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Myeloproliferative Disorders (MPDs)

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Title: Myeloproliferative Disorders (MPDs)


1
Myeloproliferative Disorders (MPDs)
2
MPDs1 of 2
  • Definition
  • Clonal hematopoietic stem cell diseases that
    result in expansion and excessive production and
    overaccumulation of erythrocytes, granulocytes,
    and platelets in some combination in the bone
    marrow, peripheral blood, and body tissues
  • They are grouped as myeloproliferative diseases
    because they may express common clinical
    features, laboratory changes, and pathogenetic
    similarities
  • Chronic conditions

3
MPDs2 of 2
  • Most common diseases included in the
    classification of MPDs
  • Chronic myelogenous leukemia (CML)
  • Polycythemia rubra vera (PRV or PV)
  • Essential thrombocythemia (ET)
  • Clinically, patients with MPD present in a
    clinically stable phase that may transform to an
    aggressive cellular growth phase such as acute
    leukemia or just a more aggressive form of MPD

4
Polycythemia rubra vera(PV)
  • A neoplastic, clonal overproduction of
    erythrocytes, granulocytes, and platelets to some
    degree (primary problem is too many RBCs)
  • The clonal neoplastic transformation arises in a
    pluripotential hematopoietic stem cell
  • RBCs are very sensitive to erythropoietin for
    cell growth
  • Clinical diagnosis increased red cell mass,
    maybe increased arterial O2 saturation, and
    splenomegaly, increased platelet counts or WBC
    counts
  • Disease progresses to acute leukemia in 15 of
    cases
  • Primary treatment is therapeutic phlebotomy

5
Essential Thrombocythemia(ET)
  • Manifests with increased megakaryocytopoiesis and
    peripheral blood thrombocytosis
  • PLT counts greater than 600,000/µL
  • Complications include thromboembolism and
    hemorrhage

6
Lymphoproliferative Disorders
7
Lymphoproliferative Disorders1 of 2
  • Refers to a large group of neoplastic lesions of
    the lymphoid system
  • Normal lymph nodes are composed of a cortex and
    medulla Follicles of varying size and shape are
    usually present in the cortex
  • Biopsy of lymph node may be submitted for
    histologic evaluation in formalin additional
    fresh tissue may be submitted for ancillary
    tests, including immunophenotyping, cytogenetics,
    and molecular diagnostics

8
Lymphoproliferative Disorders2 of 2
  • Most common diseases included in the
    classification of Lymphoproliferative Disorders
    are
  • Hodgkin lymphoma
  • Non-Hodgkin lymphoma
  • Plasma cell dyscrasias
  • Chronic lymphocytic leukemias (CLLs)
  • Other lymphoid proliferations that may behave in
    an aggressive fashion
  • Neoplastic lymphoproliferative disorders are
    broadly divided into Hodgkin and non-Hodgkin
    lymphomas

9
Malignant Lymphoma
  • Classification systems correctly used for
    diagnosis include Working Formulation, Revised
    European-American Classification of Lymphoid
    Neoplasms (REAL), and World Health Organization
    (WHO)
  • Subtypes of malignant lymphoma are broadly
    divided into B-cell and T-cell neoplasms
  • AIDS patients are at high risk for
    lymphoproliferative disorders, especially
    aggressive high-grade malignant lymphomas
  • Transplant patients may develop a wide array of
    post-transplant lymphoproliferative disorders

10
Hodgkin Lymphoma
  • A malignant lymphoproliferative disorder
    associated with fevers, night sweats, and
    cervical adenopathy
  • Diagnosis is characterized by the Reed-Sternberg
    cell (a large cell with a bilobed or polylobated
    nucleus and thick nuclear membrane, and looks
    like owl eyes)
  • Extranodal Hodgkin lymphoma may be found in bone
    marrow, liver or spleen

11
Non-Hodgkin Lymphoma
  • Includes a heterogenous group of disorders that
    differe in microscopic appearance, immunologic
    origin, and biologic behavior

12
Myelodysplastic Syndromes
13
Myelodysplastic Syndromes(MDSs) 1 of 3
  • A group of acquired clonal hematologic disorders
    characterized by progressive cytopenias in the
    peripheral blood, and dyspoiesis in the
    erythroid, myeloid, and/or megakaryocytic cell
    lines
  • The dyspoiesis is evidenced by abnormal
    morphologic appearance and abnormal function of
    the cell lines affected
  • Affect primarily the older population, occurring
    most frequently among people over 50 Uncommon
    in children

14
Myelodysplastic Syndromes(MDSs) 2 of 3
  • Three types of MDS have been recognized
  • Primary (de novo)
  • Therapy-related (TR-MDS)
  • Hereditary
  • All are the result of proliferation of abnormal
    stem cells
  • Currently, the classification of these syndromes
    is determined by a strict set of morphologic
    guidelines (FAB), although a new classification
    system has been proposed that includes molecular,
    cytogenetic, and immunologic characteristics (WHO)

15
Myelodysplastic Syndromes(MDSs) 3 of 3
  • Treatment depends on prognosis, and includes
  • Supportive therapy
  • Chemotherapy
  • Bone marrow transplant (the only cure)
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