Case 114 – Summary!

Acute Promyelocytic Leukaemia

Thank you for everyone’s contributions throughout our case this week!

It started as a seemingly simple advice call regarding a platelet count of 51. However the clinical history of marked bleeding was inconsistent with this platelet count prompting a prompt review. Further investigations identified a progressive pancytopenia and DIC. Acute Promyelocytic Leukaemia (APL) was diagnosed following a bone marrow biopsy.

The patient had non-high risk APL (WCC <10) and began induction therapy with ATRA/Arsenic. Our patient experienced a WCC rise from 3.6 to 18 secondary to therapy prompting the need for dexamethasone prophylaxis for differentiation syndrome. Unfortunately she developed prolonged QTc and arrhythmias. Her arsenic was temporarily withheld until the QTc <460ms. She had also been commenced on other medications that prolong the QTc which were stopped – highlighted the importance of cautious prescribing.

Below is a summary of APL:

APL is a subtype of acute myeloid leukaemia (AML) accounting for approximately 5-8% of all AML cases. It has better outcomes than most forms of AML with ~90% achieving long-term remission.

Clinical features:

  • Bleeding / bruising (haemorrhagic manifestations are a common presentation and often more marked than non-APL AML due to DIC)
  • Symptoms of anaemia
  • Infections
  • Thrombosis (less commonly)
  • Often symptoms have developed reasonably rapidly prior to diagnosis (days / weeks)

 

Investigations:

FBC: typically cytopenias. The WCC is not usually greatly elevated.

Coag screen (Clauss fibrinogen), DDimer

U+Es, LFTs, CRP.

Viral screen

Blood film: may not be diagnostic.

Bone marrow

 

Morphology

Acute hypergranular promyelocytic leukaemia

  • Abnormal promyelocytes: large cells, pink/purple granules, faggot cells (bundles of Auer rods)

Variant hypogranular form

  • WCC is usually elevated
  • Abnormal promyelocytes : fine granules or agranular. Nucleus is frequently bilobed.

Immunophenotype: CD45+, CD33+, MPO+, CD117+, CD13+/-, CD34-, HLA-DR-, CD2+/-, CD56+/-

Molecular diagnosis of PML-RARA fusion

  • Mandatory
  • t(15;17) resulting in PML-RARA fusion protein (NB rarer variants of this are also reported). This functions as a transcriptional repressor resulting in arrested differentiation.
  • Various techniques available
    • Reverse transciptase polymerase chain reaction – must be performed to enable definition of type of PML/RARA isoform and quantification for subsequent MRD evaluation. Other techniques provide a more rapid diagnosis and are often performed in tandem.
    • Conventional karyotype
    • FISH
    • (Alternative for genetic diagnosis: PML nuclear staining in leukaemic cells using anti-PML monoclonal antibodies)

 

Management

 Blood transfusion Support – To be started as soon as APL suspected

  • Essential as haemorrhage is main cause of early death
  • To be initiated prior to genetic confirmation of diagnosis
  • Frequent monitoring of coagulopathy (coag screen/DDimer) required
  • Platelet transfusion: aim plt >30-50×109/L
  • FFP/cryoprecipitate/fibrinogen: aim INR <1.5, fibrinogen >1-1.5g/l
  • Invasive procedures should be avoided whilst coagulopathic

 

All trans retinoic acid (ATRA) – To be started as soon as APL suspected

  • ATRA causes the differentiation of the abnormal promyelocytes. In rare variants RARA is fused to other genes and ATRA is not successful.
  • To be started as soon as APL suspected (do not wait until genetic confirmation)
  • Prophylactic corticosteroids to be considered if WCC >5-10 x 109/L (or if WCC increases after ATRA started due to ATRA-induced differentiation). However no definitive evidence showing clinical benefit.
  • Monitor for Differentiation syndrome
    • Unexplained fever, weight gain, peripheral oedema, hypoxia, interstitial pulmonary infiltrates, hypotnsion, acute kidney injury
    • Management: dexamethasone 10mg IV twice daily. Temporarily withhold ATRA/ATO in severe cases.

 

Non-high-risk APL (WCC <10×109/L)

  • NB risk based on WCC not on other leukaemic characteristics e.g CD56 expression, FLT3 mutation
  • ATRA and ATO for induction (daily until CR or for a maximum of 60 days)
  • ATRA + ATO vs ATRA + chemo: associated with greater efficacy, less myelosuppression, fewer infections but increased rates of deranged LFTs and QTc prolongation.
  • If WCC rises >10 x 109/L after ATRA/ATO started this should be considered as ATRA/ATO-induced differentiation. The case should not be reclassified as high-risk disease. The median peak occurs at ~10 days after starting treatment
  • Consolidation: 4 courses of ATO (4 wks on, 4 off) and 7 courses ATAR (2wk on, 2 off)
  • No maintenance required

 

Specific treatment considerations with ATO:

  • If significant WCC rise (10 x 109/L) cytoreductive therapy can be considered (hydroxycarbamide, or in severe hyperleukocytosis idarubicin or mylotarg)
  • Maintain serum K>4, Mg >1.8
  • Perform ECG minimum x2/week. Calculate QTc using Bazett formula
  • Avoid medications that prolong QTc interval if possible
  • If QTc >500ms or develop syncope, tachycardia or arrhythmia
  • Admit patient
  • Optimise electrolytes
  • Temporarily withhold ATO
  • Stop other medications that prolong QTc interval if possible
  • When QTc <460ms and electrolytes replete restart ATO at 50% with intention of increasing to 100% if stable.
  • Time to achieve complete remission (CR) is often longer than with ATRA+ATO vs ATRA+chemo. Treatment should be continued until CR is achieved – this can be up to 8-10 weeks.

 

High-risk APL (WCC >10X109/L)

  • ATRA + chemotherapy (idarubicin or daunorubicin alone or in combination with cytarabine)
  • (In Europe ATO is not approved for high risk APL but otherwise could consider ATRA/ATO + chemo regimens)
  • Cytoreductive chemotherapy should be started promptly even if molecular confirmation is still awaited.
  • Avoid leukapheresis due to the risk of haemorrhage

 

MRD monitoring

  • Use RT PCR with sensitivity 104
  • Based on bone marrow sample.
  • To be performed at completion of consolidation
  • In non-high risk patients who achieve CR MRD negativity prolonged MRD monitoring is not required due to very low risk of relapse
  • In high risk patients perform BM MRD every 3 months for 3 years post completion of treatment
  • If PCR+ post completion of consolidation repeat within 2 weeks.

 

Relapsed disease

  • Molecular relapse (2 successive PCR+ assays analysed in 2 laboratories) is highly predictive of frank haematological relapse and should be treated promptly
  • Salvage therapy is based upon on patients 1st line therapy (if CR1 lasted <2years):
    • Relapse post ATRA/ATO treat with ATRA+chemo
    • Relapse post ATRA+chemo treat with ATRA/ATO
    • Aim to consolidate with autologous HSCT providing MRD negativity achieved
  • If CR1 >2 years can consider repeating original regimen
  • If CNS relapse weekly triple intrathecals should be performed alongside systemic therapy until by complete clearance of blasts in CSF. A further 6-10 intrathecals should be performed.

 

References

  1. Sanz MA et al. Management of acute promyelocytic leukaemia: updated recommendations form an expert panel of the European LeukaemiaNet. Blood. 133(15): 1630-1643. 2019.
  2. Bain BJ et al. Bone Marrow Pathology. Fourth Edition. Wiley-Blackwell. 180-183. 2014.
  3. Sanz MA and Montesinos P. How we prevent and treat differentiation syndrome in patients with acute promyelocytic leukaemia. Blood. 123:2777-2782. 2014.

 

 

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Case 114 – Update 5!

Thanks to everyone’s advice our patient didn’t develop differentiation syndrome following administration of dexamethasone prophylaxis. She also received hydroxyurea in addition to ongoing ATRA / arsenic to reduce the WCC. Her DIC is also improving. She has had issues with nausea/vomiting which is predominantly controlled on antiemetics and a infection for which she is on oral antibiotics.

However she is currently week 4 of induction and informs the staff that for the last few days she has been experiencing intermittent marked dizziness. She is currently asymptomatic and her observations are stable. There is no evidence of sepsis.

Questions:

  1. How would you want to investigate the dizziness? What is your likely differential?

 

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 on Twitter 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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Case 114 – Update 4!

Thank you for the ongoing contributions to our case. Following everyones input our 43 year old female patient has been commenced on intravenous arsenic alongside ATRA. This decision was made as her APL is considered non-high risk as initial WCC <10. However on day 8 her WCC has increased to 18.

She continues to have ongoing blood product support to achieve:

  • Plts >30-50
  • Clauss Fib >1-1.5
  • INR <1.5

Questions:

  1. What is the likely cause of the WCC rise?
  2. Do we need to alter treatment and if so how?
  3. How long would you aim to maintain the above plt / coag thresholds provided the patient doesn’t have any significant bleeding?

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 on Twitter 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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Case 114 – Update3!

Thanks for everyones contributions to our case so far! We now have confirmation of our suspected diagnosis of Acute Promyelocytic Leukaemia by FISH. We have already began discussing initial management. Following the suggestions to date we have instituted the following:

  • ATRA treatment. With monitoring for differentiation syndrome. We have not started prophylactic dexamethasone as WCC 3.6 when ATRA started.
  • Intensive blood product support

There is some debate from responses of what definitive treatment to commence between arsenic, chemotherapy or combination of arsenic and chemotherapy.

Questions:

  1. How do we risk stratify patients with APL? How does this influence our treatment decision?
  2. What other factors/investigations would you consider/perform prior to commencing your chosen treatment?

 

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 on Twitter 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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Case 114 – Update 2!

As highlighted by our followers the patients degree of bruising is out of proportion to a platelet count of 51. As requested a thrombin time and ddimer have been performed and are both prolonged.

Thrombin time: 19 Ddimer 6320 (reminder: PT 27, APPT 45, Clauss Fib 0.9)

We’ve also been informed that the patient is also developing new mucosal blood blisters. A repeat FBC is more deranged Hb 95, plts 34, WCC 3.6, Neuts 0.6. Film still not diagnostic. Viral screen as requested already in progress.

Questions:

  1. What is the explanation for the clotting results?
  2. How would you want to further investigate this progressive pancytopenia and above deranged clotting?

 

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Case 114 – Update 1!

Thank you for all your contributions. Following your requested information and initial investigations you now know:

Our 43 year old patient has previously been fit and well with no prior significant medical history. Over the past 2 weeks she has developed marked bruising – the largest of which is approximately 10cm on her thigh. She has no history of trauma and no personal or family bleeding history. She is feeling tired but has no other associated symptoms. She has no relevant travel history. She is not on any regular medications and does not drink in excess. She has Apart from the bruises her examination is unremarkable.

FBC: Hb 106, Plts 51, WCC 3.7, Neuts 1.2.

Blood film: confirms the above FBC differential but is otherwise unremarkable.

Coag: PT 27, APPT 45, Clauss Fibrinogen 0.9.

LFTs, UEs, Haematinics all normal.

Questions:

  1. What further investigations would you want to perform in this outpatient?

 

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 on Twitter 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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Case 114 – The Beginning

You are the haematology registrar on-call and a GP contacts you for advice regarding investigation and management of a platelet count of 51. The GP performed a FBC on a 43 year old woman who had presented to him with marked bruising.

FBC differential: Hb 106, Plts 51, WCC 3.7, Neuts 1.2

Questions:

  1. What further information from the history would you want?
  2. What initial investigations would be relevant?

 

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 on Twitter 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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Case 113 – Summary

Our patient was found to have systemic anaplastic lymphoma kinase (ALK) positive anaplastic large cell lymphoma (ALCL).

ALK-positive ALCL is a rare, mature T-cell non-Hodgkin lymphoma (NHL).

Key learning points:

1. Epidemiology

  • 3% of all adult NHL
  • 10 – 20% of all childhood NHL
  • More common in males 1.5:1 (male:female)
  • Commonly occurs before the age of 30

2. Clinical features

  • Frequently involves extranodal sites such as skin, soft tissue, liver, lung, bone
  • Rarely involves the mediastinum, gut or CNS
  • Majority of patients present with advanced disease (stage III/IV) ~70%
  • Majority of patients present with B symptoms ~75%

3. Pathology

    Lymphoid cells are usually large with abundant cytoplasms and pleiomorphic ‘horseshoe-shaped’ nuclei, this is known as then ‘common pattern’. Variant histology includes the small cell and lymphohistiocytic patterns.
    Pathological cells contain a chromosomal translocation that involves the ALK gene and express ALK protein and CD30.

4. Prognosis

  • Risk stratification should be completed using IPI index
  • ALK positivity is an important prognostic marker as 5 year overall survival is greater in patients with ALK+ ALCL (~80%) than in ALK- ALCL (50%), however this may reflect the comparatively younger patient age group, rather than ALK expression

5.Treatment

First line

  • CHOP – cyclophosphamide, doxorubicin, vincristine, prednisone
  • CHOEP – as above plus etoposide

Consolidation

  • Auto-SCT

Second line

  • GDP – gemcitabine, dexamethasone, cisplatin
  • Brentuximab vedotin (NICE approved in the UK for a minimum of 8 and maximum of 16 cycles)

Consolidation

  • Auto/allo

Clinical trials:

  • Brentuximab vedotin as 1st line therapy?

References:

WHO classification of Tumours of haematopoietic and lymphoid tissues (2017) Swerdlow et. al.

The biology and management of systemic ALCL, Hapgood et. al. 2015 Jul 2;126(1):17-25. doi: 10.1182/blood-2014-10-567461

https://www.ncbi.nlm.nih.gov/pubmed/25869285

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Case 113 – update 1

Thank you for your contributions so far…

Initial work-up:

Our patient has expressed a short history of significant weight loss, fatigue and night sweats. In addition he has palpable superficial adenopathy. He has no clear infective history.

Examination reveals widespread lymphadenopathy.

His initial blood work shows a normal FBC, biochemistry profile, LDH and viral screen.

Specialist review:

Our patient is referred to Haematology for further assessment of suspected lymphoma.

A CT neck to pelvis confirms extensive, size significant lymphadenopathy above and below the diaphragm, with radiological changes suggestive of pulmonary involvement.

Pathology:

A needle core lymph node biopsy (groin) reveals abnormal nodal architecture with a predominant population of large cells with kidney-shaped nuclei and an eosinophilic region nears the irregular nuclei.

Questions:

1) Would you request any further staging investigations?

2) Based on the abnormal lymph node morphology findings, which immunohistochemical stains would you request?

3a) What is your preferred likely diagnosis?

3b) What is the most important prognostic marker in this case?

Join us on Twitter @TeamHaem and let us know what your thoughts are, what questions you have and what you want to do as we see this case evolve over the next week. 

Remember to use #teamhaem on all your posts to help us follow the case! 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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Case 113 – the beginning

Welcome to our new #TeamHaem case!

Tim, a 31 year old teacher, has made an urgent GP appointment to come and see you. He tells you that he feels exhausted and is falling behind in his work. In the last 4 weeks he has also noticed a lump in his left groin.

  • What further questions would you ask Tim?
  • What would you look for on examination?
  • What investigations would you arrange?

Join us on Twitter @TeamHaem and let us know what your thoughts are, what questions you have and what you want to do as we see this case evolve over the next week. 

Remember to use #TeamHaem on all your posts to help us follow the case! 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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Case 112 SUMMARY

Our case presented a 34 year old lady with known sickle cell disease to the A&E department complaining of shortness of breath and chest pain.  The case starts by reminding us of the basic management of a patient with sickle cell disease.

  • Oxygen
  • ABG
  • Fluids
  • Analgesia
  • antibiotics
  • chest x-ray
  • Physiotherapy – incentive spirometry
  • Involvement of HDU/ITU team
  • FBC, Basic biochemistry, group and screen, blood cultures
  • sputum culture
  • Consideration of top up transfusion or exchange

 

Whilst treating the patient for ACS, suspicion was raised of a possible PE.    VTE in adults with sickle cell disease is a recognised important clinical complication and is likely in part to be related to the hypercoagable state. Pulmonary embolism may present with chest pain, dyspnoea and hypoxia. If there is a high clinical suspicion of pulmonary embolism (i.e. sudden onset unilateral pleuritic pain that is not typical of sickle pain) treat for both conditions pending imaging. ACS may be complicated by pulmonary embolism or may occur secondary to pulmonary embolism and treatment will be required for both conditions simultaneously.

Risk factors for VTE in sickle (reference 1)

  1. Sickle cell disease is an ongoing risk factor for VTE.
  2. Genotype with HbSS and HbSbeta0 having the highest risk compared to HbSC or SBeta+.
  3. Gender – women are at higher risk
  4. Number of crises – > 3 admissions per year
  5. Hospitalisation within 90 days
  6. elevated tricuspid regurgitant jet velocity on cardiac ECHO
  7. Indwelling catheter

 

Use of d-dimer levels at diagnosis are uncertain, as baseline levels in patient with sickle cell disease who are clinically well, are often elvated. Therefore performing a d-dimer is unlikely to be helpful.

Imaging (reference 1)

  • CTPA – results may be confounded in SCD disease patient experiencing ACS due to high prevalence of in situ pulmonary thrombosis.  There can also be small subsegmental filling defects secondary to sicklingnin the absence of ACS
  • CTPA may lead to contrast induced kidney damage
  • V/Q – minimises radiation exposure, and there is no risk of kidney damage, however it may be less useful in patients exhibiting abnormalities on chest x-ray

Treatment options for sickle patients are no different to that of the general population but thought must be given to the higher risk of recurrence in this population and their bleeding risk. Data has suggested a high bleeding risk in patients with sickle after a VTE, with a rate of 2.9% at 6 months and 5 % at one year. (Reference 3). This is in line with patients treated for cancer related thrombosis on anticoagulation.

Risk of recurrence of VTE in sickle patients is similar to those individuals in the general population who have had a unprovoked VTE. Individuals factors need to be considered. Patients with >3 admissions per year had a recurrence rate of 37%. Therefore a careful balance is required between bleeding risk and risk of recurrence.

In this case the patient was female of child bearing age which may add further complications to the treatment plan. This simply highlights the importance of an individualised plan for each patient, which considering the impact of the underlying sickle cell disease.

‘How I treat and diagnose thromboembolism in sickle disease’ provides an excellent resource. Here is a summary of diagnosis and treatment from this document available in blood journal.

(Reference 2)

 

References

1. How I treat and diagnose thromboembolism in sickle cell disease. Shet, A and Wun, T. Blood 2018.

http://www.bloodjournal.org/content/132/17/1761.long?sso-checked=true

2. Sickle cell disease: an inherited thrombophilia. Wun, T, Brunson, A. Haematology Am Soc Hematol edut program 2016

3. Increased incidence of VTE in sickle cell disease patients: risk factors, recurrence and impact on mortality. Brunson A et al. British journal of haematology 2017

.

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Case 112 update 2

Imaging has confirmed a pulmonary embolism.  How would you manage this patient?  What would be our long term management?

 

She has been expressing the desire to become pregnant, what you be your advise?

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Case 112 update 1

Baseline observations – sats 89% room air, HR 126, BP 111/65, RR33, temp 36.5

Chest x-ray – NAD

D-dimer – 775 ( normal range 208-318 ng/ml)

Hb 67 g/L

Retics – 4%

HbS 40%

eGFR 70 ml/min

Urinalysis 1+ protein

 

You manage to find out a little more about the background of the sickle cell disease:

HbSS

Recent hospital admission for acute chest crisis, approximately 3 weeks ago.  2 further admissions for painful crisis in past year.

Medications:  Hydroxycarbamide, penicillin, folic acid

Baseline Hb 7g/L

Proteinuria noted on last annual review and mild decline in eGFR, however planned for continued monitoring currently.

Vaccinations up to date

Echo performed 6 months ago had no significant findings

Noted to have red cell antibodies

 

You have commenced treatment for acute chest syndrome, but you want to rule out PE.  Does the elevated d-dimer help you in this diagnosis?

What are her risk factors for VTE?

How would you investigate further?

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Case 112

Welcome to our next case.

 

A 34 year old female presents to A&E with increased shortness of breath and chest pain.

You are contacted as the haematology registrar oncall, by the SHO working in A&E as the patient has informed the team she has sickle cell disease.

 

What do you advise?

What investigations are required?

What are you differentials?

 

 

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Case 111 – transfusion related complications – summary

So thanks for all of your input into our 2 short cases which focused on transfusion in cases of anaemia and some of the complications which you may get asked about.

Most important points to make are that blood transfusion is now very safe but preventable morbidity and death still occurs and it is therefore important to try to reduce this risk by avoiding unnecessary transfusion and minimising transfusion where it is required.

Acute transfusion reactions include allergic reactions of varying severity to anaphylaxis and life-threatening events, acute haemolytic transfusion reactions especially ABO incompatibility, bacterial contamination of the blood unit which can lead to fatal septic shock, TACO and TRALI

Severe acute transfusion reactions cause major morbidity and it is imperative to stop the transfusion and assess the patent clinically and resuscitate if necessary.  Check that the blood product compatibility label matches the patient’s ID band and that it is the correct patient and inspect the unit for clumps or discolouration.

Unless it is a mild allergic or febrile reaction, FBC, renal and liver function, urine Hb should be performed. In case of significant temperature increase or sustained febrile reaction, rigors, myalgia, vominitng or loin pain, investigations may include: FBC, renal and liver function, urine Hb, repeat group and save and cross match/compatibility testing, DAT, LDH, haptoglobin, blood cultures, coagulation screen. Blood products in question should  always be returned to the lab for further investigation.

Transfusion Associated Circulatory Overload is the most commonly reported cause of transfusion-related mortality and major morbidity with 92 cases reported to SHOT in 2017. A formal pre-transfusion risk assessment for TACO should be undertaken looking at factors including: pre-existing diagnosis of heart failure, regular diuretics, undiagnosed respiratory symptoms, significant positive fluid balance, concomitant IV fluids, peripheral oedema, renal impairment, and body weight. Consider if the transfusion can be avoided and other treatment may be appropriate eg. haematinic replacement, giving prophylactic diuretics and also review after each unit if more blood is required.

Transfusion related lung injury (TRALI)  usually presents within 2 hours of transfusion with non-cardiogenic pulmonary oedema, and sometimes hypotension, fever and rigors. CXR may show bilateral nodular shadowing. Patients may require high flow oxygen and ventilation but diuretics may worsen the situation.

Acute haemolytic reactions vary in severity but ABO-incompatible transfusions are the most serious causing intravascular haemolysis, shock, acute renal failure and DIC.  They may deteriorate within minutes with loin, chest or abdominal pain and a feeling of ‘impending doom’. They may become tachycardic and hypotensive with fevers, rigors, collapse, flushing or urticaria. Start ABC resuscitation, get help and involve ITU if necessary, contact the lab immediately and return the transfusion pack.

Treat suspected anaphylaxis immediately with 0.5ml 1:1000 IM adrenaline and escalate to ITU, consider steroids and antihistamines later, and contact the lab immediately and return the transfusion pack. Liaise with immunology regarding future transfusions.

If bacterial contamination is suspected start antibiotics quickly (usually as per the neutropenic sepsis protocol) and undertake a septic screen including blood cultures, contact the lab immediately and return the transfusion pack. The lab will contact the blood service to discuss recall of related blood products as well as perform microbiology testing.

Moderate reactions may include a temperature >= 39 degrees or an increase of >=2 degrees or other moderately severe symptoms. If symptoms are new after initiation of transfusion, discontinue the transfusion and investigate. If symptoms are consistent with the patients pre-transfusion condition, consider continuing the transfusion at a slower rate and symptomatic management.

Mild reactions such as an isolated temperature of >= 38 degrees or rise of 1-2 degrees or itch or rash only, consider bacterial contamination as a possibility, review the patient’s pre-transfusion condition and transfusion history. and monitor closely. Consider symptomatic treatment such a paracetamol and continue the transfusion.

Moderate and severe reactions should be discussed with the transfusion team and reported to SHOT/MHRA as appropriate.

References:

Handbook of Transfusion Medicine, fifth edition. Available at http://www.transfusionguidelines.org.uk.

PHB Bolton-Maggs (Ed) D Poles et al. on behalf of the Serious Hazards of Transfusion (SHOT) Steering Group. The 2017 Annual SHOT Report (2018).

 

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Case 2 – update 1

He is 2 weeks into his first cycle of intensive chemotherapy. You have been asked to review him by the nursing staff as he has a unit of red cells running but his temperature is now 38.9 degrees and he is rigoring. What is your response?

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Case 2 – Introduction

A 34 yr old gentleman is an inpatient on the haematology ward. This is his film 2 weeks ago at diagnosis. Anyone want to comment?

image.png

image.png

 

 

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Case 1 – update 3

It is nearly midnight and the patient wakes from sleep feeling breathless and scared. The nurses ask you to come and assess the patient. His RR is 32, sats are 91% on RA, BP is 145/89 and HR is 134. What more do you want to know/do? What are your concerns?

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Case 1 – update 2

Thanks for all your comments on the blood film – you mentioned  hypersegmented neutrophils, tear drop cells as well as a macrocytic anaemia, possible ovalocytes and some rouleaux. 

You add a B12 and folate level as well as immunoglobulins onto the bloods.

You peruse the hospital records system to try to find out more about the patient. Previous letters suggest alcohol excess and previous pancreatitis.

The folate level comes back at <2ug/L and vitamin B12 is 112 pmol/L.  By the time these are back you find that the patient is already completing his third unit of blood on the admissions suite. Do  you have any further advice for the admission suite?

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Case 1 – update 1

Thanks for all your requests for more information!

The gentleman regained consciousness before the ambulance crew arrived and is now GCS 14 – he remains confused and is unsure of how he came to be in hospital.  He says he feels fine and wants to go home, and denies any past medical history or use of medications. He denies a high alcohol intake but is becoming quite agitated and you suspect the history you are getting from him is likely to be inaccurate.

Observations on admission show a tachycardia of 111bpm, BP 92/65, RR 13, sats 97% on room air and apyrexia. A&E noted that the gentleman looked a little dishevelled, cachectic and has spider naevi across his chest. They also noted a soft flow murmur.

Other blood results as requested:

Hb36g/L, MCV 139fL, WBCs 7.2×10*9/L, Plts 153×10*9/L, Retics 42×10*9/L, DAT negative

Na 132 mmol/L, K3.1 mmol/L, Urea 8.8 mmol/L, Creatinine 58 umol/L,

Bilirubin 32 umol/L, ALT 64 U/L, ALP 32 U/L

LDH 267 U/L, Urate 432 umol/L, Haptoglobins in progress

CRP 19 mg/L

ferritin 453ng/ml

TFTs in progress

ESR in progress

Blood film:image.png1 - 1.jpg

What do you think of the blood results and the film? (apologies for the background marks which are artefact). Are there any further tests you would like to request at this point?

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