Haem Misc Flashcards
Why might there be a high MCV in a sickle cell crisis
Reticulocytosis
Preventative medication in sickle cell anaemia?
Hydroxycarbamide - increases HbF level therefore decreasing percentage of HbS
Start if 2 or more sickle cell crises in a year
What are smear cells? What are they seen in?
Lymphocytes in which the membrane has burst in preparation of the film
Seen in CLL
What electrolyte abnormality can occur with transfusion of packed RBC’s?
Hyperkalaemia
More assoc w high volumes and old blood (cell lysis releases potassium)
What blood pattern is usually seen in alcoholics?
Macrocytic anaemia and thrombocytopaenia
Management of neutropaenic sepsis?
Piperacillin + Tazobactam (Tazocin)
Add Vancomycin if the patient has a central line
Add Gentamicin if severe sepsis (NEWS > 6)
If severe sepsis
When is a patient treated for neutropaenic sepsis?
When does it most commonly occur?
Temperature >38C, Neutrophils <0/5
OR
Neutrophils <1 and chemo in last 21 days
Most commonly 7-14 days after chemotherapy
Prophylaxis for neutropaenic sepsis if it is anticipated with treatment?
Consider giving fluoroquinolone e.g. Ciprofloxacin
What to give in neutropaenic sepsis if penicillin allergy?
Teicoplanin + Aztreonam
Likely cause of isolated thrombocytopaenia?
ITP
What happens to total protein level in multiple myeloma?
Increased
Is the neutropaenic sepsis treatment different in different hospitals?
No, same across all health boards
Why do you get prolonged aPTT in von willebrand disease?
vWF stabilises factor 8
What to do if DVT likely (Wells score >2)?
Arrange proximal leg doppler USS within 4 hours
If one CANNOT be arranged within 4 hours, then test D-dimer and give prophylactic LMWH to cover until USS
What to do if DVT is unlikely (Wells score 1 point or less)?
Do D-dimer
If raised then arrange doppler USS within 4 hours
If it CANNOT be done within 4 hours, give LWMW
Management of confirmed DVT?
Give LMWH or Fondaparinux
Start on Warfarin and continue LMWH for 5 days or until INR >2
Continue Warfarin for 3 months if provoked DVT, or 6 months if unprovoked/cancer
Does tamoxifen therapy increase VTE risk?
Yes
What is the main complication of CLL?
Recurrent infection due to Hypogammaglobulinaemia
Which haem malignancy is thrombosis more common in?
CML
What can CLL transform into?
High grade non-Hodgkin’s lymphoma (Richter’s transformation)
Rarely also AML
What type of haemolysis is CLL associated with?
Warm haemolysis
What is Waldenstrom’s Macroglobulinaemia caused by?
Lymphoplasmacytic Lymphoma
It is an IgM paraprotein disease (Myeloma very rarely causes IgM paraprotein)
How can Waldenstrom’s macroglobulinaemia present?
Hyperviscosity (e.g. stroke) Paraproteinaemia Lymphadenopathy, Splenomegaly Normal renal function No lytic bone lesions on skeletal survey
How do Rivaroxaban and Apixaban work?
Direct Xa inhibitor
How does Dabigatran work?
Direct thrombin (IIa) inhibitor
How long after transfusion does Graft vs Host disease appear?
What is it associated with?
What does it present with?
Management?
4-30 days (subacute)
Non-irradiated blood being given to immunosuppressed patients
Presents with fever, erythroderma, desquamation, diarrhoea, abnormal LFTs
Rx: steroids
What cells mediate transfusion-related acute lung injury?
Management?
Neutrophils (occurs when patient has antibodies to donor leucocytes)
Normally respond to fluid and oxygen therapy
Treatment of what haem malignancy often results in tumour lysis syndrome?
What causes the syndrome?
How does it present?
Burkitt’s lymphoma
Tumour cell lysis releasing large quantities of intracellular substances such as K, uric acid and phosphate
Feeling unwell, confusion and leg cramps about 24 hours after starting chemo
Blood test results for tumour lysis syndrome?
High potassium and phosphate
Low calcium
Treatment of tumour lysis syndrome?
IV/PO Allopurinol
When to suspect aplastic crisis in sickle cell?
Usual cause?
Fall in Hb with no reticulocytosis
Usually Parvovirus infection
Temperature increase with non-haemolytic febrile transfusion reaction?
<1.5C
Patient must also be feeling well and all other obs normal
Pathogenesis of myelodysplasia?
A line of myeloid blasts are produced rapidly, so do not mature and usually die, causing a deficiency of those cells
What may myelodysplasia transform into?
How does this present?
AML - usually once the bone marrow becomes >20% myeloid blasts - occurs in 1/3 of pts
Hx of myelodysplasia - become tired, SOB, feverish etc
46 y/o man has 4 month Hx of fatigue and weight loss with splenomegaly and conjunctival pallor. Blood results:
Hb LOW PLT HIGH WBC normal NEU HIGH LYM LOW EOS normal
What do you suspect?
CML
Pathophysiology if Factor V Leiden?
Activated factor V is inactivated 10x more slowly by protein C
What are Pappenheimer bodies?
What are they found in?
Basophilic iron granules found in RBC’s
Hyposplenism, post-splenectomy
What abnormal RBC’s are expected in hyposplenism?
Target cells
Howell-Jolly bodies
Pappenheimer bodies
Acanthocytes (spiky membrane)
Which NOAC can be used in AF to prevent thrombosis?
Dabigatran (IIa inhibitor)
5 things that should be done in acute allergic transfusion reaction?
Stop transfusion permanently IM adrenaline IV antihistamine IV corticosteroid Bronchodilators
Patient with hereditary spherocytosis, otherwise well apart from upper abdominal pain, what is most likely cause?
Biliary colic from pigment gall stone
In a transfusion, what is delayed haemolytic reaction caused by?
How long does it take to present?
Antibodies to the Rh antigen
Usually 5-10 days
Tests to confirm acute haemolytic reaction to transfusion?
Direct Coomb’s test
Also unconjugated bilirubin, haptoglobin, serum and urine haemoglobin
Investigation for Males with iron deficiency anaemia and Hb<110?
Urgent referral for Upper + Lower GI gastroscopy within 2 weeks
What anticoagulant should be used in patients with AF who have CKD?
Why?
Apixaban
Due to mainly faecal drug excretion, minimal is renally excreted
Does DVT/PE after infection (e.g. pneumonia) warrant 3 or 6 months Warfarin therapy?
Why?
3 months
It is provoked - clotting factors are acute phase proteins so increased level with infection
Which haematological malignancy is most likely to cause ASYMMETRICAL spreading lymphadenopathy?
Hodgkin’s lymphoma
What do Ree-Steenberg cells look like on histology?
Large multinucleate cells with prominent eosinophilic nucleoli
Transmission of which type of infection is more likely with platelet transfusion?
Why?
Bacterial
Platelets are usually stored at room temperature so are favourable for bacterial growth
What gene translocation is associated with Burkitt’s lymphoma?
c-myc translocation
t(8;14)
Young boy presents with jaundice and SOB a week after being treated for UTI, cause?
G6PD deficiency
Nitrofurantoin can cause oxidative stress
What is seen on blood film with G6PD deficiency?
Heinz bodies RBC fragments (schistocytes)
How should Tranexamic acid be administered?
1g bolus then 1g slow-infusion over 8 hours
If granulocytes at different stages of maturation (e.g. bands) are seen on blood count, what does this suggest?
CML
What are mirror image nuclei?
Another way of describing Reed-Steenberg cells - Hodgkin’s Lymphoma
Should compression stockings be offered after proximal DVT?
No
Woman with SLE presents with jaundice and tiredness. Blood count reveals Hb low, LDH high, bilirubin high. Film reveals spherocytes. Direct Coomb’s test +ve. What is the cause?
Autoimmune haemolytic anaemia
SLE can cause this
LDH suggests lysis.
What is post-thrombotic syndrome? (5 features)
Management?
After DVT, venous outflow obstruction/venous insufficiency ensues, causing:
- Painful, heavy calves
- Pruritis
- Swelling
- Varicose veins
- Venous ulceration
Rx: graduated compression stockings
Most common lymphoma in the UK?
Diffuse B-cell lymphoma (non-Hodgkin’s)
A man has itching and stinging on his trunk soon after starting RBC transfusion. You observe red, raised welts over abdo and chest. All vitals are normal and no angioedema. What is going on? Management?
Urticarial reaction
Temporary cessation of transfusion
Administer antihistamine e.g. chlorphenamine
What should you investigate if a 48y/o male has DVT with no obvious precipitating factor, no RF and no FHx?
Underlying malignancy
Antiphospholipid antibodies
What is the reversal agent of Dabigatran?
Idracuzimab (humanised monoclonal antibodies against dabigatran)
What is the reversal agent of Heparin?
Protamine
A 62 y/o man presents with headaches. On exam he is found to have widespread bilateral wheeze, facial plethora, HR 90, BP 110/60, SpO2 88%. He has a 60 pack-year smoking Hx. Currently on Amlodipine and bendroflumethiazide for hypertension. Haematocrit 0.58 (0.41-0.50) and RBC 8.8 (4-6). What is the cause of his polycythaemia?
COPD
Low SpO2 causes EPO release from kidneys, therefore, stimulating RBC production from Hypoxic Drive
How long are packed red cells transfused over?
In non-acute - 2 hours (3 hours if CCF)
In acute setting - STAT
What is Hand-Foot Syndrome?
Common presentation of sickle cell crisis in kids
Painful, tender, erythematous swelling of the hands and feet bilaterally. Lasts 1-2 weeks.
Not usually seen above 5y/o because by this age haematopoiesis in the small bones of hands and feet has ceased
Rx: supportive
What is high HbA2 diagnostic of in a 23y/o female with lethargy?
Beta-Thalassaemia Trait
In multiple myeloma, what are the blood results for:
- Calcium
- Phosphate
- Alk phos
Ca - high
Phos - normal
Alk phos - normal
A 28y/o is in Gynaecology for a D+C following incomplete miscarriage. She previously had pulmonary embolic events. After the procedure she is persistently bleeding. Her aPTT is 52 seconds. What is going on?
She has antiphospholipid syndrome
Hx of clots + bleeding and high aPTT
Check blood for lupus anticoagulant
What is the purpose of irradiated blood?
Depleted T lymphocytes - reduce chance of graft vs host disease
Most commonly used if compromised immune system e.g. intra-uterine transfusion, bone marrow transplant, immunosuppression e.g. chemo
Staging of Hodgkin’s lymphoma?
I - one lymph node region/structure (e.g. spleen) affected
II - 2 or more regions affected on the same side of the diaphragm (i.e. contained above/below thoracic diaphragm)
III - Lymph node regions on both sides of the diaphragm
IV - Involvement of extra-nodal sites
What is the Hb limit for transfusing red cells in patients with acute coronary syndrome?
80g/L
As anaemia can worsen hypoxic damage to the cardiac tissue