Myeloproliferative
Disorders (MPDs)
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MPDs
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► 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
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MPDs
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► 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
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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
►
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Essential Thrombocythemia
(ET)
► Manifests
with increased
megakaryocytopoiesis and peripheral blood
thrombocytosis
► PLT counts greater than 600,000/μL
► Complications include thromboembolism
and hemorrhage
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Lymphoproliferative
Disorders
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Lymphoproliferative Disorders
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► 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
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Lymphoproliferative Disorders
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► 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
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Malignant Lymphoma
► Classification
systems correctly used for diagnosis
include Working Formulation, Revised EuropeanAmerican 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
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Hodgkin Lymphoma
►A
malignant lymphoproliferative disorder
associated with fevers, night sweats, and
cervical adenopathy
► Diagnosis is characterized by the ReedSternberg 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
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Non-Hodgkin Lymphoma
► Includes
a heterogenous group of disorders
that differe in microscopic appearance,
immunologic origin, and biologic behavior
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Myelodysplastic
Syndromes
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Myelodysplastic Syndromes
(MDSs)
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►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
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Myelodysplastic Syndromes
(MDSs)
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► 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)
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Myelodysplastic Syndromes
(MDSs)
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► Treatment
includes:
depends on prognosis, and
 Supportive therapy
 Chemotherapy
 Bone marrow transplant (the only cure)
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