Category Archives: Haemoglobinopathy

Case 73 – summary

This week our case involved the care of a 10 year old girl with SCD who had transfered her care to the UK. She presented in outpatient clinic for the first time and we had to determine her management. During … Continue reading

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Case 73 – update 2 

Given the girls high Right MCA doppler at screening the patient started regular transfusion for prevention of CVA. This was initiated due to Adams et al 1998 NEJM paper that showed a 92% risk reduction in patients with high dopplers … Continue reading

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Case 73 – update 1

Thanks for all your suggestions so far. When the patient was seen for the first time the following was established: History: She is now age 10 and moved to the UK from west Africa a few months ago with her … Continue reading

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Case 73 – the beginning

A 10 year old girl attends paediatric outpatient clinic with a history of Sickle Cell Disease. She was born abroad and has just moved to the UK.  What history should you establish?  What should you note on examination? How would … Continue reading

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Case 50 part 2 – summary

In haematology it is important integrate laboratory investigations to clinical presentations. A blood transfusion was requested in a young patient with macrocytic anaemia with no previous blood count information. The blood film was reviewed rapidly:   The diagnosis of a … Continue reading

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Case 50 part 2 – the beginning

A 21 year old female presents with chest pain. You are asked to authorise a blood transfusion as she is anaemic. Hb 69g/L (115-160) MCV 111fL (79-98) WCC 12.6×10*9/L (4-11) Neuts 10.6×10*9/L (2-7) PLT 578×10*9/L (150-400) Here is her blood … Continue reading

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Case 49 – summary

Thanks to everyone who  tweeted for our case! The learning points are summarised below.   The case was based around a nulliparous 27 yr old women with sickle cell disease.  She wished to seek prior to conception.  Ideally pregnancy’s should … Continue reading

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Case 49 – update 2

The patient presents to a&e with shortness of breath at 22weeks gestation.   how would you assess this patient? management? differential?

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Case 49 – update 1

Partner testing reveals beta- thal trait.  How would you council this lady? The patient was commenced on hydroxycarbamide two years ago due to recurrent acute chest crisis.   As suggested by one of our followers – advice would be to … Continue reading

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Case 49 – the beginning

Our case this week focuses around a 27 year old lady who is know to have sickle cell disease.  She attends the joint haematology/obstetric clinic for preconceptual counselling.   As the junior haematology registrar in this clinic, consider how you … Continue reading

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Case 36 – update 2

Following testing of the father, he is also found to be a carrier of Beta-thalassaemia.  The pregnancy continues, and following counselling the family decline any further investigation until the fetus is born. What would be the options for testing prior … Continue reading

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Case 36 – update 1

Patients’ blood film looks like this: Ferritin level was also checked : 10 ug/l Family origin questionnaire has been mentioned and should be performed at all antenatal visits.  However all women – in both high prevalence and low prevalence areas … Continue reading

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Case 36 – the beginning

A 22 year old women has her antenatal booking in bloods taken. Her FBC shows Hb 10.2 g/dl MCV 71 fL (77-95fL) MCH 22 pg (26-33 pg) MCHC 33 g/dl (31-36g/dl) RDW 12.5% (11.5-14.5%) What features may be present on … Continue reading

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Case 34 – summary

This was a varied case centered around an unwell patient with sickle cell anaemia post surgery. There were a number of themes to cover in this case reminding us that patients with sickle cell anaemia can be complex and require … Continue reading

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Case 34 – update 4

Our patient with sickle cell anaemia has been investigated for a delayed haemolytic transfusion reaction. However, the reticulocyte count was not elevated and the direct antiglobulin test was not positive as would be expected. Similarly there was no new antibody … Continue reading

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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 … Continue reading

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Case 34 – update 2

We have a patient with sickle cell anaemia post op who is now more anaemic. She also has a sickle cell crisis with abdominal pain. She has a rather elevated CRP which may be post op or due to the … Continue reading

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Case 34 – update 1

Thanks for all of the great contributions on Twitter via #teamhaem We have initiated basic treatment for patients with a potential sickle cell crisis: Oxygen if hypoxic Hydration Analgesia Keep warm Low threshold for antibiotics Septic screen Our patients investigations show: … Continue reading

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Case 34 – the beginning

Welcome back to #teamhaem. Great you have you on board with this new case. We start in the emergency department. A 16 year old female with known homozygous sickle cell anaemia presents to your hospital with abdominal pain and feeling lightheaded. … Continue reading

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Case 31 part 5 – summary

The patient’s blood film shows target cells, sickle cell, Howell Jolly bodies. It is difficult to say what the underlying condition is other than a sickling syndrome with hyposplenism. Target cells are often found in all of these disorders, but … Continue reading

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