Haem Flashcards
reversal agents for warfarin
low dose vitamin K or rapidly with prothrombin complex concentrate.
how can Tumour Lysis syndrome be pre-treated?
IV allopurinol or rasburicase, to reduce the risk of damage from hyperuricaemia.
what is the triad of dyskeratosis congenita
nail dystrophy (nail loss, longitudinal ridging)
oral leukoplakia (white patches in the mouth)
skin pigmentation (lace like hyperpigmentation of skin creases)
with associated bone marrow failure (aplastic anaemia) and two other internal signs (like pulmonary fibrosis).
what mutation leads to dyskeratosis congenita
telomere
What is the most sensitive biomarker for iron deficiency anaemia?
serum ferritin
common cause of blood loss in developing countries
parasitic worms
how should unexplained iron def anaemia be investigated?
Unexplained IDA should have OGD, Colonoscopy, Urine dip and investigations for coeliac disease.
If there is profound renal failure, which anticoagulant is used
warfarin
most common lymphoma in 15-40 year olds
Hodgkins
most common lymphoma in >40 year olds
Diffuse Large B cell lymphoma
most common lymphoma in <15 year olds
Burkitt’s lymphoma
most common adult leukaemia
CLL
which cell lines are affected by Shwachman Diamond syndrome
neutrophils
which cell lines are affected by dyskeratosis congenita
all (pancytopenia)
which cell lines are affected by Diamond Blackfan anaemia?
red cells (anaemia)
which cell lines are affected by fanconi anaemia
all (pancytopenia)
What stereotypical description relating to poikilocytotic erythrocytes can be seen on a blood film with a patient with myelofibrosis?
dacrocyte
treatment of essential thrombocythaemia
anagrelide, aspirin
What is the term given to describe the transformation of chronic lymphocytic leukaemia into a high grade lymphoma?
Richter’s transformation
namely into Diffuse Large B cell
main two drugs used in CLL treatment
ibrutinib (TK inhibitor)
venetoclax (BCL2 inhibitor)
reversal agent for dabigatran
Idarucizumab
top 2 causes of pancreatic exocrine dysfunction in children
- cystic fibrosis
- Shwachman Diamond Syndrome
the M3 subtype (acute promyelocytic leukaemia) can be treated with
all-trans retinoic acid.
The myeloproliferative syndromes, which include essential thrombocytosis, all carry a risk of transformation to what life threatening malignancy?
acute myeloid leukaemia
main feature on peripheral blood film in CLL
smear/smudge cells
In heparin induced thrombocytopenia, what are antibodies produced against?
heparin and platelet factor 4
what is the overall effect of heparin induced thrombocytopenia
prothrombotic
heparin-P4 complexes bind to other platelets causing them to become activated leading to clot formation
what are the malignant cell in Hodgkins lymphoma predominantly
mature B cells from a lymph node’s germinal centre
Cairo Bishop scoring for TLS
Potassium >6mmol/L
Phosphate >1.125 mmol/L
Calcium LESS than 1.75mmol/L
Urate >475umol/L
Which cofactor is required for synthesis of the anticoagulant proteins C and S?
Vitamin K
describe a neutrophil
Very common
3x larger than erythrocytes
Multisegmented nucleus (2-5 lobes)
Nucleus looks “speckly”/ isn’t solidly stained because chromatin is condensed.
describe a monocyte
Relatively common
>3x larger than erythrocytes
Large, kidney bean shaped nucleus.
describe a basophil
Rare
3x large than erythrocytes
Deep blue (basic) granules
Bilobed nucleus
describe an eosinophil
Rare
3x larger than erythrocytes
Reddish purple (acidic) granules
Bilobed nucleus
describe a lymphocyte
About the same size as erythrocytes (so, small)
Giant nucleus taking up most of the cell
No granules. Nucleus is not kidney shaped.
t(8;14) [cmyc]
Burkitt’s lymphoma
t(15;17)
APML
t(9;22) [BCR-ABL]
CML
t(11;14)
Mantle Cell Lymphoma
t(14;18) [BCL-2]
Follicular lymphoma
what translocation is a very poor prognostic marker in ALL
t(9;22)
reversal agent of LMWH
protamine sulphate
what does heparin increase the action of
antithrombin III
which factors does antithrombin III inactivate
9, 10, 11
how is LMWH monitored
not required
how is UFH monitored
activated partial thromboplastin time
which clotting pathway does heparin affect
intrinsic
what are the blood results in Diamond Blackfan [3]
anaemia
low reticulocytes
elevated fetal haemoglobin
A bone marrow biopsy would also reveal decreased erythroid precursors.
treatment of CML
imatinib
What is the appropriate imaging modality for staging of lymphoma?
CT-PET
what is secreted by the liver to inhibit the transport of iron through ferroportin in the gut
hepcidin
What amino acid is glutamate substituted for in sickle cell disease?
valine
how does the endemic form of Burkitt’s lymphoma present?
Massive mandibular swelling, cervical lymphadenopathy
how does the sporadic form (UK) of Burkitt’s lymphoma present?
children/young adults with predominantly abdominal/inguinal lymphadenopathy. Nonspecific GI symptoms (N+V, diarrhoea, vomiting).
What oncogene is typically found in Burkitt’s lymphoma and is created by a translocation between chromosome 8 and 14?
C-MYC
what age range does mantle cell lymphoma affect
middle age
what age range does Hodgkins lymphoma usually affect
15-40
What are inclusions of clusters of nuclear DNA within erythrocytes commonly called when seen in a peripheral blood film?
Howell-Jolly bodies
how is moderate to severe thrombocytopenia treated
steroids or IVIG
what proteins does vWF bind
collagen
factor 8
platelets
By binding these three things together, it stabilises the platelet plug and increases the strength of the initial platelet plug in primary haemostasis.
In circulation, what protein in VWF bound to
factor 8
If vWF is not present, then Factor VIII is rapidly degraded.
why are platelet transfusions useless in chronic ITP
due to circulating autoantibodies most of the platelets will be destroyed within a few hours.
Platelet transfusions may be indicated before emergency surgery or in haemorrhage.
why are platelet transfusions useless in chronic ITP
due to circulating autoantibodies most of the platelets will be destroyed within a few hours.
Platelet transfusions may be indicated before emergency surgery or in haemorrhage.
INR target of metallic valve
3.5
A thalassemia patient presents with malaise and erectile dysfunction
transfusion related haemosiderosis
what are the two delayed adverse reactions to transfusions
- delayed haemolytic transfusion reaction (IgG mediated)
- GvHD
which Ig mediates anaphylaxis in transfusion reactions
IgA
which Ig mediates ABO incompatibility in transfusion reactions
IgM
thrombocytopenia in the first trimester is likely to be
ITP
thrombocytopenia in the third trimester is likely to be
gestational thrombocytopenia
which haematological factors are normally decreased in pregnancy? [5]
platelets
haemoglobin
haematocrit
Protein S
factor 11
which haematological factors are normally increased in pregnancy? [5]
plasma volume
red cell mass
MCV
WCC
factors 7,8,9,10,12
Difference between Coomb’s test and Direct Antigen test
Coomb’s: detect antibodies attached to red blood cells
DAT: detect antigens on red blood cells
which test is used to detect autoimmune haemolytic anaemia
Direct Antigen Test
haemolysis as a result of antimalarials like primaquine
G6PD deficiency
which GI disease are you more likley to see B12 def
Crohn’s disease
which GI disease are you more likely to see folate def
coeliac disease
what is the treatment for hyper-viscosity in Waldenstrom’s macroglobinaemia
plasmapheresis
what type of lymphoma is Waldenstrom’s macroglobinaemia
who does it affect typically
low grade NHL
elderly men
what is produced by the lymphoplasmacytoid cells in Waldenstrom’s macroglobinaemia
monoclonal IgM infiltrate the lymph nodes and bone marrow
what is Waldenstrom’s macroglobinaemia also known as
lymphoplasmacytoid lymphoma (LPL)
symptoms of Waldenstrom’s
Weight loss
fatigue
hyperviscosity syndrome (visual problems, confusion, CCF, muscle weakness)
treatment of active Waldenstrom’s
Rituximab
Ibrutinib
What is the most common cause of thrombocytopenia/low platelets in Pregnancy
gestational thrombocytopenia
A cause of neonatal thrombocytopenia
maternal idiopathic thrombocytopenia Purpura
What is done to blood donations to reduce risk of GvHD in immunosuppressed patients
irradiation
leukodepletion
A patient with renal impairment is on low molecular weight heparin, what do you measure to monitor this
factor Xa assay
56 y/o lady with SLE, has spherocytes, low Hb, raised bilirubin
how do you test for diagnosis
DAT
Viruses that blood products are screened for
HIV
Hepatitis B
cytomegalovirus
Chimeric antigen receptor T-cell therapy against CD19: what type of haematological malignancy does it target?
B cell lymphoma/leukaemia
teardrop/ dacrocytes are a feature of which conditions
myelofibrosis
which condition has a dry tap on BM aspiration
myelofibrosis
HTLV1 virus is associated with which cancer
adult T cell lymphoma
indolent lymphoma
follicular, marginal zone
Which investigation would confirm a diagnosis of hereditary spherocytosis after seeing spherocytes on a blood film
eosin-5’-maleimide dye test
In Multiple myeloma with restrictive cardiomyopathy what will you see on heart biopsy
amyloid deposits
Associated with longstanding coeliac disease
Enteropathy Associated T cell lymphoma
What would be the most important investigation to carry out in a 65 year old man with iron-deficient anaemia
OGD
Person who had a DVT many years ago (or recurrent DVTs), presents with recurrent dark bruising, and swelling over the course of 5 years, and now had pain in their leg. What is the possible cause
post thrombotic syndrome
An African man with Burkitt’s lymphoma is given Rasburicase. He develops haematuria with irregularly contracted cells. What is the cause
G6PD deficiency
first line drug in multiple myeloma
bortezomib (proteasome inhibitor)
Inhibits intracellular protein degradation, build-up and amino acid shortage kills cell
what platelet threshold triggers massive transfusion e.g. trauma
< 75 x10^9
what platelet threshold triggers transfusion to prevent bleeding post chemo
< 10 x10^9
what platelet threshold triggers transfusion during sepsis
< 20 x10^9
what platelet threshold triggers transfusion to prevent bleeding during surgery
< 50 x 10^9
what platelet threshold triggers transfusion to prevent bleeding at a critical site like the eyes or CNS; or in polytrauma
< 100 x10^9
What is the mechanism of hyperacute allograft rejection
Pre-formed antibodies attach the graft
condition with leukoerythroblastic picture
myelofibrosis
condition with pseudo Pelger Huet cells
myelodysplastic syndrome
what does parvovirus cause in sickle cell disease
aplastic crisis
your bone marrow suddenly stops making red blood cells so you develop severe and potentially life-threatening anemia + low reticulocytes
what is found in excess in beta thalassaemia
alpha chains
high HbA2 and HbF
