Myeloproliferative Disorders Flashcards
(44 cards)
Too Many Red Cells
Polycythemia vera (PCV)
Too Many Platelets
Essential Thrombocytosis (ET)
Too Many Neutrophils
Chronic Myelogenous or Chronic Myeloid Leukemia (CML)
Myeloproliferative Syndromes
Symptoms?
Clonal Disorders of Blood Cells that would fall into the Neoplastic Spectrum
Fatigue, Abdominal Discomfort, Itching or Profuse Sweating
Generally Associated with Splenomegaly
Extra-medullary Hematopoiesis is Common
Disease Control for Many Years is Common
Cure is Not Possible at this Time
Polycythemia vera (PCV) Labs
Elevated RBC Mass Associated with a Mild Increase in WBCs and Platelets
Incidence 2/100,000 – 18/100,000 adults.
Incidence increases with increasing age.
Janus Kinase 2 (JAK2) plays a key role
symptoms of Polycythemia vera
Fatigue, headache, puritis (itching), flushing, abdominal discomfort due to splenomegaly.
Less common Sx: TIA, Retinal arterial occlusion, mesenteric or hepatic venous thrombosis or arterial thrombosis in a variety of organs.
Other Sx: Epistaxis, easy bruising, digital ischemia
2 things that are found on physical exam suggesting polycythemia vera?
Ruddy complexion
Mild splenomegaly
How to diagnose polycythemia vera?
When there is significant mature erythrocytosis (Hct > 60%) with some modest increase in mature platelets and neutrophils
The WBC & Platelets will be Normal and Hct > 52% in males or > 48% in females, but < 60%.
what are the definitive tests that are hard to obtain in the field for polycythemia vera?
are those of Total Body Plasma Volume and Total Body RBC Volume or Mass.
What are the tests that will be normal/ low or elevated in polycythemia vera?
Serum Erythropoietin Levels ( should be low)
Splenic Ultrasound: May show splenomegaly
Fe/TIBC (should be normal), B12 ( Should be normal but may be elevated). Folate Levels (should be normal)
Test Peripheral Blood for JAK2 mutation
near normal HCT
decreased MCV
P. Vera prognosis?
Most patients with P. vera will live for many years with their disease.
most patients with P. vera will evolve into an End Stage Picture of Myelofibrosis with myeloid metaplasia.
**ultimately this condition is fatal.
Treatment for PCV
Lower Hct to Avoid Symptoms d/t increased Whole Blood Viscosity.
The Simplest & Safest Way to Do this is to Phlebotomize the Patient with PVC till they are Fe Deficient.
Chemotherpeutic drugs
Anagrelide
Jakifi
Essential Thrombocytosis (ET) platelet count is what?
Platelet Count > ~600 x 103/ mm3 Without Other Etiologies (WHO)
4 Other Etiologies to Be Excluded from ET?
Fe Deficiency
Co-existent Infectious or Inflammatory Processes
Bleeding
Other Myeloproliferative Disorders
Signs and symptoms for essential thrombcytosis?
Tends to be more common in Females
Headache &/or Dizziness. Rarely TIAs
Occasionally Easy Bruising or Small Vessel Thrombi
Occasionally Bruising & GI Bleeding
Physical Exam is usually Normal (Mild Splenomegaly may be present)
What are the laboratory findings associated with essential thrombocytosis?
Hgb & Hct should be Normal
WBC may be slightly elevated with Normal Differential
Platelet Must Be & Some Giant Platelets are Expected
JAK2 V617F may be present
R/O Chronic Myelogenous Leukemia
Normal Fe/TIBC; Normal Ferritin; Normal Sedimentation Rate (Sed. Rate)
What can elevated platelet levels effect?
spurious increase K+ on Routine Chem Profile due to Release or K+ from Platelets when the Clot Forms in the Serum (Red Top) Tube
Spurious increase pO2 on ABGs due to the Hypermetabolic State in the ABG Syringe if not quickly Cooled and put on Ice.
Treatment of Essential Thombocytosis?
treatment is based on symptoms
Observation–asymptomatic
The Patient should be Cautioned About Sx to Watch for
There should be Routine Follow-up in the absence of Sx
Most hematologists would Rx if Platelets were > 10^6
The primary Goal of Rx is to lower the Platelet Count till the Patient is Asymptomatic
Anagrelide ( Agrelin) or Hydroxyurea (Hydrea) are the Mainstays of Rx
Chronic Myelogenous Leukemia or Chronic Myeloid Leukemia (CML)
(Myeloproliferative Disorders)
an Elevated White Blood Count with the Spectrum of Immature Blood Cells Normally seen in the Bone Marrow, Now Present in the Peripheral Blood.
Thrombocytosis and Anemia are also fairly Common
Basophilia is Almost Diagnostic
Signs and symptoms of CML
Fatigue, SOB, Unexplained Weight Loss,&/or Abdominal Discomfort Due to Splenomegaly
Thrombocytosis of > 106 associated with Signs or Sx of Wet Purpura, GI Bleeding or Thrombosis Requires Urgent Treatment
Splenomegaly will be present in most patients
Must Exclude Evidence of Infection or Inflammation
What is the chronic phase of CML?
Modest Sx Mild- Moderate Splenomegaly < 5% Blasts on CBC Easily Rx’d Ph1 + Only Chromosomal Abnormality Post TKI Rx: Average Not reached at > 8 Years
Accelerated phase of CML?
increased Sx Wt Loss, Bleeding, Splenomegaly
increased WBC with10 - 20 % Blasts on CBC
Worsening Anemia
Not Responding to Conventional Rx
Multiple Cytogenetic Abnormalities
Post TKI Rx: Salvage TKI Rx 12 -18 months average
Blast phase or blast crisis of CML?
Clinical Picture Looks Like Acute Leukemia Evolving Out of CML
after TKI treatment pt has survival of ~ 6 months
Treatment of CML?
Call the Hematologist
The Goal of Rx is Complete Molecular Remission
