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
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
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.
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
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
Reed Sternberg cells in LN biopsy
Slight to moderate neutrophilia
Eosinophilia in 20%
Micro/Hypo in advanced disease
Contiguous LN spread
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