Haematology Flashcards
Symptoms and signs of anaemia
Symptoms Fatigue Dyspnoea Faintness Palpitations Headache Tinnitus
Signs Pallor Hyperdynamic circulation - Tachycardia - Flow murmur: apical ESM - Cardiac enlargement Ankle swelling ̄c heart failure
Cut off for anaemia
Men <13.5
Women <11.5
Children <11
Newborns<15
Classification of anaemia and causes
Microcytic - TAILS Thalassaemia Anaemia of Chronic Disease Iron deficiency Lead poisoning Sideroblastic anaemia
Normocytic
- recent blood loss
- BM failure
- Early ACD
- Renal failure
- Pregnancy (↑ plasma volume)
- Hypothyroidism (may also be macrocytic)
- Haemolysis (may also be macrocytic)
Macrocytic - Megaloblastic Vit B12/ folate deficiency Anti-folate drugs: phenytoin, methotrexate Cytotoxics: hydroxycarbamide
- Non-megaloblastic Reticulocytosis Alcohol or liver disease Hypothyroidism Myelodysplasia or Marrow infiltration
Bodies response to anaemia
- ↑ CO (positive chronotrophy and inotrophy)
- ↑ 2,3-BPG production - to reduce Hb affinity for O2 and shift saturation curve to the right,
- ↑ erythropoesis levels
Iron deficiency anaemia - signs; causes; ix and mx
Signs
• Koilonychia
• Angular stomatitis / cheilosis
• Post-cricoid Web: Plummer-Vinson
Causes
- ↑ Loss Menorrhagia; GI; bleeding Hookworms
- ↓ Intake
Poor diet/ vegetarian
- Malabsorption. Coeliac; Crohn’s; gastrectomy
- ↑ utilisation Growth; pregnancy
Ix
- Haematinics: ↓ferritin, ↑TIBC, ↓ transferrin saturation
- Film: Anisocytosis, poikilocytosis, pencil cells
- Upper and lower GI endoscopy
Rx
- Dietary advice
- Ferrous sulphate 200mg PO TDS
o SE: GI upset and black tarry stools
Sideroblastic anaemia - pathophysiology, signs; causes; ix and mx
Ineffective erythropoiesis
o ↑ iron absorption
o Iron loading in BM → ringed sideroblasts
o Haemosiderosis: endo, liver and cardiac damage
Causes - Congenital - Acquired o Myelodysplastic / myeloproliferative disease o Drugs: chemo, anti-TB, lead
Ix
- Microcytic anaemia not responsive to oral iron
- ↑Ferritin, ↑ se Fe, ↔TIBC
Rx
- Remove cause
- Pyridoxine may help
Thalassaemia - pathophysiology
Pathophysiology Point mutations (β) / deletions (α) → unbalanced production of globin chains → precipitation of unmatched globin → membrane damage → haemolysis while still in BM and removal by the spleen
β Thalassaemia trait and major - sx, ix and mx
β Thalassaemia Trait ( Heterozygosity) - Mild anaemia which is usually harmless
• ↓ MCV (“too low for the anaemia”): e.g. <75
• ↑ HbA2 (α2δ 2) and ↑HbF (α2γ2)
β Thalassaemia Major Features develop from 3-6mo • Severe anaemia • Jaundice • FTT • Extramedullary erythropoiesis o Frontal bossing o Maxillary overgrowth o HSM • Haemochromatosis after 10yrs (transfusions)
Ix
• ↓Hb, ↓MCV, ↑↑HbF, ↑HbA2 variable
• Film: Target cells and nucleated RBCs
Rx
• Life-long transfusions
• SC desferrioxamine Fe chelation
• BM transplant may be curative
α Thalassaemia- sx, ix and mx
Trait
o Asymptomatic
o Hypochromic microcytes
HbH Disease –/-α
o Moderate anaemia: may need transfusions
o Haemolysis: HSM, jaundice
Hb Barts –/–
o Hydrops fetalis → death in utero
Ix for macrocytic anaemia
Ix
Film o B12/Folate Hypersegmented PMN Oval macrocytes o EtOH/Liver Target cells
Blood
o LFT: mild ↑ bilirubin in B12/folate deficiency
o TFT
o Se B12
o Red cell folate: reflects body stores over 2-3mo
BM biopsy: if cause not revealed by above tests
o Megaloblastic erythropoiesis
o Giant metamyelocytes
Causes of haemolytic anaemia
Acquired
Immune-mediated DAT+ve
- AIHA: warm (IgG mediated - extravascular haemolysis–> rx = immmunosupression +/- splenectomy), cold (IgN - intravasc haem –> rx = avoid cold +/- rituximab),
- Drugs: penicillin, quinine, methyldopa
- Allo-immune: acute transfusion reaction, HDFN
PNH (Paraoxysmal nocturnal haemoglobinuria)
- abscence of RBC anchor molecule - IV lysis; sx = venous thrombosis of liver, mesentary and CNS; RX - anticoagulation
Mechanical:
- MAHA:
- ->DIC
- -> HUS (E.Coli O157:H7 - MAHA + Thrombocytopenia + renal failure; resolves spont.)
- -> Thrombotic Thrombocytopenia Purpura ( sx - Fever; confusion/seizures; MAHA; Thrombocytopenia; Renal failure –> rx - plasmapheresis/ immunosup)
- Heart valve
Infection: malaria
Burns
Hereditary
Enzyme: G6PD and pyruvate kinase deficiency
Membrane: HS, HE
Haemoglobinopathy: SCD, thalassaemia
Pathophysiology of haemolytic anaemia
↑ red cell breakdown
- Anaemia ̄c ↑ MCV + polychromasia = reticulocytosis
- ↑ unconjugated bilirubin
- ↑ urinary urobilinogen
- ↑seLDH
- Bile pigment stones
Intravascular
- Haemoglobinaemia
- Haemoglobinuria
- ↓ se haptoglobins
- ↑ urine haemosiderin
- Methaemalbuminaemia
Extravascular
1. Splenomegaly
Causes and mx of folate deficiency
Reduced intake: Poor diet, alcoholics
Reduced absorption: Coeliac, Crohns, gastrectomy
Increased demand: Pregnancy, lactation,
Increased cell turnover – malignancy, chronic inflammation, dialysis, SCD or hereditary spherocytosis
Drugs – trimethoprim, methotrexate, sulphasalazine
Mx - assess for underlying cause; folate PO
Causes of B12 deficiency
- ↓ intake – vegans
- ↓ absorption – Gastrectomy, pernicious anaemia, Crohns, ileal resection
- Increased utilization – Tapeworm
- Drugs – metformin, Nitrous oxide.
Drugs that interfere with iron absorption
- Cimetidine
- Rantidine
- Cholestyramine
What drugs can Iron decrease the absorption of?
- Tetracyclines
- Quinolone Abx
- Bisphosphonates
- Levodopa
- Levothyroxin
Causes of Anaemia of Chronic Disease
- Malignancy
- Inflammation – RA, temporal arteritis
- Chronic Infection – TB
- Renal Failure
Pernicious anaemia cause, ix and mx
AI atrophic gastritis caused by autoAB agaisnt pareital cells or intrinsic factor –> achlorhydria and low intrinsice factor
- B12 needs intrinsic factor for absorption and is absorbed in terminal ileum.
Ix - parietal cell and intrinsic factor Ab
Mx - Give B12 injection
Complications of blood transfusions
IMMEDIATE -stop transfusion
- haemolytic due to ABO incompatibilioty (IV haemolysis) Sx - Agitation; Fever; Abdo/chest pain; ↓ BP → shock; DIC → haemorrhage Renal failure
-bacterial contamination (sx - ↑↑ temp + rigors; ↓BP)
- febrile non-haemolytic (slow transfusion)
- anaphylaxis (Anti-IgA IgE)
- TRALI (ADRS - Anti-WBC ab)
- Fluid overload (CCF)
- Massive transfusion (Sx - ↑K
↓ Ca (citrate chelation); dilutional coagulopathy –>↓ F5 + F8 +↓ plats; Hypothermia); ?lactic acidosis
DELAYED
- delayed haemolytic (Jaundice; Anaemia / ↓ing Hb Fever± Haemoglobinuria - extravas haem)
- Fe overload –> Desferrioxamine SC
- Post-transfusion Purpura (?thrombocytopenia - rx - IVig and plat transfusion)
- GvHD (sx - d; skin rash; liver failure; pancytopenia)