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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Case 111 – Introduction

This week we will run a few short basic cases which you might come across in general medicine.

Case 1)  A 72 yr old man is admitted to A&E with a collapse. He has a macrocytic anaemia with Hb 36g/L. What more information would you like to help manage this gentleman?

 

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 110 – summary

Thankyou for all your help this week

This week we had a look at 4 films where there were visible organisms.

Film 1 – babesia

Film 2 – malaria – p.vivax

Film 3 – malaria – p.falciprum

Film 4 – diplococci – neisseria meningitidis

When looking at films where there is a suspicion on infection clinical details are very important. This will enable the appropriate tests and blood film preparations to be made to aid diagnosis. Travel history is very important to aid diagnosis.

Film 1 – babesia.

Babeosis is a tick borne protozoan infection that is see in Europe, Asia and north west and north eastern america. Humans are usually affected by babesia microti. Many patients will have no, or only mild symptoms. Symptoms can include fever, headache, myalgia and nausea/vomiting. In patients who are asplenic or immunocompromised severe disease may occur.

Symptoms are usually related to the parasitaemia of red cells by babesia. Infected red cells can contain more than one merozoite. The ring forms may be mistaken morphologically for p.falciprum malaria so travel history is very important when looking at individual cases.

Haemolytic anaemia and haemoglobinuria can be present in severe infections. Some patients may also develop ARDS and/or multi organ failure.

Although the majority of cases are transmitted via bites from infected ticks. Transmission has also been seen from blood transfusions from infected donors. Perinatal and transplacental infection can also occur.

Symptoms can persist for several months after initial infection, and can also recur, particularly in asplenic patients and those on immunosuppressive treatment.

In patients with lower parasitaemia multiple thin films or stained buffy coat preperations may be needed to identify the parasite. Wright or giemsa stained thin films can show the ring forms. In some cases the ‘maltese cross’ made up of merozoites. P.falciprum can be mistaken for babesia, Maltese crosses are not seen in p.falciprum, there is also a lack of hemozoin in babesia.

Serological testing and PCR to investigate for babesia can be performed.

Patients with babeosis will need treatment with antibiotics and may need HDU/ITU care in severe cases. All suspected cases should be discussed with local infectious disease/microbiology team. In severe cases exchange transfusion may be considered.

Case 2 – p.vivax malaria

Case 3 – p.falciprum malaria

Travel history is again very important when malaria is suspected. Not only when trying to establish the likely parasite, but also establish the likelihood of drug resistance.

P.vivax is the most common human malaria transmitted globally, although p.falciprum has a higher mortality.

Patients may present with fever, myalgia, headache, nausea/vomiting, and can present with symptoms of haemolysis and organ failure in more severe cases. Occasionally patients can present with features of cerebral malaria including seizures and altered consciousness.

When examining blood films identification of parasites can be made on thin films, however it is important to look at think films as low levels of parasites may be missed on thin films. Thick films should be stained with giemsa or field stain, and thin films with giemsa or leishman stain. A thick film should be examined for around 10 minutes, which equates to around 200 oil immersion fields before it is declared negative. Each thick film should be reviewed by 2 people.in p.falciprum and p.knowelsi the percentage of cells with parasites should be calculated and reported as this may effect the choice of treatment. If there is doubt as to whether parasites can be seen on the thick film, the entire thin film should be reviewed.

Rapid diagnostic tests(RDT) can be used when suspecting malaria, but are not a substitute for microscopy.

Quantification of parasites should be done daily until no parasites are seen

Patients who have traveled to the Asia-Pacific area who are thought to have p.malariae should have samples sent urgently to the malaria reference lab for pcr as p.malariae and p.knowlesi are very difficult to distinguish from one another.

Samples should be sent to a malaria reference lab if there are positive results, if there is discrepancy between films and

RDT or if there is strong clinical suspicion after discussion with ID team but no positive results. If films are negative then team should consider repeating the film in 12-24 hours, and then again at 24 hours.

PCR is more sensitive than microscopy for malarial parasites however is not widely available, but is used in the reference lab, and can be particularly useful in patients with mixed infection.

All labs involved in testing for malarial parasites should be involved in EQA schemes for parasites.

When diagnosing p.vivax, it is important to consider testing for g6pd deficiency as treatment may involve primaquine which can precipitate haemolysis in patients with g6pd deficiency.

As p.vivax can lie dormant in liver cysts recurrent infections can occur.

The treatment of p.falciprum is guided by the severity of the disease. Severe infection can be rapidly fatal so prompt treatment can be life saving.

In severe cases of malaria red cell exchange can be considered.

Links at the bottom show some aids to diagnosing malaria from the CDC. There is also a link to the bsh guidelines for malaria diagnosis.

Film 4 – diplococci – neisseria meningitidis

In this case a patient was admitted collapsed and unwell with no collateral history and they were subsequently diagnosed with neisseria meningitidis bacteraemia and suspected meningitis.

The blood film demonstrates diplococci. It is unusual to see bacteria on blood films and when this is seen it should rapidly be reported to the caring physician to enable appropriate treatment as these patients are usually extremely unwell. If the patient has no signs or symptoms of infection then contamination can be considered.

Thankyou for all your help this week. This week we looked at 4 cases where microscopy was important in the diagnosis of infections.

Are there any questions following this week’s 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

Bsh guideline on laboratory diagnosis of malaria

https://onlinelibrary.wiley.com/doi/full/10.1111/bjh.12572

CDC information on malaria

https://www.cdc.gov/dpdx/malaria/index.html

CDC bench aid to p.vivax diagnosis

https://www.cdc.gov/dpdx/resources/pdf/benchAids/malaria/Pvivax_benchaidV2.pdf

CDC bench aid to p.falciprum diagnosis

https://www.cdc.gov/dpdx/resources/pdf/benchAids/malaria/Pfalciparum_benchaidV2.pdf

CDC bench aid to p.ovale diagnosis

https://www.cdc.gov/dpdx/resources/pdf/benchAids/malaria/Povale_benchaidV2.pdf

CDC bench aid to p.malariae diagnosis

https://www.cdc.gov/dpdx/resources/pdf/benchAids/malaria/Pmalariae_benchaidV2.pdf

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Case 110 – part 4

Welcome to the last part of this case.

You get a phone call from the lab for a patient who has attended a&e. The patient has no fixed abode and has been found collapsed in the steet. They are unable to give a history, but have attended the department after being found collapsed following drug overdoses previously.

Hb 110 plt 20 wcc 17 neuts 12

What does the film show. How would you proceed?

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 110 – part 3

Here is the 3rd part of this week’s case.

A patient has come in to a&e very unwell, with fever and has had a seizure whilst in the department. The patient came back yesterday from a trip to Malawi.

Hb 80 plt 30 wcc 12 neut 12

We have been asked to look at the film.

What does the film show? How would you proceed?

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 110 – part 2

Welcome to the second part of this week’s morphology based case.

A&E have asked for a film for a patient who has come in with fever.

Hb 90 plt 50 wcc 15 neuts 8

What does the film show? What other investigations should be done? What would you do next?

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 110 – part 1

Welcome to this week’s case!

This week we will have some short morphology cases.

Our first case starts with a call from the lab.

A patient has attended a&e unwell and the lab have made a film. There is something unusual on it and we have come in to have a look.

Hb 90 plt 50 wcc 14 neuts 8

How would you report this? What could the diagnosis be? What would your next steps be?

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 109 – summary

Thanks for all your help this week. This week’s case focused on Thrombocytopenia pregnancy that ultimately was ITP responsive to steroid therapy.

Mild gestational thrombocytopenia (with platelet counts <150×10^9/L) is common affecting up 5-10% of pregnant women.

Pregnant women with mild thrombocytopenia of platelets 100-150×10^9/L and normal blood film/screening blood tests should stay in primary care with monthly FBC and advice to refer to secondary care if platelet counts fall below 100×10^9/L.

Only 1% of pregnant women will have a platelet count less than 100×10^9/L which is the international working groups definition of thrombocytopenia and this is the range where further specialist investigation and referral to secondary care is appropriate.

A suggested approach to thrombocytopenia in preganancy is outlined below:

History:

• Previous FBC results: Thrombocytopenia prior to pregnancy points to causes other than gestational thrombocytopenia.

• Family history: Type IIb VWD although rare can present as thrombocytopenia in pregnancy.

• Medication History: Any changes in medications. Many drugs in common use can cause isolated thrombocytopenia – worth checking a patients medications.

• Features of Connective tissue diseases (CTD): Fatigue, Rashes, Joint stiffness, sore eyes etc.

• Gestation at onset: Later in pregnancy more likely to be due to pregnancy specific causes like Pre-eclampsia, HELLP, Acute Fatty liver of pregnancy etc. Gestational thrombocytopenia is more common in second and third trimesters.

• Personal Bleeding history: BAT score may help indicate if underlying bleeding disorder, particularly useful if no previous blood tests available and family history of bleeding.

 

Initial investigations:

• FBC repeat plus CD61 platelet count or citrate count: Confirm genuine thrombocytopenia.

• Blood film: Film crucial to exclude underlying marrow disorder and to look for fragmentation, signs of haemolysis, hereditary thrombocytopenia with giant platelets or white cell inclusions or for signs of infection.

• U&E, LFT’s: May be abnormal if HELLP/Pre-eclampsia/HUS.

• Coagulation screen inc D-Diner: Abnormal if DIC/HELLP.

• VWD screen: Useful particularly if family history to exclude type IIb VWD.

• Antiphospholipid antibodies

• ANA

• HIV, Hep B, Hep C

• B12/Folate: Rarely a cause of isolated thrombocytopenia

• Physical examination: Look for mucosal bleeding petichiae and any signs of Connctive tissue diseases.

Optional testing depending on findings:

  • TFT’s: Thyroid problems may be associated with ITP in pregnancy as part of autoimmune spectrum.
  • Ig levels: If history of frequent infections.
  • Other viral serology: if indicated from history or film.
  • H.Pylori serology.
  • DAT, LDH and Retics: If concern Evans syndrome due to spherocytes on film.
  • ADAMTS 13 testing if fragmentation on film.
  • Bone Marrow: If suspicion of primary blood disorder from film.

 

Establishing the Diagnosis:

Broadly speaking thrombocytopenia in pregnancy is best thought of in five distinct categories depending on the investigations above.

 

1) Normal Blood film and other investigations (most likely scenario):

• Gestational thrombocytopenia (GT) – accounts for 75% total

• ITP – 5%

 

Useful features to distinguish the two diagnoses:

Rare for GT to cause platelet count <50×10^9/L.

GT usually occurs mid-late second trimester and during the third trimester where as ITP can occur earlier (as in this case).

There may also be a history of previous thrombocytopenia in pregnancy that fully resolves post-partum in GT. hence very important to monitor platelet counts post partum.

 

2) Atypical lymphocytes on film, raised inflammatory markers:

• Infection

 

3) Microspherocytes, agglutination or clumping on film and positive DAT:

• Evans syndrome

 

4) Blasts on film

• Pimary bone marrow disorder

 

5) Schistocytes on film – 15% of patients

• TTP

• HUS

• DIC

• Pre-eclapsia/HELLP/Acute fatty liver of pregnancy

 

The rest of this case focuses on our lady who had a normal blood film and isolated thrombocytopenia. It is important that the possible cause of thrombocytopenia is established before embarking upon management as the approaches for management of HELLP or TTP vary greatly to the management of ITP for example.

 

Monitoring patients with Isolated thrombocytopenia thought to be ITP:

All patients should be referred for secondary care review if platelets <100×10^9/L and have an antenatal anaesthetics review.

There is little evidence to suggest what appropriate monitoring should be and it should be tailored to platelet count and the rates of decline seen rather than a rigid time based check for example every 4 weeks. The consensus seems to be monitoring should be somewhere between 2-6 weekly Depending on the individual. In the third trimester particularly after 34 weeks weekly checks if plt <80×10^9/L are suggested especially if treatment is given.

Patients with thrombocytopenia in pregnancy should be advised of who to contact in case of bleeding or concerns. They should also be asked to avoid IM injections, though BCSH guideline makes it clear low dose aspirin prescribed for obstetric reasons should not be automatically withheld unless high bleeding risk.

All patients should have repeat FBC 1-3 months post-delivery to check for spontaneous resolution of thrombocytopenia. This is important particularly if GT was thought to be the cause of thrombocytopenia as by this timepoint the thrombocytopenia should have resolved.

Treating patients with Isolated thrombocytopenia thought to be ITP:

BCSH guidelines suggest treatment for ITP should be in order to achieve “safe” platelet counts and not necessarily normalise platelet counts.  BCSH guidelines state that although no robust trials it is reasonable to leave asymptomatic pregnant women with platelet counts of 20-30×10^9/L without treatment until the third trimester.

Treatment approaches for ITP during pregnancy vary slightly but generally treatment is with prednisolone first line starting at 10-20mg dose of prednisolone with IVIG reserved in case of bleeding or as second line therapy.

Treatment is usually prednisolone for a week, adjusting to minimum dose of prednisolone that maintains a response. Response time is usually around 3-7 days. It is recommended that tapering of doses is suspended near term as often platelet count falls at term. Slow taper also recommended post-partum as quick tapering can affect the mother’s psychological state.

The treatment aims to achieve  a minimum platelet count of 50×10^9/L for vaginal delivery. Platelet counts of >80×10^9/L will usually be needed for neuroaxial anaesthesia.

Although steroids are relatively safe in pregnancy common side effects are impaired glucose tolerance, hypertension, mood change and weight gain.

Second line therapies and agents for ITP are less clear and would require discussion in an MDT setting depending on the individual patient.

Delivery planning for patients with ITP:

Mode of delivery should be based on obstetric indications there is no need for Cesarean section in ITP patients as no evidence to show this reduced ICH rates in thrombocytopenic neonates.

Ideally delivery should be in a hospital with Haematology and obstetric care.

General consensus both in How I treat and RCOG review of thrombocytopenia in pregnancy is that a platelet count >50×10^9/L is required for vaginal and operative delivery. For epidural anaesthesia platelet count needs to be >80×10^9/Land an anaesthetic review before delivery is recommended to discuss alternative analgesia if thrombocytopenia at delivery.

Platelet transfusions should be available in case of complications but should not be routinely administered.

Neonates born to mothers who have ITP do have a risk of thrombocytopenia due to passage of Ig G Ab across the placenta. 15-30% of babies born to mothers with ITP are thrombocytopenic though only 5% have counts <20×10^9/L. It is worth stating that antenatal steroids and IVIG have no effect on the fetal platelet counts. The passage of antibodies is unpredictable therefore mothers platelet counts won’t always indicate the likelihood of associated thrombocytopenia in the baby. A history of sibling with thrombocytopenia at birth, mother with previous splenectomy or platelet count <50×10^9/L at delivery are predictors of an increased risk of neonatal thrombocytopenia. This means all babies born to mothers with presumed ITP should be treated as possibly being thrombocytopenic. Fetal scalp electrodes and instrumental deliveries are ideally avoided and cord blood should be taken at delivery to confirm the platelet count. IM vitamin K should also be avoided. Platelet counts should also be checked at day 1 and 4 post delivery, as platelet nadir around this time. If platelets less than 50×10^9/L or symptomatic transcranial USS should be performed.

Postnatal care should include daily FBC of mother until day 5 post partum.

Prenatal counselling of mothers with ITP:

Pre-pregnancy counselling for a lady with ITP as recommended by RCOG should include:

• Discussion that ITP relapses may occur during pregnancy.

• Around a third of women will need treatment in pregnancy usually in third trimester.

• An epidural may not be possible if thrombocytopenic.

• There is an increased chance of a sibling being affected with thrombocytopenia if a mother has had splenectomy or previous baby with neonatal thrombocytopenia.

• Risk of adverse delivery outcomes for mother or major bleeding in neonates is thankfully low with correct planning and monitoring.

 

References:

Provan et al. International consensus report on the investigation and management of primary immune thrombocytopenia. Blood 2010 115:168-186.

B Myers. Diagnosis and management of maternal thrombocytopenia in preganancy. BJHaem, 2012, 158, 3-15.

B.Myers. Review Thrombocytopneia in pregnancy. RCOG 2009.

T Gernsheimer et al. How I treat thrombocytopenia in pregnancy. Blood 2013. 121.

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Case 109 – update 4

Thanks for your help so far. The lady thankfully had an uneventful normal vaginal delivery. She had chance to have an anaesthetics review prior to labour and had been attending joint obstetric and haematology clinic. Her pre prepared birth plan included the following points:

Mode of delivery based solely on obstetric indications.

Delivery should ideally be in a hospital with Haematology and obstetric care.

Target platelet count >50×10^9/L for vaginal and operative delivery.

For epidural anaesthesia platelet count needs to be >80×10^9/L.

Platelet transfusions should be available in case of complications.

Haematology team to be contacted if any concerns regarding platelet count or bleeding.

Neonates born to mothers who have ITP do have a risk of thrombocytopenia due to passage of Ig G Ab across the placenta. 

15-30% of babies born to mothers with ITP are thrombocytopenic. All babies born to mothers with presumed ITP should be treated as possibly being thrombocytopenic. When writing birth plans and the following points need to be communicated to obstetric and paediatric team as happened in this case.

Foetal scalp electrodes and instrumental deliveries are ideally avoided.

Cord blood should be taken at delivery to confirm the platelet count.

IM vitamin K should also be avoided.

If platelets less than 50×10^9/L on cord blood or symptomatic transcranial USS should be performed.

Our lady had a healthy baby boy with a normal platelet count of 370×10^9/l on cord blood sampling.

Does the baby require any further platelet count monitoring? If so when would you check platelet count again?

The lady is keen to know of the implications of her ITP on future pregnancies, how likely is she to need treatment for ITP in further pregnancies?

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Case109 – update 3

The lady’s platelet count responded to 20mg Prednisolone and is now at 65×10^9 at 38 weeks gestation. 

The obstetric team are keen for a plan for delivery what advice would you give the team? 

What advice would you give to the neonatal team?

How common is transient neonatal thrombocytopenia in mothers who have ITP?

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Case 109 – update 2

You suspected ITP or possibly gestational thrombocytopenia and embarked on monitoring her platelet counts every few weeks. The lady is now 34 weeks pregnant and is otherwise well. Her platelet count has started to fall over the past 6 weeks and is now 30×10^9/L. She is well with no bleeding.

What is the most likely diagnosis now?

How would you manage this patient at this stage?

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