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4 Meyloproliferative disorders

Polycythemia vera
Essential primary thrombocytopenia
Chronic Myelogenous Leukemia

Meyloproloferative disorders: unifying concepts

Clonal expansion from pleuripotent stem cell
Multiple cell lines usu affected though can be just one

Polycythemia vera: Etiology/incidence

increased Hgb and RBC mass
60 year old median onset

Relative PV

Low plasma volume
EPO Normal

Secondary PV

"Reactive PV"
Hypoxia, smoking, altitude, lung Dz
EPO Increased

Primary PV (vera)

Not self limiting
Tx = phlebotomy
Significant increases in all formed elements
EPO Decreased

When do we consider PV in the Dx?

Hct > 52% (18g/dl) in white males
47% (16g/dl) in AA population and females
Also when RBC, WBC, Platelets are all high with splenomegaly

PV: Diagnostic criteria

Last slide
Increased uric acid
EPO low or undetectable
RBC > 6 million/mm3
Hypercellular BM
RBC survival time decreased by 25%

What is the PB picture for PV?

Immature WBC's
Immature RBC with POIK/ANISO
May see DACROCYTES, microcytes, elliptocytes
Thrombocytosis, neutrophilia

Define Meyloid Metaplasia

Extramedullary hematopoiesis
Cells are formed in the liver and spleen

Define Meylofibrosis

Bone marrow that becomes fibrotic
Leads to difficulty making cells

What is the PB for Meylofibrosis?

Mild poik/polychromatophilia
NRBC's (nucleated RBC's)
WBC's: leukocytosis
Platelets: THROMBOCYTOPENIA (late)

Distinguish primary thrombocytopenia

Idiopathic platelet count above 500,000/microL
--Above in the absence of other meyloproliferative disorder features

Clinical presentation for Meylodysplastic Syndrome

Sxs nonspecific.
Fatigue, weakness, anorexia, weight loss, abd fullness.

CBC for MDS:

Monocytosis and up to 5% blasts

Define Leukemia

Malignant neoplasms of the blood-forming tissues.
Uncontrolled proliferation of a malignant clone and eventual marrow replacement with that line

Leukemic cells increase dt decreased reate of apoptosis

Acute Lymphocytic Leukemia: ALL

T-ALL: adolescents
B-ALL: <4 yo

LYMPHOBLASTS common in smear

More than 30% blasts in the smear usually means:


Always do what in ALL

BM Exam

Acute Myelogenous Leukemia: AML

MEYLOblasts in PB


Chronic Meylocytic Leukemia: CML

Philadelphia Chromosome
ABSOLUTE EOS/BAS increased!!

Age 20-50, rare in kids
Platelets high in 60%, low in 10%

Chronic Lymphocytic Leukemia: CLL

Middle age and older
LOW Platelets
WBC 20-150,000: mature lymphocytes

CLL DX is made by what factor?

Sustained ABSOLUTE lymphocytosis of >5000/ul for longer then 6mo

Refer to a hematologist when:

You see any blast cell on a PB smear!!

Hodgkin's Dx:

Reed Sternberg cells in LN biopsy
Slight to moderate neutrophilia
Eosinophilia in 20%

Micro/Hypo in advanced disease
Contiguous LN spread

What exacerbates Hodgkin's?


Define NHL:

Clonal proliferation of lympoid cells in LN, BM, Tonsils, slpeen, liver, GI

Features of NHL:

Anemia present in 30%
Non-contiguous spread
LN biopsy

Lab findings: Multiple Meyloma

RBC's Rouleaux
-> ESR elevated >100 mm/hr
55% make IgG
40% Bence Jones proteinuria

Anemia: Normo/normo

Waldenstrom's Labs:

Clonal expansion of IgM secreting plasma cells.
Cryoglobulins, RHfactor, cold agglutinins

Hx Raynauds

IgG or IgA?

Multiple Meyloma



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