EXAM 3 - pathopharm II - hematopoietic disorders

what is hematopoiesis?

adequate blood cell production

what is immunocompetence?

normal immune system function capable of defending

what is immunodeficiency?

inadequate or impaired hematopoiesis or immune function, leading to a high risk of infections and cancer

what are pluripotent stem cells?

stem cells that are capable of becoming different types of cells

what are colony forming units (CFU)?

committed stem cells

what are cytokines?

hematopoietic growth factors

what do cytokines do (7)?

("mom cells")

1. reproduction

2. growth

3. differentiation of stem cells and CFUs

4. cell maturation

5. cell-cell communication

6. help with inflammation and wound healing

7. regulate all cellular activity

where are cytokines produced?

bone marrow and WBCs

what are the three cytokines?

1. colony-stimulating factors

2. interferons

3. interleukins

what are colony-stimulating factors?

a cluster pattern of hemopoietic cytokines that stimulate the bone marrow to produce and mature WBCs

what are interferons?

cytokines that interfere with viral replication and help cells communicate to trigger immune response

when interferons do their job, what symptoms occur?

symptoms of infection (flu-like symptoms)

what are interleukins?

cytokines that activate B and T lymphocytes that recognize antigens and invaders in immune responses

interleukins are also called...

antigen-activated

what is erythropoiesis?

production of adequate red blood cells

what is erythropoietin?

a hormone secreted by the kidneys that stimulates bone marrow production of RBCs

how is erythropoietin signaled?

decreased oxygen level detected by kidney cells

in kidney injury or disease, why does anemia often occur?

erythropoietin cannot be signaled for production because the kidneys are damaged

what is MPV?

average size of platelets

what does MPV show about anemia?

the lower the MPV, the higher the potential for anemia

what labs do we monitor for immune function?

WBCs, neutrophils, monocytes, and lymphocytes

how does absolute neutrophil count (ANC) measure immune function?

the lower the ANC, the higher the risk for infection and sepsis

what is anemia?

decreased RBCs

what does anemia result in?

decreases oxygen carrying capacity and tissue hypoxia

what are the s/s of anemia?

tachycardia, muscle weakness, fatigue

what symptom would we see in severe anemia?

pale skin

how do you calculate ANC?

WBCs x % of neutrophils

what is severe neutropenia?

ANC < 500

what is normal ANC?

3000-6000

what ANC indicated neutropenia precautions?

ANC < 2000

what is hypoproliferative anemia?

defect in production of RBCs

what are the 5 hypoproliferative anemias?

1. iron deficiency anemia

2. anemia in renal disease

3. anemia in inflammation

4. aplastic anemia

5. megaloblastic anemia

what is iron deficiency anemia?

when the body cannot take in adequate iron or meet the demands of the body

what are common causes of iron deficiency anemia?

GI bleeding and menstruation

what is anemia of prematurity?

anemia that occurs in preterm infants when there is impaired iron transfer from mom to baby

what is anemia in renal disease?

kidneys cannot produce erythropoietin and thus cannot produced RBCs

when we will begin seeing anemia effects in renal disease?

when the GFR < 30

what is anemia in inflammation?

nonprogressive anemia that results from chronic inflammation that decreases the process of erythropoiesis

what is aplastic anemia?

rare disease where the body's T cells are destroying and inhibiting the bone marrow stem cells

what else occurs in aplastic anemia?

neutropenia and thrombocytopenia (because these are also produced in bone marrow)

what is megaloblastic anemia?

deficiency of vitamin B12 or folic acid, causing abnormally large RBCs

what is the lifespan of a RBC?

120 days

what is hemolytic anemia?

erythrocyte breakdown/destruction

what are the 5 hemolytic anemias?

sickle cell disease, thalassemia, glucose-6-phosphate dehydrogenase deficiency, immune hemolytic anemia, hereditary hemochromatosis

what occurs in sickle cell disease?

abnormally crescent-shaped RBCs stack and clump together

what results in sickle cell disease?

tissue hypoxia and pain because blood cannot get to body parts and tissues

how do we treat sickle cell disease?

oxygen, fluids to swell the cells, and hydroxyurea

what is thalassemia?

group of hereditary disorders that have smaller than normal erythrocytes, which the body attacks and destroys

what is glucose-6-phosphate dehydrogenase deficiency?

when the body is exposed to certain foods, drugs, or stress, the body reacts and breaks down RBCs

what is immune hemolytic anemia?

immune system is attacking RBCs

what is hereditary hemochromatosis?

too much iron absorption causes destruction of RBCs

what is the prototype erythropoiesis-stimulating agent?

epoetin alfa

how does epoetin alfa work?

makes RBCs through bone marrow stimulation

epoetin alfa is not effective unless there is adequate...

iron

what should we monitor in patients on epoetin alfa?

iron stores

what does epoetin alfa treat (3)?

anemia, anemia in chemotherapy with hgb<10, anemia with CKD

what is the goal of epoetin alfa?

decreases need and cost associated with blood transfusions, lessen risk of infections passed via the blood transfusions, and enhance quality of life

what are the AE of epoetin alfa?

HTN, N/V/D, arthralgia

what patients should avoid taking epoetin alfa?

pts with uncontrolled hypertension

what are the 2 BBWs on epoetin alfa and why?

avoid use in patients with Hgb of 12 or higher; no benefit and causes harm if cancer patient isn't receiving chemo

why should a patient avoid use of epoetin alfa if their Hgb is higher than 12?

epoetin alfa increase Hgb, and if Hgb is too high our blood is too thick and can cause heart attacks and strokes

what is another type of erythropoiesis-stimulating agent and what is the difference between that one and epoetin alfa?

epoetin beta; BBW - NOT indicated or recommended for treatment of chemo-induced anemia

what is the prototype granulocyte colony-stimulating factor?

filgrastim

what does filgrastim do?

stimulates maturation, production, and activation of neutrophils in the bone marrow

what does filgrastim treat?

patients with neutropenia to prevent infections

what are the AE of filgrastim?

drowsiness, fatigue, flu-like symptoms, nausea, bone pain

what patients should avoid taking filgrastim?

pts with a known sensitivity to E. coli-derived proteins

what should filgrastim not be given within 24 hours of?

cytotoxic chemo

why can't filgrastim be given alongside cytotoxic chemo?

filgrastim would act as a growth factor for any tumor

what is the prototype interferon?

interferon alfa 2B

what does interferon alfa 2B treat?

chronic hepatitis B and C and several viruses

how does interferon alfa 2B work?

inhibits viral replication and boosts immune response

what are the AE of interferon alfa 2B?

flu-like symptoms, neutropenia, anemia, and thrombocytopenia

how is interferon alfa 2B administered?

IV, SQ, IM, intralesionally

what patients should avoid taking interferon alfa 2B?

pts with hepatic impairment or autoimmune hepatitis

what is the BBW on interferon alfa 2b?

causes or aggravates fatal or life-threatening neuropsychiatric, ischemic, autoimmune, and infectious disorders

when should interferon alfa 2B be discontinued immediately?

pts who develop severe decreases in neutrophil or platelet counts

what are the two interleukins used for treatment?

Aldesleukin and Oprelvekin

what is Aldesleukin used to treat?

metastatic kidney cancer and metastatic melanoma skin cancer

what is the action of aldeleukin?

activates cellular immunity

what is the BBW on aldesleukin?

only administered to patients with normal cardiac and pulmonary function and it should occur in a hospital setting with critical care expertise and under supervision of oncologist

what is oprelvekin used to treat?

stop platelet transfusion needs with specific nonmyeloid cancer

what is the BBW on oprelvekin?

high risk for allergic or hypersensitivity reactions, including anaphylaxis

what is the prototype antineoplastic?

hydroxyurea

what should hydroxyurea be taken with?

folic acid

what does hydroxyurea treat?

sickle cell disease

how does hydroxyurea work?

interferes with DNA synthesis of RBCs to reshape them

what is the goal of hydroxyurea?

decrease sickling, pain, and the need for transfusions

what are the AE of hydroxyurea?

leukopenia, secondary malignancy, decreased fertility in males

what important precaution should you keep in mind when administering hydroxyurea?

wear gloves when handling