PMF Flashcards
GIPSS
Genetically inspired international prognostic scoring system
- Karyotype
- Absence of CALR
- Molcular avaluation:
- ASXL1
- SRSF2
- U2AF1 Q157
MIPSS 70
Mutation and Karyotype-Enhanced International Prognostic Scoring System for Primary Myelofibrosis in adults 70 and younger
Only for pts younger than 70
Karyotype
High molecular risk (HMR) mutations
Absence of CALR
B symptoms
Anemia severity
> 2% blasts
median age
67
General clinical manifestations
Fatigue- 50-70%
Synptomatic splenomegaly 25-50%
B symp 5-20%
PHTN
Pruritus
Thrombotic complications
Vonous ans arterial
13% at or pre-diagnosis
10% post-diagnosis
Extramedulary hematopoesis
Hepato-splenomegaly
Lymphadeopathy
Surrounding the vertebral column
Skin, lung, pleura, pericard
Retroperitoneum
And any other site
Bone and joint involvement
Osteosclerosis
Periostetis
Gout
CBC
Anemia
Thrmobocytosis/Thrombocytopenia (as disease progress more cytopnia)
Leukocytosis/leukopenia
CD34+
PPV and NPV of 98% and 85% percent, respectively.
Increases as disuse progresses
Related to OS and blast crisis- but not independent prognostic factor
Abnormal laboratory tests
ALKP
LDH
uric acid
Leukocyte alkaline phosphatase
Vitamin B12
Gene mutations
JAK2 mutation – 60-65%
CALR mutation – 20-25%
MPL mutation – 5%
Triple negative – 8-10%
Risk factors for transformation
Blasts > 3%
Thrmbocytopenia < 100K
WHO grading
Based on fibrosis of BM
MF - 0 scattered
MF- 3 diffuse
Cytogenetic features
Very high risk (VHR): single or multiple abnormalities of -7, i(17q), inv(3)/3q21, 12p-/12p11.2, 11q-/11q23, or other autosomal trisomies not including +8/+9 (eg, +21, +19)
Favorable: normal karyotype or sole abnormalities of +9, 13q-, 20q-, chromosome 1 translocation/duplication, or sex chromosome abnormality including -Y
Unfavorable: all other abnormalities
High molecular risk (HMR) mutations
ASXL1 (38%)
SRSF2 (14%)
U2AF1Q157 (8%)
EZH2 (7%)
IDH1/2 (4%)
