Haematology Flashcards
B symptoms are a sign of…
what cancer? (1)
what prognosis? (1)
lymphoma
poor prognosis
What are the B symptoms in Hodgkins lymphoma? (3)
weight loss > 10% in last 6 months
fever > 38ºC
night sweats
Afro-carribeans have worse prognosis of what haematological cancer
ALL
Hodgkins lymhpoma:
what cells
malignant proliferation of lymphocytes characterised by the presence of the Reed-Sternberg cell.
Vitamin B12 and folate deficiency together - what is important to do in treatment?
treat B12 first otherwise –> subacute combined degeneration of spinal cord
where is B12 absorbed? (1)
what condition should you consider this in? (1)
terminal ileum (impacted in Crohns/ following resection)
most common INHERITED thrombophilia
factor 5 leiden (–> clotting!/ VTE)
most common ACQUIRED thrombophilia
antiphospholipid syndrome
Antiphospholipid syndrome in pregnancy:
how to manage? (2)
aspirin + LMWH
(low-dose aspirin should be commenced once the pregnancy is confirmed on urine testing,
low molecular weight heparin once a fetal heart is seen on ultrasound. This is usually discontinued at 34 weeks gestation)
a reversal agent for dabigatran (used to treat AF)
idarucizumab (monoclonal antibody)
vWD:
management if bleeding (1)
other possibles (2)
tranexamic acid for mild bleeding
desmopressin (DDAVP): raises levels of vWF by inducing release of vWF from Weibel-Palade bodies in endothelial cells
factor VIII concentrate (F8 can be low)
vWD:
types (3)
type 1: partial reduction in vWF (80% of patients)
type 2*: abnormal form of vWF
type 3**: total lack of vWF (autosomal recessive)
ITP (Immune (or idiopathic) thrombocytopenic purpura (ITP)):
trigger (1)
investigations (2)
management (2)
immune reaction –> low platelets post infection/ vaccination
FBC
blood film (bone marrow no longer needed)
1st line= prednisolone
2nd= pooled normal human immunoglobulin (IVIF) to raise count if urgent or active bleeding
Evan’s syndrome
ITP in association with autoimmune haemolytic anaemia (AIHA)
major criteria determining the use of cryoprecipitate in bleeding (1)
what does it contain? (3)
fibrinogen level
Contains: fibrinogen, factor VIII, and von Willebrand factor
when to give Fresh frozen plasma (FFP) (specific ratio)
prothrombin time (PT) ratio or activated partial thromboplastin time (APTT) ratio > 1.5
When to use prothrombin complex concentrate
A REVERSAL
emergency reversal of anticoagulation in severe bleeding or a head injury with suspected intracerebral haemorrhage
Can be prophlyactic if emergancy surgery
Fever, abdominal pain, hypotension during a blood transfusion → what should you be concerned about? (1)
acute haemolytic reaction
Blood transfusion complications
immunological: acute haemolytic, non-haemolytic febrile, allergic/anaphylaxis
infective
transfusion-related acute lung injury (TRALI)
transfusion-associated circulatory overload (TACO)
other: hyperkalaemia, iron overload, clotting
Non-haemolytic febrile reaction:
main features (2)
what to do (1)
fever+ chills
stop/ slow transfusion and paracetamol
Thought to be caused by antibodies reacting with white cell fragments in the blood product and cytokines that have leaked from the blood cell during storage
Minor allergic reaction
features
management
itch urticaria
stop+ histamine + monitor
Anaphylaxis:
features (4)
management (1)
hypotension, SOB, wheeze, angioedeam
stop+ adrenaline+ABC
Acute haemolytic reaction
what is it? (1)
Sx? (3)
test to confirm? (1)
management? (1)
ABO incompatable
abdo pain+ hypotension+ fever
STOP
confirm with Coombs test
fluid resuscitation (supportive care)
Transfusion-associated circulatory overload (TACO)
PO due to overload
diuretics
Transfusion-related acute lung injury (TRALI)
Non-cardiogenic pulmonary oedema thought to be secondary to increased vascular permeability caused by host neutrophils that become activated by substances in donated blood
Hypoxia, pulmonary infiltrates on chest x-ray, fever, hypotension
complications acute haemolytic reaction (2)
renal failure and DIC
Post thrombotic syndrome:
what is it? (1)
management (1)
venous problems post DVT
compression stockings
(only offered if post thrombotic not for DVT routienly)
do you rescan patients post DVT
no!
DIC typical picture
plaetles
fibrinogen
APTT and PT
D Dimer
↓ platelets
↓ fibrinogen
↑ PT & APTT
↑ fibrinogen degradation products
schistocytes due to microangiopathic haemolytic anaemia
causes of DIC (4)
sepsis
trauma
obstetric complications e.g. aminiotic fluid embolism or hemolysis, elevated liver function tests, and low platelets (HELLP syndrome)
malignancy
Prothromibin time, APTT, bleeding time and platelet count of:
warfarin
aspirin
heparin
DIC
Warfarin- prolonged PT but rest normal
Aspirin- prolonged bleeding time but rest normal
Hepatin prolonged APTT but rest normal
DIC: prolonged everything and low platelets
Tonsilitis and neutropenia
Epstein-Barr virus may result in neutropaenia
(infectious mononucleosis)
An increase in the number of circulating activated T and B lymphocytes seen in the virus causes lymphocytosis.
Neutropenia:
what classes mild/ moderate/severe? (3)
mild= 1-1.5
moderate= 0.5-1
severe= <0.5
neutropenia causes:
viral (3)
drugs (3)
ethnic (1)
haematological (1)
other (4)
viral
HIV
Epstein-Barr virus
hepatitis
drugs
cytotoxics
carbimazole
clozapine
benign ethnic neutropaenia
common in people of black African and Afro-Caribbean ethnicity
requires no treatment
haematological malignancy
myelodysplastic malignancies
aplastic anemia
rheumatological conditions
systemic lupus erythematosus: mechanisms include circulating antineutrophil antibodies
rheumatoid arthritis: e.g. hypersplenism as in Felty’s syndrome
severe sepsis
haemodialysis
Hodgkins: High potassium, high phosphate, and low calcium - what causes this (1)
prophylaxis for this? (1)
who to suspect in? (1)
timor lysis syndrome (post chemo) due to the cancer cells breaking down
ALLOPURINOL
suspect in any patient with AKI and high phopshate/ uric acid level
Tumor lysis syndrome:
how to treat
IVF
rasburicase (higher risk)
alluring (lower risk)
rasburicase and allopurinol should not be given together in the management of tumour lysis syndrome as this reduces the effect of rasburicase
Definition tumor lysis syndrome
laboratory tumour lysis syndrome plus one or more of the following:
increased serum creatinine (1.5 times upper limit of normal)
cardiac arrhythmia or sudden death
seizure
‘tear drop’ poikilocytes on blood film causes (3)
thalassaemia, megaloblastic anaemia and myelofibrosis.
(the bone is all fibrosed so the blood cells have to squeeze out - making them tear drop shaped)
tear drop’ poikilocytes + weight loss/ fatigue + large spleen
myelofibrosis
reduces the ability of the bone marrow to produce normal cells, thus causing thrombocytopenia, anaemia and extramedullary haematopoiesis in the spleen.
Cryoprecipitation contains
factor VIII, fibrinogen, von Willebrand factor and factor XIII
Indications include massive haemorrhage and uncontrolled bleeding due to haemophilia
When to do exchange transfusion in sickle cell
if neurological complications
Long term management sickle cell (2)
Hydroxyurea (prophylaxis)
Pneumococcal polysaccharide vaccine every 5 years
A grossly elevated APTT (3)
may be caused by heparin therapy, haemophilia (bleeding) or antiphospholipid syndrome (clotting)
Haemophilia A and B factors
A= Factor VIII
B= Factor IX
blood tests haemophilia - what is elevated? (1)
prolonged APTT, rest normal
Lymphoma: why special irradiated blood products?
Irradiated blood products are often treated with gamma or x-ray radiation to deplete T-lymphocytes in order to prevent transfusion-associated graft versus host disease
Blood film in hyposplenism
target cells
Howell-Jolly bodies
Pappenheimer bodies
siderotic granules
acanthocytes
common cause of hyposplenism
Coeliac disease
Iron def anaemia:
what do the cells look like? (2 items)
if combined with B12/folate def? (1)
target cells
‘pencil’ poikilocytes
if combined with B12/folate deficiency a ‘dimorphic’ film occurs with mixed microcytic and macrocytic cells
Sickle cell crisis:
Thrombotic crisis what is it?
also known as painful crises or vaso-occlusive crises
precipitated by infection, dehydration, deoxygenation (e.g. high altitude)
painful vaso-occlusive crises should be diagnosed clinically - there isn’t one test that can confirm them although tests may be done to exclude other complications
infarcts occur in various organs including the bones (e.g. avascular necrosis of hip, hand-foot syndrome in children, lungs, spleen and brain
Sickle cell:
what is acute chest syndrome
vaso-occlusion within the pulmonary microvasculature → infarction in the lung parenchyma
dyspnoea, chest pain, pulmonary infiltrates on chest x-ray, low pO2
management
pain relief
respiratory support e.g. oxygen therapy
antibiotics: infection may precipitate acute chest syndrome and the clinical findings (respiratory symptoms with pulmonary infiltrates) can be difficult to distinguish from pneumonia
transfusion: improves oxygenation
the most common cause of death after childhood
Sickle cell:
what is aplastic crisis? (1)
incidator on bloods? (2)
infection with parvovirus
sudden Hb cfall
bone marrow supprossion–> reduced reticule count
Sequestriation crisis
Spleen or lungs
increased reticulocyte count
What blood film associated with DIC?
schistocytes
splenomegaly:
causes (5)
myelofibrosis
chronic myeloid leukaemia
visceral leishmaniasis (kala-azar)
malaria
Gaucher’s syndrome
Myeloid leukaemia vs lymphoid leukaemia
Myeloid: neutrophils
Lymphoid: lymphocytes
ALL –> CML –> AML –> CLL
acute= immature blast cells
chronic= mature cells
ALL (Tdt)–> CML (Philadelphia chromosome, splenomegaly) –> AML (Auer rods)–> CLL
AML cells
Auer rods (large uncles with rods)
(A)uer rods and (M)yeloperoxidase positive
Why is CML a problem
if it progresses to AML
(AML in 80%, ALL in 20%)
CML treatment
- imatinib is now considered first-line treatment
inhibitor of the tyrosine kinase associated with the BCR-ABL defect
very high response rate in chronic phase CML - hydroxyurea
- inferno alpha
- allogenic bone marrow transplant
Raised haemoglobin, plethoric appearance, pruritus, splenomegaly, hypertension
polycythaemia vera
Polycythaemia vera mutation
JAK2
Iron studies in iron deficiency anaemia
↓ Ferritin, ↑ total iron-binding capacity, ↓ serum iron, ↓ transferrin saturation
The total iron-binding capacity (TIBC) increases as the body produces more transferrin (the iron transport protein) to maximise iron uptake. Serum iron levels fall due to depleted stores. Transferrin saturation, which is calculated as (serum iron ÷ TIBC) × 100, will be low due to the combination of low serum iron and high TIBC.
Transferrin is like a delivery truck that carries iron around your body. When you need more iron, there are more delivery trucks on the road. If you have too much iron, there are fewer trucks driving around. TIBC (Total Iron Binding Capacity) is like the number of seats on the delivery trucks. If you need more iron, you add more seats to the trucks. If you have too much iron, the seats on the trucks are mostly empty.
How is myeloma diagnosed
urine protein electrophoresis and serum-free light-chain assay
Rouleaux formation blood film
3-month history of tiredness, back pain and both increased urination and thirst. The GP requests a urine protein electrophoresis and a serum-free light-chain assay, which confirm the diagnosis.
myeloma (rouleaux formation)
Multiple myeloma CRAB features
‘CRAB’ features of multiple myeloma = hyperCalcaemia, Renal failure, Anaemia (and thrombocytopenia) and Bone fractures/lytic lesions
Hb transfusion threshold for patients with ACS
80
(without ACS= 70)
most aggressive hodgkin lymphoma
Lymphocyte-depleted Hodgkin lymphoma
what type of anaemia is sickle cell disease?
normocytic anaemia with raised reticulocyte count - due to haemolysis
Cancer patients with VTE
- 6 months of a DOAC
beta-thalassaemia major management (1)
what to give alongside (1)
repeated transfusion
this leads to iron overload → organ failure
iron chelation therapy is therefore important (e.g. desferrioxamine)
what is behcets syndrome
a rare and chronic condition that causes inflammation of the blood vessels (vasculitis) throughout the body. It can lead to numerous symptoms, including painful mouth sores, genital sores, and eye inflammation. it can increase the risk of venous thromboembolism due to the inflammation of the blood vessels. This inflammation can cause damage to the vessel walls leading to activation of the coagulation cascade and the formation of blood clots.
Iron studies and infection: what to remember
Ferritin HIGH in infection REGARDLESS of iron status
microcytic aneamia causes (5)
Causes
iron-deficiency anaemia
thalassaemia*
congenital sideroblastic anaemia
anaemia of chronic disease (more commonly a normocytic, normochromic picture)
lead poisoning
A question sometimes seen in exams gives a history of a normal haemoglobin level associated with a microcytosis. In patients not at risk of thalassaemia, this should raise the possibility of polycythaemia rubra vera which may cause an iron-deficiency secondary to bleeding.
The antidote for heparin
protamine sulfate.
sickle cell crisis main management
analgesia, oxygen, and IV fluids (+ consider Abx if infection and transfusion if Hb low)
Wells score DVT Cut off
If a 2-level DVT Wells score is ≥ 2 points then arrange a proximal leg vein ultrasound scan within 4 hours
Paraproteinaemia:
benign causes (2)
malignant causes (4)
Benign paraproteinaemia:
Monoclonal gammopathy of undetermined significance (MGUS)
Transient paraproteinaemia (e.g., following an infection)
Malignant paraproteinaemia:
Multiple myeloma
Waldenstrom macroglobulinemia
Primary amyloidosis (AL)
B-cell lymphoproliferative disorders (e.g., chronic lymphocytic leukaemia, non-Hodgkin lymphoma)
Oaraproteinuriea
Clinical features
Hyperviscosity syndrome
Neuropathy (e.g., sensory, motor, or autonomic dysfunction)
Renal dysfunction
Hematologic abnormalities (e.g anaemia, thrombocytopenia, or leukopenia)
Bone pain or pathologic fractures (in the context of multiple myeloma)
intense itching which usually occurs after exposure to hot water or hot and humid weather.
Polycythaemia vera is a neoplasm of the bone marrow which results in the production of excessive red blood cells. The classic symptom of this condition is intense itching which usually occurs after exposure to hot water or hot and humid weather. It is believed that this is due to abnormal histamine or prostaglandin production.
Polycythaemia:
primary (1)
relative (2)
secondary (4)
polycythaemia rubera vera
relative: stress/ dehydration
secondary: COPD, altitude, sleep apnoea, excessive erythropoietin
–> look at blood cell mass to tell
most common cause of: prolonged APTT (Activated Partial Thromboplastin Time) but normal PT (Prothrombin Time) and platelet count
Haemophilia A (factor VIII) MORE common than factor B (IX)
inheritance haemophilia A/B
X-linked recessive disorder of coagulation
AP and APTT
PT signifies the extrinsic pathway (Play Tennis Outside=EXtrinsic). Since the extrinsic pathway has only factor VII which is rarely deficient in isolation, PT would be a more generalised test for clotting (liver disease, DIC, warfarin, Vit K deficiency)
APTT signifies the intrinsic pathway (Play Table Tennis Inside= INtrinsic). This consists of factors VIII, IX and XI.
Haemophilia A- Factor VIII (X linked recessive)
Haemophilia B- Factor IX (X linked recessive)
Haemophilia C- Factor XI (autosomal recessive)
Von Willebrand as vWF binds to factor VIII
EXCEPTION- antiphospholipid syndrome can cause increase APTT despite being a clotting disorder
Sickle cell crisis + fever = what to do?
admit urgently as increased risk overwhelming sepsis by encapsulated organisms
When do you do exchange transfusion in sickle cell?
indications include: acute vaso-occlusive crisis (stroke, acute chest syndrome, multiorgan failure, splenic sequestration crisis
(rapidly reduce the percentage of Hb S containing cells)
Which blood products have the highest risk of bacterial§ contamination?
platelets
Raised haemoglobin, plethoric appearance, pruritus, splenomegaly, hypertension
Polycythaemia vera
polycythaemia vera - mutation
JAK2 mutation
‘CRAB’ features of multiple myeloma
hyperCalcaemia, Renal failure, Anaemia (and thrombocytopenia) and Bone fractures/lytic lesions
Lymphocyte-depleted Hodgkin lymphoma - aggressive or protective?
most aggressive
sickle cell anaemia FBC
low Hb
normal MCV
raised reticulocytes
hereditary spherocytosis
a genetic disorder that affects red blood cells, causing them to become spherical instead of their normal biconcave shape –> more fragile
(most common hereditary haemolytic anaemia in people of northern European descent)
autosomal dominant
the normal biconcave disc shape is replaced by a sphere-shaped red blood cell
presentation of hereditary sperocytosis
failure to thrive
jaundice, gallstones
splenomegaly
aplastic crisis precipitated by parvovirus infection
degree of haemolysis variable
MCHC elevated
acute haemolytic crisis:
treatment is generally supportive
transfusion if necessary
longer term treatment:
folate replacement
splenectomy
Important when interpreting FERRITIN result
goes up in infection
APML bone marrow
Auer rods
commonest cause of perennial allergic rhinitis
house dust mite
hyposensitisation is of proven benefit in..
wasp venom anaphylaxis
bruises, recurrent infection and anaemia..
Aplastic anaemia
bone marrow failure –> pancytopenia and marrow hypoplasia
aplastic anaemia causes
autoimmune
drug related (antibiotics, phenytoin, chemo…)
viruses (parvovirus, EBV)
irradiation
inherited
aplastic crisis in sickle cell - how to identify
infection with parvovirus
sudden drop in Hb
reticulocytes won’t be raised (but should be as this is a sign the body is trying to make more cells)
if renal impairment is severe (e.g. < 15/min) what anticoagulant should be used for VTE
LMWH, unfractionated heparin or LMWH followed by a VKA
in antiphospholipid syndrome what anticoagulant to give
LMWH followed by a VKA
dyspnoea, chest pain, cough, hypoxia and new pulmonary infiltrates seen on chest x-ray
BG of known anaemia
sickle cell disease
myeloma features - CRABBI: and what else?
CRABBI:
calcium, renal, anaemia, bleeding, bones, infection
amyloidosis e.g. macroglossia
carpal tunnel syndrome
neuropathy
hyperviscosity
X-rays: ‘rain-drop skull’ vs ‘pepper pot skull’
RAIN DROP = myeloma
PEPPER POT= hyperparathyroidism
imaging in myeloma
whole body MRI to look for boney lesions
diagnostic criteria myeloma
One major and one minor criteria or three minor criteria:
Major criteria
Plasmacytoma (as demonstrated on evaluation of biopsy specimen)
30% plasma cells in a bone marrow sample
Elevated levels of M protein in the blood or urine
Minor criteria
10% to 30% plasma cells in a bone marrow sample.
Minor elevations in the level of M protein in the blood or urine.
Osteolytic lesions (as demonstrated on imaging studies).
Low levels of antibodies (not produced by the cancer cells) in the blood.
most common ACQUIRED vs INHERITED thrombophilia
ACQUIRED= antiphospholipid syndrome
INHERITED= factor V leiden
Thrombotic crisis in sickle cell are also known as…
also known as painful crises or vaso-occlusive crises
precipitated by infection, dehydration, deoxygenation (e.g. high altitude)
painful vaso-occlusive crises should be diagnosed clinically - there isn’t one test that can confirm them although tests may be done to exclude other complications
infarcts occur in various organs including the bones (e.g. avascular necrosis of hip, hand-foot syndrome in children, lungs, spleen and brain
how does thrombotic crisis differ from haemolytic in terms of blood levels
haemolytic will have drop in Hb
post splenectomy: what infections at risk of
ENCAPSULATED bacteria
SHiN SKiS
Streptococcus pneumonia
Haemophilus influensza
Neisseria meningitidis
group B Strep
Klebsiella
Salmonella typi
THATS WHY THEY THEY VACCINES
for meningitis, influenza, HiB and pneumococcal
megaloblastic anaemia
B12
terminal ileum resection
gastrectomy
folate deficiency
drugs - sulfonamides
normoblastic anaemia
hypothyroid
alcohol
liver diease
myelodysplasia
drugs- anticonvulsants
Heinz bodies - what does it indicate
G6PD deficiency
commonest cause eosinophilia in UK
asthma
difference of active vs latent TB treatment
Active = RIPE (rifampicin, isonizaid, pyrazinamide, ethambutol)
Latent = RI
microcytic anaemia
iron def
thalamassaemia
sideroblastic anaemia
pica
during blood transfusion: fever, chills, nausea, burning at cannula site, chest tightness, restless, joint and back p[ain
acute haemolytic transfusion reaciton
collapse during blood transfusion
tachycardia
hypotensive
temp 40oC
bacterial contaminationH
“collapsing attacks” where fall to ground
legs feel stiff and weak
unable to reduce walking without period of rest
anxious, emotional and aggressive at these times
skin rash on legs and face
new diet
Hartnup disease
rare genetic disorder
inborn abnormality of renal and intestinal transport
(similar to pellagra-like dermatitis and neurological involvement)
myeloma is malignant disease of which CELL type
plasma cell
8yo Caucasian
mild aenamia
jaundic
normal MCV but red cell osmotic fragility increased
congenital sperocytosis
(Coomsb -ve)
can –> gallstone and aplastic crisis (parvoviral infection B19)
AFP is a tumor marker for
hepatocellular carcinoma
nonseminomatous germ cell tumor
B-hCG is a tumor marker for
nonseminomatous germ cell tumor
gestational trophoblastic disease
