Haematology Flashcards
which Ig is raised in acute infection
IgM
what are the iron storage compounds
hemosiderin (tissues) and ferritin (blood)
what inhibits iron absorption from small bowel? where is it secreted from?
hepcidin
liver
what can cause iron overload?
haemochromatosis (low hepcidin)
thalassaemia
excess intake - diet, oral therapy, transfusions
what does a low mean cell HB (MCH) suggest?
hypochromic anaemia
- iron deficiency, thalassaemia, AOCD
what does a high//low reticulocyte count suggest
high: haemolytic anaemia, active bleed
low: impaired RBC production (kidney failure, chronic disease, aplasia - BM disease)
causes of microcytic anaemia
hypochromic: iron deficiency / sideroblastic / thalassaemia
normochromic: chronic disease
blood findings in anaemia of chronic disease
microcytic
normochromic
low reticulocytes
investigations in iron deficient anaemia
microcytosis
low iron and ferritin
GI investigations - endoscopy, colonoscopy
management of iron deficient anaemia
oral ferrous sulfate aiming for >10 Hb increase per week
continue for 3/12 after return to normal level
may use IV in kidney disease/ poor absorption
how much should 1 unit RBCs increase Hb?
10g/L
causes of sideroblastic anaemia
congenital / dysplasia - myeloma / MDS
blood findings in sideroblastic anaemia
high iron and ferritin(cant be made into Hb)
low Hb
low haematocrit
hypochromic
causes of normocytic anaemia
aplastic
haemolytic: SCA, GPD6, autoimmune, infection
what is aplastic anaemia, presentation and management
normocytic anaemia
pancytopenia
infections , anaemia, thrombocytopenia - bleeding and bruising
neutropenic regimen and transfusions
blood findings in haemolytic anaemia
raised reticulocytes, bilirubin and LDH
normocytic anaemia with raised reticulocytes and coombs test +ve? treatment
acquired immune mediated haemolytic anaemia
IgG = warm type - steroids
IgM = cold type - keep warm
haemolytic anaemia , coombs test -ve ?
non immune mediated anaemia
infection, trauma, DIC, TTP, HUS, liver disease
haemolytic anaemia with heinz bodies on blood films? causes?
GPD6 deficiency
x linked - triggered by aspirin, antimalarials, cephalosporins, fava beans, infection
management of GPD6 deficieny
folic acid
normoblastic causes of macrocytic anaemia
pregnancy, liver disease, myelodysplasia, hypothyroidism
causes of megaloblastic macrocytic anaemia
pernicious anaemia, B12 defic, folate defic
a macrocytic megaloblastic anaemia without neuro signs is due to what? (+hypersegmented neutrophils)
folate deficiency
causes of folate deficiency, symptoms and treatment ?
malnutrition, alcoholism, bowel disease/malabsorption, pregnancy, methotrexate, trimethoprim, antiepileptics
macrocytic anemia, weight loss, anorexia, tachycardia
PO folic acid for 4 months (correct B12 first)
should you correct B12 or folic acid first ? and Why?
b12
subacute degeneration of the cord
what can cause B12 deficiency?
malnutrition, alcoholism, malabsorption, pernicious anaemia
presentation of B12 deficiency?
macrocytic anaemia lemon skin - jaundice and pallor posterior column degeneration - upgoing plantars, loss of reflexes paresthesia ataxia sub acute degeneration of the cord
triad of symptoms in B12 deficinecy
anemia
neuropsych
peripheral neuropathy
management of B12 deficieny
IM hydroxocobalamin + folic acid
investigations in pernicious aneamia
intrinsic factor Ab, antiparietal cell Ab serum gastrin (raised) schillings test - B12 uptake scan
what are the myeloproliferative disorders
polycythaemia (RBCs)
CML (WBCs)
myelofibrosis (stroma)
management of sickle cell bone crisis
cross match blood analgesia O2 warm fluids Abx blood transfusion
management of sickle cell chest crisis
oxygen spirometry broad spec Abx transfusion fluids
diagnosis of sickle cell
new born blood screen
presents around 4 months - howell jolley bodies on film and Hb electrophoresis
male toddler bleeding into muscle and joints?
haemophilia
teenager having mucocutaneous bleeding +/- menorrhagia?
von willebrand’s disease
will APTT be normal/high/low inhameophillia?
increased
management of hemophilia A and B
A: recombinant factor 8
B: recombinant factor 9
management of VWD
recombinant factor 8
young woman with mucocutaneous bleeding and solitary low platelets? management
immune thrombocytopenia
IVIg , prednisolone, platelets
pentad in thrombotic thrombocytopenic purpura
- thrombocytopenia,
- microangiopathic haemolytic anaemia,
- fever,
- renal abnormalities,
- neurological abnormalities
presentation of thrombotic thrombocytopenic purpura
child with prodrome of bloody diarrhoea (e. coli/ shigella)
fever, AKI, anaemia, thrombocytopenia
ill patient with generalised bleeding for >3 sites? management?
DIC
ICU, platelets, FFP
investigations in DIC
decreased platelets, fibrinogen, clotting factors (5&8)
increased PT, aPTT, D-dimer
causes of acquired thrombophilia
antiphospholipid syndrome
OCP
pregnancy
causes of inheritied thrombophilia
factor V leidin protein C defic protein S defic antithombin defic prothrombin mutation
what should be screened for in unprovoked VTE?
factor V leidin protein C defic protein S defic antithombin defic prothrombin mutation
how do you reverse warfarin?
vitamin k - takes 24h
prothrombin complex
which drugs affect platelet function and which affect coagulation cascade?
platelets: aspirin and clopidogrel
coagulation: warfarin (factors 2,7,9,10) heparin
causes of massive splenomegaly
CML
malaria
myelofibrosis
what is factor V leiden?
activated protein C deficiency
how does thalassemia present?
microcytic anaemia
skull bossing
hepatosplenomegaly
diagnosis of thalassaemia
Hb electrophoresis
how does leukemia present?
pancytopenia - infections, anaemia, bleeding/ bruising
what do you see on peripheral film in leukaemia
blast cells
most common leukaemia in children? investigations?
acute lymphoblastic
CXR and CT - mediastinal widening
LP - CNS involvement
management of ALL
chemo (remission inducing, consolidation, maintenance - 2 years)
CNS prophylaxis - methotrexate
allogenic bone marrow transplant
most common form of leukaemia in adults? investigations?
chronic lymphocytic anaemia
raised lymphocytes
peripheral film - smudge and smear cells
CT - binet staging
management of CLL
stage A-B: WW
C-D: chemo and BM transplant
what haematological malignancy is associated with the philadelphia chromosome?
chronic myeloid leukaemia
investigations and management in CML?
FISH cytogenetics - philadelphia
BM biopsy - hypercellular/ hypergranular
imatinib - tyrosine kinase i
what is AML associated with?
auer rods on biopsy
rapidly progressive
what can trigger hodgkins lymphoma
EBV, SLE, RA
what differentiates HL from NHL
HL has reed sternberg cells
staging in lymphoma?
Ann Arbour
some examples of non hodgkin’s lymphoma and triggers
MALT - H pylori diffuse B cell - HIV Burkitt's - EBV also primary CNS and follicular other triggers: SLE, RA, sjogren's, coeliac
diagnosis should be queried in unresolving back pain
multiple myeloma
investigations when ?multiple myeloma
ESR, blood film, electrophoresis
diagnosis of multiple myeloma
monoclonal proteins in blood/urine (bence jones) on electrophoresis
increased plasma cells on BM biopsy
end organ damage (kidney, hypercalcaemia, anaemia)
osteolytic bone lesions
management of multiple myeloma
manage the symptoms chemo dexamethasone bone marrow transplant VTE prophylaxis plasmapheresis
monitor 6/12 with urine
osteoporosis + raised ESR is what until proven otherwise?
multiple myeloma
