Haematology Flashcards
What is Immune thrombocytopenia?
This is either an acute or chronic condition.
- Acute - follows an URTI
- IgG antibodies against GPIIb/IIIa
Symptoms:
- epitaxis
- menorrhagia
- petechiae bruising
- *normal spleen size
Labs:
- low platelets
- antibody screen positive
- Megakaryocytes seen on bone marrow
Treatment:
- prednisolone
- Immunoglobulin therapy
- Rituximab
Emergency:
- platelet transfusion
- Splenoectomy
What is:
Thrombotic Thrombocytopenic Purpura?
- TTP
A type of microangiopathic haemolytic anaemia.
- acquired or congenital (ADAMTIS13 deficiency, allowing vWBF to promote platelet aggregation)
*causes multisystem thrombotic occulsions due to activation of the platelets
Most common in females following an URTI
Symtpoms:
Pentad:
- Anaemia
- Fever
- thrombocytopenia
- renal failure
- CNS involement
Labs:
- reduced platelets
- LDH increased
- Haptoglobin decreased
- Clotting studies - normal
- Blood smear - schiztosites
**test for shigella toxins as this may be haemolytic uraemic syndrome caused by E.Coli 0157
Treatment:
Haematological emergancy:
- plasma infusion
- plasma exhange
- steroids
- rituximab
Beta thalassamias:
Point mutations on chromosome 11. defects in beta globulin chain.
Beta thalasemia trait:
carrier state with one chain missing.
- usually symptomatic
Beta thalasemia Mjor
both globulin chain defects.
- 1st year of life
- severe anaemia
- failure to thrive
- skull bossing (extra medullary haematopoesis)
- splenomegaly
Blood film:
- HbA2
HbF
*no HbA
Treatment:
promote healthy lifestyle
folate supplements
2-4 weekly tranfusions *Iron overload is a problem often causes endocrine issues (hypothyroidism, hypocalaemia)
- splenoectomy
What will 1 unit of packed RBCs equate to in blood volume?
1 unit = 350ml
1 unit = ~ 1g/L Hb in 70kg adult
How long a life span do platelets have in the bag?
5 days
What temperture must FFP be kept at?
how long does it take to thaw and when does it need to be used by?
-18Celius
20mins to thaw
2 hours
What drug can be used in sickle cell disease to help promote production of HbF?
Hydroxycarbamide
What drug can stimulate production of Von Williebrand in people who have Von Williebrand disease?
Desmopressin
What gene is associatted with Burkits lymphoma?
MYC gene with 8;14 translocation
List some causes of Haemolytic anaemias:
Acquired:
Autoimmune Haemolytic Anaemia:
- Warm IgG
- Cold IgM
Drug Induced
- RBCs autoantibodies
Microangio haemolytic anaemias:
- Haemolytic uraemic syndrome - E.Coli
- TTP - ADAMST13
Infection:
- Malaria
Paraoxysmal Notctual Haemoglobinuria:
Heridatiry:
Glucose - 6 - phosphate deficiency
Pyruvate deficiency
Heridatory spherocytosis
Sickle cell disease
Thalassaemias
What is Cryoprecipitate and when is it used?
Preperaed plasma contianign:
- fibrinogen
- von williebrand
- factor VIII
- Factor XIII
Medical emergancies for massive bleeding. Can be used in DIC
also used for haemophilae
What infective transmission may occur in blood transfusion and what steps have been put in place to reudce its spread?
vCJD
> 1999 donation have White cells removed
>1999 plasma sourced from abroad
>2004 recipitants of blood donation are excluded form giving blood
What anticoagulant can be used in pregnancy?
LMWH
If a male has explained Hb <110g/L what should be done?
Urgent endoscopy and colonoscopy
<2 weeks
Which DOAC can be used in the prevention of thromboembolism from AF?
Dabigatran
What are the risk factors for devleoping ALL?
Ionizing radiation
- especially during pregnancy
Down’s syndrome
Being young
- most common cancer in childhood
What is the classfication system used and how is the prognosis worked out in ALL?
Chromosomal and molecular analysis
Immuno-Phenotyping
What are some differentials for petechia bleeding?
*Leukaemia
*Vasculitis - Henock Scholien
*Microangio Haemylytic syndroes
- TTP
- E.Coli
*Menigitis
- N. menigitis
What investigations should be done in suspected leukaemia?
- FBC
- this should be done with 48 hours of suspected leukaemia.
*note young adults or children with ptechiae should be referred to hospital immediatley
- will usually see a high WCC (although they won’t be functioning WCs)
- Blood film
- looking at abnormal cells
- LDH
- this will show increased cellular turnover
- Bone Marrow Biopsy
- with phenotyping. this is definitive diagnosis
- Chest x-ray
- looking for mediastinal masses
- CT/ PET CT
- LP
- if suspected CNS involvement
What is the treatment of ALL?
Support:
- Blood transfusions
- IV fluids
Allopurinol - tumour lysis syndrome
**insert hickman line
Prophylatic antibotics and antifungals
Neutropenic regieme
- hygeine
- high calorie intake
- full barrier treatment
- daily checks
Chemotherpay + steroids
- remission induction
- consolidation
- Maintance
Marrow transplant
- young adults
What are some symptoms seen in AML that help to distinguish it?
Anaemia
Bruising
Infections
Symptoms that help differentiate:
- DIC
- Gum hypertrophy / bleeding
*these infilatrate
What are the symptoms of CML?
Mostly chronic insideous symptoms.
weight loss
tiredness
fever
night sweats
Splenic enlargemnt
- abdominal discomfort
**often the spleen is massive in CML
What is the natural history of CML?
- *Chronic phase**
- last years
- *Accelerated phase:**
- increase in symptoms
- increase in splenic size
- *Blast Crisis:**
- AML tranformation
- features of leukaemia. death is likely
What is the most common leukaemia over all? and how does it often present?
CLL
Often CLL is asymptomatic and is found out by incidental finding.
Patient may be prone to:
- infections
- bleeding
- large rubbery non tender lymph nodes.
What are some complications of CLL?
- Autoimmune haemolysis
- over production of B cells leads to production of antibodies against RBCs.
* - Warm IgG antibody anaemia* - Hypogammaglobulinaemia
- Marrow failure
- Richter’s transformation
What is the natural history of CLL?
1/3 never progress
- or even regress
1/3 progress slowly
1/3 Progress rapidly into aggressive lymphoma called:
- RIchter’s lymhoma
*majority die from infection or transformation
When is a blood transfusion indicated pre-operatively?
if a patient is unable to take oral Iron, then how should they be managed pre-operatively?
Hb: <8g/dL
IV Iron - ferric carboxymaltase 1g/ weekly
What is the most common type of Hodgkins lymphoma?
Nodular sclerosing
Any supcision of leukaemia in a young person should be managed how?
FBC within 48hours
What is the most important factor in deciding whether cyroprecipitate should be given?
Low fibrinogen level
What are the symptoms of lead poisoning?
Muscle weakness
Neuropychiatric symptoms
Anaemia
Blue gums
- mainly seen in children
Adbominal pain/ constipation
Outwith a high MVC, what other feature may pointi n the direction of megoblastic anaemia?
Hyper - segmented neutrophil polymorphs
Glucose 6 phosphate defiency is inherited by which means?
X linked
What can cause haemolysis Glucose - 6 - phosphate dehydrogenase deficiency?
Infection
Fava beans
Drugs - nitrofurantoin
Acidosis
What disease is associated with stary sky appearance on H&E stain
Burkits lymphoma
Why is parvovirus worrying in sicklecell patients?
It can cause aplastic crisis, which is a poor response of the bone marrow to respond to the drop in Hb
What is the blood tranfusion cut off for patients without ACS and those who present with ACS?
Non ACS - <7g/dL
ACS - <8g/dL
If a patient presents with a painful swollen leg and meets the criteria of Well Score >2, what should the next course of action be?
Carry out leg ultrasound scan within 4 hours.
if the scan is negative D Dimers should be done. If these are positive the LMWH should be started
What type of infection is most likely to transmitted via platelets?
Bacterial
- stored at room temperture making them more likely to devleop bacterial infections
What investigation should be done to estbalish polycaemthemia ruba vera?
JAK 2 mutation screen
