Case 130 – the beginning

It is 5pm. You are the haematology SpR and you have just congratulated yourself on clearing the blood films and aspirates to report – good job! But just as you are taking off your lab coat, a transfusion BMS pops round to discuss an issue they have.

A sample has been sent for Group and Screen (G&S) on a patient not previously known to the trust. The clinical details state: ‘Patient recently moved to area. For 2 units RBC ASAP.’ The lab have performed ABO and D typing as well as an antibody screen and subsequent panel. The results of these are shown below:

ABO group:

ABO

Antibody screen:

Abscreen

Antibody panel:

Abpanel

The BMS has sent a sample to the Red Cell Immunohaematology (RCI) lab at NHSBT to investigate further, but wants your advice on how to proceed clinically….

A few things to think about:

  1. What ABO group is the patient?
  2. What does the antibody screen and panel demonstrate?
  3. What further clinical information do you require?
  4. Would you request any additional tests?

 

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 129 – Summary

This weeks case was based around Cerebral venous sinus thrombosis (CVST). Thanks for everyone’s help really lovely that so many of you contributed your thoughts.

Incidence:

This is a rare disorder affecting 2-4/million of the adult population a year but is becoming increasingly common with more widespread availability of MRI scanning.

It is more common in females (accounting for up to 75% of cases), particularly in pre-menopausal women.

Risk factors for CVST :

  • Hormonal contraceptives/Pregnancy
  • Inherited Thrombophillias
  • Acquired Prothrombotic states: Malignancy, Nephrotic syndrome, Myeloproliferative disorders, TTP, PNH etc
  • Head Injury
  • ENT and CNS infections
  • PEG Aspariginase in ALL therapy

Presentation:

This case illustrates that not all cases of CVST will present in the same way. The majority of patients will have headaches, however they are not always localised and of gradual onset and there are case reports of CVST mimicking Subarachnoid haemorrhage as in our case. Other important presentations not to miss include encephalopathy, generalised seizures and even new cranial nerve palsies.  Therefore it is important that a clinician remains alert to this in a differential diagnosis particularly in pre-menopausal women who are the highest risk group.

Pathophysiology:

The pathophysiology of CVST helps explain the varied clinical presentations that are seen:

  1. The thrombosis can occur at any site within the dural vein network.
  2. Initially in CVST collateral venous pathways and capillary dilation mitigates the effects, however these systems can be rapidly overwhelmed.
  3. The thrombosis obstructs the blood drainage from brain parenchyma. This increase in pressure leads to high venous and capillary back pressure causing oedema as plasma leaks into the interstitial space. The extra pressure then causes capillaries to rupture leading to local haemorrhage formation.
  4. Increased pressure in the venous system results in increased intravascular pressure causing a reduction in cerebral profusion which can cause ischaemic infarction.
  5. There is also a loss of CSF reabsorption that leads to elevated intracranial pressure and the symptoms and signs associated with this such as vomiting, visual loss, papilloedema and cranial nerve palsies.

Understanding these mechanisms can help to understand why anticoagulation is recommended even in the presence of cerebral haemorrhage.

Initial Management:

Initial management is with either unfractionated or more commonly LMWH. Evidence for recommendations are weak and based on the findings of a 2011 Cochrane review that looked at 79 patients in two RCT and it concluded

Based upon the limited evidence available, anticoagulant treatment for cerebral venous sinus thrombosis appeared to be safe and was associated with a potentially important reduction in the risk of death or dependency which did not reach statistical significance”.

The European Stroke organisation guidelines recommend treatment with heparin in acute setting even with those who have intracerebral haemorrhage at baseline. BSH guidelines are in agreement with the european guidelines and also suggest duration of initial LMWH should be for at least 7 days

Thrombolysis is generally not recommended unless clinical deterioration despite anticoagulation and it is clear this is deterioration isn’t related to new intra-cranial bleed. Surgical decompression is also an option in raised ICP with risk of herniation.

Our patient had an elevated BMI and therefore it is important to follow local trust protocol or summary of product characteristics of the LMWH for safe prescribing. Generally the advice is use a weight based therapeutic dose but split dose (to reduce bleeding risk). Anti-Xa monitoring should occur to ensure therapeutic levels are reached. Initial monitoring should be 3-4 hours after third dose.  In renal impairment unfractionated heparin would be a suitable alternative.

Long term anticoagulation:

Oral anticoagulation is to be avoided until patient is stable. There is no recommendation to re-evaluate with imaging but if concerns this could be considered especially if ongoing symptoms. In some centres this is done routinely prior to moving to long term anticoagulation. As with all Haemostasis and thrombosis situations the plan needs to be based on the specifics of the case and there is a lack of trial data to support decision making.

Recommendations for duration of longer term anticoagulation are based on the individual precipitating features for the CVST.

If there was a clear transient provoking factor then European stroke guidelines suggest 3-12 months of anticoagulation is sufficient. BSH guidance also suggests at least 3 months anticoagulation should be used. This guidance is based on two important observations:

  1. Recurrence of CVST is generally low between 1.5% to 4.4% based on retrospective studies.
  2. There is also evidence to show the majority of patients even those without anticoagulation do recanalise following thrombosis by 4 months.

 

Practically speaking patients with clear provoking factors generally have 3-6 months anticoagulation and unprovoked events may be anticoagulated up to 12 months or even longer term depending on perceived risks and benefits. Patients with recurrent CVST should be considered for long term anticoagulation.

Thrombophillia screens are generally not recommended in the guidelines and are unlikely to alter management unless strong family history of thrombosis. A young female patient with unprovoked event who may be contemplating pregnancy should have anticardiolipin antibodies checked as a minimum in line with RCOG Green top 37a guidance. It should also be pointed out that thrombophillia screening should not occur during acute phase of thrombosis or be performed on anticoagulation. Patients who have been deemed appropriate for long term anticoagulation should not have thrombophillia screening as unlikely to alter management in these cases.

At present warfarin is recommended as the anticoagulant of choice for longer term anticoagulation. However, in context of active malignancy LMWH may be a more appropriate choice at preventing recurrent VTE. In pregnancy again LMWH should be continued.

In our patients case Direct oral anticoagulants are not suitable given the patients elevated BMI. There are however new studies that have been preformed looking at the possible safety of Dabigatran and other DOACs in CVST. Most of the evidence prior to 2019 had been based on single centre observational studies with less than 20 patients which concluded that Rivaroxaban and Dabigatran appeared safe.

The RE-SPECT CVT study which was a prospective randomised open-label trial with a blinded end-point adjudication reported in JAMA neurology in 2019. It recruited 120 patients in 9 countries across 51 sites. It found that Dabigatran and Warfarin were associated with low risk of recurrent VTEs (No VTE in both arms over 6 months follow up)  and that risk of bleeding was similar (1 Intestinal bleed on Dabigatran and 2 ICH in warfarin group) . The authors therefore conclude that “Dabigatran may be safe and effective in preventing recurrent VTEs”.  This trial was published after the European stroke guidelines were released as yet in Europe this approach has yet to be adopted into practice. It should be highlighted that DOACs are not recommended if thrombosis in context of antiphospholipid syndrome.

In summary CVST is an interesting condition with varied presentation and clinical course. Generally it has low recurrence rates and can be managed with short term anticoagulation. The mainstay of therapy is anticoagulation and haemorrhage associated with CVST is not a contraindication to therapy. In the future there may be a role for use of DOACs in the management of CVST but this remains the subject of clinical trials and is yet to be adopted by the guidance in UK and Europe.

The end of our case 129….

With all this evidence in mind our patient was reviewed in clinic. Her history revealed she had several possible risk factors for the CVST she has an elevated BMI and was on the COCP at the time of the event. She also had a finding of an isolated lupus anticoagulant at presentation without anticardiolipin or beta 2 glycoprotein 1 antibodies. Her antibodies were repeated while on anticoagulation and were negative at 12 week time point. She had no family history of VTE or personal history of thrombosis. She reported no issues on warfarin therapy and had been established on therapy for 8 months. She reported no neurological sequale or headaches.

She was clear she wished to become pregnant and that she wanted to stop her warfarin therapy. She was now 8 months from initial diagnosis and not on COCP so this was agreed. After discontinuing warfarin therapy her APTT was normal and her DRVVT was within normal range. The positive DRVVT at diagnosis of CVST was transient and not present on repeat sampling therefore she did not fulfil the criteria for Antiphospholipid syndrome (which would require two or more positive lupus anticoagulant tests more than 12 weeks apart in presence of VTE).

She was counselled about dietary advice as it was explained that high BMI was a modifiable risk factor for VTE. She was informed of the need to avoid hormone based contraceptives in future given the CVST. She was encouraged to address her lifestyle first before trying to conceive as this would improve her chances of a successful pregnancy outcome. It was explained to her that given her history of VTE while on hormonal contraception and her elevated BMI she would require thromboprophylaxis throughout her pregnancy and 6 weeks postpartum in line with RCOG Green top guidelines 37a.

Thanks for your help with the case and please feel free to post any comments.

References:

UpToDate cerebral venous sinus thrombosis section.

Coutinho  J, de Bruijn  SFTM, deVeber  G, Stam  J. Anticoagulation for cerebral venous sinus thrombosis. Cochrane Database of Systematic Reviews 2011, Issue 8. Art. No.: CD002005.

Ferro et al. European Stroke Organization guideline for the diagnosis and treatment of cerebral venous thrombosis – endorsed by the European Academy of Neurology. European Journal of neurology 2017; 24 (10) 1203-1213.

https://onlinelibrary.wiley.com/doi/full/10.1111/ene.13381

Miyakis S et al. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). Journal of thrombosis and haemostasis.2006;4 (2) pp295-206.

https://onlinelibrary.wiley.com/doi/full/10.1111/j.1538-7836.2006.01753.x

RCOG Green Top Guideline 37a. reducing the risk of VTE during pregnancy and puerperum. April 2015.

https://www.rcog.org.uk/globalassets/documents/guidelines/gtg-37a.pdf

Tait C et al. Guidelines on the investigation and management of venous thrombosis at unusual sites.BJHaem 2012;159 (1) 28-38.

https://onlinelibrary.wiley.com/doi/full/10.1111/j.1365-2141.2012.09249.x

Ferro et al. Safety and Efficacy of Dabigatran Etexilate vs Dose-Adjusted Warfarin in Patients With Cerebral Venous Thrombosis A Randomized Clinical Trial. JAMA Neurol 2019;76(12) 1457-1465.

https://jamanetwork.com/journals/neur/articlepdf/2749167/jamaneurology_ferro_2019_oi_190070.pdf

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Case 129 – update 3

Thanks for everyone’s thoughts in line with the consensus on the feed our patient was established on split dose LMWH and her Anti Xa levels are therapeutic. She is nearing the point of discharge home and the medical team contact you regarding the plan for longer term anticoagulation they tell you they feel this was a provoked event caused by BMI and Oral contraceptive pill usage.

What would you do regarding her single positive DRVVT result? 

What are her options for anticoagulation?

How long would you continue anticoagulation for?

Any other advice for the patient if she was planning on pregnancy after completing anticoagulation? 

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Case 129- update 2

Having established the patient has never had Lupus anticoagulant screening before and has no history of VTE you advise doesn’t fulfil criteria for antiphospholipid syndrome. You recommend repeat LA ratio and antibodies in 12 weeks. You explain a LP should be safe given the results and to proceed with Lumbar puncture. You quietly congratulate yourself on swiftly sorting out the coagulation issues and go back to bed for some well earned sleep.

Several days later you receive an on call bleep regarding the same lady. It transpires that the LP was normal and the following day the patient developed new numbness and tingling in her left arm. An MRI Brain was organised and this confirmed the diagnosis of a cerebral saggital sinus thrombosis with associated small intracerebral haemorrhage.

The medical team are concerned about anticoagulation given the small intracerebral haemorrhage. The patient weighs 130kg.

What would you advise regarding anticoagulation for this lady’s cerebral sinus thrombosis?

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Case 129 – update 1

Thanks for everyone’s thoughts So far we have now established the following:

No Anticoagulants
No ETOH excess
Normal liver function
No previous coagulation results available for this lady
No personal history of bleeding
No FHx of bleeding disorder

The lab performed the following extra tests at your request:

Repeat Coag results:

PT 12.4, APTT 49.6, APTT 50:50 Mix 46, Fib 2.9, DRVVT Ratio 1.5 (0.8-1.2)

Anticardiolipin and Anti beta 2 glycoprotein 1 Ab are pending
What is the likely cause for prolonged APTT?
Is it safe to proceed with planned LP on the basis of the above tests?
Anything further to recommend to the clinical team?

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Case 129 – The beginning

You are the haematology registrar on call and you get contacted regarding a 28 year old lady who presented to the A&E department on Saturday evening. She presented with a sudden onset severe headache that had been present for 12 hours with associated vomiting. Her CT Head has been provisionally reported over the phone to A&E as “normal” and  the medical registrar wants to perform an urgent lumbar punctureto investigate for possible subarachnoid haemorrhage.

Blood tests so far (Ref range in brackets):

Wcc 11.6 (3.6 – 11), Hb 140 (115-165), Plt 400 (140-400), Neu 10.2 (1.7-7.5)

PT 12.6 (10-14)

APTT 49 (22-36)

Fib 3 (1.5-4)

Is it safe to proceed with the planned lumbar puncture?

What other history would you ask the registrar on the phone?

Any further tests you would request from the lab?

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Case 128 – Summary!

Thank you for everyone’s contributions with case 128!

We discussed a complex case of a 65 year who was critically unwell with severe pancreatitis. During her admission she developed anaemia and marked thrombocytopenia. Our discussions centred upon the potential differential diagnoses, focusing in more detail on pancreatitis associated thrombotic thrombocytopenia purpura (TTP) and post transfusion purpura (PTP). At a later stage she also had a further complication of bilateral pulmonary emboli and portal vein thrombosis directing our thoughts to the management of these in the setting of her thrombocytopenia. This case highlighted the challenges (and frustrations!) that we are often faced with in our clinical roles where the diagnosis is not clear cut but immediate management is essential prior to confirmation of the diagnosis – if this can be achieved! Please see below for information on the main topics we discussed throughout this case.

Potential differential diagnosis of bi-cytopenia (Hb + Plts) with acute onset includes:

  1. Severe acute illness
    1. Rx: underlying cause. Transfusion support as required
  2. DIC (with either bleeding / acute illness to account for anaemia)
    1. Rx: underlying cause. Transfusion support for coagulopathy /thrombocytopenia if bleeding / surgery
  3. Drug-induced immune mediated thrombocytopenia
    1. Rx: Stop causative agent. Consider IVIg +/- steroids. Transfusion support as required.
  4. Drug-related marrow suppression
    1. Rx: Stop causative agent. Transfusion support as required.
  5. Microangiopathic haemolytic anaemia (including TTP)
    1. Rx see below for pancreatitis-associated TTP
  6. Post-transfusion purpura
    1. Rx see below for PTP
  7. Acute haematological malignancies e.g acute leukaemia
  8. Marrow infiltration

 

Pancreatitis-associated TTP

  • Rare complication of pancreatitis. Typically occurs during acute episode but can occur shortly after resolution
  • ADAMTS13 is only moderately reduced
  • Good outcomes following plasma exchange and steroids

Post-transfusion purpura (PTP)

  • Extremely rare complication of transfusions with blood components containing platelets and should be considered in those who have developed thrombocytopenia within 14 days of a transfusion.
  • Since leucodepletion has been introduced the incidence of PTP is <1 in 700,000.
  • PTP occurs when a blood transfusion stimulates an anamnestic response of HPA antibodies in a previously sensitised patient. The most common initial sensitising event is pregnancy but immunisation by HPA alloantigen can also occur from previous transfusions.
  • The marked thrombocytopenia associated with PTP is secondary to the antibody mediated destruction of both the donor platelets AND patients own platelets.
  • The presence of IgG allo-HPA antibodies confirms the diagnosis of PTP. Anti-HPA-1a is most frequent antibody implicated in PTP. If PTP is strongly suspected management should be initiated prior to serological confirmation.
  • Management
    • IVIg 2g/kg over 2-5 consecutive days
    • Consider plasma exchange if IVIG alone not effective
    • During acute episode of PTP no benefit of transfusing blood components from HPA compatible donors. Unfortunately random platelet transfusions are also not effective and multiple transfusions are often required in setting in haemorrhage.
    • Following acute episode transfusion of blood components should be from HPA compatible donors.
  • Report to SHOT / MHRA

 

Potential differential diagnosis of acute onset thrombocytopenia and macrovascular venous thromboembolism includes:

  1. Critical illness
  2. Malignancy
  3. PNH
  4. Antiphospholipid syndrome
  5. HITT

 

Management of venous thromboembolism in context of marked thrombocytopenia

  • Individualised approach as always! Assessment of risk of VTE vs bleeding
  • Management of thrombosis
    • Optimise risk reduction of VTE by non-pharmacological means i.e hydration, mobilisation
  • Anticoagulation options
    • consider bleeding risk, renal / liver function
    • In our case with marked thrombocytopenia and low eGFR unfractionated heparin (UFH) likely to be the safest option due to short half life and reversal agent available. However UFH needs regular monitoring of anti-Xa levels (or APTTr levels if baseline APTT within normal range) and if this can not be achieved than its use is often unsafe and a twice daily low molecular weight heparin may be better option
  • Management of bleeding risk
    • Need close monitoring of platelets and manage cause of thrombocytopenia
    • Consider platelet transfusions to maintain platelet count >50 (depending on cause of thrombocytopenia – avoid unless bleeding in HITT / TTP / PTP / ITP etc)

References:

1. Scully et al. Guidelines on the diagnosis and management of TTP and other thrombotic microangiopathies. British Journal of Haematology. 2012.

1. Massey E. NHS Blood and Transplant clinical guidelines. Post transfusion purpura.  2011. https://nhsbtdbe.blob.core.windows.net/umbraco-assets-corp/14873/inf153-post-transfusion-purpura.pdf

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Case 128 – Update 4!

Thank you for everyone’s ongoing contributions!

The patient underwent further intra-abdominal surgery without significant bleeding complications following the management below:

  1. Vitamin K 10mg IV
  2. Co-trimoxazole stopped
  3. IVIg 2g/kg over 2 days
  4. Platelet transfusion (random ABO and D compatible with good 15 minute increment – plt count went from 25 to 51)
  5. 2 units packed red cells

Unfortunately 3 days later our 65 year old patient has deteriorated and is now on critical care having been intubated and ventilated. A CTPA and CT abdo-pelvis was performed and this has shown:

  • Bilateral segmental PEs
  • Fully occluded portal vein thrombosis
  • Mild improvement regarding appearance of pancreatitis

Repeat bloods are as follows:

  • Hb 78 Plts 39 WCC 5
  • PT 18 APTT 39 Clauss fib 3
  • eGFR 31 Bili 125 ALT 320 Alk phos 110 CRP 265

Questions:

  1. How would you manage our patient now (from haem perspective)?

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.

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Case 128 – Update 3!

I think we’re all in agreement that we currently can’t give a definitive diagnosis as is frequently the case in complex critically unwell individuals. From your responses the most likely differentials for our patient’s bi-cytopenias include the following (or combination of the following):

  1. Severe sepsis / critical illness (marrow suppression, consumptive coagulopathy, liver dysfunction etc)
  2. Drug induced immune mediated thrombocytopenia
  3. Post transfusion purpura

Repeat bloods:

Hb 74 Plts 25 WCC 6 PT 20 APTT 41 Clauss fib 3

Questions:

  1. How would you manage our patient generally and also if planned for further surgery (laparotomy)

 

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 128 – Update 2!

Thank you for everyone’s ongoing contributions!

We now have the outstanding results to help diagnose our patients bi-cytopenias (and old results for reference) and some additional history as requested:

Her bloods from this morning are as follows:

  • Hb 71 (several transfusions required throughout this admission)
  • Plts 20 (plt count was within normal range upto 3 days ago)
  • PT 19, APTT 40, Clauss fib 5.2
  • DAT negative. LDH 326
  • Stage 2 AKI, bilirubin 84 (ULN 21), ALT 250 (0-40), Alk phos normal

 

Outstanding results now in:

  • Blood film: Normocytic normochromic anaemia with unremarkable red cell morphology including NO red cell fragments or features of haemolysis. Thrombocytopenia appears genuine with no plt clumps. Plts morphologically normal. left shifted neutrophils with toxic granulation.
  • Retics: upper level of normal.
  • HIT assay: negative (and intermediate 4Ts score)
  • B12 / folate: normal
  • Autoimmune screen: negative
  • HIV / hep B / C: negative
  • Serum electropheresis: no paraprotein.
  • The patient has received 10 units packed red cells over the past 2 weeks for support several surgeries but has also been given appropriate associated FFP / platelet support.
  • (NB: an ADAMST13 has not been requested given the above findings and plasma exchange had not been initiated.)

Questions:

  1. What are the most likely differentials now?
  2. Any others related to the transfusion history and how could you investigate?
  3. Can you give a definitive diagnosis for cause of anaemia / thrombocytopenia?

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 128 – Update 1!

Thank you for everyone’s contributions so far!

Below is the additional information you requested to help determine the cause of our 65 year old patients anaemia and marked thrombocytopenia.

  • Her severe pancreatitis is secondary to gallstones.
  • She has a previous history of cholecystitis (due to gallstones) and well controlled hypertension.
  • She has no significant alcohol history.
  • She has been on amlodipine for years and takes no other medications or OTC treatments. She has however been on multiple new medications this admission including starting prophylactic low molecular weight heparin throughout admission and co-trimoxazole started approximately 5 days ago.
  • A CT abdo-pelvis this admission has demonstrated pancreatitis with marked necrosis, cholecystitis. The liver and spleen appear normal. Low volume lymphadenopathy (~2cm) in the porta hepatis but nil elsewhere.

 

Her bloods from this morning are as follows:

  • Hb 71 (several transfusions required throughout this admission)
  • Plts 20 (plt count was within normal range upto 3 days ago)
  • PT 19, APTT 40, Clauss fib 5.2
  • DAT negative. LDH 326
  • Stage 2 AKI, bilirubin 84 (ULN 21), ALT 250 (0-40), Alk phos normal
  • Blood film, retics, serum electropheresis, autoimmune screen, viral screen all pending

With this new information what are your differential diagnoses?

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.

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Case 128 – The beginning!

You are the haematology registrar oncall and are contacted by the surgical team regarding a 65 year old woman with severe pancreatitis. The patient is critically ill. They are after some advice regarding the following FBC results:

Hb 72, Plts 31, WCC 5.5, Neuts 4.4

Questions:

  1. What other information would you want to ascertain from the clinical team?
  2. What initial investigations would you want to review/perform to look into the cause of her cytopenias?

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 127 – the summary

Thanks for all your input this week, as we worked through the investigation and management of CML; highlighting a few areas for discussion as we went!

See below summary notes on CML – mainly taken from the European Leukaemia Network (ELN) and ESMO guidelines. Any mistakes are my own!

Screen Shot 2020-04-30 at 19.23.13Screen Shot 2020-04-30 at 19.23.30Screen Shot 2020-04-30 at 19.23.39

Key references:

Hochhaus, A., Baccarani, M., Silver, R.T. et al. European LeukemiaNet 2020 recommendations for treating chronic myeloid leukemia. Leukemia 34, 966–984 (2020). https://doi.org/10.1038/s41375-020-0776-2

Hochhaus, A. et al. Chronic myeloid leukaemia: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-upAnnals of Oncology, Volume 28, iv41 – iv51

 

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 127 – Update 2

Our 31 year old female patient with newly diagnosed CML in chronic phase is commenced on dasatinib 100 mg OD with the aim of gaining a faster deep molecular response (DMR).

3 years later (patient is now 34)…..

  • She has tolerated the drug well – no evidence of pleural effusions
  • She has had a complete haematological response (CHR)
  • Her BCR-ABL1 to ABL1 ratio has been hovering around 0.05% for the last 2 years. She is now asking to stop the drug to start trying for a family

What is the evidence around stopping TKI therapy? How would you counsel the patient in the best way forward?

 

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 127 – update 1

Our 31 year old patient has repeat bloods including a few extra. FBC confirms leucocytosis with WCC 38, and basophilia 3.8. Platelets are normal. U&Es and LFTs are normal. No organomegaly on clinical exam.

Cytogenetics confirms the Ph chromosome t(9;22) and RT-PCR demonstrates BCR-ABL1 to ABL1 transcript ratio as 56%

This confirms a diagnosis of CML in chronic phase

On further questioning, this 31 year old female patient has no relevant PMH. She has a FH of familial hypercholesterolaemia but maintains regular exercise, good diet and doesn’t smoke. She works as a primary school teacher, has been married for 2 years and has no children, but is planning a family in the near future.

How would you explain the disease/prognosis to this young patient? What would be the options for 1st line 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 127 – The beginning

You are the haematology SpR reviewing routine blood films in the lab at 4pm when you come across the following FBC differential and film from a GP request. The patient is a 31 year old female. Clinical details state ‘for bloods.’

FBC:

  • Hb 115 g/l
  • MCV 90 fl
  • Plt 152 x 109/L
  • WCC 19.3 x 109/L
  • Neuts 10.4 x 109/L
  • Lymph 1.4 x 109/L
  • Monocytes 4.2 x 109/L
  • Eosinophils 1.1 x 109/L
  • Basophils 2.2x 109/L

Blood film as shown below:

CML

What does the FBC and blood film demonstrate? What are the next actions you would take?

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Case 126 – Update 3!

Thank you for everyone’s contributions so far!

As advised by yourselves we have commenced our 57 year old gentleman who has newly diagnosed T-PLL on Alemtuzumab with the aim of consolidating with an allogenic stem cell transplant. He had been tolerating it well having only  experienced mild infusion reactions with cycle 1 and 2. However he has now presented with marked diarrhoea post his 4th cycle of treatment.

Questions:

  1. What is the differential diagnosis for this?
  2. What investigations would you perform?

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.

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Case 126 – Update 2!

Thank you for everyone’s thoughts on the blood film. Think we’re all in agreement that blood film shows small to medium sized lymphocytes with high nuclear/cytoplasmic ratio and folded nucleoli. There are also occasional forms with nucleolus and cytoplasmic protrusions.

The flow plots are now available. Can you help interpret to ascertain the immunophenotype and ??diagnosis.

TPLL Flow1

TPLL Flow2TPLL Flow3

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Case 126 – Update 1!

 

We advised the GP to repeat the asymptomatic 57 year old patient FBC (with film) in 4-6 weeks and to refer to haematology if absolute lymphocyte count was >10 or if he had associated cytopenias / B symptoms / lymphadenopathy / hepatosplenomegaly.

Unfortunately after 4 weeks the GP contacts you again to say the patient re-presented with drenching night sweats. On examination he has an macular rash over his thorax and the spleen is now palpable 3cm below the costal margin.

Repeat FBC: Hb 125 Plts 110 WCC 62 Neuts 4 Lymphocytes 56

Blood film:

TPLL1

TPLL2

Questions:

  1. What does the blood film show? Diagnosis??
  2. What further investigations would you request?

 

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

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Case 126 – The beginning!

You are the on-call haematology registrar who has been contacted by a conscientious GP regarding a fit and well 57 year old man who has an incidental finding of lymphocytosis. The FBC was performed as part of his monitoring for hypothyroidism.

Hb 131 MCV 90 Plts 180 WCC 13 Neutrophils 4.5 Lymphocytes 7

Questions:

  1. What further information would you want to clarify with the GP?
  2. How would you advise the GP regarding the lymphocytosis?

 

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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