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The Chronic Myeloproliferative Disorders (MPD) PowerPoint PPT Presentation


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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.

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The Chronic Myeloproliferative Disorders (MPD)

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The chronic myeloproliferative disorders mpd l.jpg

The Chronic Myeloproliferative Disorders (MPD)

John Matthews


Mpd concepts l.jpg

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


1 myeloproliferative disorders are clonal and the fibrosis is reactive l.jpg

1. Myeloproliferative disorders are clonal and the fibrosis is ‘reactive’


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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


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Essential Thrombocythemia


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Normal Regulation of Platelet Numbers by Thrombopoietin - TPO

  • Constitutive production of thrombopoietin by liver

  • Bound by platelets

  • Excess stimulates megakaryopoiesis


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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


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ET-Typical Blood Count

WBC x 109/L10.0[4-11]

Hb g/L156[140-180]

MCV fl85[80-100]

Platelets x 109/L1560[150-450]

Neuts x 109/L7.0[2-7.5]

Lymphs x 109/L2.0[1.5-4]

Monos x 109/L0.8[0.2-0.8]

Eos x 109/L0.1[0-0.7]

Basos x 109/L0.1[0-0.1]

Film Comment: many large and abnormal platelets present


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Normal


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Essential Thrombocythemia

note giant platelets


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ET – clinical features

  • None

  • Erythromelalgia

  • Peripheral Vascular Occlusion

  • Transient Ischemic Attack (TIA)

  • Stroke

  • Bleeding (esp surgical)


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ET – differential diagnosis

  • reactive thrombocytosis

  • primary thrombocytosis

    • ET

    • PV, MF

    • Chronic Myeloid Leukemia


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ET- treatment

  • None in low-risk cases

  • Anti-platelet agents (aspirin)

  • Platelet reduction treatment


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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.


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PRV - typical blood count

WBC x 109/L18.0[4-11]

Hb g/L200[140-180]

HCt0.62[.42-.51]

MCV fl75[80-100]

Platelets x 109/L850[150-450]

Neuts x 109/L14.6[2-7.5]

Lymphs x 109/L2.0[1.5-4]

Monos x 109/L0.8[0.2-0.8]

Eos x 109/L0.1[0-0.7]

Basos x 109/L0.5[0-0.1]

Film: microcytosis: large and abnormal platelets present


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PRV - clinical features

  • Headaches

  • Itch

  • Vascular occlusion

    • Venous thrombosis

    • TIA, stroke, MI

  • Splenomegaly


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PRV - diagnosis

  • exclude secondary polycythemia

  • look for features of primary polycythemia

  • measure erythropoietin

  • JAK-2 mutation analysis


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PRV – differential diagnosis

  • secondary polycythemia

  • spurious polycythemia


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PRV - treatment

  • phlebotomy to control hematocrit

  • low-dose aspirin

  • hydroxyurea if necessary

  • avoid iron


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(Primary) Myelofibrosis (MF)

  • neoplastic (clonal) hemopoietic stem cell disorder

  • bone marrow failure

  • myeloid metaplasia (extra-medullary hemopoiesis)


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MF - typical blood count

WBC x 109/L2.4[4-11]

Hb g/L88[140-180]

MCV fl85[80-100]

Platelets x 109/L60[150-450]

Neuts x 109/L1.0[2-7.5]

Lymphs x 109/L1.0[1.5-4]

Monos x 109/L0.2[0.2-0.8]

Eos x 109/L0.1[0-0.7]

Basos x 109/L0.1[0-0.1]

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


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myelocyte

nucleated rbc


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Tear Drop Cells (or Tear Drop Poikilocytes)


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normal marrow trephine

cells

fat

bone


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myelofibrosis

fibrosis

new bone (arrows)


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MF - clinical

  • Marrow failure

  • splenomegaly


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MF - diagnosis

  • typical blood picture

  • splenomegaly

  • dry aspirate

  • fibrosis on trephine biopsy

  • absence of other cause


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MF - treatment

  • supportive care

  • splenectomy or hydroxyurea if necessary

  • consider allo-BMT


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