Haematology Flashcards
What is von Willebrand’s disease
A disease characterised by a lack of vWF, which leads to a reduced life of factor 8
Sx von Willebrand’s disease
Sx include epistaxis and menorrhagia but many people are asymptomati
Mx von Willebrand’s disease
TXA and COCP
Sx haemophilia
haematoma, haemarthrosis, melaena
Inheritance pattern of haemophilia
X-linked recessive
Mx haemophilia
Give deficiency factor and reduce bleeding risk (no NSAIDs or IM injections
Difference between haemophilia A and B
A is deficient factor 8, B is 9
blood results for haemophilia
Long APTT but everything else normal
What kind of anaemia is anaemia of CKD?
Normocytic and normochromic
Why does CKD give you anaemia?
Reduced EPO means no RBC made
Is there raised reticulocyte count in anaemia of CKD?
No
Mx anaemia of chronic kidney disease
Treat underlying disorder or give synthetic EPO in CKD
Causes of megaloblastic macrocytic anaemia
FOlic acid/b12 deficiency
Causes of folic acid deficiency
Coeliac, pregnancy
How do you treat megaloblastic macrocytic anaemia
Supplement deficiency vitamin
In which order should you give folate and b12 supplements?
B12 then folate
What do folic acid and b12 normally do
mature and condense RBC DNA = large immature cells
causes of b12 deficiency
gastrectomy
coeliac
number 1 is pernicious anaemia
What is pernicious anaemia
autoimmune destruction of parietal cells = no IF
treatment for pernicious anaemia
tydroxycobalamin
sx b12 deficiency
damage to dorsal columns = peripheral neuropathy
causes of normoblastic macrocytic anaemia
alcohol, pregnancy, myeloma, hypothyroidism, reticulocytosis
With which disease would you associate anti-dsDNA?
SLE
With which disease would you associate anti-mitochondrial antibodies?
primary biliary sclerosis
With which disease would you associate anti-smooth muscle antibodies
chronic hepatitis
With which disease would you associate pANCA
ulcerative colitis
With which disease would you associate anti-transglutaminase antibodies
coeliac
With which disease would you associate anti-Ro and anti-La antibodies
Sjogren’s
CHICAGO causes of splenomegaly
cancer haematological malignancies - anaemia, leukaemia, lymphoma infections - HIV, TB, malaria, EBV congestion - portal hypertension autoimmune (SLE and RA) glycogen storage disorders other - amyloidosis and sarcoidosis
From which cells do giant cells originate
Macrophages
Microcytic anaemia associated with decreased ferritin levels
Iron deficiency anaemia
Causes of iron deficiency anaemia
Mal-absorption (PPIs)
Poor diet
Blood loss
Tx iron deficiency anaemia
Ferrius sulphate
What type of anaemia is anaemia of chronic diseasw
Microcytic anaemia + normal/high ferritin
Blood test results for anaemia of chronic disease
Normal/high ferritin and raised CRP
What type of anaemia is sideroblastic anaemia?
Microcytic + normal/high ferritin
Cause of sideroblastic anaemia
Anti-TB drugs and lead poisoning
What kind of anaemia is thalassaemia?
Microcytic + high/normal ferritin
what kind of anaemia is aplastic anaemia?
normocytic with low/normal reticulocytes
sx aplastic anaemia
pancytopenia
it is a symptom of ALL and AML
Causes of aplastic anaemia
idiopathic - benzenes, hepatitis, phenytoin
Fanconi syndrome
What is fanconi anaemia
skeletal abnormalities, aplastic anaemia and cafe au last spots
what kind of anaemia is caused by blood loss
normocytic with high reticulocytes
what kind of anaemia is haemolytic anaemia
normocytic with high reticulocytes
blood results of haemolytic anaemia
high lactate dehydrogenase, reticulocytes and bilirubin
causes of intravascular haemolytic anaemia
mechanical heart valve
TTP, G6PD deficiency
Signs of G6PD deficiency
Heinz bodies = inflexible RBCs
causes of extravascular haemolytic anaemia
sickle cell, spherocytosis
what is spherocytosis
Defective RBCs are spherical - they can obstruct spleen and cause vaso-occulative crisis
test for spherocytosis
Coombs
function of thrombopoeitin
stimulate platelet creation
does prothrombin measure intrinsic or extrinsic system
extrinsic
which system does aPTT measure
intrinsic
Dabigatran MOA
inhibits thrombin
What does thrombin normally do?
Convert fibrinogen to fibrin to form a clot
How do you reverse dabigatran
idarucizumab
TXA MOA
inhibits plasminogen (used in menorrhagia)
Plasminogen MOA
plasminogen -> plasmin -> breaks down clot
rivaroxaban/apixaban MOA
inhibit factor 10 a
What does 10a normally do?
activate thrombin
what temp should RBCs be kept at before infusion
4c
Why is irradiated blood goof?
no t-lymphocytes
where does the tip of the tongue drain to
submental LNs
Which structures drain to the para-aortic LNs
testes/ovaries, kidneys, adrenal glands
Which structures drain to the superficial inguinal LNs
anal canal below pectinate line scrotum glans penis vagina perineum skin of thigh
which structures drain to deep inguinal LNs
glans penis
which structures drain to the axillary Lns
breast and upper limb
which structures drain to the superior mesenteric LNs
jejunum and duodenum
which structured drain to the internal iliac LNs
cervix, anal canal above pectinate line, lower rectum
MOA GVHD
T cells in donor tissue mount immune response against host cells
What type of hypersensitivity is GVHD
4
tx GVHD
steroids
acute GVHD
<100 days of transplant, painful and macropapular rash, jaundice, D/N/V
chronic GVHD
> 100 days of transplant, conjunctivitis, dysphagia, ulcers, lung disease, skin irritation
TTP
Deficiency of ADAMTS13 (normally cleaves vWF) results in large clumps of vWF which causes clumps of platelets
Sx TTP
fever, haemolytic anaemia, thrombocytopenia, renal failure
causes TTP
UTI, pregnancy, COCP, acyclovir, SLE, HIV
what is likely to be found in LN if sarcoidosis
asteroid bodies
sickle cell Hb
HbS HbS
anti-coagulant to use in pregnancy if at high risk of VTE
LMWH
