Anaemia Flashcards
Basic features of anaemia?
Pale conjunctivae and skin, fatigue and high RR and HR if severe
IDA on blood film features?
Aniso-poiliocytosis
Pencil Cells
Hypochromic and microcytic
Causes of IDA?
Reduced uptake: malnutrition, coeliac, IBD
Increased Loss: GI malignancy, peptic ulcer, IBD, menstruation
Increased requirement: pregnancy and breastfeeding
High plant based foods for iron?
Spinach, broccoli, lentils
Pathology of ACD?
Chronic disease causes cytokine release so increased Hepcidin to reduce iron to infection normally. Therefore decreased ferroportin and transferrin activity and increased storage in Ferritin
IDA vs ACD bloods?
Both Iron low
IDA = low ferritin and high TIBC
ACD = High or normal ferritin and decreased TIBC
TIBC = transferrin iron binding capacity
What is thalassaemia?
Globin chain quantitative defcts. Autosomnal recessive and high in Mediterranean and CYPRUS
Resistant to Malaria
Thalassaemia types?
Chr 16 = alpha and alpha O is mild anaemia, HbH is significant and needs transfusion.HbBarts is death in utero
Chr 11 = beta major needing transfusions
Investigations for thalassaemia?
Microcytic anaemia + film
Normal iron studies
Gel electrophoresis
Haemoglobin types?
HBA1 = 2alpha + 2beta HBA2 = 2 alpha and 2 delta HbF = 2 alphga and 2 gamma
What predisposes sickling in SCD?
hypoxia, dehydration, acidosis and infection
SCD features?
Point mutation on beta globin gene on CHR 11. autosomnal recessive and resistant to malaria
SCD on blood films?
Howell-Jolly bodies in RBC and sickled cells (10-20 day lifespan)
Acute Management of various SCD crises?
Sickle Acute Painful Crises: Saturate (O2), Abx, Pain Relief, Cannula (IV fluids)
Stroke: Exchange blood transfusion
Sequestration = splenectomy
Chronic cholecystitis = cholecystectomy
SCD presentation and diagnosis?
Dactylitis, acute chest syndrome, haemolytic anaemia, aplastic crisis, priapism
Hb electrophoresis + blood film
SCD chronic management?
Conservative: avoid triggers, vaccination.
Medical: Vaccinations, hydroxyurea, prophylactic Abx
Surgical: bone marrow transplant
Features of folate + B12 deficiency Blood film?
Megaloblastic anaemia from hypersegmented neutrophils and macrocytic cells
Causes of megaloblastic anaemia types?
Vit B12 = alcohol, IBD and coeliac, malnutrition and pernicious anaemia
Folate: alcohol, IBD and coeliac, anti-folate drugs and pregnancy
B12 Deficiency ?
Megaloblastic + Neuro changes +/- pernicious anaemia (either parietal or IF)
Neuro: glove and stocking parasthesiae, hyporeflexia, Rombergs +ve, Subactue combined degeneration of the spinal cord
Non-megaloblastic macrocytic anaemia causes?
Alcoholics may have liver failure
Alcohol, Myelodysplasia, Hypothyroidism, liver disease
What must be avoided in patients with G6PD deficiency?
Beans e.g. fava or broad
General features of haemolytic anaemia?
Scleral ictus and jaundice, pale conjunctivae and skin.
Blood tests = Hb low, haptoglobin low, unconjucated bilirubin raised and LDH raised
Hereditary haemolytic anaemias?
Membrane = hereditary spherocytosis Enzymes = G6PD deficiency Hb = SCD and thalassaemia
Pathology of G6PD?
Vulnerable to oxidative stress as decreased NAPDH so less glutathione.
Beans means HEINZ bodies
G6PD on blood film?
Heinz bodies and Bite cells (previous haemolysis)
Pathology of hereditary spherocytosis?
Beta spectrin or ankyrin deficiency so weak cytoskeleton so no biconcave shape
Investigations and complications of hereditary spherocytosis?
Osmotic fragility test by giving hypotonic saline which causes lysis
Coombs test Negative
Complication is aplastic crises due to Parvovirus B19
HUS classic grouping?
Child
AKI and MAHA features (jaundice, conjunctival pallor, schistocytes), thrombocytopenia
GI: abdo pain and bloody diarrhoea
Typically E.Coli producing SHiga
Pathology Microangiopathic Haemolytic Anaemia?
Microthrombi causing schistocyte formation
Can be due to DIC
Causes of DIC?
Pancreatitis, sepsis, obstetric complication, ABO reaction, trauma and cancers e.g. MM
DIC Features?
Bleeding: petechiae, ecchymoses, haematuria all due to decreased platelets and fibrinogen, increased FDPs and D-dimer
Clotting: Prolonged APTT and PT
Haemolytic: jaundice and conjunctival pallor + underlying pathology
Thrombotic thrombocytopaenic Purpura (TTP) pathology?
Dysfunctional ADAMTS-13 that cannot cleave VWF
TTP features?
COOMBS NEGATIVE
Defunct ADAMTS
Antiglobulin negative, Decreased platelets, AKI, MAHA, Temperature and Swinging CNS signs
Form Pentad with HUS triangle
MAHA components?
DIC, HUS, TTP
What is the Coombs test?
Direct antiglobulin test (DAT)
Detects presence of Ab attached to RBC surface e.g. autoimmune haemolytic anaemia
What are the other acquired haemolytic anaemias?
Autoimmune: so anti-RBC Abs
Drugs e.g. Dapsone (anti-leprosy Abx)
Infections e.g. Malaria
Describe agglutination in Coombs?
Warm >37: IgG Abs so idiopathic, SLE or CLL
Cold <37: IgM so idiopathic, mycoplasma or mononucleosis
Presentation of MM, AML, Essential thrombocytosis and Myelodysplasia?
MM has increase in WCCs, AML does not have splenomgealy but CML does, ET does not cause splenomegaly and common in 35-55YO, and myelodysplasia does not have elevated platelets or splenomgealy
Myelofibrosis presentation?
In response to BM malignancy
Myelofibrosis has characteristic dry tap of bone, in older patients with radiation exposure, low RBC< Hb and WCC with poikilocytosis and high platelets
Tear drop cells on blood film
Causes of polycythaemia?
Primary: Rubra Vera
Secondary: Appropriate (hypoxic response from altitude, COPD, heart disease) or Inappropriate (EPO-secreting tumour) e.g. lung cancer
Whats the classic mutation for PCV?
JAK2 V617F activates itself instead of needing EPO so high Hb and primary polycythaemia
Nearly 100% of PCV
Features of polycythaemia rubra vera
Philadelphia Chr negative myeloproliferative disorder.
Older>60, asymptomatic aquagenic pruritis, hyper-viscosity syndrome
Elevated Hb and haematocrit +/- thrombocytosis
Symptoms of hyperviscosity?
headaches and ligh headedness, visual disturbances and strokes
What is anti-phospholipid syndrome?
autoimmune mediated thrombosis. Often in pregnancy.
CausesL recurrent miscarriages 3+, VTE, Stoke/MIs, HTN, livedo reticularis (mottled)
By anti-cardiolipin +ve and lupus anti-coagulant test +ve
What is aplastic anamia?
Bone marrow failure causing pancytopaenia
Caused by fanconis (congential, infections e.g. parvovirus and radiation
Anaemia features + raised EPO
Thrombocytopaenia: bleeding + petechiae
Leucopoenia: sepsi + recurrent infections
BM aspirate in hypocellular
When do you get aplastic crisis?
In SCD and hereditary spherocytosis - restricted due to RBC
