Criteria/Risk/Stratification Flashcards
Systemic mastocytosis
Major
Multifocal clusters with >15% abnormal mast cells in BM or extracutaneous tissue
Minor
Elevated serum tryptase >20ng/ml
CD25+ mast cells
KIT D816V mutation
>25% atypical mast cells
Major + 1 minor
Or
3 minor
BCR::ABL1 breakpoints
BCR e1 + ABL1 a2/3 —> p190
BCR e13/14 + ABL1 a2/3 —> p210
MF diagnosis 2023
PMF initial work up
Spleen USS
Symptom score using MPN-10
Work up up ?portal HTN
BMAT with reticulum stain
JAK2, CALR, MPL
Myeloid gene panel
BCR::ABL1
MDT discussion
MF diagnosis 2023
PMF diagnostic criteria
Major
BM with mega atypia + retic 2-3/3
Clonal marker
Not CML, PV, ET or MDS
Minor
Unexplained anaemia
Leucocytosis
Splenomegaly
High LDH
Leucoerythroblastic film
All 3 major + 1 minor
MF diagnosis 2023
Post ET/PV MF diagnostic criteria
Major
Documented previous PV/ET
BM fibrosis 2-3/3
Minor
New anaemia (eg no longer venesected)
2/3 fever, weight loss, night sweats
Increasing splenomegaly
High LDH (post ET)
Leucoerythroblastic film
Both major + 2 minor
MF diagnosis 2023
Secondary causes BM fibrosis
HIV, TB, Leishmaniasis
SLE, APLS
Hairy cell
MDS, Hodgkin, CML, SM
Solid tumour mets
TPO-A
MF diagnosis 2023
MF Prognostic scores
IPSS - age >65, Hb <100, WCC >25, blasts >1%
DIPSS - same + symptoms
MIPSS70
MF diagnosis 2023
MF stratified survival
DIPSS
Low 15 years
Int-1 6.5 years
Int-2 2.9 years
High 1.3 years
MF diagnosis 2023
MF stratified treatment
Low/int-1 asympt - observe/trial
Low/int-1 symptoms - JAKib peg-IFN
Int-2/high - JAKib then alloSCT
Rux
Dosing depends on platelets
Withdraw = SIRS
Risk skin cancer
Shingrix
Anaemia may respond to EPO, danazol
Camitta criteria
NSAA
BM cellularity <25%
SAA
NSAA + 2/3
Retic <60
Plat <20
Neut <0.5
VSAA
SAA + neut <0.2
Initial work up AA
Drug history + context (eg anorexia)
FBC + film + retic + HbF + Htinics
Chromosomal breakage if <50yrs (DEB)
PNH FLAER
Assess spleen/syndromic kidneys-heart-lungs-skeleton
Autoimmune
Viral inc parvo
BMAT
Myeloid gene panel
MDT inc consider IBMF syndrome
Consider clin genetics referral
Camitta stratified treatment AA
NSAA
CSA + horse ATG if transfusion dependent, bleeding or recurrent infections
SAA/VSAA
If MSD and <40 or 40-50 and fit —> upfront HSCT
CSA + horse ATG + eltrombopag if no MSD
CSA + horse ATG + eltrombopag if MSD and >40
ATG - patient must be stable, ideally afebrile, plt >20, prophylactic antibiotics, given in experiences centre
MUD/alternative
If young/fit upfront
In others if failed CSA-ATG
Syngeneic
Consider in all
Pregnant
Supportive transfusion
Can use CSA if needed
Criteria for anti-C5 in PNH
Thrombosis
Renal impairment or pul htn 2o haemolysis
Pregnancy
Haemolysis with anaemia and LDH >1.5ULN
CMML diagnosis
Prerequisite
Persistent mono >0.5 and >10% WCC
Blasts <20%
Not CML or other MPN
Not myeloid neoplasm with TK fusion
Supporting
Dysplasia in at least 1 lineage
Clonal marker
Abnormal mono subsets
All prerequisites
If mono >1 then 1 supporting
If mono 0.5-1 then supporting 1-2
WCC >13 —> MPN type
CMML-2 —> 5-19% blasts blood (10-19% BM)
Criteria for prophylaxis in haemophilia
1o
All children with F <3 IU/dl, start after 1st bleed or before 2yr
2o
If 2x spontaneous joint bleeds
