Someone recently bought our

students are currently browsing our notes.


Myeloproliferative Disorders Notes

Medicine Notes > Haematology Notes

This is an extract of our Myeloproliferative Disorders document, which we sell as part of our Haematology Notes collection written by the top tier of University Of Leicester students.

The following is a more accessble plain text extract of the PDF sample above, taken from our Haematology Notes. Due to the challenges of extracting text from PDFs, it will have odd formatting:

Myeloproliferative Disorders A group of four disease caused by clonal proliferation of haemopoeitic stem cells
- Chronic myeloid leukaemia
- Myelofibrosis
- Polycythaemia ruba vera
- Essential throbocythaemia

Chronic myeloid leukemia Clonal proliferation of myeloid precursor cells (neutrophils, basophils, eosinophils) Chronic = abnormality further down the cell line therefore more differentiated cells
= More treatable Pathogenesis
- Presence of Philadelphia chromosome (9:22 translocation forming bcrabl gene) o Produces a protein that is thought to be oncogenic Clinical features
- 3 distinct clinical phases Primary = chronic phase - symptoms of anaemia, anorexia and weight loss May have marked splenomegaly. Neutropenia or thrombocytopenia not usually present Secondary = accelerated phase - insidious deterioration. Requires increased amount of treatment to control spleen size and WCC Tertiary = blast phase - usually fatal. Presents as an acute leukaemia Diagnosis
- Blood count o Raised WCC - usually >100/10x9 o Low Hb o Low platelets
- Film o Shows increasing numbers of morphologically normal myelocytes and neutrophils o Basophilia o Blast cells
- BM aspirate and trephine o Less useful o Cytogenetics show Philadelphia chromosome (in 95% of cases) Management
- Chronic phase o Chemo o Alpha interferon o Allogeneic stem cell transplant - only cure o Imatanib - tyrosine kinase inhibitors for Philadelphia chromosome BCR-abl gene) - targeted treatment
- Blast crisis o Management as for acute leukaemia


Can induce remission back into 'chronic phase' - temporarily

- Best predictor = initial response to treatment
- Can monitor with blood counts and cytogenetics (ph. Cr disappears therefore bcr-abl reduces)

Myelofibrosis Clonal proliferation of haemopoeitic stem cells, which release growth factors
- Particularly platelet-derived growth factor and cytokines Epidemiology
- Mean age of presentation = 60years Clinical features
- Anaemia
- Splenomegaly (often painful and massive)
- Systemic symptoms o Weight loss o Fever Investigations
- FBC o Anaemia o Raised WCC o Raised platelets
- Blood film o Immature red and white cells (leukoerythroblastic change) o Tear-drop RBC
- Bone marrow aspirate and trephine o BM often inaspirable due to fibrosis o Trephine biopsy is hypocellular with increased marrow fibrosis Management
- Supportive treatment o Blood transfusion, anabolic steroids and EPO for anaemia o Splenectomy useful in painful splenomegaly or to reduce transfusion requirements
- Hydroxyurea can be used to control high WCC and reduce spleen size o But has no impact on marrow fibrosis Prognosis
- Median survival 4 years
- Poor prognostic factors = increasing age, anaemia, leukopenia, abnormal marrow karyotype

Buy the full version of these notes or essay plans and more in our Haematology Notes.