haem Flashcards
causes of haemolytic anaemia
hereditary: spherocytosis G6PD deficiency haemoglobinopathics: SCD, thalassaemia acquired: autoimmune (cold/warm) microvascular: HUS, TTP, DIC drugs: penicillin infection: malaria blood mismatch
management haemochromatosis
cons: MDT, genetic counselling, monitor for complications (cirrhosis, HCC) med / surg: 1. venesection 2. iron chelation: desferrioxamine liver transplant
types of polycythaemia
primary: polycythaemia rubra vera (JAK2 mutation)
secondary (increased erythropoietin): hypoxia, renal cell carcinoma, erythropoietin abuse in athletes
reactive: due to reduced plasma volume
causes of thrombocytopenia
reduced production: infection, drugs (e.g. penicillamine), myelofibrosis, myelodysplasia
increased destruction:
heparin, hypersplenism, HUS, TTP, DIC, ITP
causes of thrombocytosis
reactive to infection, surgery, IDA malignancy hyposplenism essential thrombocytosis polycythaemia ruba vera
causes of neutropenia
viral infection
chemo / radiotherapy
drugs: carbimazole, clozapine
causes of lymphocytosis
viral infection
lymphoma
CLL
management of warfarin dose w/o bleed
< 6: reduce warfarin dose
6-8: omit warfarin dose + start on reduced dose
> 8: oral vit K
management of warfarin w bleed (incl dose)
stop warfarin
5-10mg IV vit K
+ prothrombin complex (e.g. beriplex) if major bleed
signs of hyposplenism on blood film
howell-jolly bodies
target cells
pappenheimer bodies (post-splenectomy)
complications + management of splenectomy
haemorrhage
pancreatic fistula
infection esp encapsulated bacteria: NHS (neisseria, haemophilus, strep)
management:
immunisations: Hib, men A+C, annual influenza, 5yrly pneumococcal
penicillin V
features of HUS
triad:
typically following E.coli gastroenteritis
1. renal failure (N&V, fluid retention, oliguria, haematuria, proteinuria)
2. MAHA (anaemia, jaundice, schistocytes)
3. thrombocytopenia (bruising)
rain-drop skull on XR
multiple myeloma
investigations for multiple myeloma
urine sample → Bence-Jones protein bloods: normocytic anaemia, blood profile (normal ALP), raised Ca, deranged U&Es (renal failure), rouleax formation on blood film skeletal X-ray serum electrophoresis → paraprotein bone marrow aspirate → plasma cells
disseminated intravascular coagulation
FBC + clotting + blood film findings
anaemia (normocytic), thrombocytopenia prolonged aPTT + PT reduced fibrinogen increased fibrin degradation products + D-dimer schistocytes on blood film
investigations haemochromatosis
bloods: FBC, iron studies (↑iron, ↑ferritin, ↑transferrin, ↓TIBC)
genetic testing: HFE
MRI liver + biopsy → Perl’s / prussian blue stain
Auer rods
AML
myeloblast granules +ve for Sudan black staining
AML
Philadelphia chromosome (t9:22), Abelson-BCR
CML
smudge / smear cells
CLL
causes of autoimmune haemolytic anaemia (AIHA)
cold (IgM): mycoplasma, mononucleosis, HIV, lymphoma
warm (IgG): CLL, SLE
causes of B12 deficiency
reduced intake e.g. in vegetarians reduced absorption (terminal ileum): IBD (esp Crohn's) pernicious anaemia: Ab against intrinsic factor (Schilling test) gastrectomy / ileal resection
presentation B12 deficiency
signs of anaemia glossitis mixed UMN + LMN signs of LL: spastic weakness, areflexia, extensor plantars (Babkinski +ve) peripheral neuropathy cerebellar signs: ataxia, +ve Romberg's
causes polycythaemia
primary: polycythaemia rubra vera (JAK2 kinase mutation)
secondary:
to hypoxia: COPD, chronic lung disease, high altitude
inappropriate production: renal cell carcinoma, erythropoietin abuse in athletes
presentation polycythaemia
pruritus after hot bath splenomegaly (massive) plethoric face erythromelalgia: tenderness/redness/painful burning of fingers, palms, toes, heels choreiform movements thrombosis headache dizziness night sweats
clinical features multiple myeloma
CRAB: hypercalcaemia (causing low PTH) renal impairment anaemia bone lesions (osteolytic: resorption by activated osteoclasts) \+ immunodeficiency
multiple myeloma vs MGUS
MM: presence of paraprotein in urine / serum (electrophoresis)
bone marrow monoclonal cells > 10%
end-organ damage (hypercalcaemia, raised Cr, anaemia, bone lesions, osteoporosis, pathological fractures)
MGUS:
paraprotein < 30g/L
bone marrow monoclonal cells < 10%
no end-organ damage
definition multiple myeloma
malignancy disease of clonal B cells resulting in bone marrow failure / destruction + overproduction of monoclonal paraprotein (bence jones proteins in urine)
