Haematology Flashcards
3 classic Sx of anaemia?
fatigue, dyspnoea, faintness
When is anaemia severe? name 2 signs ?
< 8g/dL
Tachycardia
Flow murmur
Cardiac enlargement
Increased cardiac output
what transports iron?
transferrin
where is iron stored
ferritin, haemosiderin
Name 3 things on presentation with Fe deficiency anaemia
Pallor
Nail changes - Koilonychia (spoon), brittleness
Hair loss
Mouth changes - Angular stomatitis, atrophic glossitis
Classic 3
Fatigue
Faintness
Dyspnoea
What are the general causes of iron deficiency
Inadequate intake
Poor absorption
Excessive loss
Excessive iron requirement
Name 3 Ix in Iron deficiency anaemia and 2 things seen on bloods
Hb <13g/dL (men), <12g/dL (women)
MCV - microcytic
Peripheral blood smear
Iron studies - Low iron / ferritin
Ix for cause
Coeliac (malabsorption), H. pylori (basically eats iron), endoscopy (bleed or Malab)
Why might ferritin be unexpectedly high in iron deficiency
It is an acute phase protein so will increase if inflammation, infection and malignancy
2 key DDx for iron deficiency anaemia
chronic disease sideroblastic anaemia (bone marrow produces sideroblasts rather than normal erythrocytes)
mx of iron deficiency
oral replacement - ferrous sulphate
consider transfusion
SE of ferrous sulphate
constipation, black stools, vomit
When would you think this pt has sideroblastic anaemia
microcytic hypochromic anaemia NOT responding to iron
Pathology and What causes sideroblastic anaemia
Ineffective erythropoesis - cant incorporate iron into Hb
Congenital - inherited XLSA (x-linked)
Acquired - MDS (myelodysplastic syndrome), myeloma, PRV, pyridoxine (B6) deficiency
name 3 Ix in sideroblastic anaemia. Make sure you know the last one
Hb - low
MCV - microcytic
Peripheral blood smear
Iron studies - Iron / ferritin high
Marrow aspirate - Perinuclear ring of iron granules with Prussian Blue
Mx of sideroblastic
iron chelation (gets rid of excess iron in body) - desferrioxamine Avoid alcohol / vit c (increase iron absorption)
What happens in b thalassaemia
Inherited microcytic anaemia caused by mutation in beta-globin gene
-ineffective erythropoesis
Erythroid hyperplasia
2 Effects of erythroid hyperplasia in b thalassaemia?
Bony changes - skull bossing, vertebral….
hepatosplenomegaly
Name 3 Ix in b thalassaemia
FBC Peripheral blood smear Hb anaylsis LFT Xray skull for skull bossing Abdo USS for hepatosplenomegaly
b thal mx points
genetic councelling
transfusion
Iron chelation
splenectomy
Bone marrow transplant
name 3 complications of thalassaemia
thrombotic
iron overload - heart arrhythmia, pituitary, pancreas
transfusion reactions
transfusion infections
splenectomy comps
Reticuloendothelial causes of haemolysis
Macrophages of liver
Spleen
Accelerated red cell destruction due to immune targeting by antibodies
Name 3 hereditary causes of haemolytic anaemia
Glucose 6 phosphate dehydrogenase deficiency
Hereditary spherocytosis,
Sickle cell anaemia, thalassaemia
Test for immune mediated haemolytic anaemia
direct antiglobulin +ve (Coombs’ positive)
Name 3 causes of non immune mediated haemolytic anaemia
Infection (e.g. malaria), trauma,
microangiopathic haemolytic anaemia (DIC, TTP, HUS, HELLP)
hypersplenism, liver disease
Paroxysmal nocturnal haemoglobinuria
trauma - eg mechanical heart valve
complement mediated lysis
Dx of Paroxysmal nocturnal haemoglobinuria
urinary haemosiderin
People with this condition have paroxysmal episodes of haemolytic due to things like infection
3 Ix in haemolytic anaemia
FBC - low Hb
Unconjugated bilirubin
Urinanalysis - haemaglobinuria
Peripheral blood film
Direct coombs test = test antibodies ON red blood cells
In haemolytic anaemia there is increased breakdown - what do you see in blood (and one other place) as a result of this?
Increased bilirubin, increased urobilinogen [not blood obvs], increased LDH
In haemolytic anaemia there is increased production - what do you see as a result of this?
Increased MCV as there are increased reticulocytes (bigger)
marrow hyperplasia
How might you differentiate between Extravascular or intravascular haemolytic anaemia
Intravascular - increased free plasma Hb and decreased haptoglobin, increased haemoglobinuria (no blood cells)
Extravascular - splenomegaly
What supplement is usually given in haemolytic anaemia
folic acid
How does G6PDD present? Triggers?
Presents with prolonged neonatal jaundice
In older:
Jaundice
Pallor
Dark urine (suggests intravascular haemolysis)
Nausea, vomiting, dehydration, AKI (haemoglobin precip)
Infection
Broad beans
Certain drugs (causing oxidative stress) e.g. sulphonamides, *cephalosporins
mx of G6PDD
Fluid intake
folic acid
blood transfusion if severe anaemia
renal support if needed
what is anaemia of chronic disease
Anaemia and evidence of immune system activation
- decreased RBC production + decreased survival
Whats seen on lab studies of anaemia of chronic disease?
Normocytic normochromic/microcytic hypochromic Low reticulocyte count Low serum iron Low TIBC Low transferrin saturation Elevated ferritin
1 test that can differentiate anaemia of chronic disease and iron deficiency
ferritin - increased in ACD
How does aplastic anaemia present?
What is it?
Autoimmune destruction of RBCs.
Neutropenia - infections
Anaemia - fatigue, pallor, dyspnoea, faintness
Thrombocytopenia - bleeding, bruising
Name 2 associations with aplastic anaemia
pregnancy, coeliac, SLE, paroxysmal nocturnal haemoglobinuria
Seen on bone marrow biopsy of aplastic anaemia
Hypocellular marrow with no abnormal cell population
2 parts of Non pharma mx of aplastic anaemia
MASSC risk assessment if NP< 0.5
[risk of febrile neutropenia]
Full barrier nursing
Avoid IM injections
Look for infection
Check bloods and cultures
Vitals 4 hourly
Medical Mx of aplastic
allogeneic BM transplant
+ RBC and Pt transfusion
+ ABX
2 issues with crescent shaped Hb in sickle cell
Disrupt blood flow and break (haemolysis)
- Painful crises
- Organ damage
- Increased vulnerability to infection
Cause varying degrees anaemia
what triggers sickle cell crisis?
CHIDS:
Cold Hypoxia (extreme exercise) Infection Dehydration Stress
3 areas affected in exam land by sickle cell crisis?
Acute chest syndrome: pneumonia like syndrome (due to sickling in pulmonary vasculature) - chest pain, fever, dyspnoea, tachypnoea
Bone infarction and avascular necrosis femoral head
Acute abdomen - mesenteric ischaemia
When is sickle cell usually Dx? Mx at this point?
newborn screening - blood spot
pneumococcal vaccine and penicillin prophylactically
genetic counselling
parental education
mx of sickle cell crisis
cross match analgesia O2 IV vluids Abx -broad Blood if needed
Chronic Mx of sickle cell
Supportive
- Pain
- Pneumococcal vaccination and penicillin prophylaxis
- Trigger avoidance
- Folic acid if severe haemolysis
Hydroxyurea - Increases fetal Hb production
Repeat transfusions if Hb <10g/dL
Name 3 comps of sickle cell
crisis anaemia liver - jaundice / gallstones iron overload from transfusion leg ulcers
What causes megaloblastic anaemia?
B12/folate deficiency + drugs (hydroxyurea)
This causes defective DNA synthesis which also leads to leukopenia and thrombocytopenia
Where is folate found naturally
green veg
nuts
liver
4 causes of folate deficiency
Poor diet - eg alcoholic , poverty
Increased demand - eg pregnancy renal disease
Malabsorption - coeliac/ tropical spure
Drugs, alcohol and methotrexate
signs of folate deficiency
Headache - hallmark of megaloblastic
Anaemia
Pallor, fatigue, dyspnoea, faintness, tachycardia, heart murmur
Gi
Loss of appetite/wt loss
Skin
glossitis, exfoliative dermatitis
Mx of folate deficiency
oral folic acid
What do you get with b12 deficiency
macrocytic anaemia with peripheral neuropathy and neuropsych complaints
foods rich in b12
meat, fish, dairy
[vegans always lacking]
which cell and what do they produce is needed for b12 absorption
parietal - intrinsic factor
neuro sx of b12 deficiency
Subacute degeneration of spinal cord
[peripheral neuropathy, demetia, ataxia, paraethesia]
triad of signs Subacute degeneration of SC?
Upgoing plantars
Loss knee jerk
Loss ankle jerk
mouth signs b12 deficiency
Glossitis (painful), angular cheilitis
mx of b12 deficiency if severe (pancytopenia + anaemia + neurological)
IM hydroxycobalamin (cobalamin = b12) oral folic acid
blood transfusion
admit neuro / haem
Mx of b12 deficiency if no neuro o
IM hydroxycobalamin
What is pernicious anaemia
Autoimmune atrophic gastritis -> Atrophy of gastric mucosa with failure of IF and acid production due to autoantibodies to IF
Association wit pernicious anaemia? increased risk of ?
AI diseases - thyroid, DM, vitiligo….
Gastric Ca
Specific Ix for pernicious
IF antibody
antiparietal antibody
Schilling test (radio b12)
What is leukaemia
Excess of abnormal white cells in peripheral blood - myeloid or lymphoid
Acute leukaemia 3 Sx categories?
Tumour related:
Bone pain, fever, lethargy, night sweats, wt loss
BM sx
Anaemia
thrombocytopenia
neutropenia
Circulating cell sx
headache, lyphadenopathy, hepatosplenomegally
Dx of AML
Bone marrow blasts > 20% or peripheral blood
[may be complication of chemotherapy]
[?most common in old age]