Myeloproliferative Disorders

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Myeloproliferative disorders are characterized by proliferation of marrow stem cells, which
differentiate into erthyroid, granulocytic, megakaryocytic or fibroblastic cells. There
is frequent overlap between diseases, with transformation of one into another,
and termination of disease as ANLL.
Included in this group of diseases is polycythaemia rubra vera, which mainly occurs
after the age of 40 years, causing polycythaemia with all its complications,
particularly vascular thrombosis. The marrow contains increased numbers of erythroid precursors,
as well as increased numbers of megakaryocytes and fibroblasts. Splenomegaly is common, and 15%
of patients develop myelofibrosis,10% developing ANLL.
With treatment, overall survival is about ten years.
In primary thrombocythaemia there is thrombocytosis associated with bleeding tendency
or thrombosis. Splenic infarction and secondary hyposplenism is common.
Myelofibrosis is characterized by proliferation of fibroblasts in the marrow,
which is a response to growth factors secreted by abnormally proliferating myeloid cells.
As the marrow is replaced by fibroblasts and collagen,extramedullary haemopoiesis develops in the
spleen and liver, causing hepatosplenomegaly.
Clinically, patients develop anaemia and the signs and symptoms of a massively enlarged spleen.
The peripheral blood shows anaemia with abnormally shaped red cells and a leukoerythroblastic picture.
Patients typically survive for about 3 years, with approximately 10% developing ANLL.
Chronic granulocytic leukaemia is also a form of myeloproliferative disease with
splenomegaly, anaemia, and neutrophilia.
The bone marrow is replaced by myeloid cells in varying stages of differentiation.
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