Haem Flashcards
which HL carries the best prognosis
lymphocyte predominant
which B symptoms indicate a worse prognosis in Lymphoma
night sweats
fever >38
weight loss >10% over 6 months
which HL carries the worst prognosis
lymphocyte depleted
what is a cause of post radiotherapy pancytopenia
myelodysplastic syndrome
what leukaemia does myelodysplasia often transform into?
AML
what anticoagulant is used in cancer patients
DOACs
if a DVT is likely, what investigation should be done
Proximal Leg vein USS within 4 hours
what should be done if Proximal Leg Vein USS is negative?
A D-dimer should be done
What should be done if proximal leg vein USS is not available within 4 hours
interim anticoagulation before vein USS within 24 hours
what if the proximal leg vein USS is negative but D-dimer is positive
stop interim anticoagulation
offer repeat scan in 6 to 8 days
what should be done if Wells score is low and DVT is unlikely
D-dimer
what should be done if D-dimer is positive in a low likelihood DVT
proximal leg vein USS within 4 hours
if this is not done, start interim anticoagulation
what anticoagulant is used in the renally impaired for DVT
UFH or LMWH with VKA
what anticoagulant is used in antiphospholipid syndrome patients with DVT
LMWH with VKA
Key investigation for child suspected of leukaemia
Urgent FBC within 48 hours
pentad of TTP
swinging fevers
neuro signs
renal failure
thrombocytopenia
haemolytic anaemia
9 positive features of Well Score
active cancer
immobilisation
bed ridden
tenderness along vein
leg swollen
calf swollen
previous DVT
pitting oedema
superficial contralateral veins
reticulocytes level in aplastic anaemia
LOW
reticulocytes level in acute sequestration
HIGH
also seen in haemolysis
how is a definitive diagnosis of sickle cell made
Hb electrophoresis
main imaging in multiple myeloma
whole body MRI
characteristic findings in SCD blood smear
Howell Jolly Bodies
Waldenstroms features
hyperviscosity
monoclonal IgM paraprotein
cryoglobulinaemia
hepatosplenomelagy
systemic upset
3 drugs that causes agranulocytosis
carbimazole
carbamezapine
clozapine
which amyloidosis is associated with chronic inflammation such as rheumatoid arthritis
what do they tend to present with
AA
renal disease e.g. nephrotic syndrome, proteinuria, renal failure
heptosplenomegaly
which amyloidosis is associated with multiple myeloma
AL
indirect marker of lymphoma proliferation
raised LDH
poor prognosis
what sort of lymphoma is associated with H.pylori
gastric MALT
what sort of lymphoma is associated with EBV
Burkitt’s
what sort of lymphoma is associated with HIV
CNS lymphoma
what sort of lymphomas is associated with Hep C
diffuse large B-cell lymphoma and splenic marginal zone lymphoma.
what sort of lymphoma is associated with HTLV-1
T-cell lymphoma.
which conditions are associated with lymphoma [4]
coeliac disease
Sjogrens
post-transplant
AIDS
sickle cell crisis vs aplastic anaemia
aplastic: low reticulocytes, pancytopenia
crisis–> haemolysis: high reticulocytes to compensate
pathology behind sub acute combined degeneration of spinal cord
vitamin B12 deficiency caused by degeneration both the dorsal columns and corticospinal tracts (pain and temperature sensation are typically preserved)
Causes of basophilic stippling [6]
megablastic anaemia
myelodysplasia
thalassaemia alpha
sideroblastic anaemia
alcohol abuse
lead poisoning
causes of Howell Jolly Bodies
Hyposplenism
causes of schistocytes
MAHA: HUS, TTP, DIC
what does left shift mean
presence of immature cells
(including so-called band cells - neutrophils with an unlobed, band-shaped nucleus)
what is the significance of the number of lobes in the neutrophil nucleus
index of maturity
causes of left shift in neutrophils [4]
acute infection
myeloproliferative: CML, myelofibrosis, acute leukaemia
what does right shift mean
signifies the prevalence of hypermature neutrophils with greater than five nuclear lobes
In some cases, right shift may involve the presence of band cells, which are immature neutrophils with an unlobed, band-shaped nucleus
causes of right shift in neutrophils [3]
chronic infections
G-CSF
megaloblastic anaemic
what does the presence of reticulocytes indicate
high turnover of red cells matched by increased marrow production and release
causes of reticulocytosis [2]
acute bleeding
haemolysis
causes of target cells [3]
obstructive liver disease
haemoglobinopathies (thalassaemia and sickle cell disease)
post-splenectomy
causes of roleaux formation [3]
multiple myeloma
Waldenstrom’s macroglobulinemia
inflammatory disorders and malignancies.
what is meant by a leukoerythroblastosis
A leukoerythroblastic film described to combined presence of immature (including nucleated) red blood cells and left shift (immature white cells)
causes of leukoerythroblastosis
marrow fibrosis or invasion, such as: primary myelofibrosis, metastatic cancer, TB and Gaucher’s disease.
most common cause of anisocytosis
iron deficiency
causes of acanthocytes/spur cells
liver disease and neuroacanthocytosis (including abetalipoproteinemia and homozygous familial hypobetalipoproteinemia)
causes of Echinocytes or burr cells
liver disease, vitamin E deficiency, end-stage renal disease, and the haemolytic enzyme disorder pyruvate kinase deficiency.
difference between acanthocytes and echinocytes
Acanthocytes or spur cells are red blood cells that appear irregularly spiked on a blood film.
They are distinct from echinocytes (or burr cells) which are also spiked but with smaller projections in a more regular distribution than those seen on acanthocytes
Echinocytes or burr cells are red blood cells with small, regularly distributed projections across the cell surface. They are distinct from acanthocytes (or spur cells) which appear irregularly spiked or thorny.
how do you prevent painful episodes/crises in sickle cell disease
hydroxyurea
increases HbF
investigations for multiple myeloma
1) Bloods
full blood count: anaemia
peripheral blood film: rouleaux formation
urea and electrolytes: renal failure
bone profile: hypercalcaemia
2) Protein electrophoresis
raised concentrations of monoclonal IgA/IgG proteins will be present in the serum
in the urine, they are known as Bence Jones proteins
3) Bone marrow aspiration
confirms the diagnosis if the number of plasma cells is significantly raised
4) Imaging
historically a skeletal survey has been done to look for bone lesions
however, whole-body MRI is increasingly used and is now recommended in the 2016 NICE guidelines
X-rays: ‘rain-drop skull’ (likened to the pattern rain forms after hitting a surface and splashing, where it leaves a random pattern of dark spots). Note that a very similar, but subtly different finding is found in primary hyperparathyroidism - ‘pepperpot skull’
which blood film abnormalities may be seen in coeliac disease [2]
Howell Jolly and target cells
how should children with new onset purpura be managed
admitted immediately for investigations as it may be a sign of meningococcal septicaemia or acute lymphoblastic leukaemia
how should children with new bruising be treated
urgent FBC
features of acute chest syndrome [4]
dyspnoea
chest pain
pulmonary infiltrates on chest x-ray
low pO2
platelet transfusion threshold for clinically significant bleeding (World Health organisation bleeding grade 2- e.g. haematemesis, melaena, prolonged epistaxis)
< 30 x10^9
platelet transfusion threshold for patients with severe bleeding (World Health organisation bleeding grades 3&4), or bleeding at critical sites, such as the CNS.
< 100 x10^9
when should a platelet transfusion not be done [4]
Chronic bone marrow failure
Autoimmune thrombocytopenia
Heparin-induced thrombocytopenia, or
Thrombotic thrombocytopenic purpura.
Platelet level aim for most patients before/after surgery
> 50 ×10^9/L for most patients
Platelet level aim for patients with high risk of bleeding before/after surgery
50-75 x 10^9
Platelet level aim for patients having surgery at critical sites before/after surgery
> 100 x10^9
causes of spherocytes [2]
Hereditary spherocytosis
Autoimmune hemolytic anaemia
causes of Heinz Bodies [2]
G6PD deficiency
Alpha-thalassaemia
cause of tear drop cells
myelofibrosis
test for hereditary spherocytosis
EMA binding test
how is tranexamic acid given in major haemorrhage
IV bolus followed by slow infusion
medication to reduce risk of tumour lysis syndrome
allopurinol or rasburicase
how does MCV compare to Hb in thalassaemia
disproportionately low in comparison
most common inherited bleeding disorder
von Willibrand
raised aPTT and prolonged bleeding time
blood film features in hyposplenism (post splenectomy, coeliac)
target cells
Howell-Jolly bodies
Pappenheimer bodies
siderotic granules
acanthocytes
rheum conditions that cause neutropenia
SLE and RA
what can polycythaemia rubra vera progress to [2]
AML or myelofibrosis
treatment of polycythaemia vera [3]
aspirin
reduces the risk of thrombotic events
venesection
first-line treatment to keep the haemoglobin in the normal range
chemotherapy
hydroxyurea - slight increased risk of secondary leukaemia
phosphorus-32 therapy
Sickle cell patients should be started on long term ___________ to reduce the incidence of complications and acute crises
Sickle cell patients should be started on long term hydroxycarbamide to reduce the incidence of complications and acute crises
features of acute intermittent porphyria
management?
abdominal: abdominal pain, vomiting
neurological: motor neuropathy
psychiatric: e.g. depression
hypertension and tachycardia common
avoiding triggers
acute attacks
IV haematin/haem arginate
IV glucose should be used if haematin/haem arginate is not immediately available
transfusion threshold in someone with ACS
without ACS?
<80 (target 80-100)
<70 (target 70-90)
Ann-Arbor staging
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
cause of thrombocytopenia
Causes of severe thrombocytopenia
ITP
DIC
TTP
haematological malignancy
Causes of moderate thrombocytopenia
heparin induced thrombocytopenia (HIT)
drug-induced (e.g. quinine, diuretics, sulphonamides, aspirin, thiazides)
alcohol
liver disease
hypersplenism
viral infection (EBV, HIV, hepatitis)
pregnancy
SLE/antiphospholipid syndrome
vitamin B12 deficiency
purpose of irradiated blood
depleted of T-lymphocytes and used to avoid transfusion-associated graft versus host disease (TA-GVHD) caused by engraftment of viable donor T lymphocytes.
features of TACO
Hypertension, raised jugular venous pulse, afebrile, S3 present
features of TRALI
Hypotension, pyrexia, normal/unchanged JVP
what does cryoprecipitate contain
contains factor VIII, fibrinogen, von Willebrand factor and factor XIII
which leukaemia undergoes Richters transformation
what is the result of a richter’s transformation
CLL
becomes a NHL
in a non-urgent scenario, a unit of RBC is usually transfused over _____
in a non-urgent scenario, a unit of RBC is usually transfused over 90-120 minutes
is CLL associated with warm or cold autoimmune haemolytic anaemia
warm
The universal donor of fresh frozen plasma is ___ RhD ______ blood
The universal donor of fresh frozen plasma is AB RhD negative blood
