Myeloproliferative Disorders (MPD)
Introduction
·
Myelo = marrow
·
4 types:
o Polycythaemia rubra vera
o Essential thrombocythaemia
o Myelofibrosis
o Chronic granulocytic leukaemia (=chronic myeloid leukaemia)
·
Primary, clonal proliferation of
myeloid (marrow) cells
·
Arises at pluripotent stem cell
level:
· Leads to mixed picture as diseases merge
· Variation is based on degree and type of proliferation:
· Leucoerythroblastosis:
o Nucleated RBC and immature neutrophils in blood
o Disturbance of blood/marrow barrier (normally stops immature cells
getting out)
o Causes:
§ Immature barrier: common in new born
§ Toxic: septicaemia
§ Hypoxia: respiratory failure
§ Marrow
function
§ Mechanical damage: e.g. infiltration of metastatic cancer (breast, lung,
prostate go to bones)
§ Extramedullary haematopoiesis:
·
Marrow cells outside marrow (e.g.
spleen, liver, lymph nodes)
·
Common in MPD, especially
myelofibrosis
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