MRCP Haem Flashcards
Triad:
Haemolytic anaemia
pancytopenia
Large vessel thrombosis
- Dark urine
- microscopic haematuria
- no red cell in microscope
PNH
paroxysmal nocturnal haemoglobinuria
intravascular haemolysis
associated with VTE
Mx: eculizumab monoclonal antibody inhibit complement
Ferritin
Normal- not iron deficient
In inflammation may mask IDA
Beta thalassaemia
trait
- microcytic anaemia, raised HbA2
major
- no beta globulin, chr 11, microcytic anaemia, raised HbA2 and HbF, absent HbA
NHL
painless LN
B symptoms
cytopenia
extranodal disease
raised LDH in proliferative
Most common extranodal lymphoma
upper GI tract
Haematological cause of nephrogenic DI
sickle cell
PNH gene defect in…
PIG A gene
Von willebrand disease types
Types
type 1: partial reduction in vWF (80% of patients)
type 2*: abnormal form of vWF
type 3**: total lack of vWF (autosomal recessive)
Management of von willebrand disease
Management
- 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
Heparin-induced thrombocytopaenia (HIT)
bind to the PF4-heparin complexes on the platelet surface and induce platelet activation
Anaemia and jaundice
HAEMOLYTIC ANAEMIA
Raised reticulocytes mean…
intravascular haemolysis
When is osmotic fragility high?
hereditary spherocytosis
Howel jolly bodies indicate…
hyposplenism
Crohns patient with macrocytic anaemia
terminal ileal disease
B12 and folate deficiency
without b12, RBC cannot mature -> haemolyse
When to do haemoglobin electrophoresis
non white patient with microcytic anaemia
Aplastic anaemia
pancytopenia
hypoplastic bone marrow
Mx: haematopoietic stem cell transplant
Lead poisoning associated with…
normocytic anaemia with sideroblastic change
accumulation of **iron around nuclei **of developing RBC
Invasive aspergillosis test
galactomannan test via ELISA
vitamin K dependent clotting factors
1972
2, 7, 10, 9
Poor prognosis for AML
AGE > 60 years
more than > 20% blasts after first course of chemo
cytogenetics: deletions of chromosome 5 or 7
Acute promyelocytic leukaemia M3
associated with t(15;17)
fusion of PML and RAR-alpha genes
presents younger than other types of AML (average = 25 years old)
Auer rods (seen with myeloperoxidase stain)
DIC or thrombocytopenia often at presentation
good prognosis
CML
BCR/ABL fusion gene (philadephia)
9:22
tyrosine kinase production
mx: imatinib (tyrosine kinase inhibitor)
Acute transfusion reaction
- non haemolytic febrile reaction
- paracetamol - minor allergic reaction
- antihistamine - anaphylaxis
- stop transfusion IM adrenalione - Acute haemolytic reaction (ABOincompatible)
- direct coombs test, stop transfusion - TACO
- stop and give diuretics - TRALI
- stop transfusion
- anti HLA antibodies donor
Acute haemolytic reaction (ABOincompatible) presents as…
tachycardia
back pain
Which immune cell does CLL affect
B cell disorder
blood film: smudge cells (also known as smear cells)
Thrombocytopenia with VTE risk and miscarriages
Antiphospholipid syndrome
Polycythaemia
primary:
- JAK 2+ve
- polycythaemia vera
- pruritis and splenomegaly
secondary:
- JAK -ve
- hypoxia —> EPO
- COPD/fibrosis/altitude
Haemophilia A and B inherited pattern
X linked recessive
BOYS get it
women can be carriers
Ann arbor staging for Hodgkins
I: single lymph node
II: 2 or more lymph nodes/regions on the same side of the diaphragm
III: nodes on both sides of the diaphragm
IV: spread beyond lymph nodes
Type:
A = no systemic symptoms other than pruritus
B = weight loss > 10% in last 6 months, fever > 38c, night sweats (poor prognosis)
Hodgkin 2 types
nodular lymphocyte predominant
- good prognosis
classical hodgkins lymphoma
- nodular sclerosis
- mixed cellularity
- lymphocyte rich
- lymphocyte depletes
Coagulation factor deficiency…
prolonged PT and APTT
Causes of cold agglutinin
autoantibodies react at <37 degrees
Lymphoproliferative
- non hodgkins
infections
- mycoplasma
- EBV
What vitamin aids iron absorption
vitamin C
HRT risk proportion
DVT: 2-3 times risk
Breast ca: 1 in 1000/yr
CLL investigation
**blood film: smudge cells **(also known as smear cells)
immunophenotyping is the key investigation
most cases can be identified using a panel of antibodies specific for CD5, CD19, CD20 and CD23
Another name for immunophenotyping
Flow cytometry
Antiphospholipid syndrome management of clots
lifelong with target INR 2-3
Diagnostic criteria for antiphospholipid syndrom
antibody should be rechecked after 3 months to ensure persistence
Anaemia causes…
retinopathy including haemorrhage
Haemolytic anaemia due to primaquine
G6PD deficiency
heinz bodies (denatures haemaglobin)
Side effects of vincristine
peripheral neuropathy
DIC
prolonged clotting time
low fibrinogen
Sickle cell anaemia
howell jolly body (hyposplenism)
frontal bossing of skull
Spleen size difference between sickle cell and thalassaemia
sickle cell- hyposplenism from infarct
thalassaemia- megaly due to haematopoiesis
Secondary polycythaemia due to hypoxia…
increased affinity haemoglobin
What is reactive thrombocytosis
infection/inflammation/surgery etc causing increased plt
Complication of multiple blood transfusion
iron overload –> pericarditis
Mx: iron chelator desferrioxamine
Italian women with Hypochromic microcytic anaemia
nucleated RBC
Thalassaemia
Pancytopenia
Bone marrow disorder
Elderly
lymphocytosis
splenomegaly
pancytopenia
CLL
Diarrhoea
abdo pain
AKI
rash in lower limbs
HUS
ITP
isolated thrombocytopenia
brusing and bleeding
Myeloproliferative - myelofibrosis
fibroblast overstimulation
splenomegaly
high WCC and plt
tear drop poikilcytes blood film
dry tap on marrow biopsy
Isolated prolonged APTT
antiphospholipid syndrome
Idiopathic hypereosinophilic syndrome
steroids to treat
AML chromosome associated with///
5 or 7
Myeloproliferative disorder may transform into…
AML
Irradiated blood is for…
inactivated donor lymphocyte
CLL on purine analogue are T cell depleted hence risk of graft vs host
GVHD ….
Allogenic bone marrow transplant
T cell in donor mount immune on host
NOT a transplant rejection
Acute - within 100 days (skin, liver, GI)
Chronic- after 100 days (+lung, eyes)
Managmenent of GVHD
Steroids
reversal of alteplase (tPA)
FFP
Beta thalassaemia common in which ethnic groups
Mediterranean
Middle east
Central and south east asia
Auer rods seen in…
AML
(Richter’s transformation)
CLL transformation to high-grade lymphoma
(Richter’s transformation)
lymph node swelling
fever without infection
weight loss
night sweats
nausea
abdominal pain
Most common inherited prothrombotic disorder
Factor V Laden
Cytotoxic Purine analogue abx prophylaxis
affects t cell function
co-trimoxazole for PCP
Capecitabine vs 5-FU
5-FU -fluiiidd (intravenous)
capecitabine (prodrug of 5FU)
Common side effect of imatinib
nausea
Tumour lysis syndrome prevention
- IV fluids
- higher risk s- allopurinol or rasburicase
- rasburicase- high risk group
a recombinant version of urate oxidase - allopurinol- lower-risk groups
- rasburicase and allopurinol should not be given together
G6PD hallmarks
red blood cell enzyme defect Mediterranean and Africa
inherited in an X-linked recessive drugs can precipitate a crisis as well as infections and broad (fava) beans
reduced NADPH
Haemolytic anaemia
reduced haptoglobulin
raised reticulocytes
Waldenstrom’s macroglobulinaemia
Low grade lymphoma
monoclonal IgM (Walden and M) paraprotein
hyperviscosity syndrome
cryoglobulinaemia e.g. Raynaud’s`
W upside down M
Koilonychia
IDA
CD20 targetted therapy for..
lymphoma (B cell)
Rheumatoid arthritis
Anicytosis
variation in RBC size
macrocytic anaemia due to B12 deficiency
Gallstones and anaemia
Hereditary spherocytosis
Which medication should be checked with Haem for VWD?
NSAID (affects plt aggregation)
Most common sx of myelofibrosis
fatigue
Burkitts lymphoma
c-Myc
high grade NHL
T(11;14)
Mantle cell lymphoma
t(14;18)
follicular lymphoma
Aplastic crisis
parvovirus
joint pain
fever
facial rash
ITP antibody
Antibodies against the glycoprotein IIb/IIIa or Ib-V-IX complex.
Evan’s syndrome
ITP + autoimmune haemolytic anaemia (AIHA)
Mx: steroid
Management of ITP
Prednisolone
IVIG
Splenectomy
ALL poor prognosis
age < 2 years or > 10 years
WBC > 20 at diagnosis
T or B cell surface markers
non-Caucasian
male sex
presence of philadelphia Chr
Fat embolism syndrome
recent traumatic fracture
petechiae in upper body and axillae
Hodgkins
supraclavicular
mediastinal
young patient
Prolonged PT
Lack of vit K dependent clotting factors
1972
False positive VDRL
Antiphospholipid syndrome
VWD
autosomal dominant
post op bleed
nose bleed
menorrhagia
Most common inherited clotting disorder
F V Laden
Most common inherited bleeding disorder
VWD
Most common cause of death in CLL
Infection
LMWH action
potentiates antithrombin 3
Major complication of polycythaemia vera
bleeding
thrombosis
Platelet target
more than 20 if fever
more than 10 if no fever
Bite cells on blood film
G6PD
rbc life span
120 days
17 days in sickle
TTP pentad
FATRN
fever
anaemia
thrombocytopenia
renal
neurological symptom
mycosis fungoides
T-cell lymphoma that affects the skin.
Fragment cells on blood film
TTP
Hairy cell leukaemia
BRAF mutation
Sideroblastic anaemia
genetic
increased iron stores in RBC (mitochondria)
- ringed sideroblasts around the nuclei
Myelodysplastic syndrome
older population
can lead to AML
unexplained cytopenia and macrocytosis
Ix: bone marrow biopsy + cytogenetic
Haemophilia A and B
A- 8
B- 9
haemophilia A complication
factor 8 inhibitor can happen in multiple factor 9 infusions
Methaemaglobinaemia
drugs: sulphonamides, nitrates (including recreational nitrates e.g. amyl nitrite ‘poppers’), dapsone, sodium nitroprusside, primaquine
chemicals: aniline dyes
methylene blue
Cabot rings
lead poisoning
Warfarin reduced effect by…
azathioprine
COCP
carbamazepine
Poor prognosis of hodgkins
LYMPHOCYTE DEPLETED
Post transplant complications
most common:
- skin cancer
second most common:
- lymphoma
Management of essential thrombocytosis
aspirin and hydroxycarbamide (reduce plt)
Long term risk of radiotherapy
secondary cancer
Which leukaemia can lead to DIC
AML
Complication of heparin induced thrombocytopenia
- THROMBOCYTOPENIA
- THROMBOSIS
Painless lymph node in neck
PUO
HOdgkins
Which heparin has higher risk of HIT
UFH > LMWH
Thrombocytopenia on chemotherapy
myelosuppression
What is the most important prognostic factor for AML
cytogenetics:
- poor - Chr 5 or 7
- good - t(8:21) or 16
Action of desmopressin in haemophilia A
leads to release of pre-formed VWF –> bind to factor 8 and prolong factor 8 lifespan
Complication of myeloproliferative disorder
gout
Myeloproliferation
raised wcc
raised plt
Long term complication of chemotherapy and radiotherapy
leukaemia
Why do you see raised LDH and BR
haemolysis
basophilic stippling
lead poisoning
impaired Hb synthesis
alcoholism
megaloblastic anemias
Pappenheimer body
- iron containing inclusion body in reticulocyte
myelodysplastic syndrome (MDS), sideroblastic anemia
hemolytic anemia
lead poisoning
sickle cell disease.
adult T cell leukaemia/lymphoma
HTLV1 virus
skin lesion
lymphadenopathy
Lymphoma diagnosis
EXCISION biopsy
Felty syndrom
neutropenia
splenomegaly
rheumatoid arthritis
G6PD drug induced
heinz body –> oxidised –> removed by spleen –> extravascular haemolysis –> bite cells
Black water fever
falciparum + malariae spp
acute jaundice, haemolysis, AKI
due to acute anaemia
follows acute treatment for malaria
Significant splenomegaly
CML
- raised WCC
Myelofibrosis
- pancytopenia
Most useful test for haemolysis vs aplastic anaemia
reticulocyte count
- high in haemolysis
- low in aplastic anaemia
Methaemoglobinaemia
oxidation of Fe2+ to Fe3+
Antiphospholipid syndrome with x2 VTE
Lifelong warfarin
TTP treatment
urgent plasma exchange
TTP
deficiency of ADAMTS13
overlaps with haemolytic uraemic syndrome (HUS)
Prolonged bleeding time does not occur in
Haemophilia
beta thalassaemia trait
low HbF
high HbA2
Parvomyxovirus
measles
mumps
Aplastic crisis
pancytopenia
bone marrow failure
parvovirus 19
Polycythaemia vera complications
- thrombotic events
- myelofibrosis
- acute leukaemia (risk increased with chemotherapy treatment)
Complication of myelofibrosis
AML
Primary Polycythaemia EPO level
LOW
(negative feedback)
Paraprotein in multiple myeloma
IgG
Gestational thrombocytopenia
no intervention
observe
fatty bone marrow
aplastic anaemia
Blood products in UK negative for
Hep B and C
syphillis
HIV
Which virus must be negative for pregnant women for blood transfusion
CMV
DAT (coombs test)
direct antiglobulin test
- for haemolytic anaemia
- warm vs cold (acitivity)
- warm- IgG
- cold- IgM
Warm coombs
SLE
CLL
Lymphoma
Cold coombs
mycoplasma
EBV
Influenza
non hodgkins
MAHA
microangiopathic haemolytic anaemia
small vessel
red cell breakdown
- RBC shredded and sheared from platelet plug (mechanism not a disease)
HUS
Haemolytic uraemic syndrome
MAHA + renal failure + thrombocytopenia
Ecoli–> shiga like toxin –> endothelial damage –> plt plug forms –> RBC shears –> plt being consumed in plug formation –> renal failure
TTP
Thrombotic throbocytopaenic purpura
clot form + low plt + rash
VWF sticky –> stick to healthy endothelium –> reduced ADAMTS13 (usually present) –> persistence of sticky VWF –> plug form –> consume plt –> MAHA
NOTE: Brain (confusion and neuro)
DIC
disseminated intravascular coagulation
all over body + within blood vessel + clotting
sepsis/tumour/pancreatitis –> XS tissue factor –> XS extrinsic clotting cascade –> lots of clots form –> deplete plt and clotting factors –> wide spread bleed
ADAMTS13 role
protein that cleans up dodgy sticky VWF
Extrinsic clotting cascade
exposed tissue factor on damaged tissue –> activate F7 to F7a –> eventual fibrinogen to fibrin –> fibrin mesh
VWF role
form initial plt plug
bind to factor 8 and stabilise
(therefore F8 is low in VWD)
Management of acute promyelocytic leukaemia
ATRA
all transretinoic acid
Hodgkins vs non hodgkin
Hodgkin:
- b cell only
- single group of LN usually
- reed steinberg cell
Non hodgkin
- 90% of all lymphoma
- b or t cell
- multiple groups of nodes
- painless LN, B symptoms, pruritis
MGUS
Smouldering multiple myeloma
multiple myeloma
spectrum of plasma cell disease
MGUS and smouldering - premalignant
MGUS:
- M protein <3
- Marrow plasma cell <10%
- no CRAB sx
Smouldering
- M protein >3
- marrow plasma cell 10-60%
- no CRAB sx
myeloma
- CRAB
- Marrow plasma >60%
- more than 1 lesion on MRI
Multiple myeloma
explanded MONOCLONAL clone of plasma cells and paraprotein (immunoglobulin)
Non hodgkins lymphoma types
Follicular
MALT lymphoma (H.pyelori)
Mantle lymphoma (11)
Diffuse large B cell lymphoma
Burkitt (8)
Peripheral T cell lymphoma
Active bleed INR 8
PCC and Vit K
Myelodysplasia
abnormal differentiation of cells in bone marrow
therefore pancytopenia
can progress to AML (1/3)
Myelofibrosis
cloncal expansion -> fibrotic scar tissue
pancytopenia
tear drop cell
dry tap
massive splenomegaly (outside cell production)
ATRA mechanism
promote APML cell to mature to granulocyte
apoptosis of these cells
Most common infection in plt transfusion
s.aureus
stored in room temp for up to 5 days
RBC storing
fridge
FFP storing
frozen