haem continued Flashcards
what are the lymphoid cells
t cells b cells nk cells
what cells produces platelets
megakaryocytes
embryological origin of stem cells
mesoderm
how does liver disease cause thrombocytopenia
thrombopoeiten regulates growth adn is produced by liver
how do you identify lymphoid cell type
immunophenotyping
where are b cells and t cells produced
b cells- bone
t cells - thymus
blood film findings in hypospelnism
howell jolly bodies
causes of hypospleinis
sick cell
graves
coeliac
SLE
amyloid
what cells does AML come from
common myeloid proginator
what cells does CLL - chronic lymphocytic leukaemia come from
b cells
what cell does myeloma come from
myeloma
what cells does acute lymphoblstic leukaemia come from
common lymphoid precusor
what does leukaemia and lymphoma cause in terms of loss of fuction
leaukaemia - loss of haempoeisis
lymphoma - loss of immune function
difference in AML and CML
both have proliferation of abnormal projinators
AML = block in differentiation or maturation
CML = no block in differentiation or maturation
what mutation cause haematological cancers
driver mutations
signs of high grade haemotological cancer on histology
large cells with high nucelus:cytoplasmic ration
prominent nucleoli and rapid prolif
what percentage of blasts in blood/bone is indicative of acute leukaemia
> 20%
what is the most common chidhoos cancer
Acute lymphoblastic leukaemia (CLP)
what is involved in pancytopenia
anaemia-
thrombocytopenia
leukopenia
differentials for non blanching petechia
meningococcal septicaemia
Leukaemia
HSP
NAI
ITP
vasculitis
what haemotological cancer is downs syndrome assoc with
acute lymphoblastic leukaemia
features of ALL
those assoc with marrow failure
thrombocytopenia- petechia
neutropenia- infections
anaemia - pallor, fatigue
bone pain (secondary to bone marrow infiltration)
splenomegaly
hepatomegaly
fever is present in up to 50% of new cases (representing infection or constitutional symptom)
testicular swelling
what genetics can be seen in ALL
phildelphia chromosome 9:22 trasnlocation
transformation of a myeloproliferative disorder causes what cancer
acute myeloid leukaemia
what haemotological malignancy can present with DIC
acute myeloid leukaemia
auer rods
AML
blast cells indicate
an acute leukaemia
what are auer rods
abnormal granules in blast cells
what neurophil count is seen in neutropenic sepsis
<0.5
most common cause of neutropenic sepsis
coag neg gram pos
STAPH EPIDERMIDIS
Treatment of neutropenic spesis
pipraciilen adn tazobactam
prophylaxis of tumour lysis syndrome adn when given
IV Allopurinol or IV rasburicase
immediately prior and during first days
findings in tumour lysis syndrome
AKI
high potassium, high phosphate, high uric acid
low calcium
what chemo agents casue dilated cardiomyopathy
anthracyclines
what are b symptoms
night sweats
fever
weigth loss
utch without rash adn alcohol induced pain
hodgekins
is lymphadenopathy tender in cancer
no
how is CLL diagnosed
immunophenotyping
what cell does hodkins come from
b-cell
reed sternberg cells
hodgkins
what leukemia can transform to lymphoma and what is this called
CLL - richters trasnformation
smear or smudge cells
CLL
what blood test is a good marker of rpognosis in lymphoma
LDH
what are reed sternberg cells and where are they seen
hodgkins lymphoma
binucelated cells - ‘owl eyes”. with eosinphilic inclusions
most common type of hodgkins lymphoma
nodular sclerosing
risk factors for hodgkins lymphoma
HIV EBV
staging system in lymphoma + describe
ANN ARBOUR
1- 1 lymph node
2- 2 lymph nodes on same side
3- nodes on both sides
4- spread
whats more common hodgkins or non hosgkins
non hodgkins
what cell is involved in burkitts lymphoma
b-cell high grade
genetic association of burkitts
c-myc trasnlocation (t18:4)
starry sky lymphoma
burkitts lymphoma
inherited causes of pancytopenia
fanconis anaemia
acquired causes of bone marrow failure/pancytopenia
idiopathic aplstic anaemia - autoimmune
myelodysplastic syndrome
acut emyeloid/lymphoblastic leukaemia
hypercellular marrow and marrow failure
myelodysplastic syndrome
b12/folate deficiency
hypersplenism
hypocellular marrow
aplastic anaemia
what can myelodysplastic syndrome progress to
AML
what ig is a pentamer adn what one is a dimer
pentamer - igM
dimer
IgA
in the body should antibodys be monoclonal or polyclonal. what is a monoclonal anitbody also called
polyclonal
monoclonal- undelying b cell plasma cell disorder
= paraprotein
what test is used to detect immunoglobulins
electrophoresis
what test is used to classify paraproteins
immunofixation
where would you find bence jones protein
urine
causes of paraproteinaemia
MGUS
myeloma
Amyloid
waldstrom
others eg lymphoma
what is MGUS
Monoclonal gammopathy of undetermined significance
an asymptomatic paraproteinaemia that can progress to myeloma
what is rouleoux formation and when is it seen
stacks of rbc seen in myeloma
how is myeoma classified adn whats most common
based on what Ig it produced - IgG most common
what monoclonal antibodies are raised in myeloma
IgA and IgG - called bence jones in urine
symptoms of myeloma
CRABBI
calicum - hypercalcaemia
Renal - failure due to light chain accumulation
A- anaemia (marrow failure)
B- bone lytic lesion
B-bleeding -thrombocytopenic -marrow failure
i infection reduced norm ig
how do lytic lesions occur in myeloma
plasma cells produce IL-6 which upregs osteoclasts
what can be given to stop light chain production
steroids
what can be used to measure response in myelom atx
paraprotein levels
tx for bone pain in myeloma
OPIATES not NSAIDS
what is the paraportien level seen in MGUS
<30
apple green bifringement on congo red stain
amyloid
what is walderstorms
neoplasm producing IgM paraprotein
why does waldenstroms cause hyperviscosity
IgM pentamer thickens serum
what is th ephiladelphia chromososme
t(9:22)
types of myeloproliferative neoplasm
CML
Essential thrombocythaemia
polucthaemia rubra vera
primary myelofibrosis
what is a myeloproliferative disorder
increase in one or more type of haemopoietic cells
what gene is involved i myeloproliferative disorders
BRC-ABL 1
what myeloproliferative disorder is BCR-ABL1 positive
CML
what is there too much f in chronic myeloid leukaemia
too many granulocytes/WCC
first line treatment of CML
imatinib
diagnostic testing for CML
genetic testign fro philadelphia chromosome
features of polcythaemia rubra vera
headache, fatigue, itch post shower
hyperviscosity therefor srterial and venou thrombosis
mutation seen in polycythaemia rubra vera
JAK2
treatment of polycythaemia
venesect till haematrocrit is <0.45
aspirin
hydroxycarbamine
essential thrombocytossi characteristic symptom
burning in hands
treatment of essential thromboytosis
hydroxyurae/carbamine
tear drop poikilocytes
myelofibrosis
treatment of autoimmune haemolytic anaemia
steroids
most common inherites thrombophilia
Factor V leiden
