Haematology Flashcards
Plummer-Vinson syndrome
Post-cricoid web that is associated with iron deficiency anaemia
Iron deficiency anaemia haematinics
↓ferritin, ↑TIBC, ↓ transferrin saturation
Sideroblastic anaemia haematinics
↑Ferritin, ↑ se Fe, ↔TIBC
Rx: Can give pyroxidine
B Thalassaemia trait
↓ MCV
↑ HbA2 (α2δ 2) and ↑HbF (α2γ2)
B Thalassaemia Major
↓Hb, ↓MCV, ↑↑HbF, ↑HbA2 variable
Film: Target cells and nucleated RBCs
Folate deficiency
Typically decreased intake or high demand (pregnancy or medication e.g. methotrexate/phenytoin)
Rx = give B12 first unless this is normal as can worsen SCDC; give folate PO 5mg/d
Subacute Combined Degeneration of the Cord
Combined symmetrical dorsal column loss and corticospinal tract loss with a mixed UMN and LMN picture.
Pain and temperature intact
Usually only caused by pernicious anaemia
G6PD deficiency triggers
Broad (Fava) beans
Mothballs (naphthalene)
Infection
Drugs: antimalarials, henna, dapsone, sulphonamides
Can see Heinz bodies
Warm AIHA
IgG mediated with extravascular haemolysis and spherocytes and is DAT +ve
Linked to SLE, RA and Evan’s
Rx = immunosuppression +/- splenectomy
Cold AIHA
IgM mediated with intravascular haemolysis and is DAT +ve
Linked to mycoplasma
Rx = avoid cold and rituximab
Paroxysmal cold haemoglobinuria
IgG “Donath-Landsteiner” Abs bind RBCs in the cold → complement-mediated lysis on rewarming
Associated with Measles, mumps, chickenpox
Paroxysmal nocturnal haemoglobinuria
Features
Visceral venous thrombosis (hepatic, mesenteric, CNS)
IV haemolysis and haemoglobinuria
Ix
Anaemia ± thrombocytopenia ± neutropenia
FACS: ↓CD55 and ↓CD59
Rx
Chronic disorder therefore long-term anticoagulation
Eculizumab (prevents complement MAC formation)
HUS
E. coli O157:H7 from undercooked meat
Bloody diarrhoea and abdominal pain precedes:
MAHA (schistocytes)
Thrombocytopenia
Renal failure
Spontaneously resolves
TTP
Pentad = fever, CNS, MAHA, thrombocytopenia, renal failure
Rx = Plasmapheresis, immunosuppression, splenectomy
Hereditary spherocytosis Ix + Rx
↑ osmotic fragility
Spherocytes
DAT-ve
Rx = folate + splenectomy (after childhood)
Pyruvate Kinase Deficiency
Features: splenomegaly, anaemia +/- jaundice
Rx = often not required
SCD presentation
SICKLED Splenomegaly Infarction - stroke, AVN, spleen (hyposplenism) Crises - pulmonary, mesenteric, sequestration crises (splenic pooling --> shock and severe anaemia) Kidney disease Liver disease Erection Dactylitis
Increased PT
Warfarin / Vit K deficiency
Hepatic failure
DIC
Increased APTT
Lupus anti-coagulant Haemophilia A or B vWD (carries factor 8) Unfractionated heparin DIC Hepatic failure
Increased bleeding time
↓ plats number or function
vWD
Aspirin
DIC (increased thrombin time as well)
Haemophilia A Ix and Mx
Ix
↑APTT, normal PT, ↓F8 assay
Mx
Avoid NSAIDs and IM injections
Minor bleeds: desmopressin + tranexamic acid
Major bleeds: rhF8