Myeloproliferative disorders Flashcards
underlying pathology
uncontrolled proliferation of single type of stem cell
3 ones to remember
primary myelofibrosis
polycythaemia vera
essential thrombocythaemia
what proliferates in primary myelofibrosis?
hematopoietic stem cells
what proliferates in polycythaemia vera?
erythoid cell line
what proliferates in thrombocythaemia?
megakaryotic cell line
what do megakaryocytes produce?
thrombocytes
what can these progress into?
AML
gene mutations associated
JAK2
MPL
CALR
JAK2 inhibitors
ruxolitinib
what happens in myelofibrosis?
proliferation of cell line
fibrosis in bone marrow
replaced by scar tissue
myelofibrosis - cytokine
FGF
what can the fibrosis lead to?
anaemia and leukopenia
what can happen when bone marrow replaced with scar tissue?
extramedullary haematopoeisis
what is extramedullary haematopoesis?
haematopoiesis in liver and spleen to produce blood cells in other areas
other complications of myelofibrosis
hepatomegaly
splenomegaly
portal hypertension
spinal cord compression
myeloproliferative disorder presentation
fatigue
weight loss
night sweats
fever
which one does not have anaemia?
polycythaemia vera
which is thrombosis common in?
polycythaemia
thrombocythaemia
signs and symptoms of underlying complications
thrombosis infection bleeding and petechiae portal hypertension - ascites, varices abdo pain - splenomegaly
FBC - polycythaemia
raised haemoglobin
primary thrombocythaemia FBC
raised platelets
myelofibrosis FBC
anaemia
leucocytosis or leucopenia
high or low platelets
blood film myelofibrosis
teardrop shaped RBCs
poikliocytosis
blasts
poikliocytosis
varying sizes of RBCs
diagnosis
bone marrow biopsy
Genetic testing - JAK2
managing primary myelofibrosis
allogenic stem cell transplant
chemotherapy
supportive management
managing polycythaemia vera
venesection
aspirin
hydroxycarbamide
managing essential thrombocythaemia
asprin and chemotherapy
chemotherapy used
high risk - over 60, previous VTE
hydroxycarbamide
