myoproliferative disorders Flashcards
what are myeoproliferative neoplasms
- group of chronic conditions
- clonal proliferation of marrow precursor cells
- includes polycythaemia, thrombocythamae, myelpfibrosis, chronic myeloid leukemia
how does polycythmia present
- puiritis (itching) after bath
- Erythromelalgia (burning)
- dizziness + headaches
investigations for polycythmia
- FBC- inc WCC, inc RBC, inc platelets + inc haemocrit, inc haemoglinb
- JAK2 V617F mutation — where positive in addition to a clear clinical history and haematological features it is considered definitive of polycythaemia vera.
- <0.48 (F), <0.49 (M)
what is normal level range of EPO (IU)
5-19 IU
who is polycythemia treated
- Venesection to maintain the haematocrit at less than 0.45.
- asprinin 75mg
- chemo (hydroxycarbamide) (also used for sickle cell)
what is the normal platelet count range (x10^9/L)
150 - 400 (x10^9/L)
how do you investigatet for thrombocytosis
- FBC- more than 450 platelets
- of detect low iron test with iron
what condition if defined as having a platelet cound to less than 150x10^9
throbocytopenia
what are the primary causes of thrombocytosis
- bone marrow disorder
*e,g poly
what are the secondary causes of thrombocytosis
- Acute- blood loss, infection/inflamm/alcohol cessaation
- chronic- iron deficiency, haemolytic anemia, asplenia(no spleen/ damaged spleen), IBD
how to treat thrombocytosis
Primary
* acetylsalysilic acid
* anagralide
* hydroxyurea
secondary
* treating route cause
*
what would a blood fopilm for JACK2 myelofibrosis show
- Leukoerythroblastic cells.
- Teardrop poikilocytes.
how do you treat myelofibrosis
- EPO injections
- jack 2 inhibitors (ruxolitnib)
- stem cell transplantation (curatuve)
- alagesics for spleed pain
- blood transfuction (pantopenia)
what causes primary myleofibrosis
JACK2 gene mutation
what 3 disease does JACK2 gene give rise too
- essential thrombocythemia
- polycythemia
- myelofibrosis
