TeamHaem Saves Christmas Part 2

Jingle bells, jingle bells

Jingle all the way

Oh what fun it is to tweet TeamHaem’s case today!

 

Father Christmas is so worried

Jingle sorts out both his lists

He checks on who’s behaving

And makes sure no-one’s missed.

 

Jingle bells, jingle bells

Jingle all the way

Oh what fun it is to tweet TeamHaem’s case today!

 

Blood cultures have grown nothing

The blood film’s ready for review

There is no clear infectious cause

Please we all need help from you!

 

Jingle bells, jingle bells

Jingle all the way

Oh what fun it is to tweet TeamHaem’s case today!

 

Please review the blood film and suggest next steps

What tests should be performed?

What treatment would you recommend?

 

Falciparumimage003

Posted in Uncategorised

TeamHaem help us save Christmas 2019! 🎄❄️ Part 1

Jingle bells, jingle bells

Jingle all the way

Oh what fun it is to tweet TeamHaem’s case today!

 

A day or two ago 

Father Christmas needed help

Jingle, his top packing elf,

Just wasn’t feeling himself

 

Jingle bells, jingle bells

Jingle all the way

Oh what fun it is to tweet TeamHaem’s case today!

 

His temperature was high

He was shaking head to toe

Mumbling, stumbling and confused

To the hospital he did go, Oh!

 

Jingle bells, jingle bells

Jingle all the way

Oh what fun it is to tweet TeamHaem’s case today!

 

‘Oh my muscles are so achy’

‘My head is pounding bad!”

They took some blood tests from his arm

And his platelets were down a tad.

 

Jingle bells, jingle bells

Jingle all the way

Oh what fun it is to tweet TeamHaem’s case today!

 

What are our first steps?

 

Elf blood count: Hb 110, WBC 13, Neut 11, PLT 99

(Please note, elf physiology is extremely similar to humans)

 

Posted in Uncategorised

Case 121 – Summary!

This week we followed our patient with relapsed/refractory DLBCL through CAR T-cell therapy. We managed her cytokine release syndrome (CRS) and she is in remission thus far.

CAR T-cell therapy

Chimeric Antigen Receptor (CAR) T-cells are modified T cells in which the receptors are targeted against a specific tumour antigen. There a several ‘brands’ of CAR T-cells directed against CD19 which hold great promise in the treatment of relapsed/refractory B cell malignancies, including diffuse large B cell lymphoma (DLBCL), which has previously represented an area of unmet need.

Within the UK, eligibility and appropriateness for CAR T-cell therapy is discussed in MDT meetings at designated CAR T centres. Current products are licensed in DLBCL after two or more prior lines of treatment. Patients often require bridging therapy due to the timeframe of organising CAR T cell therapy (including MDT discussion, cell harvest, manufacture and production) and the therapy used is often down to clinician choice.

Patients receive lymphodepleting conditioning prior to infusion of CAR T-cell therapy. There is increasing recognition of specific toxicities related to CAR T-cell therapy:

 

Cytokine-release syndrome (CRS) is an escalated immune response triggered by CAR T-cell release of inflammatory mediators (cytokines) which often presents with fever, hypotension, hypoxia with or without evidence of end-organ dysfunction.

The severity of CRS can be graded according to the ASBMT Consensus Grading System

Table 1Table from Neelapu SS. Managing the toxicities of CAR T‐cell therapy. Hematological Oncology. 2019;37(S1):48–52. https://doi.org/10.1002/hon.2595

Centres which administer CAR T-cell therapy should have an algorithm for assessment and management of patients receiving CAR T-cell therapy to ensure early recognition and intervention of acute toxicities.

Management will often be guided by the grade of CRS. Supportive measures should be used including anti-pyretics, IV fluids and oxygen. It is vitally important to consider infection and treat accordingly.

Hypotension or hypoxia refractory to adequate fluid challenges or oxygen therapy, or those with Grade 2 CRS, can be considered for management with tocilizumab, an anti-IL-6 therapy. Depending on response patients may require intensive care admission with cardiovascular support. Corticosteroids can be considered for patients with Grade 3 or 4 CRS or those refractory to treatment above.

Patients receiving CAR T-cell therapy should be monitored closely with assessment of fluid balance, organ systems and CRS grade at least twice daily, or more frequently if there is a change in clinical status

 

Immune effector cell-associated neurotoxicity syndrome (ICANS) represents a toxic encephalopathic state and often presents with CNS signs including confusion, impaired higher cognitive function and delirium.

The severity of CRS is often graded using the ASBMT consensus grading system which incorporates the ICE score (Immune effector Cell‐associated Encephalopathy).

Table 2Table from Neelapu SS. Managing the toxicities of CAR T‐cell therapy. Hematological Oncology. 2019;37(S1):48–52. https://doi.org/10.1002/hon.2595

Management of ICANS is based on toxicity grade and options include supportive care, anti-IL-6 therapy (tocilizumab) when associated with CRS, or corticosteroids if not.

Patients receiving CAR T-cell therapy should be monitored closely with assessment of fluid balance, organ systems and ICANS grade at least 8 hourly, or more frequently if there is a change in clinical status. Both CRS and ICANS are reversible in most patients with the correct treatment, and so close monitoring and awareness of local policy is key.

There is also increasing recognition of medium term toxicities related to CAR T-cell therapy including prolonged cytopenias, risk for opportunistic infections, and B‐cell aplasia and hypogammaglobulinaemia.

 

References/further reading:

  • Neelapu SS. Managing the toxicities of CAR T‐cell therapy. Hematological Oncology. 2019;37(S1):48–52. https://doi.org/10.1002/hon.2595
  • Yakoub-Agha, I et al. Management of adults and children undergoing CAR t-cell therapy: best practice recommendations of the European Society for Blood and Marrow Transplantation (EBMT) and the Joint Accreditation Committee of ISCT and EBMT (JACIE). Haematologica Nov 2019, haematol.2019.229781; DOI: 10.3324/haematol.2019.229781

 

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.

Posted in Uncategorised

Case 121 – update 3!

We manage our patient with CAR T-cell therapy related CRS/ICANS with supportive care, including organ support from our critical care colleagues, and tocilizumab (anti-IL-6) therapy. She responds well to therapy and is stepped down to the haematology ward for ongoing care.

She is now 2 weeks following infusion of CAR T-cell therapy and observations are stable and she feels well.

How would you monitor this patient on discharge from hospital? What long term toxicities may you encounter?

 

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.

Posted in Uncategorised

Case 121 – Update 2!

We suspect cytokine-release syndrome (CRS) in our patient 5 days post CAR T-cell infusion. We manage her with aggressive fluid boluses, low-flow nasal cannula oxygen and paracetamol. We also complete a septic screen and cover her with broad-spectrum antibiotics. Her observations initially improve and she remains on the haematology ward. The next afternoon at 2pm you are asked to review the patient again.

She continues to spike high-grade fevers despite regular paracetamol and oxygen requirement has increased to 40% via facemask.

On further assessment, you also note that our patient appears a little muddled and less orientated.

What would be your next steps in the acute investigation and management of this patient?

 

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.

Posted in Uncategorised

Case 121 – Update 1!

Our 48 year old patient with relapsed/refractory DLBCL is referred for consideration of CAR T-cell therapy. She is managed with IVE chemotherapy as a ‘bridging treatment’ and is subsequently admitted for CAR T-cell infusion.

You are the on-call haematology SpR. You are called at 2am regarding our patient. She is 5 days post CAR T-cell infusion, and the SHO on-call is concerned she is ‘septic.’ Current observations are:

Temp 39.2

BP 84/60

HR 78

SpO2 92% O/A

What would be your next steps in the acute investigation and management of this patient?

 

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.

Posted in Uncategorised

Case 121 – The Beginning!

Welcome to another TeamHaem clinical case! Get involved and help shape the care of our fictional patient……

You review a 48 year old patient in lymphoma clinic. She was diagnosed with stage IVA diffuse large B cell lymphoma (DLBCL), with no evidence of MYC rearrangement, and completed treatment with six cycles of R-CHOP. End-of-treatment PET-CT demonstrated complete metabolic remission (CMR).

6 months following this she re-presented with stage IV relapsed disease and is currently post 2 cycles of R-GDP as ‘salvage chemotherapy.’ On review of a CT scan to assess disease response, she has progressive disease. She has no significant co-morbidity and ECOG performance status is 0.

What are the treatment options for this lady? Would you arrange any further investigations at this point?

 

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.

Posted in Uncategorised

Case 120 – Summary!

Thank you for everyone’s contributions this week. We discussed the management of a 57 year old lady who was suffering a major haemorrhage secondary to a road traffic accident.

The key points of managing a major haemorrhage (from haem perspective) are highlighted below:

  1. Identify Major Haemorrhage
    1. Early recognition essential to enable prompt provision of blood products
    2. Definitions:
      1. Bleeding which leads to heart rate >110bpm +/- systolic BP <90mmHg (NB be aware of patients baseline HR / SBP as individuals may be significantly compromised at the above parameters if their baseline levels vary from the general population)
      2. Loss of 1 blood volume in 24 hours / 50% blood volume in 3 hours / 125ml blood loss in 1 minute (NB these definitions are generally considered less useful as often detected retrospectively)
  1. Activate major haemorrhage protocol
    1. All hospitals within the UK should have a protocol
    2. Typically involves senior clinician, senior nurse, anaesthetist, transfusion lab, porter
    3. Ideally the team leader should designate one member of staff to liaise with the haematology lab

 

  1. Initial Management
    1. ABC approach and local control of bleeding
    2. Bloods:
      1. FBC, coag and clauss fibrinogen, biochem
      2. Group and save (x2 different times)
        1. Minimum identifiers: name, DoB, unique patient identifier number
        2. If unknown patient: Unique patient identifier number and gender
      3. Transfusion support (see below)
      4. Ascertain if on anti-platelets / anticoagulation (if possible)

 

  1. Transfusion Support
    1. Urgent blood / blood component transfusion. Major haemorrhage packs should be available as part of the major haemorrhage protocol. The documented ‘packs’ below are a guide and will vary slightly between hospitals such as 1 pool platelets may be available in pack. Need to switch to group specific / cross-matched packed red cells (PRCs) asap. Use cell salvage if available. Ongoing PRCs and components should be guided by blood results and near patient tests as soon as available alongside the ongoing clinical situation.
    2. Pack 1 of major haemorrhage pack
      1. PRCs and FFP in 1:1 ratio or 2:1 ratio (based on PROPPR trial – which improved deaths from bleeding but note no benefit in overall survival)
      2. 4 units PRCs
        1. If female of child bearing potential (<50 yrs) give Group O Rh D-ve Kell-ve
        2. If adult male or female >50yrs can consider Group O Rh D+ve
        3. Switch to group specific as soon as possible
      3. 4 units FFP
        1. Ideally Group AB but often in short supply in which case Group A (negative for high-titre anti-B)
    3. Pack 2 of major haemorrhage pack
      1. PRCs and FFP in 1:1 or 2:1 ratio (4 units PRCs : 4 units FFP)
      2. 1 pool platelets (consider giving platelets earlier if known to be on anti-platelets)
      3. Consider cryoprecipitate
    4. Transfusion based on blood results / near patient tests
      1. Aim to do traditional coagulation tests (PT, APTT, clauss fib) approximately every 30 mins. Be aware that these tests are not real-time so don’t represent the current clinical picture
      2. TEG / ROTEM provide real time results but should be used alongside traditional coag tests.Aim parameters
        1. Falling Hb: PRCS
        2. PTr / APTTr >1.5 : FFP 15-20ml/kg
        3. Clauss fibrinogen <1.5 (2 if obstetric haemorrhage) : 2 ppols of cryoprecipitate
        4. Platelets <50 : 1 pool platelets
  1. Pharmacological agents
    1. Tranexamic acid
      1. If trauma <3hrs / or risk of major haemorrhage / or not contraindicated as per CRASH 2 trial which demonstrated increased overall survival with TXA use. Give 1g TXA bolus then further 1g over 8 hours.
    2. Reversal agents for anti-coagulation (i.e protamine for heparin, praxbind for dabigatran)
  1. Other supportive measures
    1. Optimisation of hypothermia (aim >36.5C), acidosis, hypocalcaemia to reduce coagulopathy.
  1. Measures post major haemorrhage
    1. When bleeding has ceased – DEACTIVATE PROTOCOL (inform blood bank)
    2. Return unused stock to blood bank
    3. Monitor for complications from transfusions such as TACO
    4. Consider VTE prophylaxis when considered safe from bleeding perspective

 

References:

  1. Hunt BJ et al. A practical guideline for the haematological management of major haemorrhage. British Journal of Haematology. 170 (6). 788-803. 2015.
  2. Norfolk D. Handbook of Transfusion medicine 5th Edition. TSO. 2013.
Posted in Uncategorised

Case 120 – Update 4!

Well done team – with your input I’m pleased to say she has now stabilised and has stopped bleeding!!! To achieve this she has been transfused:

  1. 8 units red blood cells
  2. 8 units FFP
  3. 1 pool of platelets
  4. 2 pools cryoprecipitate

She undergoes surgery to stabilise her pelvis and is likely to be an inpatient for some time whilst she undergoes rehabilitation.

Questions:

  1. Now she is stable what would be the threshold to transfuse red blood cells? What her Hb target?
  2. What methods would you use to reduce her risk of thromboembolism?
  3. How would you ensure your hospital is managing major haemorrhages appropriately?

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.

Posted in Uncategorised

Case 120 – Update 3!

Thank you for everyone’s ongoing input. Our patient is more haemodynamically stable but continues to have ongoing bleeding. She is now receiving group specific/compatible blood products. She has currently received:

  1. 6 units packed red cells.
  2. 6 units FFP
  3. 1g tranexamic acid with a further 1g TXA being infused

A repeat FBC / coagulation screen is in progress. Her latest TEG is below (dotted line is normal TEG for comparison).

TEG

R time: 8min (4-8min), K time: 5min (1-4min), a-angle: 30 (47-74), MA: 35 (55-73mm)

Questions:

  1. What blood products would you give based on the TEG?
  2. Based on the traditional FBC / coagulation screen what values would you be aiming for? e.g PT/APTT/fibrinogen/platelets greater than ?

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.

Posted in Uncategorised

Case 120 – Update 2!

Thanks for everyone’s contributions so far!

Our 57 year old female patient is haemorrhaging following a road traffic accident. The multidisciplinary team are trying to establish source control of the bleeding. The major haemorrhage protocol has been activated. She has received packed red cells and FFP in a 1:1 ratio. She has received the following products:

  1. 4 units packed red cells: 1 unit Group O D negative, 3 units Group O D positive
  2. 4 units FFP: 2 units Group AB and 2 units Group A (negative for high-titre anti-B)

The baseline blood results are now available:

Hb 81, Plts 60, WCC 5. PT 19, APTT 42, Clauss Fib 1.4

The ABO group and D status result is available for interpretation (antibody screen in progress):

Forward Group Reverse Group
Anti-A Anti-B Anti-D1 Control A1 cells B cells
5 0 5 0 0 5

Questions:

  1. What ABO group is this patient? What is the Rh D status?
  2. What is the potential impact of the potential blood products that have already been given? How would you manage this?

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.

Posted in Uncategorised

Case 120 – Update 1!

Thanks for everyone’s contributions. Following recommendations from yourselves our patient  is being managed by a multidisciplinary team and the major haemorrhage protocol has been activated. The only information the clinical team know about our patients that she is called ‘Betty and she is 57 years old (DoB unknown).

Questions:

  1. What patient identifiers are required prior to issuing blood for our patient?
  2. What blood / blood products would you want in the major haemorrhage packs?
  3. What group / special requirements are required for the blood / blood products prior to establishing the patients blood group / antibody status?

 

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.

Posted in Uncategorised

Case 120 – The Beginning!

A 57 year old female is in Accident and Emergency following a road traffic accident. She has multiple injuries including a suspected pelvic fracture. She is haemodynamically unstable (heart rate 130, BP 86/54, oxygen saturations 97%). The clinical team are concerned she is still actively bleeding.

What is your initial investigation and management of this patient?

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.

Posted in Uncategorised

Case 119 – Summary

 Many thanks for all your great suggestions and contributions this week. We highlighted the multidisciplinary treatment of our patient with mycosis fungoides, who eventually required systemic treatment with brentuximab vedotin for his CD30+ disease.

Primary cutaneous T cell lymphoma (CTCL) is a type of non-Hodgkin lymphoma (NHL) which presents in the skin. It is the second most common group of extranodal NHL but remains a rare disorder, with an estimated annual incidence of 1 per 100,000 of the population in the UK, with a higher male incidence (1.6:1.0) and peak age between 50 and 74. Mycosis Fungoides (MF) is the most common subtype of CTCL, comprising around 40% of these conditions. Other less common subtypes include primary cutaneous anaplastic large cell lymphoma (pcALCL) and Sezary syndrome.

Patients with MF at different clinical stages present with skin patches/plaques, tumours or erythroderma; accurate staging relies on clinical, histological, immunophenotypical and genetic data.

All patients with suspected or proven primary cutaneous lymphomas should be reviewed at a regional Specialist Skin Cancer Multidisciplinary Team (MDT) meeting, with close involvement of haematology MDT in the presence of systemic disease. The current staging system in use in the UK is the International Society for Cutaneous Lymphomas (ISCL) EORTC revised staging system. This system distinguishes patches and plaques, incorporates a molecular assessment of lymph node and peripheral blood, and provides a quantitative method for assessing peripheral blood disease in SS (see links at bottom).

Treatment options will often be dictated by the clinical stage of disease. Options include skin-directed therapies (SDT) such as topical agents, phototherapy and radiotherapy, and systemic therapies including biologics, chemotherapy and stem-cell transplantation.

Early stage MF (Stage IA – IIA) is often treated with skin-directed therapy including topical steroids and phototherapy. Patients with infiltrative plaques or tumours (stage IIB) may also be offered local radiotherapy.  Those with more systemic disease may be offered skin-directed therapies in conjunction with systemic biologic therapies e.g. interferon alfa or retinoids. Total skin electron beam therapy has also been used in this group. Those with advanced and refractory disease often require antibody therapy or chemotherapy. Allogeneic stem cell transplant can also be considered.

Extra reading:

ESMO clinical practice guideline: https://www.esmo.org/Guidelines/Haematological-Malignancies/Primary-Cutaneous-Lymphoma

British Association of Dermatologists guideline: http://www.bad.org.uk/shared/get-file.ashx?id=6265&itemtype=document

Posted in Uncategorised

Case 119 – Update 2

Our patient with Stage IIB Mycosis Fungoides was discussed at the relevant MDT and his care remained primarily under the dermatology and clinical oncology service. They instigated topical treatment alongside localized radiotherapy to his tumour site on the right arm. He also required systemic treatment with oral methotrexate. He responded well and achieved a good partial response (PR) with resolution of his severe itch.

2 years later he is referred urgently to haematology clinic. Several new lesions have appeared, at the original site of disease (right arm) but also on his chest wall and left ankle. These lesions initially responded to radiotherapy but recurred within a few weeks. His skin disease is less responsive to topical treatment.

He feels generally fatigued and describes unintentional weight loss over the last few months. On examination, he has nodular, ulcerating lesions in the distribution above, as well as bilateral axillary and inguinal lymphadenopathy.

Re-staging investigations demonstrate Stage IVA disease.

What treatment options would you consider?

 

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.

Posted in Uncategorised

Case 119 – Update 1

We are assisting the dermatology team in their investigation of a 68 year old man, with a longstanding history of eczema, presenting with erythematous, well defined patches and plaques over his trunk and limbs, a 2cm firm lesion on his right arm and right axillary lymphadenopathy.

Many thanks for everyone’s input thus far. Summary of requested investigations:

  • Bloods look unremarkable except for non-specific increase in inflammatory markers
  • Viral serology, including HIV and Human T-cell lymphotropic virus type 1, is negative
  • Skin biopsy reported as demonstrating an epidermotropic infiltrate of T cells (CD3+ CD4+ CD30+)
  • Peripheral blood flow cytometry does not indicate an excess of Sezary cells and there is no evidence of a clonal TCR gene rearrangement
  • Lymph node biopsy demonstrates reactive changes
  • PET-CT confirms a right axillary lymph node measuring 2.7 cm with moderate FDG avidity (SUV max, 3.9). Increased uptake at the right arm corresponding with the clinical lesion.

As you have stated, investigation findings are suggestive of cutaneous T cell lymphoma (CTCL), specifically mycosis fungoides (MF).

How would you go about staging and risk stratifying the disease? What treatment options would you consider at this point, and under which medical team?

 

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.

Posted in Uncategorised

Case 119 – The beginning

Welcome to a new case for TeamHaem!

You get a phone call for advice from a dermatology registrar who has seen a 68 year old man in outpatient clinic.

The patient has a 20-year history of eczema which has been managed by his GP with topical treatment. He has experienced gradual worsening control of his skin over recent months and now complains of unbearable itching. On examination the SpR noted erythematous, well defined patches and plaques over his trunk and limbs. She also noted a 2cm firm lesion on his right arm with associated right axillary lymphadenopathy, and would therefore value your input.

What further information would you like to know? What investigations would you suggest that the team arrange?

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.

Posted in Uncategorised

Case 118 – the summary

This is a case of a 3 month old infant girl presenting with evidence of haemolysis.

Blood film showed irregularly contracted cells, target cells and polychromasia.  The reticulocyte count was raised and direct antibody test (DAT) was negative.

This is suggestive of a congenital haemolytic anaemia.  The causes of congenital haemolytic anaemia can be divided into two causes: membrane or enzyme deficiency.

Membrane causes include amongst others, hereditary spherocytosis and hereditary elliptocytosis.  There was no evidence of such causes on this blood film.

Enzyme causes include G6PD deficiency but this is unlikely in a female patient (it is inherited in an X-linked fashion).  Other possibilities include pyruvate kinase deficiency or triosephosphate isomerase deficiency.

In this case the diagnosis was triosephosphate isomerase deficiency.

  • inheritied in an autosomal recessive manner
  • due to mutations in the TPI1 gene
  • presents in infancy with pallor and jaundice
  • associated with movement disorders in older patients (usually apparent by 2 years of age)

 

Further information can be found here:

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5503903/pdf/br-52-84.pdf

Posted in Uncategorised

Case 118 – update 2

You ask the paediatrician about the baby’s history and discover:

  • she has never had a blood tranfusion
  • mum does not have an allogenic antibody
  • there is no known family history of haemolysis
  • there are signs of jaundice

Further tests show

Reticulocyte count 436 (reference range for age 50-100)

Direct antibody test is negative

What is the most likely differential diagnosis and what further tests could be done to make the diagnosis?

Posted in Uncategorised

Case 118 – update 1

You review a peripheral blood film on the sample (shown below).  What is your differential diagnosis?  What questions to you want to ask the paediatrician?

50HD0998.JPG

Posted in Uncategorised