Anaemias Flashcards
What is Aplastic Anaemia?
A pancytopenia due to destruction of the HSC
Name three causes of Aplastic Anaemia
Genetic - Fanconi Anaemia
Radiation
Infection - HIV/EBV
Name two other features of Fanconi Anaemia
Predisposition to malignancy
Short Stature
Name two investigations for Aplastic Anaemia
Bloods (inc EPO)
BM Biopsy (dry tap)
How is Aplastic Anaemia managed?
<50 - SCT
>50 - Immunosupression +/- GCSF +/- Transfusions
What characterises autoimmune haemolytic anaemia?
Anaemia
Increased Reticulocytes
Reduced Haptoglobin
+ve Coombs
Sphero and Reticulocytes
What is Warm AHA?
IgG causes haemolysis at extravascular sites at body temperature
Name two associations of Warm AHA
CLL
SLE
What is Cold AHA?
IgM causes haemolysis at 4 degrees intravascularly generally causing raynaud symptoms alongside typical haemolytic anaemia picture
How is AHA managed?
Steroids +/- Rituximab
What is Beta Thalassaemia major?
Comlete absence of beta globulin chains presenting in first year of life with failure to thrive
How is Beta Thalassaemia physiologically compensated?
Increase in HBA2 and HBF
How is Beta Thalassaemia Major managed?
Repeated Transfusion
Desferrioxamine
How does Beta Thalassaemia trait present?
Typically asymptomatic as only reduced production
Name three hereditary haemolytic anaemias
Sickle Cell
G6PDH
Hereditary Spherocytosis
Name two immune haemolytic anaemias
AHA
Transfusion related
Name two non immune haemolytic anaemias
DIC
TTP/HUS
What is Hereditary Spherocytosis?
Most common hereditary HA
Autosomal abnormality in cytoskeleton - then destroyed by spleen
Name three features of H.Spherocytosis
Failure to thrive
Jaundice
Splenomegaly
How is H.Spherocytosis diagnosed?
Family History +Suggestive bloods + Symptoms
Describe the long and short term managements of H.Spherocytosis
Acute - Supportive +/- Transfusion
Long term = folate and splenectomy
What age group has the highest incidence of iron deficiency anaemia?
PreSchool
What is characteristic of iron deficiency anaemia on blood film?
Anisopoikilocytes
Name four causes of macrocytic anaemia
Megaloblastic - B12 deficiency, Folate Deficiency
Non Megaloblastic - Alcohol, Liver Disease
What is Methaemaglobinaemia?
Haemoglobin becomes oxidised so cannot carry oxygen
Associated - congenital, nitrates, dapsone
How does Methaemaglobinaemia present?
CHocolate blood
Dyspnoea
Headaches
How is Methaemaglobinaemia investigated?
Normal pO2 and reduced sats
How is Methaemaglobinaemia managed?
Ascorbic Acid +/- Methylene Blue
What are Myelodysplastic Syndromes?
Essentially Carcinoma in Situs
Can be Primary or Secondary (radiation or chemo)
How do Myelodysplastic Syndromes present?
Reduction in various blood counts
Can either remain stable or progress to AML
What is Myelofibrosis?
Overgrowth of megakaryocytes causing release of platelet growth factors and fibrosis
How does myelofibrosis present?
Anaemia
Extramedullary Hameatopoiesis
Hypermetabolic sx (weight loss, night sweats)
Name two characteristic investigations for myelofibrosis
Tear drop poikilocytes on blood film
Dry Tap from BM
Give three possible managements for Myelofibrosis
JAK2 inhib
Thalidomide
Periodic Infusions
What levels define Polycythamia
Red cells >35ml in males and 32ml in women
Name two causes of relative polycythaemia
Dehydration
Stress
Name two causes of secondary polycythaemia
COPD
ALtitude
What is Polycythaemia Vera?
95% due to JAK2 mutation causing clonal proliferation
Name three presenting features of PV
Itchiness (worse afterbath)
Plethora
Splenomegaly
How is PV managed?
Hydroxyurea+ Aspirin +Therapeutic Venesection
Describe the different phenotypes of Hb in Sickle Cell
HbAA
HBAS - Trait
HBSS - Sick Cell
Why does sickle cell not develop until 4-6m?
As that is when foetal hB reduces
How is Sickle Cell Diagnosed?
Hb Electrophoresis
What is the long term management of sickle cell?
Hydroxyurea - increases HbF and reduces crises
Pneumococcal Vax
Name the five types of sickle cell crises
THrombotic
Acute Chest
Aplastic
Sequestration
Haemolytic
In general - how are sickle cell crises managed?
Supportive
Oxygen
Exchange Transfusions
Opiates
What is Sideroblastic Anaemia?
Red cells fail to form haem properly
Can be congenital or acquired (lead, alcohol)
What is characteristic of sideroblastic anaemia?
basophillic stippling
How is sideroblastic anaemia managed?
Supportviely
How is Vit B12 deficiency managed?
Ig IM hydroxycoalbumin 3 times a week for 2 weeks, then once every 3m
Treat B12 deficiency before folate (SCDC)