Polycythemia Pg. 901

Polycythemia: number of RBCs in blood is greater than normal. Pt is hyperviscous (thicker than normal blood)

Can be temporary or chronic.

Polycythemia VeraPg. 901

Patho: PV: a disease with sustained increase in blood hgb levels to 18g/dL, an RBC count of

6mil, or a hct of 55% or greater. Fatal if left untreated.

PC is a cancer of RBCs with three major hallmarks:o Massive production of RBCso Excessive Leukocyte productiono Excessive production of platelets

Extreme hypercellularity (cell excess) of the peripheral blood occurs Pts facial skin and mucous have dark flushed appearance.

o Appear cyanotic b/c of poor oxygenation Intense itching Hypertension resulting from blood viscosity and increase demands on the heart Superficial veins distended Thrombosis in smaller vessels, occlusion

o Leads to hypoxia, anoxia, and later to infarction and necrosis. o Tissues most at risk:

Heart Spleen Kidneys

Cell life spans are shorter than normalo Cause rapid turnover of circulating cells

Increases the amount of cell debris which adds to the “sludging” of blood. Debris includes uric acid and potassium

o Which cause symptoms of gout and hyperkalemia Assessment

o Bone Marrow Aspiration Manifestations

o Poor oxygenation Hypoxia

o Bleeding problems b/c of platelet impairment

Patient-Centered Collaborative Care Malignant disease, progresses in severity.

o If untreated, pt lives 2 years after diagnosis

Treatment o Repeated phlebotomies (2 to five per week)

Pt may live 10 to 15 years longero Increase hydration and promoting venous return

Help prevent clot formationo Use of anticoagulantso Aspirin therapy may be used if benefits are weighed against the risk for GI

bleeding.o Hydroxyurea can be prescribed for severe manifestations.

Pt and Family Education

Drink 3L of fluid/dailyAvoid tight and constricting clothingwear gloves when outdoors when temps are lower than 50degreesKeep all health appts.Contact physician at first sign of infectionTake anticoagulants as prescribedwear support hose while you are awake and standing upElevate feet whenever seatedExercise slowly and only on the advice of physicianstop activity at first sign of chest painuse electric shaveruse soft bristeld tooth brushDo not floss btw teeth.

Myelodysplastic SyndromePg 901-902

MDSs- group of disorders caused by the formation of abnormal cells in the bone marrow.

o The abnormal cells are usually destroyed shortly after they are released into the blood

As a result, pts with MDS have decrease in blood cell lines Anemia is the most common problem (90% of MDS pts have it) Neutropenia and Thrombocytopenia are also present in pts with MDS Most common in people of 60 years or older Has cancer-like features

o Considered to be a precancerous stateo Arises from single population of abnormal cells, like cancer.

30% of pt tend to develop leukemia Problems can range from unresponsive anemia to cytopenia with increased blast cells Cause is unknown Risk factors:

o Normal physiologic changes with agingo Chemical exposures

Pesticides Benzene

o Tobacco smokeo Exposure to radiation o Chemo drugs

Diagnosiso Made by examining the chromosomes and genes within the chromosome

(Cytogenetic testing) of bone marrow cellso Peripheral blood smears

Used to assess level of maturation and proportion of abnormal cells Tx:

o Symptom management and supportive care Supportive care includes:

Blood transfusions and platelet transfusions Erythropoietin may be given

o Chelation therapy may be needed to reduce iron overload b/c of so many transfusions

o Drugs used for iron overload include deferasirox (Exjade) and deferomaxmine mesylate (Desferal, Desferrioxamine)