Haematology 1 Flashcards
most common inherited bleeding disorder?
- lab:
1. Bleeding time?
2 Platelet count?
3 APTT? - what might be reduced?
Management:
1. mild bleed =
2. **
Von Willebrand’s disease
Investigation
1. prolonged bleeding time
2. APTT may be prolonged
3. Normal platelets
4. factor VIII levels may be moderately reduced
defective platelet aggregation with ristocetin
Management
1. tranexamic acid for mild bleeding
2. desmopressin
3. factor VIII concentrate
Neutropenic Sepsis
Should be suspected in a person with: (4)
1. cause ie…
2
3
4
Pathogen?
Prophylaxis- whats given if neutrophil count < 0.5 x 10^9
- known cause for neutropenia (recent cancer treatment)
- presumed or confirmed infection
- temperature >38ºC
- respiratory rate >20 breaths per minute
coagulase-negative, Gram-positive bacteria are the most common cause, particularly Staphylococcus epidermidis
Prophylaxis- fluoroquinolone
Management of neutropenic sepsis/ suspected
- If febrile after 48 hours…
- No respeonse after 4-6 days…
- Abx IV **Pip-taz ( aka tazocin) immediately
+/- Vancomycin is central venous access - If febrile after 48 hours… alternative abx such as vancomycin or meropenim
- No respeonse after 4-6 days… investigate for fungal infection
Platelet transfusion for thrombocytopaenia
BLEEDING RULES
1. mod bleed
2. severe bleed or…
PRE-INVASIVE RULES
1.
2.
3.
NO BLEEDING OR PLANNED INVASION RULES
BLEEDING
1. offer if platelet < 30 x 10^9 and bleeding grade 2- e.g. haematemesis, melaena, prolonged epistaxis
- Severe bleeding- platelets < 100 x10^9 or bleeding at critcal site such as CNS
PRE-INVASIVE RULES
1. > 50×109/L for most patients
2. 50-75×109/L if high risk of bleeding
3. >100×109/L if surgery at critical site (CNS)
NO BLEEDING OR PLANNED INVASION RULES
threshold of 10 x 10^9 except where platelet transfusion is contradindicated or there are alternative treatments for their condition
CI of platelet transfusion (4)
- Chronic bone marrow failure
- Autoimmune thrombocytopenia
- Heparin-induced thrombocytopenia
- Thrombotic thrombocytopenic purpura.
If you see on the blood film- Increase in granulocytes at different stages of maturation +/- thrombocytosis
CML
Painless, ASYMMETRICAL node swelling in the neck → ?
Hodgkin’s lymphoma
Iron deficiency anaemia 2ww rule (1)
men of any age with a Hb below 110g/L should be referred for upper and lower GI endoscopy as a 2ww.
Iron def anaemia give me 4/5 reasons why it might happen
Features rogue
mail changes-
Serum ferritin high or low?
TIBC low or high?
blood film you see (2)
- Most common pre-school age due to increase reuirement
- Heavy bleeding- menorrhagia
- GI blled- malig
- Poor diet iron
- Malabsorption
rogue
1. koilonychia (spoon-shaped nails)
2. Atrophic glossitis
3. Post-cricoid webs
4. Angular stomatitis
Serum ferritin- low
TIBC- high
Blood film
- target cells, ‘pencil’ poikilocytes
DVT investigation: if the scan is negative, but the D-dimer is positive …. (2)
- Stop anticoag
- Repeat USS in 1 week
- Look at lymphocytes
- Look at WBC
- Others hint (blast cell/ bands)
Example
1.Low Lymphocytes -> the answer should be AML or CML
2. WBC > 100 -> Chronic causes so Consider CML (rule out AML)
3. Presence of bands cell -> confirm CML
*blast -> Acute
*bands -> Chronic
Just recveived a transplant of somesort
- painful maculopapular rash is a common feature of
graft versus host disease
After PPH and resus complete- what blood products should be given first?
max you can give?
FFP
- request ‘Clotting studies and a platelet count ugently’ and give FFP while you wait for them.
up to 4 units of FFP and 10 units of cryoprecipitate
What is the definition of aplastic anaemia (3)
Drug case of aplastic anaemia?
normocytic anaemia, leukopenia and thrombocytopenia
Phenytoin
Disproportionate microcytic anaemia - think beta-thalassaemia trait- raised HbA2 points to a diagnosis of beta-thalassaemia trait
A normocytic anaemia with low serum iron, low TIBC but raised ferritin in a patient with a chronic illness is typical of
anaemia of chronic disease
Ileocaecal resection may result in what type of anaemia and why?
GLOSSITIS IS ALSO A PATHONOMIC FEATURE
Vitamin B12- as it is absorbed in the terminal ileum, therefore resection decreases
GLOSSITIS- tongue also appears beefy-red and is sore
porphyria what is it?
Mx:
1. Conservative=
Acute attack:
1
2. if not available =
are autosomal dominant condition causes abnormal synthesis of haem- therfore piss out Hb
Mx:
1. Conservative= avoiding triggers
Acute attack:
1 IV haematin/haem arginate
2 if not available = IV glucose
If giving blood products to someone with Hodgkins lymphoma what do you give them?
Irradiation: to prevent transfusion-associated graft versus host disease (taGVHD), for the rest of their life
Irradiated blood destroys all nucleated cells (such as leucocytes), therefore, eliminating the risk of taGVHD.
ITP in adults- usually a more chronic condition in adults compared to kids which is more acute following virus
*what do you see on bloods?
management:
1st line-
2nd- (also if active bleeding as it acts quicker) =
splenectomy is less commonly used
- isolated thrombocytopenia
management:
1st line- Oral prednisolone
2nd- (also if active bleeding as it acts quicker) = IV IG pooled normal human immunglobs
splenectomy is less commonly used
Management of autoimmune haemolytic anaemia (CLL!!)
Corticosteroids
(More common)Haemophilia A = lack of..
Haemophilia B (Christmas disease) = lack of..
Haemophilia A = lack of.. VIII
Haemophilia B = lack of… IX
Howell-Jolly bodies and target cells think…
- Sickle cell ( hyposplenism)
- Coeliac Disease
What canEBV cause on bloods?
neutropaenia
STEM- Recurrent tonsillitis in a young person should raise suspicions
Factor V Leiden mutation results in (pathophysiology?)
activated protein C resistance
Mirror image nuclei =
Reed-Sternberg cells
When should you do G6PD enzyme assays? (2)
At time of presentation AND repeated around 3 months after acute haemolytic episodes to avoid false negatives
Methotrexate therapy may result in what kind of anaemia and why?
Folate deficiency!!
Macrocytic megaloblastic anaemia s
how does Factor V Leiden increase VTE?
activated factor V (which causes clotting) is INactivated 10 times more SLOWLY by activated protein C than normal
Therefore Factor V can clot for longer thus increasing VTE risk
ADMIT EVERYONE WITH SICKLE CELL CRISIS UNLESS: (2)
- 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.
Both Hereditery Spherocytosis and G6PD def get INCREASE RISK OF…
GALLSTONES!!
