The chronic myeloproliferative disorders mpd
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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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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



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MPD - inclusions and nomenclature is ‘reactive’

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 is ‘reactive’


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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) TPO

  • 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- TPOTypical 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


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Normal TPO


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Essential Thrombocythemia TPO

note giant platelets


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ET – clinical features TPO

  • None

  • Erythromelalgia

  • Peripheral Vascular Occlusion

  • Transient Ischemic Attack (TIA)

  • Stroke

  • Bleeding (esp surgical)


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ET – differential diagnosis TPO

  • reactive thrombocytosis

  • primary thrombocytosis

    • ET

    • PV, MF

    • Chronic Myeloid Leukemia


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ET- treatment TPO

  • None in low-risk cases

  • Anti-platelet agents (aspirin)

  • Platelet reduction treatment


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Polycythemia (Rubra) Vera (PV, PRV) TPO

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 TPO

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


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PRV - clinical features TPO

  • Headaches

  • Itch

  • Vascular occlusion

    • Venous thrombosis

    • TIA, stroke, MI

  • Splenomegaly


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PRV - diagnosis TPO

  • exclude secondary polycythemia

  • look for features of primary polycythemia

  • measure erythropoietin

  • JAK-2 mutation analysis


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PRV – differential diagnosis TPO

  • secondary polycythemia

  • spurious polycythemia


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PRV - treatment TPO

  • phlebotomy to control hematocrit

  • low-dose aspirin

  • hydroxyurea if necessary

  • avoid iron


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(Primary) Myelofibrosis (MF) TPO

  • neoplastic (clonal) hemopoietic stem cell disorder

  • bone marrow failure

  • myeloid metaplasia (extra-medullary hemopoiesis)


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MF - typical blood count TPO

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


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myelocyte TPO

nucleated rbc



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normal marrow trephine TPO

cells

fat

bone


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myelofibrosis TPO

fibrosis

new bone (arrows)


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MF - clinical TPO

  • Marrow failure

  • splenomegaly


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MF - diagnosis TPO

  • typical blood picture

  • splenomegaly

  • dry aspirate

  • fibrosis on trephine biopsy

  • absence of other cause


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MF - treatment TPO

  • supportive care

  • splenectomy or hydroxyurea if necessary

  • consider allo-BMT


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