Case 103 – update 1

We have found out that our patient has just started adjuvant chemotherapy for breast cancer following surgery.

Five days prior to admission she had a 2 unit blood transfusion, which had no immediate complications.

She had no transfusions prior to this.

4 previous pregnancies with uncomplicated delivery. All babies were born at term with no jaundice or anaemia.

She self discharged from the urology ward and was given a course of antibiotics for a presumed UTI.

She attends her oncology day unit later that day for review following her chemotherapy. This is a different hospital to her local hospital where she attended for the haematuria. She mentioned that she had been feeling increasingly tired and had been admitted under urology for a blood transfusion and antibiotics following blood in her urine which had resolved on admission.

Her Hb is found to be 67g/l and her creatinine 288, with a bili of 106. She is admitted to the oncology ward for iv fluids and antibiotics and she is crossmatched and transfused 2 further units of blood, antibody screen negative. The lab has not been informed regarding the previous possible transfusion reaction. During the 2nd unit of blood she begins to feel very, but none specifically, unwell, and has some chills. She remains haemodynamically stable. The transfusion is stopped and the patient has piriton, hydrocortisone and paracetamol. The iv fluids are continued. The patient is catheterised and her urine again appears to be red.

Our patient has now had a total of 6 units of red cells transfused in the last 8 days. Following the most recent transfusion her bloods show –

Hb 80g/l

Plt 180

Wcc 11

Neuts 5.5

Urea 12

Creatinine 400

Bilirubin 90

Pt 14

Aptt 35

Fib 3.5

LDH 400

Retic 35 (50-100×109/l)

Haptoglobin undetectable

DAT negative

Blood cultures pending

What would you do next? Would you like any more information?

What do we think is happening?

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 103 – the beginning

Welcome to this week’s teamhaem case!

This week we start with a 50 year old lady who has presented to A&E complaining of feeling tired and she has, over the previous couple of days, noticed blood in her urine, however this has now stopped.

Bloods

Hb 77g/l

Plt 200

Wcc 11

Neuts 5

She is transferred to the urology ward where she is transfused 2 units of blood. After the 2nd unit of blood our patient began to feel unwell and had a rigor. She remained haemodynamically stable. She was assessed by the urology team and started on antibiotics. They have also taken some bloods to check for transfusion reaction.

What types of transfusion reactions are there? What investigations would you do if a transfusion reaction were suspected?

What questions would you like to ask about our patient’s history?

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 102 – The Summary

 

Thank you for all the contributions to the case this week!

This week we discussed a 29 year old lady who presented with an extensive left arm DVT. She had been on the COCP for 2 years. Further investigations detected a large mediastinal mass that was confirmed to be Stage 2 Primary Mediastinal B cell Lymphoma. From our discussions we covered the following topics that will be discussed further below:

  • Upper extremity DVT
  • Cancer-associated thrombosis
  • Primary mediastinal B cell lymphoma

Upper Extremity DVT1,2

  • Account for upto 10% of all DVTs. The incidence is increasing predominantly due to the increasing use of central venous catheters and pacemakers.
  • Include radial, ulnar, brachial, axillary, subclavian, brachiocephalic and jugular veins.
  • Aetiology
    • Primary
      • Idiopathic
      • Thoracic outlet syndrome (compression of neurovascular bundle secondary to first rib, clavicle or muscle bulk)
      • Paget-Schroetter syndrome (vigorous effort causes microtrauma to the vessel resulting in thrombosis)
    • Secondary
      • Foreign body within vascular system i.e central venous catheter, pacemaker (most common risk factor)
      • Malignancy
      • Surgery, immobilisation
      • Acquired thrombophilia i.e anti-phospholipid syndrome
      • Inherited thrombophilia i.e anti-thrombin deficiency
      • Others (extensive list as for lower limb DVT / PE although Combined oral contraceptive pill / hormonal replacement therapy have not been convincingly found to be risk factors but no RCTs).

Clinical presentation:

  • Venous congestion e.g swelling, pain, dilation of the superficial veins

Investigations:

  • Compression ultrasound / venography
  • Investigations of underlying cause (directed by history / examination)

NB: DDimer has NOT been validated for the use of upper limb DVT

Complications:

  • PE
  • Post thrombotic syndrome
  • Superior vena cava syndrome
  • Recurrence

Management:

  • Anticoagulation
    • Various options regarding the type of anticoagulation exist including
      • LMWH
      • LMWH for 5 days followed by warfarin
      • DOACs are being increasing used (note studies regarding the efficacy of DOACs were not specific for upper limb DVT)
    • The optimal duration of anticoagulation is unknown. Periods of anticoagulation of 3-6 months tend to be associated with low risk of recurrence (<5%) but the risk needs to be individually assessed.
  • Catheter directed thrombolysis / thrombectomy can be considered in specific circumstances
  • Treatment of risk factor e.g resection of cervical rib, scalenotomy

 

Cancer-associated Thrombosis3,4:

  • Initial anticoagulation should be for 6 months if tolerated. In on-going active malignancy anticoagulation should be continued (with consideration of patient preference, bleeding risk etc)
  • Anticoagulation options
    • LMWH (lower VTE recurrence rate vs warfarin -7.9% vs 15%)
    • DOACs4
      • 2 RCTs for edoxaban and rivaroxaban (HOKUSAI Cancer and SELECT-D respectively) demonstrated non-inferiority vs LMWH in recurrence of VTE in cancer patients. However there was increased risk of bleeding especially in gastrointestinal and urological malignancies. Therefore in setting of thrombocytopenia one may opt for LMWH.
      • The effects of cancer treatment needs to also be considered when deciding re use of edoxaban or rivaroxaban in this setting. For example, whether will be absorbed and drug-drug interactions.
  • Anticoagulation in setting of thrombocytopenia3:
    • Platelets <50 x 109/l: Give plt transfusions to elevate plt >50 to allow full dose anticoagulation (especially in acute period 4-6 weeks)
    • Platelets 25-50 x 109/l: Frequent assessment of anticoagulation should occur
    • Platelets <25 x 109/l: Avoid full dose anticoagulation.

 

Primary Mediastinal B Cell Lymphoma (PMBCL)5

  • Subtype of DLBCL (~10%), derived from a thymic B cell
  • Females > males
  • Typically presents in 3rd and 4th decades

Clinical presentation

  • Bulky anterior mediastinal mass
  • 75% stage 1 or 2
  • Symptoms relating to local compression e.g airway compromise, superior vena cava obstruction
  • Symptoms relating to local invasion
  • B symptoms
  • 50% have pleural or pericardial effusions. CNS / bone marrow involvement not uncommon.

Investigations:

  • FBC, U+Es, LFTs, bone profile, LDH, urate
  • HIV, hepatitis B+C
  • CT neck/chest/abdo/pelvis
  • PET CT
  • Biopsy
    • Significant histopathological overlap between PMBCL and nodular sclerosing Hodgkin lymphoma
    • Morphology: Medium-large lymphoid cells, often sclerosis and some cells can resemble Reed-Sternberg cells. Often extensive fibrosis.
    • Immunophenotype: CD20+, CD22+, CD79a+, sIg-, CD30weak, CD15-, CD5-.

Management:

There is no single approach to treatment currently and the need for consolidative radiotherapy and role of PETCT after R-chemo remains controversial. It is important to optimise upfront therapy as salvage treatment once disease has relapsed is rarely curative. However, the longer-term toxicities need to be considered in this young patient cohort.

Chemo-immunotherapy options:

  • DA-EPOCH-R (6 cycles)

3 year PFS 83-93% (Dunleavy 2013, Kryachok 2016 ESMO). May be able to omit radiotherapy if end of treatment PET shows complete metabolic remission but ongoing IELSG 37 trial to assess this. Would currently recommend discussion with oncologist to discuss potential options. Note this regime contains high anthracycline doses.

  • R-CHOP (6 cycles)

3 year PFS ~70% (Rieger 2011 MInT, Kryachok 2016 ESMO). Typically require radiotherapy.

  • (R-)V/MACOP/B – less commonly used.

Thromboprophylaxis

  • To be considered as high risk of thrombotic complications (28.2% – Roth 2017, Lugano) including upper extremity DVT, superior vena cava, intracardiac etc.

Supportive therapies:

  • Blood product support
  • Antimicrobial support
  • Pyschological

References

  1. Tait C et al. Guidelines on the investigation and management of venous thrombosis at unusual sites. British Journal of Haematology. 2012. 159(1): 28-38.
  2. Heil J et al. Deep Vein Thrombosis of Upper Extremity. Deutsches Arzteblatt International. 2017. 114(14): 244-249.
  3. Watson H et al. Guideline on aspects of cancer-related venous thrombosis. British Journal of Haematology. 2015. 170(5): 640-648.
  4. Khorana A et al. Role of Direct Oral Anticoagulants in the treatment of cancer-associated venous thromboembolism: guidance from the SSC of the ISTH. Journal of Thrombosis and Haemostasis. 2018. 16:1891-1894.
  5. Giulino-Roth L. How I treat Mediastinal B-cell Lymphoma. Blood. 2018. http://www.bloodjournal.org/content/bloodjournal/early/2018/07/05/blood-2018-04-791566.full.pdf?sso-checked=true

 

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 102 – Update 3.

We now have further results for our 29 year old female who presented with an extensive left arm DVT and SOB. She was commenced on therapeutic LMWH. This thrombosis was provoked as she has been found to have a 11cm anterior mediastinal mass.

FBC, U+Es, calcium and phosphate: within normal range. LDH: 700.

CT CAP and PET CT confirms stage 2 disease.

Histology (core biopsy): Medium to large lymphoid cells with abundant cytoplasm.

Immunophenotype: CD20+, CD22+, CD79a+, sIg-, CD30 weak, CD15-.

Questions:

  1. What is the diagnosis?
  2. How would you manage this patient?

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 102 – Update 2.

Thanks for everyone’s contributions!

We have been discussing the management of a predominantly idiopathic upper limb DVT in our young lady on the combined OCP. She is on therapeutic LMWH and currently continues on the combined OCP. However, your requested investigations have now been completed and are as follows:

  1. CTPA: No evidence of PE or accessory rib but large mediastinal mass (11cm).
  2. Antiphospholipid screen: Normal.

Questions:

  1. What are your main differential diagnoses?
  2. What further investigations do you want?

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 102 – Update 1.

Thanks for everyones contributions!

Following everyone’s suggestions we now know that this 29 year old with an extensive upper limb DVT, has been short of breath over the past few weeks and non-specifically unwell. She has been on the combined oral contraceptive pill for 2 years and rarely misses any doses. She has had no other recent relevant personal history including no venous catheters, surgeries and does not undergo excessive exercise regimes. She has no relevant family history.

She has been commenced on therapeutic anticoagulation. An antiphospholipid screen and CTPA (to assess for both PE and an accessory rib) are pending.

Questions:

  1. What risk would you attribute to the COCP and thrombosis?
  2. What would you advise about the use of OCP to this lady now?
  3. If this appears to be idiopathic upper limb DVT with no other known risk factors how would you manage?

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 102 – The Beginning

Welcome to the new #TeamHaem Case!

You are working in the acute medical day unit. A 29 year old female has been referred in with a week long history of progressive left arm swelling. An ultrasound has confirmed an extensive DVT in the axillary and subclavian vein.

Questions:

1. What further history and investigations would you like?

2. What immediate management would you suggest?

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 101 – summary

This is a case of super warfarin overdose. It was complicated by the lack of accurate history from the patient due to presentation with an intra cranial haemorrhage.

Super warfarin are easily available as rodenticides. The most common in the UK are difenacoum, bromodialone and brodifacoum. All have long half lives. In the case of difenacoum, it’s half life in the plasma is approximately 20 days and 60 days in tissue.

Frequently many months of daily treatment with higher doses of vitamin K are required. Initially in this case daily intravenous doses of 50 – 100 mg are likely to be needed, followed by many months of 100mg a day orally.

Suggestions for further reading:

Card, D.J., Francis, S., Deuchande, K. and Harrington, D.J., 2014. Case Report: Superwarfarin poisoning and its management. BMJ case reports, 2014.

King, N. and Tran, M.H., 2015. Long-acting anticoagulant rodenticide (superwarfarin) poisoning: a review of its historical development, epidemiology, and clinical management. Transfusion medicine reviews, 29(4), pp.250-258.

Spahr, J.E., Maul, J.S. and Rodgers, G.M., 2007. Superwarfarin poisoning: a report of two cases and review of the literature. American journal of hematology, 82(7), pp.656-660.

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Case 101 – update 3

Further history is sort and her partner confirms the presence of rat poison in the house.

Repeat factor levels show:

Factor II – 24%                         Factor VII – 9%

Factor X – 30%                        Factor V – 101%

Factor VIII – 156%                   Factor IX – 27%

Difenacoum is detected in the plasma

A diagnosis of super warfarin overdose is made.

Question: how should this case be managed?

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Case 101 – update 2

She is treated for vitamin K deficiency and prescribed 10mg vitamin K IV for a further 2 days.

36 hours after admission you are called because she had developed epistaxis and frank haematuria.

Repeat bloods show:

  • Hb 89  g/l
  • MCV 82 fl
  • WCC 14.5 x 109/l
  • Platelets 455 x 109/l
  • PT 132 seconds
  • APTT 102 seconds
  • Fibrinogen 6 g/L

Questions:

  1. How do you interpret these results?
  2. What further investigations do you want to perform?
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Case 101 – update 1

She is given prothrombin complex concentrate and vitamin K 10mg IV.

She has a history of asthma and depression, she has taken sertraline for the last five weeks and salbutamol inhalers infrequently.  There is no history of anticoagulant use and no previous history of abnormal bleeding.

Her pre-treatment factor levels return as:

Factor II – 11%                      Factor VII – 18%

Factor X – 7%                        Factor V – 101%

Factor VIII – 156%                Factor IX – 2%

Liver and renal function are normal.

Peripheral blood morphology is normal.

D-dimer is 350 ng/mL

A pregnancy test is negative.

Questions:

How do you interpret these results?

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Case 101 – the beginning

Welcome to the new #TeamHaem Case!

You are a medical doctor working in a busy emergency department.

You are called to review a 34 year old woman who has been brought in by ambulance.  She was found by her partner lying on her sofa confused and disorientated.

There are no signs of external injury.  Her Glasgow Coma Score is 11.  An urgent CT head is performed and shown below:

Subdural1 (1)

Initial blood tests show:

  • Hb 102  g/l
  • MCV 87 fl
  • WCC 12.5 x 109/l
  • Platelets 301 x 109/l
  • PT 123 seconds
  • APTT 98 seconds
  • Fibrinogen 7 g/L

Questions:

1. How do you interpret these results?

2. What further history and investigations would you like?

3. What immediate management would you suggest?

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 100 (part D) – summary

The patient had mild thrombocytopenia and has suffered epistaxis. Epistaxis is relatively common and therefore structural causes should also be looked into. This is especially so if the nose bleeds are unilateral. There is a wide differential of why someone may have a low platelet count, including:

  • Any viral illness including HIV and hepatitis
  • Laboratory artefact e.g. platelet clumping
  • Sepsis
  • Medications
  • Nutritional
  • Liver disease and hypersplenism
  • Autoimmune disease
  • Immune thrombocytopenia
  • Anti-phospholipid syndrome
  • Marrow infiltration e.g. leukaemia
  • Inherited bleeding disorders e.g. type 2b vWD and platelet disorders
  • DIC
  • Micro-angiopathic haemolytic anaemia e.g. TTP, HUS
  • Evans syndrome
  • Post transfusion purpura
  • Heparin-induced thrombocytopenia
  • Pregnancy related including gestational thrombocytopenia and consumption due to pre-eclampsia etc.
  • Kasabach-Merritt syndrome
  • Inherited bone marrow failure syndromes e.g. thrombocytopenia with absent radii, Fanconi anaemia, Wiscott Aldrich etc.
  • Dilutional or consumption e.g. major haemorrhage

Most of these can be ruled out by history and examination and some basic tests. For most further blood tests may not be required but below gives a vague guide.

History

  • Bleeding using bleeding assessment tool
  • Eczema
  • Diet and drugs
  • Birth
  • Recurrent infections

Family history

  • AML and MDS
  • Bleeding

Examination

  • Hearing loss
  • Malformations including arms, dental, face, heart
  • Pigmentation
  • Developmental delay

Laboratory (not all usually necessary)

  • Coagulation screen
  • Clauss fibrinogen
  • vWF, VIII, IX, XI
  • FBC, reticulocytes and film
  • LFTs, renal function
  • Inflammatory markers
  • Immunoglobulins
  • Haematinics

In our cases the blood film showed enlarged platelets with a neutrophil inclusion consistent with a Döhle body. May Hegglin

Macrothrombocytopenia

This can be seen in:

  • Bernard Soulier syndrome
  • Paris-Trousseau syndrome
  • MYH9 syndromes
  • Arthrogryposis renal dysfunction and cholestasis syndrome
  • Giant platelets and velo-cardio-facial syndrome
  • Macrothrombocytopenia with dyserythropoiesis/anaemia/beta thalassaemia (GATA1)
  • Grey platelet syndrome

 

May Hegglin and MYH9 abnormalities

The combination of mild thrombocytopenia with mild bleeding and leucocyte inclusions makes one think of MYH9 abnormalities which are rare, autosomal dominant conditions due to mutation in MYH9 on chromosome 22 – codes for heavy chain of non-muscle myosin IIa – involved in contractile activity of cytoskeleton. Patients have thrombocytopenia with enlarged hypergranular platelets. They may have mild neutropenia but no increase in infection. Bleeding is uncommon and can usually be treated with tranexamic acid. Structural problems (in this cause ne had abnormal vasculature in the nose) should be looked for. Some MYH9 conditions may result in renal disease and hearing loss (Epstein syndrome) and cataracts (Fetchner syndrome) so these should be looked for.

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 Inherited bleeding, Laboratory morphology, Paediatric haematology, Platelet disorders | Tagged , , , , , , , ,

Case 100 (part D) – update

You repeat the FBC and it shows similar results

  • Hb 141g/l (130-180)
  • MCV 89fl (82-98)
  • WCC 7.5×109/l (4-11)
  • Neutrophils 4.7×109/l (1.7-7.5)
  • Lymphocytes 2.1×109/l  (1.5-4.5)
  • Platelets 70×109/l (150-450)
  • Coagulation screen – normal
  • U&E/LFT – normal

Blood film:

May Hegglin

Questions

  • What is the problem here?
  • How is it managed?

 

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 Inherited bleeding, Laboratory morphology, Paediatric haematology, Platelet disorders | Tagged , , , , , ,

Case 100 (part D) – the beginning

You are a GP evaluating a 12 year old boy with persistent nose bleeds. He otherwise feels well with no past medical history. He is on no medications. FBC shows:

  • Hb 140g/l (130-180)
  • MCV 90fl (82-98)
  • WCC 8.5×109/l (4-11)
  • Neutrophils 5.7×109/l (1.7-7.5)
  • Lymphocytes 2.3×109/l  (1.5-4.5)
  • Platelets 74×109/l (150-450)

Questions

  • How do you investigate this further?

 

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 Inherited bleeding, Paediatric haematology, Platelet disorders | Tagged , , ,

Case 100 (part C) – summary

Our patient had a mild lymphocytosis. The differentials include:

  • Reactive – especially viral illnesses
  • Hyposplenism
  • Smoking
  • Polyclonal B cell lymphocytosis – binucleate lymphocytes with isochrome 3q
  • Lymphoproliferative neoplasms such as CLL and other types of lymphoma

In the first instance repeating the FBC is all that is required as it will often normalise. Patients with persistent lymphocytosis without reactive causes should have a history and examination to assess for any complications of CLL/lymphoma e.g. lymphadenopathy, splenomegaly and B symptoms. The rest of the blood count should be reviewed to look for any cytopenias or haemolysis. There are no absolute guidelines of when to refer to a haematologist or when to obtain confirmatory flow cytometry/immunophenotyping. The key is that most of these disorders are slow -growing and most will never need any treatment and therefore mild to moderate lymphocytosis can just be monitored. If the cells appear atypical on the blood film then a haematologist may reflex immunophenotyping.

In this case the lymphocytosis was very mild but increased over time and the morphology was not typical for CLL. By flow there was a kappa restricted B cell population = 19.2 x 109/L which was CD19+, FMC7+, CD23 negative, CD5+, CD79b+, CD10 negative, CD103 negative, CD11c negative, CD38 negative, sIg+ (CLL score 1/5). FISH showed an abnormal CCND1 rearranged signal pattern in 54 of 100 nuclei scored, consistent with a clone with t(11;14)(q13;q32) translocation or other rearrangement of CCND1 (11q13), typical of mantle cell lymphoma.

Mantle cell lymphoma is a type of lymphoma that behaves in both an indolent and aggressive fashion. Patients can undergo active monitoring in the early stages but for this type of lymphoma often treatment is required. This includes R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine and prednisolone) and for fitter patients a regime containing high dose cytarabine followed by an autologous stem cell transplant. Rituximab maintenance therapy is used. Other options for treatment in less fit patients includes bendamustine-rituximab, VR-CAP (rituximab, bortezomib, cyclophosphamide, doxorubicin and prednisolone), R-CVP (rituximab, cyclophosphamide  and prednisolone) or rituximab-chlorambucil. Ibrutinib is licenced in the UK for relapsed patients.

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 Chronic leukaemia, Laboratory morphology, Lymphoma | Tagged ,

Case 100 (part C) – update 1

Our patient has a mildly elevated lymphocyte count and this is persistent on repeat. In the absence of reactive causes the most likely explanation is a low grade lymphoproliferative neoplasm such as CLL. After a period of monitoring the patient’s lymphocyte count rises:

  • Hb 140g/l (115-165)
  • MCV 95fl (82-98)
  • WCC 30.4×109/l (4-11)
  • Neutrophils 5.7×109/l (1.7-7.5)
  • Lymphocytes 23.3×109/l  (1.5-4.5)
  • Platelets 232×109/l (150-450)

Here is the film:

Case 100 part c

A haematologist contacts you as the laboratory have performed flow cytometry:

Case 100 flow1Case 100 flow3Case 100 flow4Case 100 flow5

Questions

  • What is the most likely diagnosis?
  • What questions are you going to ask?
  • What test can confirm the diagnosis?

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 Chronic leukaemia, Laboratory morphology, Lymphoma | Tagged , , , ,

Case 100 (part C) – the beginning

You are in a GP surgery and are asked to perform a full blood count on a 60 year old male who is awaiting a knee replacement for osteoarthritis. He has a past history of glaucoma, hypertension and a myocardial infarction a few years ago.

  • Hb 147g/l (115-165)
  • MCV 95fl (82-98)
  • WCC 10.4×109/l (4-11)
  • Neutrophils 3.7×109/l (1.7-7.5)
  • Lymphocytes 5.8×109/l  (1.5-4.5)
  • Monocytes 0.6 ×109/l  (0.2-1.0)
  • Eosinophils 0.2 ×109/l  (0-0.5)
  • Basophils 0.1 ×109/l  (0-0.1)
  • Platelets 252×109/l (150-450)

 

Questions

  • How would you approach this problem?
  • What further information would you like to know or would you ask the patient?
  • What do you tell the 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.

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Posted in Chronic leukaemia, Lymphoma | Tagged

Case 100 (part B) – summary

Our patient had a mild neutrophilia. Neutrophilia can be seen in:

  • Neonates
  • Infection
    • Especially bacterial (note marrow depletion may occur leading to neutropenia)
  • Inflammation, autoimmune diseases etc.
  • Acute gout
  • Stressed states with high adrenaline e.g. myocardial infarction, exercise, acidosis, eclampsia, seizures, acute haemorrhage
  • Malignancy e.g. Hodgkin lymphoma, ovarian carcinoma
  • Leucoerythroblastic anaemia
    • Metastatic marrow infiltration
    • Myeloproliferative neoplasm
    • Miliary TB
    • Osteopetrosis
  • Drugs
    •  Steroids
      • Lead to increased neutrophils from reserve to circulating pool but reduced neutrophils from circulating pool to tissue leading to increased peripheral blood neutrophilia
    • Beta agonists
    • Lithium
    • Tetracycline
    • GCSF
  • Smoking
  • Uraemia
  • Hyposplenism
  • Myeloproliferative neoplasms
    • Chronic myeloid leukaemia
    • Atypical chronic myeloid leukaemia
    • Chronic myelomonocytic leukaemia
    • Chronic neutrophillic leukaemia
    • Myelofibrosis
    • Polycythaemia vera
  • Leucocyte adhesion deficiency
  • Familial cold urticarial and leucocytosis

 

The most common causes of neutrophilia are reactive and secondary causes should be looked for in the history and examination. Where there is a cause usually no further investigations are needed but clinicians may want to repeat the test to ensure it is resolving as expected.

Pointers towards something more serious includes:

  • Abnormalities on the blood film – tear drop cells, nucleated red cells
  • Basophilia (can be seen in reactive state but would want to monitor)
  • Non-resolution as expected and greater than 15×109/l
  • Patient unwell with systemic symptoms
  • Splenomegaly

CML annotated

  • 1 Neutrophil
  • 2 Basophil
  • 3 Myelocyte
  • 4 Eosinophil

In this case chronic phase CML was the most likely diagnosis due to the persisting leucocytosis and basophilia without reactive causes. Left shifted granulocytes were seen on the film. This was confirmed by peripheral blood fluorescence in situ hybridisation looking for t(9;22) BCRABl1 which was detected.

Tyrosine kinase inhibitors such as imatinib are used to treat CML and the outcome for patients is now excellent.

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 Laboratory morphology, Myeloproliferative neoplasm, Related to other specialities | Tagged , , , , , , ,

Case 100 (part B) – update

Our patient’s neutrophilia/thrombocytosis is attributed to his chest infection. You advise to get a repeat FBC in one month but he forgets and goes to Spain for some ‘winter sun’. On return he has a repeat FBC:

  • Hb 145g/l (115-165)
  • MCV 92fl (82-98)
  • WCC 28.6×109/l (4-11)
  • Neutrophils 22.1×109/l (1.7-7.5)
  • Lymphocytes 1.8 (1.5-4.5)
  • Monocytes 1.0 (0.2-1.0)
  • Eosinophils 0.9 (0-0.5)
  • Basophils 1.2 (0-0.1)
  • Platelets 500×109/l (150-450)

A blood film is performed:

Case 100 part b

Questions

  • What would you look for on examination?
  • What further test would you do?
  • What would you explain to the 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 Laboratory morphology, Myeloproliferative neoplasm, Related to other specialities | Tagged , , , , ,