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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)

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


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


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


Essential Thrombocythemia


Normal Regulation of Platelet Numbers by Thrombopoietin - TPO

  • Constitutive production of thrombopoietin by liver

  • Bound by platelets

  • Excess stimulates megakaryopoiesis


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

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


Normal


Essential Thrombocythemia

note giant platelets


ET – clinical features

  • None

  • Erythromelalgia

  • Peripheral Vascular Occlusion

  • Transient Ischemic Attack (TIA)

  • Stroke

  • Bleeding (esp surgical)


ET – differential diagnosis

  • reactive thrombocytosis

  • primary thrombocytosis

    • ET

    • PV, MF

    • Chronic Myeloid Leukemia


ET- treatment

  • None in low-risk cases

  • Anti-platelet agents (aspirin)

  • Platelet reduction treatment


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

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


PRV - clinical features

  • Headaches

  • Itch

  • Vascular occlusion

    • Venous thrombosis

    • TIA, stroke, MI

  • Splenomegaly


PRV - diagnosis

  • exclude secondary polycythemia

  • look for features of primary polycythemia

  • measure erythropoietin

  • JAK-2 mutation analysis


PRV – differential diagnosis

  • secondary polycythemia

  • spurious polycythemia


PRV - treatment

  • phlebotomy to control hematocrit

  • low-dose aspirin

  • hydroxyurea if necessary

  • avoid iron


(Primary) Myelofibrosis (MF)

  • neoplastic (clonal) hemopoietic stem cell disorder

  • bone marrow failure

  • myeloid metaplasia (extra-medullary hemopoiesis)


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


myelocyte

nucleated rbc


Tear Drop Cells (or Tear Drop Poikilocytes)


normal marrow trephine

cells

fat

bone


myelofibrosis

fibrosis

new bone (arrows)


MF - clinical

  • Marrow failure

  • splenomegaly


MF - diagnosis

  • typical blood picture

  • splenomegaly

  • dry aspirate

  • fibrosis on trephine biopsy

  • absence of other cause


MF - treatment

  • supportive care

  • splenectomy or hydroxyurea if necessary

  • consider allo-BMT


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