Haem Ix Mx Flashcards
Antiphospholipid syndrome (blood clots -> miscarriages)
Ix = anticardiolipin ab’s (+ anti-beta2GPI)
lupus anticoagulant
thrombocytopenia
prolonged APTT
Mx:
Primary thromboprophylaxis = low-dose aspirin
Secondary thromboprophylaxis
Initial VTE/arterial thrombosis = lifelong warfarin with a target INR of 2-3
Pregnancy = aspirin + LMWH
Recurrent VTE = lifelong warfarin; if occurred whilst taking warfarin then + low-dose aspirin, increase target INR to 3-4
Disseminated intravascular coagulation (DIC)
Ix: bloods and clotting time
↓ platelets
↓ fibrinogen
↑ PT & APTT (because platelets are used up)
↑ fibrinogen degradation products
schistocytes due to microangiopathic haemolytic anaemia
Haemolytic Uraemic Syndrome (E.coli damages vessel wall -> microangiopathic hemolytic anaemia)
Ix: Triad of MAHA, thrombocytopenia and renal failure
FBC - MAHA (Hb<8 g/dL) + thrombocytopenia + negative Coomb’s test
U&E - acute kidney injury
Blood film: schistocytes and helmet cells
PCR for E.coli Shiga toxins
Mx:
treatment is supportive e.g. Fluids, blood transfusion and dialysis if required
No role for antibiotics
Haemophilia
Ix = Blood tests
prolonged APTT (deficient in f.8 in intrinsic pathway) haemophili-eight
bleeding time, thrombin time, prothrombin time normal
Immune thrombocytopenic purpura (ITP) (ab’s against GP IIb/IIIa)
Ix: FBC = an isolated thrombocytopenia
antiplatelet autoantibodies (usually IgG)
bone marrow aspiration shows megakaryocytes in the marrow. This should be carried out prior to the commencement of steroids in order to rule out leukaemia
Mx = usually, no treatment is required, it resolves in around 80% of children with 6 months, with or without treatment
1st = oral pred (80% of patients respond)
splenectomy if platelets < 30 after 3 months of steroid therapy
IV immunoglobulins
immunosuppressive drugs e.g. cyclophosphamide
avoid trauma sports
If platlets are v low (e.g. < 10 * 109/L) then steroids, ig’s and platelet transfusions
Multiple myeloma (Calcium, Renal issues, Anaemia, Bone pain)
NSAIDs can precipitate renal failure in patients with multiple myeloma
Ix:
FBC = anaemia (+ commonly leucopenia)
peripheral blood film = rouleaux formation
urea and electrolytes = renal failure
bone profile = hypercalcaemia
Protein electrophoresis = monoclonal IgA/IgG proteins in the serum. In the urine, they are known as Bence Jones proteins
Bone marrow aspiration
confirms the diagnosis if the number of plasma cells is significantly raised (30%)
Imaging
Whole body MRI
skull X-Ray = ‘rain-drop skull’ (likened to the pattern rain forms after hitting a surface and splashing, where it leaves a random pattern of dark spots). Note that a very similar, but subtly different finding is found in primary hyperparathyroidism - ‘pepperpot skull’
Mx:
Thalidomide*/Bort + dexamethasone
+ bisphos for bones
IV saline to treat the acute effects of hypercalcaemia and stabilise the pt!
*Crabs have thalidomide hands
Myelofibrosis
differentiator between myelofibrosis and aplastic anaemia?
Ix: hypermetabolic symptoms - weight loss, night sweats
Lab;
- ‘tear-drop’ poikilocytes on blood film
- Dry tap (trephine biopsy needed)
- High urate and LDH (reflect increased cell turnover)
Also, anaemia and a high WBC and platelet count early in the disease
Mx = Hydroxycarbamide
Differentiator = Myelofibrosis has MASSIVE SPLENOMEGALY!
Polycythaemia
Ix = JAK2 mutations
Mx = aspirin, venesection, chemo
chemotherapy (hydroxyurea/phosphorus-32)
Sickle cell disease
Admit all people with clinical features of a sickle cell crisis to hospital unless they are:
A well adult who only has mild or moderate pain and has a temperature of 38°C or less.
A well child who only has mild or moderate pain and does not have an increased temperature.
Ix = haemoglobin electrophoresis + Blood film*
Mx:
Crisis management = Analgesia,
+ fluids + oxygen. ab’s if theres an infection
Longer-term management = hydroxyurea + Pneumococcal vaccine every 5 years
Hydroxyurea (aka hydroxycarbamide)- increases the HbF levels and is used in the prophylactic management of sickle cell anaemia to prevent painful episodes
*Sodium metabisulphite can be added to blood smears to mimic
accelerated deoxygenation. RBCs with high haemoglobin S concentrations
undergo sickling in a reduced oxygen environment. Further investigation
would be needed to differentiate homozygous and heterozygous sickle cell disease.
Thalassaemia
Ix = Hb electrophoresis
Mx = Repeat transfusion + iron chelation (desferoxamine to prevent iron overload)
Vitamin B12 & Folate deficiency
Ix:
Sore tongue and mouth
Neurological symptoms
Dorsal column is usually affected first (joint position, vibration) prior to distal paraesthesia
neuropsychiatric symptoms: e.g. mood disturbances
Mx:
No neuro involvement = 1 mg of IM hydroxocobalamin (B12)
(3 times each week for 2 weeks, then once every 3 months)
If pt is also deficient in folic acid => It is important to treat the B12 deficiency 1st! to avoid precipitating subacute combined degeneration of the cord
Then can give oral folic acid to replenish stores
Pt > 60 who present with IDA should be investigated for colorectal cancer
Von Willebrand’s disease
bleeding test abnormality?
Von Willebrand factor is important in platelet adhesion and Factor VIII
Patient’s therefore have normal PT and platelet counts but bleeding time
and PTT are abnormal!
CML
Ix: Philidelphia chromosone for (t.9:22), PCR for BCR-ABL gene, BM shows lots of immature myelocytes
To differentiate from AML;
granulocytes are different stages of maturation shown by immature band forms and mature neutrophils. Also the platelet count may also be raised
Mx:
1st = Imatinib (inhibitor of the tyrosine kinase associated with the BCR-ABL defect)
2nd = hydroxyurea, INF-alpha
3rd = BM transplant
CLL
Ix: FBC (lymphocytosis)
Anaemia* + thrombocytopenia:
blood film: smudge/smear clls
Gold standard = immunophenotyping ab’s for CD5, CD19, CD20 and CD23
*Associated with warm haemolytic anaemia
Epistaxis
Mx: to be edited
If the patient is haemodynamically stable, bleeding can be controlled with first aid measures. This involves:
Asking the patient to sit with their torso forward and their mouth open and avoid lying down unless they feel faint
This decreases blood flow to the nasopharynx and allows the patient to spit out any blood in their mouth
it also reduces the risk of aspirating blood
Pinch the cartilaginous (soft) area of the nose firmly
this should be done for at least 20 minutes
also ask the patient to breathe through their mouth.
If first aid measures are successful
consider using a topical antiseptic such as Naseptin (chlorhexidine and neomycin) to reduce crusting and the risk of vestibulitis
cautions to this include patients that have peanut, soy or neomycin allergies
Mupirocin is a viable alternative
admission and follow up care may be considered in patients under if a comorbidity (e.g. coronary artery disease, or severe hypertension) is present, an underlying cause is suspected
they are aged under 2 years (as underlying causes such as haemophilia or leukaemia are more likely in this age group)
self-care advice involves reducing the risk of re-bleeding
patients should be informed that blowing or picking the nose, heavy lifting, exercise, lying flat, drinking alcohol or hot drinks should be avoided
If bleeding does not stop after 10-15 minutes of continuous pressure on the nose consider cautery or packing
cautery should be used initially if the source of the bleed is visible and cautery is tolerated
it is not so well-tolerated in younger children!
ask the patient to blow their nose in order to remove any clots. Be wary that bleeding may resume.
use a topical local anaesthetic spray (e.g. Co-phenylcaine) and wait 3-4 minutes for it to take effect
identify the bleeding point and apply the silver nitrate stick for 3-10 seconds until it becomes grey-white. Avoid touching areas which do not require treatment, and only cauterise one side of the septum as there is a risk of perforation.
dab the area clean with a cotton bud and apply Naseptin or Muciprocin
packing may be used if cautery is not viable or the bleeding point cannot be visualised
anaesthetise with topical local anaesthetic spray (e.g. Co-phenylcaine) and wait for 3-4 minutes
pack the patient’s nose while they are sitting with their head forward, following the manufacturer’s instructions
pressure on the cartilage around the nostril can cause cosmetic changes and this should be reviewed after inserting the pack.
examine the patient’s mouth and throat for any continuing bleeding, and consider packing the other nostril as this increases pressure on the septum and offending vessel.
patients should be admitted to hospital for observation and review, and to ENT if available
Patients that are haemodynamically unstable or compromised = admit to the emergency department and control bleeding with first aid measures in the interim
patients with a bleed from an unknown or posterior source (i.e. the bleeding site cannot be located on speculum, bleeding from both nostrils or profuse) should be admitted to hospital.
Epistaxis that has failed all emergency management
may require sphenopalatine ligation in theatre
What is factor v leiden
Factor V Leiden
is a mutation of factor V which makes it resistant to the action of protein
C (it gets degraded 10x more slowly!)
The increased presence of factor V therefore creates a procoagulant
state predisposing to pathology such as DVTs.
Factor V Leiden is one of
the most common causes of an inherited procoagulant state!! Present in approximately 5 per cent of the Caucasian population
Differentiator between folic acid deficiency and b12 deficiency?
Similar sx but neurological symptoms are not as prominent and patients tend
to have a history of malnutrition in folic acid deficiency
Folate deficiency is commonly associated with alcohol disease as it is required to metabolize alcohol, poor diet and skin disease
Hypersegmented
neutrophils can be associated with B12 or folic acid deficiency
How to tell the difference between TRALI, TACO, non-febrile haemolytic reaction, ABO incompatibility
Febrile non-haemolytic transfusion reaction occurs due to white cell antibodies reacting with the leukocytes present in the blood transfusion
Patients usually have a history of blood transfusions or pregnancy. Patients usually present with fever, rigors and discomfort.
TACO = oedema/fluid overload + HF (raised JVP and preexisiting htn), S3 present, afebrile
TRALI = Hypotension + pyrexia + no change in JVP. mx = oxygen, fluids, escalate!
The hypertensive TACO and the burnign hot TRAIL!
ABO incompatibility is within minutes/hours
How do you differentiate between ALL and AML?
Sudan Black stain
Whats the EPO and MCV for Polycythaemia rubra vera?
Since polycythaemia rubra vera is a point mutation abnormality, the bone
marrow produces excess myeloid lineage cells.
This feeds back negatively
upon erythropoietin production from the renal cells, such that a raised red
cell mass but low erythropoietin level
Waldenstroms
Seen in older men. It is a lymphoplasmacytoid malignancy characterised by the secretion of a monoclonal IgM paraprotein
Features
systemic upset: weight loss, lethargy
hyperviscosity syndrome e.g. visual disturbance
the pentameric configuration of IgM increases serum viscosity
hepatosplenomegaly
lymphadenopathy
cryoglobulinaemia e.g. Raynaud’s
Investigations
monoclonal IgM paraproteinaemia
bone marrow biopsy is diagnostic
infiltration of the bone marrow with lymphoplasmacytoid lymphoma cells
Mx = rituximab-based combination chemotherapy
3 drugs CI in G6PD deficiency?
Some drugs causing haemolysis
anti-malarials: primaquine
ciprofloxacin
sulph- group drugs: sulphonamides, sulphasalazine, sulfonylureas (Glipizide)
Criteria for platelet transfusion? what platelt count qualifies someone to get a platelet transfusion
Offer transfusion if pt is Active bleeding and pt count is = <30 x 10 9
No active bleeding = threshold of 10 x 109
Pre-surgery;
Aim for plt levels of:
> 50×109/L = most patients
50-75×109/L = pt has a high risk of bleeding
> 100×109/L = if the surgery is at a critical site
Do not offer platelets where platelet transfusion is contraindicated or there are alternative treatments for their condition
For example, do not perform platelet transfusion for any of the following conditions:
Chronic bone marrow failure
Autoimmune thrombocytopenia
Heparin-induced thrombocytopenia, or
Thrombotic thrombocytopenic purpura.
Mx for acute haemolytic transfusion reaction
Acute haemolytic transfusion reaction should be treated with generous fluid resuscitation and termination of the transfusion.
What kind of blood is used in BM transplants and why?
Irradiated blood products are used as they are depleted in T-lymphocytes and therefore reduce the chance of graft vs host disease
