients with sickling disorders need careful attention when operations are being planned. They should be carefully pre-assesed with involvement of haematologists and anaesthetists.  Important points include:

  • Respiratory function post op – high risk of chest crisis due to atelectasis and infection. Ensure pain free but not drowsy and encourage chest physio
  • Renal function – risk of renal failure
  • Venous thromboembolism – high risk
  • Infection – most, if not all, are hyposplenic/asplenic
  • Cardiac complications – especially if pre-existing pulmonary hypertension
  • Pain management
  • Keep warm and hydrated
  • Transfusion support – either simple top up or exchange transfusion

The team are concerned that the patient is so anaemic and below her baseline.  Typically in delayed haemolytic transfusion reactions the following occurs:

  • Anaemia
  • Raised reticulocytes (may not occur if bone marrow failure syndrome or haematinic deficiency)
  • Raised bilirubin (unconjugated)
  • Raised LDH
  • Low haptoglobin
  • Positive direct anti-globulin test
  • Spherocytes on blood film

Here are some results from our patient

  • WCC 16.7×10*9/L
  • Neuts 13.3×10*9/L
  • Hb 47g/L (baseline between 75 and 90g/L)
  • PLT 562×10*9/L
  • Retics 30×10*9/L
  • LDH 540U/L (240-380)
  • Bilirubin 102umol/L – unconjugated
  • DAT -ve

Here is the red cell panel from the patient. For those of you that are unfamiliar: the patient’s plasma (with any potential nasty red cell antibody) is mixed with standard/reference red cells along with anti-human globulin. If a positive reaction is found the red cells agglutinate (i.e. clump in the tube or column) then an antibody is present. We already know the patient has an anti-C and anti-Fya. The eluate does not reveal any other antibodies.

Red cell panel

Red cell antibodies make transfusion difficult. Patients with antibodies take longer to get safe blood and in some patients it can be very difficult. For those patients who are likely to depend on blood long term (e.g. patients with haemoglobinopathy, haemolytic anaemia, bone marrow failure) it is important to try and prevent the formation of red cell antibodies. Normally blood is only matched for ABO and RhD groups but for sickle cell patients blood is routinely matched for RhC/c/E/e and Kell. Before transfusions take place it is important to find out what antigens are on the patients’ red cells so when an antibody is found it is easier to work out what it is and isn’t. This is called ‘phenotyping’ but is not possible so soon after a transfusion due to the mixture of donor and patient red cells. In this instance genotyping may be need (i.e. working out the patient’s antigen status by DNA). In our case we have found out from the other hospital transfusion lab.

Questions:

  • Hyperhaemolysis syndrome has been mooted by the team – what is this and how is it treated?
  • What are the complications of long-term transfused patients?

Please reply to us (@teamhaem) on Twitter and always include #teamhaem to allow others to follow your comments. Please join in the debate and learn about haematological problems along the way. The case will continue to  evolve over the coming week so keep checking #teamhaem for more information.

Please note – all cases on TeamHaem are entirely fictional to protect patient confidentiality.

TeamHaem are not a position of authority.  It is an educational platform to allow discussion and learning.

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