Teaching case presentation on pancytopaenia

Case presentation pancytopaenia

1. An Interesting case of pancytopaenia

2. 62F

3. What would you do next?

4. Investigations Other blood tests: Haematinics, reticulocyte count Viral serology LDH Protein electrophoresis Blood film: confirm counts are true, any atypical appearances of cells, blasts Imaging and other things to consider: - US/CT scans - *Bone marrow biopsy

5. Causes of pancytopaenia Reduced production Infiltration: malignancy (haem e.g. AML, lymphoma/non-haem e.g. prostate, breast), Myelosuppression: drugs (MTX, chemo)/infection Bone marrow failure Immune destruction (aplastic anaemia) Malnutrition Ineffective haematopoiesis (MDS, myelofibrosis) Destruction/Consumption Hypersplenism Sepsis

6. When do you need to think about transfusions/ gCSF ? This is very ‘tricky’ question, as it is all about the individual patient context but … Hb <70 Plts This really depends, if is the patient bleeding and what their clotting is doing But think about transfusions around these thresholds … <100 if intracerebral bleeds/neurosurgery <50 need to stop anticoagulants/antiplatelet therapies, if surgery required or planned <20 if febrile <10 Neutrophils <1

7. Neutropaenic Sepsis! = Potentially life threatening condition requiring prompt assessment and treatment Any patient who has received chemotherapy in past 6 weeks and/or with underlying haem malignancy or known immunodeficiency WITH Temp >38 o OR >37.5 o on 2 separate readings 1 hour apart AND/OR Clinically unwell, signs of infection, red/amber flags

8. Neutropaenic Sepsis! Urgent triage and assessment History: date of last chemo, chemo regimen Examination: focus of infection, observations Bloods: FBC, U&E, LFT, Bone, CRP, lactate, blood cultures (peripheral and lines) Consider urine/sputum/stool/wound/CSF MC&S Red flags HR ≥130 SBP ≤90mmHg Urine output <0.5ml/kg/hr RR ≥25 or new O2 requirement New onset objective altered mental state Mottled. Cyanosed Non blanching rash Amber flags HR≥90 SBP≤100mmHg Urine output 0.5-1ml/kg/hr Temp<36 o Subjective new onset mental state Clinical signs of potential infection Acute deterioration of functional ability

9. Neutropaenic Sepsis! Delivery of empiric IV antibiotics in 1 hour of arrival No penicillin allergy Non-severe penicillin allergy Severe penicillin allergy Pipercillin-Tazobactam 4.5g IV QDS + IF signs of severe sepsis or haematology patient Gentamicin IV STAT 5mg/kg (max 320mg) *If suspected line infection/MRSA/mucositis Teicoplanin 10mg/kg IV BD 3 doses, then OD Ceftazidime 2g IV TDS Teicoplanin 10mg/kg IV BD 3 doses, then OD Gentamicin IV STAT 5mg/kg (max 320mg) Ciprofloxacin 400mg IV BD Teicoplanin 10mg/kg IV BD 3 doses, then OD Gentamicin IV STAT 5mg/kg (max 320mg)

10. Back to our case!

11. History of presenting complaint: Recurrent ongoing severe nosebleeds Recent development of peripheral swelling and exertional dyspnoea Good diet, doesn’t drink any alcohol Examination: Marked telangiectasia over tongue Some bruises on arms/legs AF Significantly raised JVP Hepatomegaly (extending into the epigastric area)

12. Haemorrhagic Hereditary Telangiectasia – diagnosed in her early 20s Severe recurrent epistaxis Lung and liver AVMs On Tamoxifen AF On Bisoprolol and Warfarin (Target INR 2-2.5) Refractory to DC cardioversion ?For atrial appendage occlusion Recurrent IDA on Ferrinject (target Hb >110 and Ferritin >100) Pulmonary hypertension (2 o to liver AVM) Salt and water retention On Spironolactone and Bumetanide

13. Conclusions This is a very complex case requiring multiple specialist input But pancytopaenia is something you may come across and cytopaenias are a relatively common problem which we have to deal with as juniors on the ward Be familiar with transfusion thresholds and guidelines Blood Components App Microguide If really stuck and have done all the first line investigations, discuss with your friendly haematologist!