anaemia Flashcards
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What are features of anaemia?
Skin and mucosal pallor
Pale conjuctivae
fatigue
Increased resp rate and Heart rate
Krusty is a 54 year old Clown and fast food franchise owner. He reports 6 kg of weight loss over the last 3 months alongside ‘stomach issues’. On examination, he has angular cheilitis, koilonychia and appears pale.
A blood film shows hypochromic red blood cells with evident anisopoikilocytosis and multiple pencil cells.
What is the diagnosis?
a) Anaemia of Chronic Disease
b) Thalassaemia Major
c) Iron Deficiency Anaemia
d) Chronic Lymphocytic Leukaemia
e) Hyperthyroidism
Iron Deficiency Anaemia
What is type of anaemia that can be seen here?
What are the features?
What are the causes of it?
Iron deficiency anamia
poikilocytosis and anicytosis and pencil cell and microcytic and hypochromic cell
CAUSES:
Reduced uptake
- Malnutrition
- Coeliac
- IBD
- Achlorhydria
Increased loss
- GI Malignancy
- Peptic ulcer
- IBD
- Menstruation
Increased requirement
- Pregnancy
- Breastfeeding
Reduced prodcution:
- sideroblastic anaemia
what is the triad of Colon cancer?
Iron deficiency anaemia
PR bleeding
Weight loss
What is globally the most common cause of iron deficency anaemia?
ascariasis and schistosomiasis
Abe Simpson is 83 year old man with enlarged lymph nodes. He has lost 5kg in the last 2 months. He reports episodes of fevers and night sweats. On examination he has hepatosplenomegaly. After follow up from an urgent two week referral, he is diagnosed with Non-Hodgkin’s Lymphoma. Blood testing also identifies an anaemia related to his condition.
Which is increased in Anaemia of Chronic Disease?
a) Intrinsic factor
b) Hepcidin
c) TIBC
d) Transferrin
e) Ferroportin
Hepcidin
BECAUSE DUE TO ANAEMIA OF CHRONIC DISEASE
How does anaemia of chronic disease work
How do you differentiatate between anaemia of chronic disease and iron deficiency
Kent Brokodopolis is a news reporter from Greek Cyprus. His son is 6 months old and has stunted growth. On examination the boy is small, docile with pale conjunctivae. He has hepatosplenomegaly and skull abnormalities with ‘chipmunk’ facies.
Which investigation will confirm the likely diagnosis?
a) HLA typing
b) Haemoglobin Electrophoresis
c) Peripheral Blood Smear
d) Full Blood Count
e) Plain Skull X-ray
Haemoglobin Electrophoresis
Why do people with thalassemia and sickle cell get frontal bossing and skull abnormalities?
extramedullary haematopoiesis
ALSO GET hepatosplenomegaly
What are the different types of alpha defects?
What is the gene affected?
You have 4 genes-
Alpha+ lacking 1 gene
Alpha° lacking 2 genes
Hb H lacking 3 genes - significant anaemia
Hb Barts lacking all 4 - not compatible with life
What is the gene affected? - chr 16
What are the different types of beta thalassemia?
What chromosome is the defect on?
Beta minor
Beta intermedia
Beta major
Chr 11
What disease does thalassemia give resistance to
falciparum - malaria
How do you investigate thalassemia?
Investigations:
- Microcytic anaemia
- Normal Iron studies
Label the haemoglobin curve
Explain at what ages beta thalassemia presents
Typically presents between first 3 & 6 months
Carl Carlson is a 36 year old male with Sickle Cell Anaemia. He is admitted to Hospital while undergoing an acute painful crisis.
How should Carl be managed?
a) Fluids, Oxygen and Analgesia
b) Exchange Blood transfusion
c) Emergency Splenectomy
d) Urgent Cholecystectomy
Allogeneic stem cell transplant
a)Fluids, Oxygen and Analgesia
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What are the complication/ crisis (emergency) you can get in sickle cell and how do you treat them?
What are the symptoms of sickle cell?
What is the treatment of sickle cell
Conservative
- Trigger avoidance
Medical:
- Vaccinations
- Hydroxyurea
- Prophylactic ABx
Surgical:
- Bone Marrow Transplant (curative)
Someone with a low MCV without thalassemia or sickle cell thought to have iron deficiency anaemia was given iron but his anaemia is not getting any better.
What could he have
20% of older people have sideroblastic anaemia
condition is characterized by ineffective erythropoiesis, leading to increased iron absorption, iron loading in marrow ± haemosiderosis (endocrine, liver, and heart damage due to iron deposition)
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Marge is a 38 year old female who presented with a painless goitre, which she reports covering up for a number of years with her pearl necklace. She was recently diagnosed with Hashimoto’s thyroiditis. Her recent blood results also demonstrated some haematological abnormalities.
Which of the following patterns is most likely to be shown?
a) Low Hb, Low MCV, High RDW
b) Low Hb, High MCV, megaloblastic on film
c) Low Hb, High MCV, non-megaloblastic on film
d) Normal Hb, Normal MCV, Normal Haematocrit
e) High Hb, Low MCV, High Haematocrit
c)Low Hb, High MCV, non-megaloblastic on film
Lisa is a 12 year old girl presenting to her GP with tiredness and tingling sensations in her hands. Lisa has recently modified her diet, taking part in Veganuary this year and is following this through currently. Her mother has been diagnosed with Hashimoto’s.
On examination she appears pale. The corners of
her lips are notably sore.
Which is the most likely cause of her symptoms?
a) Dietary induced B12 deficiency
b) Pernicious Anaemia
c) Coeliac Disease
d) Folate Deficiency
e) Iron Deficiency
b)Pernicious Anaemia
What is type of anaemia that can be seen here?
What are the features?
What are the causes of it?
CAUSE
Macrocytic anaemia and hypersegmented neutrophils
B12-
- Alcohol
- IBD& celiac
- Bariatric surgery
- Malnutrition
- Pernicious anaemia
Folate
- Alcohol
- IBD and Celiac
- anti folate drugs
- pregancy
How long do your B12 researves last for?
3-4 years
What is a cause of macrcytic anaemia with neuro signs?
What neuro signs could you get
B12 deficiency
- Glove & stocking parasthesiae
- Hyporeflexia
- Romberg’s +ve
-
Subacute combined degeneration of the cord
- Lateral corticospinal tract lesioned: myopathy
- Dorsal column tract lesioned: pressure, vibration and touch sense diminished
B12 deficiency and folate deficiency usually go hand in hand
Which do you treat first?
B12 because of neuro signs
What is an autoimmune cause of B12 deficiency?
What are the antibodies present?
What are risk factors?
What are diagnostic factors?
What is an autoimmune cause of B12 deficiency?
- Pernicious Anaemia
What are the antibodies present?
- Anti-Parietal cells - produce intrinsic factor
- Anti-Intrinsic factor - forms GIT resistant complex with B12
What are risk factors?
- older(average onset is 60 yrs older
- Auto-immune PMHx
What are diagnostic factors?
- Macrocytosis
- Megaloblastic film
- Neuro signs
- Low B12
How long do folate storage last?
6 months
What are diagnostic factors for classic megaloblastic anaemia?
- Macrocytosis
- Megaloblastic film
- Low Folate
What are non megaloblastic causes of macrocytic anaemia
Alcoholic May have liver failure
Alcohol
Myelodysplasia
Hypothyrodism
Liver disease
Which of these must be avoided in individuals with known
G-6-PD deficiency?
a) Gluten products
b) Cow’s Milk protein
c) Broad beans
d) ACE inhibitors
e) Smoking
c)Broad beans
What are the feature of haemolytic anaemia?
What are the blood results
pale conjunctiva
pallor
sclera icterus
BLOOD resutls:
- low Hb
- Haptoglobin low
- high unconjugated bilirubin
- LDH raised
What are heredetary causes of haemolytic anaemia
How do you test for autoimmune haemolytic anaemia?
Direct antiglobulin test (DAT)- COOMBS test
What is this condition?
What can be seen on this image?
Why does this happen and in reaction to what?
What si the pattern of transmition
Haemolytic anaemia due to G6 PD deficiency
Heinz bodies and bite cell
x linked recessive
Aggrevated by beans, moth ball and antimalarias
What is this condition?
What can be seen on this image?
What is the pattern of transmition?
What is the pathophysiology
What is the investigation of it and complication?
Hereditary spherocytosis
autosomal dominant
Pathophysiology: Spectrin and Ankyrin deficiency
investigation: Coombs - see if autoimmune spherocytosis (- ) + Osmotic fragility test - (hypotonic solution get lysis)
Complication: Parvirus B19 - aplastic crisis
Maggie is a 14 month old infant. She has a recent 5 day history of abdominal pain which her parents believe is related to food she ate at their recent trip to the state fair. The paediatric registrar on call believes she has now developed an AKI. Her blood tests reveal a normal PT, normocytic anaemia with leucocytosis and thrombocytopaenia. A section of her blood film is shown:
What is the most likely cause?
a) ALL
b) DIC
c) HUS
d) Pyelonephritis
e) Sepsis
c)HUS
What is MAHA?
What does it stand for
What is microangiopathic haemolytic anaemia?
anaemia caused by the destruction of erythrocytes due to physical shearing as a result of passage through small vessels occluded by systemic microthrombi
IT is characteristically thrombocytopenia in the absence of defects in coagulation
What is it most likely due to?
What is the pathophysiology haemolytic uraemic syndrome?
In what demographic does it occur in?
What are the syptoms
Ecoli infection
E. coli produces shiga like toxin - binds to endothelial cells in the kidney and damages them -> damaged endothelium attracts platelets -> widespread platelet aggregation -> microthrombi shear blood vessel -> AKI with haemolytic anaemia
Haemolytic Urinary problems Shits (HUS)
SYMPTOMS:
Renal:
- oliguria
- creatine raised
Haemolytic:
- pallor
- jaundice
GI
- abdominal pain
- bloody diarrhoea
What is Disseminated intravasclar coagulation?
What are the causes?
What are symptoms?
sytemic activation of the clotting cascade-> depostition of firbin and resutls in mocrovascular thrombi -> multi organ dysfunciton syndrome
+ all the clotting factor used up -> life threatening haemorrhage
CAUSES
pacreatitis
sepsis
obstetric complications
Cancers
Trauma
ABO reaction
SYMPTOMS
Bleeding features:
- Pettechia
- Ecchymoses
- Haematuria
- Low platelets
- low fibrinogen
- High Fibrin degradation products
- high d dimer
CLotting features
- prolonged APPT/ PT
Haemolytic feature:
- pallor
- jaundice
Which of these statements is incorrect about Antiphospholipid
syndrome?
a) APS is screened for after 3 miscarriages
b) Patients have an increased risk of thrombosis
c) Lupus Pernio is a clinical sign
d) Diagnosis includes screening for the Lupus anticoagulant
e) Diagnosis includes screening for anti-cardiolipin antibodies
c)Lupus Pernio is a clinical sign
What is the pathophysiology of thrombotic thrombocytopaenia purpura?
What is the pentad?
What enzyme is dysfunctional?
What is the pathophysiology of thrombotic thrombocytopaenia purpura?
ADAMTS-13 breaks down Von Willebrand factor into monomere to prevent spontaneous clotting but antibodies block it in TTP-spontaneous clotting
What is the pentad? antibody negative, decreased plateltets, AKI, MAHA, Temperature, Swiniging CNS signs
What enzyme is dysfunctional? ADAMTS -13
When is auto imune thrombosis manifested ?
What are the clinincal features?
What are the antibodies?
When is auto imune thrombosis manifested ? - during pregnancy
What are the clinincal features?
- Recurrent miscarriages (3+)
- VTE
- Stroke/MIs, HTN (arterial problems)
- Livedo reticularis (mottled)
What are the antibodies?
- Anti-cardolipin +ve
- Lupus anti-coagulant test +ve
Which of the following mutations is classically associated with Polycythaemia Rubra Vera?
a) EHEC O157N7
b) BCR ABL1
c) TP53(17q-)
d) PML-RARa
e) JAK2 V617F
e)JAK2 V617F
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What are clinical feature of polycythemia vera?
What is the definition of it?
What would you see in the blood?
What mutation is it associated with?
What are clinical feature of polycythemia vera?
- Older (~60)
- Asymptomatic
- Aquagenic pruritis
- Hyperviscosity syndrome
What is the definition of it?
- Philadelphia chromosome negative myeloproliferative disorder.
What would you see in the blood?
- Elevated Hb & haematocrit
- +/- Thrombocytosis
What mutation is it associated with?
- Almost all PCV is JAK2 V617F +ve
Montgomery Burns is an 89 year old owner of a nuclear powerplant. He is hypertensive and managed with Amlodipine. He is referred to his doctor after his dentist notices he appears cachectic and his dentures no longer fit. On examination, Mr Burns has massive splenomegaly. A blood test and subsequent bone marrow biopsy are carried out:
RBC – Low
Hb – Low
WCC – Low
Plts – High
Film: poikilocytosis
Biopsy: ‘dry tap’
Which is the most likely cause of his symptoms?
a) Multiple Myeloma
b) Acute Myeloid Leukaemia
c) Essential Thrombocytosis
d) Myelofibrosis
e) Myelodysplasia
d)Myelofibrosis
What is this condition?
What can be seen?
In what demographic is it?
What mutation is it associated with?
What is an aplastic anaemia?
What happens?
What can be seen in bloods and patients
What causes it ?
What would a Bone marrow aspirate show
What is an aplastic anaemia?
Bone marrow failure causing pancytopaenia
What happens?
autoantibodies attack the Bone marrow
What can be seen in bloods and patients?
Anamia - high EPO
Thrombocytopaenia - bleeding & pettechia
Leucopoenia- frequent infections & sepsis
What causes it? rafiation and infection
What would a Bone marrow aspirate show - hypocellular