Haematology Flashcards
What is the most common form of haemoglobin?
Hb A - made up of 2alpha chains and 2 beta chains. forms 90% of the haemaglobin
Hb A2 is made up of 2 alpha chains and 2 delta chains and makes up 1-3% of the haemoglobin. A raised level may indicate thallassaemia
what additional test should be ordered with iron studies?
CRP
What should you think of when patients have pre existing conditions in questions?
IS IT AUTOIMMUNE!!!
Sever complication of pernicious anaemia
subacute combined degeneration of the spinal cord
Name 4 inherited aplastic anaemias? what proportion of AA’s do they make up?
10% the other 70% are idiopathic and should be treated with immunosuppression a nd supportive care - transfusion abx and iron chelation if persitant. marrow stimulation and recovery may be used also e.g g-csf.
(D)DFS - to buy them a sofa Fanconi's anaemia dyskeratosis congenita Schwachman diamond syndrome Diamond Blackfan syndrome
Triad of dyskeratosis congenita
SON
skin pigmentation
oral leukoplakia
nail dystrophy
chromasome instability and telomere shortening - BM failure
Symptoms of schwachman diamond syndrome?
primarily neutrophilia - endocrine and pancreatic dysfunction
What blood results do you see in DIC? causes?
low platelets and fibrinogen, high fibrinogen degredation products, high d dimer, prolonged prothrombin time
Causes = malignancy, trauma, burns, toxins, obstetric complications.
Treatment = FFP, platelets, cryoprecipitate
What joint is most commonly affected by pseudogout?
Knee
what is another name for pseudogout
chondrocalcinosis
what type of bifringence do crystals show?
rhomboid shape with positive bifringence? BLUE WITH the axis.
pyrophosphate crystals
true gout is YELLOW WITH the axis
What counts as MASSIVE blood loss?
entire blood volume in 24 hours, >50% in 3 hours
What is the normal cutoff for platelet transfusion?
most people say <50 however some say 75 (x10^9)
What is the difference between red cell concentration and red cell mass
red cell concentration varies with hydration status and plasma volume. q
what drives anaemia of chronic disease
cytokines such as IFN, IL1 and TNF decrease EPO recepter expression
What are downs syndrome children at risk of (haem)
AML in first 3 years of life and then ALL
What is an acute leukaemia
Acute Neoplastic process of bone marrow and blood resulting in a rapidly progressing and fatal disease which has >20% blast cells on BM biopsy.
What is the best way to differentiate acute and chronic leukaemias clinically?
Chronic types are usually diagnosed incidentally, wheras acute have more severe presentations with BM failure and cytopenias - anaemia, petechiae, infections (neutropenia), and hepatosplenomegaly, cranial nerve palsies due to CNS involvement, gum hypertrophy (AMMoL often with hypoK) lymphadenopathy and bone bain.
However CML in blast phase can resemble an acute leukaemia.
TDT +ve
ALL - displayed on pre b
Philadelphia chromasome positive
usually CML but can be ALL in adults. (30% positive)
Name 2 subtypes of acute myeloid leukaemia
Acute promyleocytic leukaemia characterised by a t(15,17) with hypergranular promyelocytes with auer rods. it causes early DIC and haemorrhage. (DIC may occur with infection as a result of any leukaemia)
requires ATRA
Acute myelomonocytic leukaemia characterised by gum hypertrophy and
Are protein C and protein S, procoagulant or anticoagulant?
anticoagulant. along with TFPI, thrombomodulin, antithrombin (these are often expressed on the vessel wall)
how long does a flight need to be before it confers an increase thrombosis risk
9 hours
Which has the largest thrombosis risk:
Factor 5 leiden
Antithrombin deficiency
family history of thrombosis
Protein C/S deficnency
C/s and antithrombin deficiency are wrst followed by 5 leiden.
What cell lines should be seen on a CML Bone marrow
Hypercellular marrow with spectrum of immature myelocytes and mature granulocytic cells in the blood. neutrophils in blood are 50-500
what are ethe three phases of CML
Chronic <5% blasts
Accellerated >10% blasts - increasing splenomegaly
Blast >20% blasts resembling acute leukaemia
(ALL transformation can occur)
whats the difference between essential thrombocythemia and thrombocytopenic purpura
high and low
How do you differentiate hodgkins and non hodgkins lymphomas
hodgkins - pain on drinking alcohol in affected nodes, reed sternberg
what is nodular lymphocytic (predominant) pertain to?
non classical type of hodgkins lymphoma. accounts for small number of cases and is indolent. not EBV associated. rarely has B symptoms. mainly lymphadenotpathy. it has atypical reed sternberg cells.
Describe the staging in hodgkins lymphoma
ANN ARBOR
stage 1: 1 lymph node region
stage 2 2 or more lymph node regions on same side of D
stage 3: 2 or more lymph nodes on opposite sides of D
Stage 4: extranodal sites e.g liver/BM
A- no constitutional sx B - fever/night sweat/weight loss
How can you treat von willebrand disease
Desmopressin can be used to increase the concentration of VwF and factor 8. this is only in type 1 and 2 disease. major disease - type 3 it would not be effective
clinical features of myelodysplastic syndromes
Fatigue, infection, bleeding. hypercellular marrow(as in CML)
qualitative cytopenias risk of AML transformation.
What is a ringed sideroblast? what myelodysplasias does it feature in?
abnormally nucleated blast cell with iron granule ring
occur in refractory anaemia with ringed sideroblasts. must have greater than 15% ringed sideroblasts. and erythroid dysplasia.
may also be part of refractory cytopenia with ringed sideroblasts. must have>15% ringed sideroblasts along with:
Cytopenia in 2 cell lines along with >10% dysplastic cells in 2 or more cell lines.
Define Refractory anaemia
anaemia, no blasts in blood. erythroid dysplasia with <5% blasts in BM
Define refractory anaemia with excess blasts 1
Blood:
cytopenias
<5% blasts
no auer rods
Bone Marrow
5-9% blasts
dysplasia
Define refractory anaemia with excess blasts 2
Blood:
cytopenias or 5-19% blasts or auer rods
Bone Marrow
dysplasia with
10-19% blasts, or
auer rods
what do MDS sufferers die from
International prognostic scoring system used to predict which takes into account rule of thirds
- infection
- bleeding
- AML
Define MDS with 5q deletion
Blood
Anaemia
normal or increased platelets
Marrow
Hypolobulated nuclei of megakaryocytes
<5% blasts
Define unclassified MDS
complex -
Blood
cytopenias with no blasts or aur rods
BM
Myeloid or megakaryocytic dysplasia
<5% blasts
what nationality get adult t cell lymphoma
caribbean and Japanese
associated with HTLV1 infection
Hypercalcaemia and lymphocytosis. skin lesions and splenomeg
Mantle cell lymphoma
t(11,14) BCL-1 locus causing overexertion of cyclin D1
They are aggressive lymphomas with angular nuclei on histology. occur in men more than women.
Diffuse Large b cell lymphoma
germinal cell lymphoma - may have undergone richter’s transformation from other low grade germinal cell lymphoma or CLL.
Very aggressive high grade with sheets of large lymphoid cells which needs to be treated with RCHOP.
What does RCHOP stand for and what is it used in?
Rituximab cyclophosphamide vincristine doxorubicin prednisolone
DLBCL and mantle cell.
What chemo is often used in hodgkins lymphoma
ABVD - adriamycin, bleomycin, vinblastine, decarbazine.
can use MOPP but makes sterile
What is erythmelalgia?
painful burning or swelling sensation in the fingers
often associated with essential thrombocythemia - megakaryocytic in the bone marrow.
excess dysfunctional platelets. dizziness, headaches, stroke, MI gangrene thrombosis.
treat with anegralide
or hydroxycarbamide.
what causes the hepatosplenomegaly in myelofibrosis
extramedullary haematopoeisis
What is seen on myelofibrosis investigations?
Blood film - tear drop poikilocytes and leukoerthyroblasts
bone marrow - dry tap
What is the cause of myelofibrosis
may be idiopathic or occur following Polycythemia or essential thrombocythaemia.
antibody found in autoimmune type 1 diabetes
Anti - GAD
CD55 and CD59 are found in what disease
PNH
what does CK vary with
exercise and race
what does albumin vary with
posture
Where is THC NOT found
saliva
Name 3 diseases which come about through constant antigenic stimulation
EATL - coeliac
Parotid lymphoma - sjogren’s
Gastric carcinoma - h.pylori
Name 3 diseases which come about through direct action and viral drive from infection rather than via antigenic stimulation
HTLV1 - adult t cell lymphoma
EBV - burkitts +?
HIV -
What CD are b cells associated with?
19? often 20 used as rituximab is common therapy
What CD are t cell lymphomas associated with
3+5
what marker is often used in DLBCL staging
LDH, age
t(8,14)
burkitts lymphoma
t(15,17)
APML
t(14,18)
follicular lymphoma
t(11,18)
MALToma
t(11,14)
mantle cell lymphoma
Acanthocytes
also known as spurr cells
signify:
liver disease
hyposlenism
abetalipoproteinaemia
What is basophilic RBC indicative of?
Increased rate of erythropoeisis or defective synthesis.
likely due to megaloblastic anaemia or myelodysplasia or LEAD POISINING
Heinz bodies
inclusions within red blood cells which give rise to bite cells - G6PD
Howell jolly body
basophilic staining of the cytoplasm and nuclear remnants in RBC
hyposlenism
sickle cell
Leucoerythroblastic anaemia
nucleated RBCs and WBC’s in the periphery.
marrow infiltration i.e myelofibrosis/malignancy
Pelger huet cells
hyposegmented neutrophils
myelodisplastic/leukaemia
polychromasia
reticulocytosis - gey blue cells.
increases in haemolytic anaemia and decreases in aplastic anaemia or with chemotherapy
rouleaoux
stacking
myeloma and paraproteinaemia
spherocytes
round - usually hereditary spherocytosis or may be due to AIHA (warm)
target cells
bulls eyes
HHH
hyposplenism
hepatic pathology
haemaglobinopathies.
What is the normal range of anaemia in men and women
135 in men and 115 in women.
What are the GI causes of bleefding
meckels diverticulum
peptic ulcers
polyps/cancers
hookworm
What is the mechanism for anaemia of chronic disease?
normocytic
reduction of EPO - IL! TNF etc
reduction of iron transportation out of gut cells - IL6
What is high in anaemia of chronic disease
ferritin - iron store
what is a sideroblastic anaemia
iron cannot be correctly incorporated into the cells and so is dotteda round the place - occurs as part of myelodysplasia and genetic conditions
what is the treatment for sideroblastic anaemia
remove the cause and give vitamin b6 to increase production
What antibodies do perncicious anaemia sufferers have
anti parietal cell - 90
anti intrinsic factor - 50
schilling test but is outdated.
Removal of what organs gievs youpernicious anaemia?
stomach removal or terminal ilium.
may be after fat or chrons surgery.
TROPICAL SPRUE
what are the signs of pernicious anaemia
b12 def
glossitis angular cheilosis
irritability, depression psychosis
tingling neuropathy, spastic paraparesis and SACD spinal cord ABSENT ANKLE REFLEX
What will make a b12 neuropathy worse
folic acid.
What are the indications of intravascular hameolysis?
free plasma hb
haemagolbinurea
reduced haptoglobin
methaemaglobinaemia
What are the indications of extravascular hameolysis
splenomegaly
How do you recognise all haemolysis
unconjugated bilirubin goes up, urobilinogen goes up(clear excreted in urine and faeces)
LDH goes up
GALL STONES
When do you get pencil cells
hereditary eliptocytosis but also in IDA
When would you see spectrin mutations
both hereditary spherocytosis and eliptocytosis
What are echinocytes
burr cells (not spurr) pyruvate kinase deficiency and EDTA change
what goes down in HUS
platelets
What does TXA2 do?
causes platelet aggregation
what does a2 macroglobulin do?
inhibits 10
what does uraemia do to platelets
reduces their function
What are the main things associated with Haemophilia A
factor 8 deficiency
causes prolonged bleeding and haemarthroses
can have varying degrees of loss.
What is VWf function in the blood stream?
to carry factor 8
when there is von willebrands disease they will have reduced factor 8 aswell as reduced platelt functioning which would be BLEEDING such as nosebleeds.
what is more common haemophilia A or B
A is 5 times more common
x linked recessive so is in men
how do you treat von willebrands
gove low purity factor 8 or desmopressin
what is d dimer also known as
type of FDP.
what factors are not made when there is liver disease?
2 5 7 9 10 11
platelts reduced function
