Haem 3 Flashcards
malignant haemopiesis is what
increased number of dysfunctional cells that have lost the normal haemopoiteic reserve
one or more of what is in malignant haemopoiesis
increased proliferation
lack of differentiation
lack of maturation
loss of apoptosis
acute leukaemia
abnormal proliferation and lack of maturation
ix for acute leukaemia
excess of blasts >20% in either peripheral blood or bone marrow
decreased/ loss of normal haemopoietic reserve
ALL is a malig disease of what
most common what
symptoms
malig disease of lymphocytes
most common childhood cancer
very severe bone pain, BM failure : infections, bleeding, anaemia
increased WCC, venous obstruction, CNS involvement
AML in who may be what of two similar to what subgroups occurs after treatment of what ix
>60s may be denovo or decondary similar to ALL DIC or gum infiltration alkylating agents auer rods
what can be done as ALL and AML look the same sometimes
BM aspirate immunophenytyping
myeloproliferative disorders is what
clonal haemopoietic stem cell disorders with an increase production of one or more haemopoitetic cells - in contrast to acute leukaemia maturation is relatively preserved
common features of myeloproliferative disorders
asymp gout weight loss fatigue sweats splenomegaly thrombus in unusual place - no reactive explanation
CML is what
over production of grnaulocutes
pattern of CML
prolif of myeloid cells, chronic phase with intact maturation 3-5y, accelerated phase, blast crisis (acute leukaemia)
when is fatal of CML
fatal without stem cell/BM transplant in chronic phase
ix for CML
normal or low Hb
leucocytes/neurtrophilia
myeldoi precursors
eosinophilia, basophilia, thrombocytosis
philapdelphia
9+22 -> tyrosine kinase which causes abnormal phosphorylation - signalling leading to haematological changes in CML
treatment of CML
imatinib
PRV is overproduction of what
overproduction of RBCs
high Hb/hc accompanied by erythrocytes
ddx of secondary polycythaemia
chronic hypoxia, smoking, erytho secreting tumour
ddx of pseudopolycythaemia
dehydration, diuretics, obesity
aquagenic pruritus
polycythaemia ruba vera
ix for PRV
FBC
film
JAK2 mutation
px with secondary won’t have splenomegaly
JAK2
kinase
activation of erythropoiesis in absence of ligand
treatment of PRV
venesection to haemocrit <0.45
aspirin to reduce thromb risk
hydroxycarbomide - oral chemo
essential thrombocytopenia
high can lead to what
uncontrolled production of abnormal platelets
at high levels can lead to VW dx
symp of ET
MPD symp and bleeding (unpredictable risk of surgery)
microvascular occlusion
ix for ET
exclude reactive thrombosis
JAK2 in 50% CALR in rest MPL mutation
characteristic bone marrow appearance
treatment for ET
aspirin
hydoxycarbamide
anagrelide
interferon alpha last line
myelofibrous idiopathic
marrow failure, marrow fibrosis, extra medullary haemopoesis, leukthrombin film
tear dropped shaped RBC
myelofibrosis
when else can you get myelofibrosis
post polycythaemia or ET
BF in ET
tear dropped shaped RBC and leucoerythroblastic
dry aspirate
fibrate on trophine biopsy
JAK2 or CALR mutation
what are the causes of a leukoerythroblastic film
reactive - sepsis
marrow infiltration - cancer
myelofibrosis
treatment of myelofibrosis
transfusion, AB, allogenic SC tranplant, splenectomy
JAK2 inhibitors
CLL is the commonest what
leukaemia
CLL who
65-75
what is CLL
clonal expansion os small lymphocytes
symp of CLL
asymp recurrent infection anaemia splenomegaly lymphocytes >5x109/L
what is pancytopenia
anaemia
thrombocytopenia
neutropenia
decreased production
inherited bone marrow failure
arise due to to defects in DNA repair/ribosome
falcon;s
fanconi’s symp
what happens in it
short, skin abnormalities, radial ray abnormalities, hypogenitilia, endocrine, GI, haem, renal
unable to repair inter stand cross links
fanconi’s 97%
52%
BM failure by 20yo
leukaemia by 40yo
acquired primary BMF
aplastic anaemia
myelodysplastic dysplasia
acute leukaemia
aplastic anaemia
AI attack against haemopoetic stem cells
hypocellular
myelodysplastic dyaslsoa
hypercellular mass
ineffective haemopoiesis
20-30% risk of AML
acute leukaemia
prevention of normal haemopoiesis
secondary BMF
drug - chemo, chloramphenicol
alcohol, B12 and folate
infiltrative - malignancy, lymphoma, viral (HIV), storage disease
increased destruction
hypersplenism
portal htn, CCF, fieltys, splenic lymphoma
clinical features of pan
anaemia - fatigue, SOB
CDV compromise, neutropenia
infections - bleeding
symp depending on cause
ix for for pan
FBC, film, B12/folate
LFTs - hyersplenism
HIV/virology
AB tests (SLE)
test for fanconis
chromosome fragility test
hypocellular pan
hypercellular pan
aplastic
MDS, B12/folate, hypersplenism
rx for pan
malig - chemo congenital - transplan idiopathic aplastic anaemia - immunosuppression supportive transfusions ABs
normla splenic RC mass
RC transit
platelets pool
5%
fast
20-40%
hyposplenic RC mass
RC transit
platelets pool
40%
slow
90%
hodgkins symptoms
painless cervical LD mediastinum spread - cough hepatospleno B symp pruritus and alcohol related pain
burkitts translocation
8 and 14
burrkitts lymphoma
very high grade
mantle cell
similar tp low grade hodgkins but very aggressive and incurable with chemo
marginal zone NHL
low grade
other types of B cell NHL
follicular
diffuse large b cell - commonest world wide
lymphplasmocytic lymphoma
major haemorrhage protocol what does the blood bank send
4 units of RBC
4 frozen plasma
1 platelets
ongoing bleeding after transfusion management ix what should be given RBC how cryprecipitate when consider further what
repeat blood samples
transfuse further RBC:FFP 2:1 (1:1 in trauma)
cryoprecipitate if fibrinogen <1 (<2 in obstetric haem)
consider further platelets
management if bleeding stopped after transfusion Hb ATTP and PT platelets fibrinogen use what
Hb>80 AtPP and PT ration <1.5 platelets >50 fibrinogen >1.5 (>2 in obstetrics) use blood warmer
when should you assume neutropenic sepsis
if received chemo within past 3 weeks, temp >=38 or clinical evidence of significant sepsis SIRS>=2
standard risk px
neutropenia and sepsis and SEWS score <6
standard risk px treatment
piperacillin/tazobactam
PA teioplanin and aztreonam
if anaphylaxis or angiooedema Teicoplanin and ciprofloxacin
high risk px
neutropenic and severe septic/septic shock or SEWS >=6
high risk px treatment
pipercillin/tazobactem and gentamicin
PA - teicoplanin and aztreonam and gent
if anaphylaxis or angiooedema teicplanin and ciprofloxacin and gentamicin
when should AB be started in neutropenic sepsis
within 1hr
don’t wait for blood culture results
