the chronic myeloproliferative disorders mpd
Download
Skip this Video
Download Presentation
The Chronic Myeloproliferative Disorders (MPD)

Loading in 2 Seconds...

play fullscreen
1 / 31

The Chronic Myeloproliferative Disorders (MPD) - PowerPoint PPT Presentation


  • 167 Views
  • Uploaded on

The Chronic Myeloproliferative Disorders (MPD). John Matthews. MPD - concepts. Neoplastic (clonal) disorders of hemopoietic stem cells Over-production of all cell lines, with usually one line in particular Fibrosis is a secondary event Acute Myeloid Leukemia may occur.

loader
I am the owner, or an agent authorized to act on behalf of the owner, of the copyrighted work described.
capcha
Download Presentation

PowerPoint Slideshow about 'The Chronic Myeloproliferative Disorders (MPD)' - bran


An Image/Link below is provided (as is) to download presentation

Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author.While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server.


- - - - - - - - - - - - - - - - - - - - - - - - - - E N D - - - - - - - - - - - - - - - - - - - - - - - - - -
Presentation Transcript
mpd concepts
MPD - concepts
  • Neoplastic (clonal) disorders of hemopoietic stem cells
  • Over-production of all cell lines, with usually one line in particular
  • Fibrosis is a secondary event
  • Acute Myeloid Leukemia may occur
mpd inclusions and nomenclature
MPD - inclusions and nomenclature

1. Polycythemia (Rubra) Vera (PRV, PV)

2. Myelofibrosis (with Myeloid Metaplasia), Agnogenic Myeloid Metaplasia (MF,MMM, AMM)

3. Essential (Primary) Thrombocythemia

normal regulation of platelet numbers by thrombopoietin tpo
Normal Regulation of Platelet Numbers by Thrombopoietin - TPO
  • Constitutive production of thrombopoietin by liver
  • Bound by platelets
  • Excess stimulates megakaryopoiesis
essential thrombocythemia et
Essential Thrombocythemia (ET)
  • Neoplastic stem cell disorder causing dysregulated production of large numbers of abnormal platelets
  • Some cases non-clonal (esp young women)
  • Abnormal platelets aggregate in vivo, causing thrombosis
  • Abnormal platelets also cause bleeding
et typical blood count
ET-Typical Blood Count

WBC x 109/L 10.0 [4-11]

Hb g/L 156 [140-180]

MCV fl 85 [80-100]

Platelets x 109/L 1560 [150-450]

Neuts x 109/L 7.0 [2-7.5]

Lymphs x 109/L 2.0 [1.5-4]

Monos x 109/L 0.8 [0.2-0.8]

Eos x 109/L 0.1 [0-0.7]

Basos x 109/L 0.1 [0-0.1]

Film Comment: many large and abnormal platelets present

slide13
Essential Thrombocythemia

note giant platelets

et clinical features
ET – clinical features
  • None
  • Erythromelalgia
  • Peripheral Vascular Occlusion
  • Transient Ischemic Attack (TIA)
  • Stroke
  • Bleeding (esp surgical)
et differential diagnosis
ET – differential diagnosis
  • reactive thrombocytosis
  • primary thrombocytosis
    • ET
    • PV, MF
    • Chronic Myeloid Leukemia
et treatment
ET- treatment
  • None in low-risk cases
  • Anti-platelet agents (aspirin)
  • Platelet reduction treatment
polycythemia rubra vera pv prv
Polycythemia (Rubra) Vera (PV, PRV)

A neoplastic stem cell disorder possessing a JAK-2 mutation, which leads to excessive production of all myeloid cell lines, but predominantly red cells. The increase in whole blood viscosity causes vascular occlusion and ischemia, compounded by the increase in platelets.

prv typical blood count
PRV - typical blood count

WBC x 109/L 18.0 [4-11]

Hb g/L 200 [140-180]

HCt 0.62 [.42-.51]

MCV fl 75 [80-100]

Platelets x 109/L 850 [150-450]

Neuts x 109/L 14.6 [2-7.5]

Lymphs x 109/L 2.0 [1.5-4]

Monos x 109/L 0.8 [0.2-0.8]

Eos x 109/L 0.1 [0-0.7]

Basos x 109/L 0.5 [0-0.1]

Film: microcytosis: large and abnormal platelets present

prv clinical features
PRV - clinical features
  • Headaches
  • Itch
  • Vascular occlusion
    • Venous thrombosis
    • TIA, stroke, MI
  • Splenomegaly
prv diagnosis
PRV - diagnosis
  • exclude secondary polycythemia
  • look for features of primary polycythemia
  • measure erythropoietin
  • JAK-2 mutation analysis
prv differential diagnosis
PRV – differential diagnosis
  • secondary polycythemia
  • spurious polycythemia
prv treatment
PRV - treatment
  • phlebotomy to control hematocrit
  • low-dose aspirin
  • hydroxyurea if necessary
  • avoid iron
primary myelofibrosis mf
(Primary) Myelofibrosis (MF)
  • neoplastic (clonal) hemopoietic stem cell disorder
  • bone marrow failure
  • myeloid metaplasia (extra-medullary hemopoiesis)
mf typical blood count
MF - typical blood count

WBC x 109/L 2.4 [4-11]

Hb g/L 88 [140-180]

MCV fl 85 [80-100]

Platelets x 109/L 60 [150-450]

Neuts x 109/L 1.0 [2-7.5]

Lymphs x 109/L 1.0 [1.5-4]

Monos x 109/L 0.2 [0.2-0.8]

Eos x 109/L 0.1 [0-0.7]

Basos x 109/L 0.1 [0-0.1]

Film Comment: a few nucleated red cells and myelocytes (leukoerythroblastic). Tear-drop poikilocytes

slide25
myelocyte

nucleated rbc

slide28
myelofibrosis

fibrosis

new bone (arrows)

mf clinical
MF - clinical
  • Marrow failure
  • splenomegaly
mf diagnosis
MF - diagnosis
  • typical blood picture
  • splenomegaly
  • dry aspirate
  • fibrosis on trephine biopsy
  • absence of other cause
mf treatment
MF - treatment
  • supportive care
  • splenectomy or hydroxyurea if necessary
  • consider allo-BMT
ad