4 Meyloproliferative disorders
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
Low plasma volume
Hypoxia, smoking, altitude, lung Dz
Primary PV (vera)
Not self limiting
Tx = phlebotomy
Significant increases in all formed elements
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
Increased uric acid
EPO low or undetectable
RBC > 6 million/mm3
RBC survival time decreased by 25%
What is the PB picture for PV?
Immature RBC with POIK/ANISO
May see DACROCYTES, microcytes, elliptocytes
Define Meyloid Metaplasia
Cells are formed in the liver and spleen
Bone marrow that becomes fibrotic
Leads to difficulty making cells
What is the PB for Meylofibrosis?
NRBC's (nucleated RBC's)
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
Fatigue, weakness, anorexia, weight loss, abd fullness.
CBC for MDS:
Monocytosis and up to 5% blasts
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
B-ALL: <4 yo
LYMPHOBLASTS common in smear
More than 30% blasts in the smear usually means:
Always do what in ALL
Acute Myelogenous Leukemia: AML
MEYLOblasts in PB
-> AUER RODS
Chronic Meylocytic Leukemia: CML
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
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!!
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?
Clonal proliferation of lympoid cells in LN, BM, Tonsils, slpeen, liver, GI
Features of NHL:
Anemia present in 30%
Lab findings: Multiple Meyloma
-> ESR elevated >100 mm/hr
55% make IgG
40% Bence Jones proteinuria
Clonal expansion of IgM secreting plasma cells.
Cryoglobulins, RHfactor, cold agglutinins
IgG or IgA?