Haematology Flashcards
Anaemia = Hb < ?
<130 M
<120 F
Causes of Anaemia
Microcytic (5)
Normocytic (5)
Macrocytic (6)
Microcytic = TAILS (thalassemia, anaemia of chronic disease, iron deficiency, lead poisoning, sideroblastic)
Normocrytic= AAA-SH (aplastic, acute blood loss, anaemia of chronic disease, sickle cell disease, haemolytic)
Macrocytic= FATRBC (folate deficiency, alcoholism/CLD, hypothyroidism, reticulocytosis, B12 deficiency, cytotoxic drugs/anti-folate drugs)
Iron deficiency anaemia is a microcytic hypochromic anaemia from increased blood loss, reduced iron intake and malabsorption.
List 5 presenting features, 2 main investigations + inv considerations and 3 mngmnt options.
Findings from FBC (Transferrin, ferritin, TIBC)
- Angular chelitis, atrophic glossitis, brittle hair, koilonychia, pica
- FBC, blood film, iron studies
- FBC - Low ferritin, low transferrin, raised TIBC
- Blood film - target cells, poikolocytes (pencil)
- investigate underlying cause (ca- colonoscopy?, OGD, bone marrow biopsy)
- iron tables (Ferrous sulfate), iron infusion (IV Cosmofer), blood transfusion
2WW Guidelines for suspected malignancy in iron defiency anaemia
- Which people would you refer for endoscopy urgent review
Men Hb <11
Post menopausal women <10
Finding on blood film - iron def anaemia
Poikolocytes (pencil)
Thalaessemia (autosomal recessive) - types of alpha (4) and beta (2)
Alpha - gene deletions
* silent - affects 1 copy of alpha globin chain
* trait - “ 2
* HbH disease - “3
* fetal hydrops (Bart’s) - “4
* Beta- gene mutations
* minor trait
* major
Inv for thalaessemia = FBC, heamoglobin electrophoresis & genetic testing.
What are the findings from hb electrophoresis for beta thalessemia (3)
Increased HbA2 and HbF
Reduced/absent HbA
HbA is the most common form of Haemoglobin (made from 2x alpha chains, 2 x beta chains.)
Note in alpha thalassemia- hb electrophoresis is typically normal.
Mngmnt thalaessemia
Iron chelation - Desferroxamine
Blood transfusion
prevents the iron overload from repeated blood transfusions
Sideoblastic anaemia:
* type
* mechanism
* cause
* blood film finding
* iron, ferritin and transferrin sats?
* bone marrow finding?
treatment supportive/underlying cause or Pyroxidine
- hypochromic microcytic
- RBCs fail to incorporate iron into haem - ring of iron forms around nucleus; biosynthesis in mitochondrion
- congenital or acquired (chemo, alcohol, myeloproliferative)
- basophilic stippling of RBCs
- high ferritin
- high iron
- high transferrin concentration
- prussion blue staining shows ringed sideroblasts
raised iron because it is not being incorporated into haem (loose)
What causes basophilic stippling (in general)
typical causes
Presence of basophils within the RBC cytoplasm –>
Disturbed erythropoeisis
Can contain fragments of DNA/mitochondria
causes
thalassemia
sideroblastic anaemia
megaloblastic anaemia
lead poisoning
etcetc
Lead poisoning
- presentation
- blood film
- fbc
- mx
- differential
- microcytic anaemia presenting with abdominal pain, peripheral neuropathy and blue stained gingiva.
- also neuropsychotic features, constipation and fatigue
- blood film = basophilic stippling and clover-leaf
- FBC: microcytic anaemia
- reqs chelation (DMSA, EDTA etc)
- acute porphyria - also presents with abdominal pain and neurological signs (but normally raises delta aminolaevulinic acid levels)
Normocytic anaemias - AAASH.
Aplastic anaemia occurs due to a problem within the bone marrow itself. What are its two main presentations?
PRCA
Pancytopenia
Aplastic can be acquired or inherited.
2 types of inherited and 2 causes of acquired?
- Diamond Blackfan & Fanconi syndrome
- Drugs (cytotoxics, antibiotics), infections (EBV, HIV), toxins (benzene).
Features of Fanconi syndrome (inherited aplastic anaemia) (6)
- Facial & skin abnormalities (e.g. microcepahly, cafe au lait spots)
- Absent thumb
- Horsehoe kidney & prox tubule dysfunction
- Aplastic anaemia - pancytopenia
- Intellectual disablity
- Short stature
General sx of haemolytic anaemia
Anaemia + jaundice + splenomegaly
?gall stones
Haemolytic anaemia can be hereditary (affecting membrane, metabolism & haemoglobin) and acquired (immune or non-immune). List 5 causes of hereditary haemolytic anaemia.
- Spherocytosis
- Elliptocytosis
- G6PD deficiency
- Thalassemia
- Si ckle Cell Disease
Key fact for
spherocytosis
- splenomegaly, jaundice & gallstones
- often presents in aplastic crisis during parvovirus inf
Key fact for
elliptocytosis
mostly asx
Triggers for H6PD deficiency (3) acute haemolytic episodes
Meds - sulfonylureas (Gliclazide), CIprofloxacin, and anti-malarials
key facts for
G6PD deficiency:
- genetics
- ethnicity
- haemolysis triggered by (3)
- findings on blood film (main + 3 others)
- reduced NADPH results in..
- ix: blood film & what else?
- management
- X linked, affected African/Medit. newborn babies (M)
- fava beans, certain drugs, infeection
- Heinz bodies (+ ghost, bite, blisters)
- reduced NADPH –> oxidative damage to RBC
- test using G6PD assay and repeat after 3 months
- treat underlying infection, avoid precipitants, transfusion may be needed
key facts for
Sickle cell anaemia
- autosomal recessive
- malaria protective
- presentation = SICKLED: note splenomegaly, priapism and dactylitis (under 3)
- crises & complications
- req tranfusion, imms, hydroxycarbamide (increased fetal hb) long term for pain and prophylaxis for crises
point mutation for SCD, on chr 11
glu- val on beta globin gene
findings on blood film for SCD
Howell-Jolly bodies (sign of hyposplenism) & target cells
presentation: SCD
Splenomegaly
Infarction
Crises
Kidney disease
Liver & lung disease
Erection (priapism)
Dactylitis
Sickle cell crises (4)
General management
1. Thrombotic “vaso-occlusive”
2. Acute Chest Syndrome
3. Sequestration crisis
4. Aplastic
General
Analgesia + O2 + IV Fluids
1. Blockage in organs (lungs, brain, hip)
2. Blockage in pulmonary microvasculature –> infarction in lung parenchyma. Treat with O2, analgesia, Abx
3. Splenic sequestration - sickling of the blood causing pooling within spleen(or lungs) - acute emergency, fall in retics.
4. Triggered by parvovirus infection: fall in Hb, fall in retics
4 - note elliptocytosis can also present like this
The acquired haemolytic anaemias can be classed as immune (Coombs +ve) and non-immune (Coombs -ve).
Name 2 types of each.
Immune
* Cold, occurs <10degrees
* Warm, more common, at >37 degrees
* Non-immune
* Paroxysmal noctural haemaglobinuria
* Microangiopathic (e.g. 2ndary to HUS)
Feature of PNH (paroxysmal nocturnal haemaglobinuria)
A type of acquired haemoltyic anaemia (non-immune, Coombs -ve)
Red urine in morning
Macrocytic anaemias can be megaloblastic (folate & B12 deficiency) and non-megaloblastic.
Folate & B12 deficiency can be caused by malnutrition, malabsorption (Crohn’s, coeliac) or autoantibodies (pernicious).
In pernicious anaemia what antibodies are present? (2)
What Ca does it increase your risk of 3x?
- Auto-abs against gastric parietal cells & intrinsic factor.
- reduced IF and reduced HCl.
- Gastric adenoca
B12 requires intrinsic factor to be absorbed
Presentation of B12 deficiency (3)
- Peripheral neuropathy
- Visual changes - reduced colour vision
- Mood changes (e.g. irritability)
- Anaemia sx - note glossitis
B12 deficiency can lead to SACDC. What tracts does this affect, and how does it present?
- Combined = both lateral corticospinal and dorsal columns.
- Sensory loss but preserved pain/temperature sensation
- Mixed UMN and LMN signs (e.g. spastic paresis)
Treatment for B12 deficiency
Can it reverse SACD?
- B12 supplementation (hydroxycobalamin - parenteral, or cyanocoblamin - oral)
- No.