myeloproliferative disorders Flashcards
these conditions occur due to
single type of stem cell
proliferating cell line: primary myelofibrosis
haematopoeitic stem cell
proliferating cell line: polycythaemia vera
erythroid cells
proliferating cell line: essential thrombocythaemia
megakaryocyte
what do myeloproliferative disorders have potential to progress to
acute myeloid leukaemia
assoc w what mutation
JAK2
myelofibrosis can be result of
primary myelofibrosis
polycythaemia vera or essential thrombocytopenia
what is myelofibrosis
where proliferation of cell line leads to fibrosis of bone marrow, and scar tissue formation
fibrosis affects production of blood cells and can lead to anaemia and leukopenia
myelofibrosis is in response to
cytokines released from proliferating cells
myelofibrosis: affect on liver and spleen
extramedullary haematopoiesis leads to hepatosplenomegaly
lead to portal htn
presentation
fatigue weight loss night sweats fever splenomegaly ascites
signs of polycythemia vera
conjunctival plethora
ruddy complexion
splenomegaly
FBC findings: polycythaemia vera
raised Hb
FBC findings: primary thrombocytopenia
raised platelet count
FBC findings: myelofibrosis
anaemia
leukocytosis or leukopenia
thrombocytosis or thrombocytopenia
