Hematopoietic Function (Exam 3) Flashcards
Leukocytes (WBCs)
key players in inflammatory response and fighting infection
normal= 5000-10000 cells/mL in blood
Leukocytosis
increased leukocytes
indicate ongoing infectious process
Leukocytopenia/Leukopenia
decreased leukocytes
indicates immunosuppression or deficiency
Neutrophils
usually first leukocyte to arrive at infection site
2000-7500 cells/mL
Neutropenia
condition where concentration of neutrophils reaches <1500 cells/mL
M: DEPEND ON SEVERITY OR CAUSE, but include infections and ulcerations (especially mucous membranes) and other signs of infection like fever; ALL SIGNS OF IMMUNODEFICIENCY
Granulocytosis (Neutrophilia)
increase in granulocytes
NEUTROPHILIA IS EVIDENT IN FIRST STAGES OF AN INFECTION OR INFLAMMATION
Shift-to-the-left (A shift to immaturity)
IF THE NEED FOR NEUTROPHILS INCREASES BEYOND SUPPLY, IMMATURE NEUTROPHILS ARE RELEASED FROM BONE MARROW
SHIFT TO THE RIGHT WHEN POPULATION RETURNS TO NORMAL
Infectious Mononucleosis
the “kissing disease” spread by oral transmission, coughing, and sneezing; self-limiting and mostly prevalent in adolescents and young adults
EPSTEIN-BARR VIRUS (EBV), HERPES FAMILY
Infectious Mononucleosis Pathogenesis
incorporated B cells produce heterophile antibodies, some remain after recovery for life and may cause asymptomatic spread
insidious onset after 4-8 week incubation, causing anorexia, malaise and chills and manifestations will intensify to leukocytosis, fever, sore throat, lymphadenopathy, risk for splenic rupture
ACUTE PHASE 2-3 WEEKS, RECOVERY MAY TAKE UP TO 2-3 MONTHS
Lymphomas
cancers affecting the lymphatic system
Hodgkin Lymphoma
less common; solid tumors contain Reed-Sternberg cells often originating in lymph nodes of upper body (neck, chest, upper arms); primarily in adults 20-30 yrs, second peak 70 yrs
disease spreads from one lymph node to next via lymphatic vessels
several subtypes, most curable with chemotherapy, radiation or surgery
S/S of Hodgkin Lymphoma
enlarged painless lymph nodes which typically begin in upper body
mediastinal mass
splenomegaly
fever
weight loss
night sweats
pruritus (severe itching)
Non-Hodgkin Lymphoma
90% of all lymphomas, 5 yr survival rate is 68%; involves nodes throughout body and may originate in T/B cells
resembles Hodgkin lymphoma in manifestation, staging and treatment; spreads and is diagnosed differently (no Reed-Sternberg cells)
Leukemia
cancer of the leukocytes, the second most common blood cancer after lymphoma
most common cancer among children
leukemia cells abnormally proliferate and crowd normal blood cells and limits normal cell functioning
Risk Factors of Leukemia
mutagens (chemical, viral, radiation)
smoking
chemotherapy
diseases (Downs syndrome)
immunodeficiencies
Acute Lymphoblastic Leukemia
most common, primarily affects children, responds well to therapy, has good prognosis
less differentiated
Acute Myeloid Leukemia
affects primarily adults, responds fairly well to therapy, reasonably good prognosis
less differentiated
Chronic Lymphoid Leukemia
affects primarily adults, responds poorly to therapy but most patients live years after diagnosis since it’s slow moving
more differentiated
Chronic Myeloid Leukemia
affects primarily adults, prognosis is improved by allogeneic bone marrow transplant
more differentiated
Leukemia Manifestations
leukopenia (frequent infections)
anemia (fatigue, activity intolerance)
thrombocytopenia (increased bleeding risk)
lymphadenopathy
joint swelling
bone pain
weight loss
anorexia
hepatomegaly
splenomegaly
CNS dysfunction
Multiple Myeloma
cancer of the plasma cells, the third most common blood cancer and often affects older adults
Multiple Myeloma Pathogenesis
- excessive # of abnormal plasma in bone marrow crowd blood forming cells, destroys bone marrow by increasing osteoblast activities
- bone destruction: hypercalcemic pathologic fractures!
- causes Bence Jones protein excretion in urine
- often advanced progression when diagnosed
Multiple Myeloma Manifestations
insidious onset
anemia
thrombocytopenia
leukopenia
decreased bone density
bone pain
hypercalcemia
renal impairment
Anemia
decreased # of erythrocytes, reduction of hemoglobin, or abnormal hemoglobin
decreases oxygen-carrying capacity and creates tissue hypoxia; acquired or inherited
Anemia Manifestations
weakness
fatigue
pallor
syncope
dyspnea
tachycardia
Anemia Classifications
size (identified by terms that end in -cytic; macrocytic, microcytic, normocytic)
hemoglobin content (identified by terms that end in -chromic; normochromic and hypochromic)
Iron-Deficiency Anemia
most common as iron is necessary for hemoglobin production; hypochromic, microcytic anemia; most often seen in women of childbearing age, children younger than 2, elderly clients
CAUSES: decreased iron intake in diet/absorption issues, increased bleeding, GI disorders affecting iron absorption like celiac, gastritis and bariatric surgery
Iron-Deficiency Anemia Manifestations
cyanotic sclera
brittle nails
decreased appetite
headache
irritability
stomatitis
pica
delayed healing
Pernicious Anemia
vitamin b12 deficiency (required for DNA synthesis), usually caused by autoimmune lack of intrinsic factor; macrocytic and normochromic
leads to decreased maturation, cell division and potentially neurologic complications caused by myelin breakdown
Pernicious Anemia Manifestations
bleeding gums
diarrhea
impaired smell
loss of deep tendon reflexes
anorexia
personality or memory changes
positive Babinskis sign
stomatitis
parasthesia
unsteady gait
Aplastic Anemia
bone marrow depression of all blood cells (pancytopenia); often ideopathic, autoimmune, medical, viral or genetic
potentially insidious, sudden and severe onset
MANIFESTATIONS INCLUDE ANEMIA, LEUKOCYTOPENIA, THROMBOCYTOPENIA SYMPTOMS
Hemolytic Anemia
excessive erythrocyte destruction (hemolysis)
several different types including sickle cell anemia and thalassemia
Hemolytic Anemia Causes
idiopathic
autoimmune
genetics
infections
blood transfusion reactions
blood incompatibility in neonate
Sickle Cell Anemia
hemoglobin S causes erythrocytes to carry less oxygen and clog vessels, leading to hypoxia and tissue ischemia; more common in african/mediterranean descent, south and central america, caribbean, middle east
HETEROZYGOUS sickle cell trait means less than half of erythrocytes are sickles
HOMOZYGOUS means sickle cell disease (almost all erythrocytes are sickled)
SICKLED CELLS OCCLUDE BLOOD VESSELS AND BLOCK BLOOD FLOW
Sickle Cell Anemia Manifestations
typically appear around 4 months of age
painful episodes of tissue ischemia and necrosis that can last for hours to days, triggered by dehydration, stress, altitude or fever
Thalassemia
most autosomal recessive, some autosomal dominant; most common in mediterranean or asian, indian and african descent
ABNORMAL FORM OR INADEQUATE AMOUNT OF HEMOGLOBIN; ALPHA OR BETA
Alpha Thalassemia
4 genes (2 from each parent) are needed to make alpha protein, severity of symptoms depends on how many genes are missing
all 4 genes are missing=severe form called alpha thalassemia major
Beta Thalassemia
2 genes (1 from each parent) are needed to make beta protein
if 1 gene affected you’re a carrier, if both are affected you have moderate to severe anemia
Thalassemia Manifestations
abortion
growth and development delays
fatigue
dyspnea
heart failure
hepatomegaly
splenomegaly
bone deformity
jaundice
infections
liver and spleen filter RBCs; RBCs that are damaged or dying are trapped by spleen and liver which destroy the cells
Polycythemia Vera
a rare neoplastic disease of abnormally high RBCs that increase blood volume and viscosity, leading to tissue ischemia and necrosis; primary, secondary and relative
bone marrow produces too many RBCs, and sometimes too many leukocytes and platelets
Primary Polycythemia Vera
mutation in JAK2 gene leads to uncontrolled hematopoietic stem cell growth
Secondary Polycythemia Vera
long term low O2 levels (severe heart disease or lung disease, smoking, high altitudes, exposure to high levels of CO)
Relative Polycythemia Vera
appearance of increased RBCs because of reduction in plasma (dehydration)
Hypercoagulable State
clogging and occlusion of blood vessels
Polycythemia Vera Complications
tissue ischemia and necrosis
thrombosis
hypertension
heart failure
hemorrhage
splenomegaly
hepatomegaly
acute myeloblastic leukemia
Polycythemia Vera Manifestations
cyanotic/plethoric skin
hypertension
tachycardia
dyspnea
headaches
vision impairment
INTENSE, PAINFUL ITCHING THAT APPEARS TO BE INTENSIFIED BY HEAT OR EXPOSURE TO WATER (aquagenic pruritus) SO PEOPLE AVOID WARM WATER WHEN SHOWERING
Platelets
vital components of coagulation cascade
normal concentration range from 150000-350000 cells/mL
Thrombocytosis
increased platelet levels
INCREASES RISK OF THROMBUS FORMATION
Thrombocytopenia
decreased platelet levels <150000/mm3
INCREASES RISK OF BLEEDING AND INFECTION
<100000: becomes clinically significant
<50000: hemorrhage from minor trauma
<15000: spontaneous bleeding
<10000: severe bleeding
Thrombocytopenia Causes
DECREASED PLATELET PRODUCTION (viral infections, drugs/radiation therapy, chronic renal failure, bone marrow hypoplasia or cancer)
INCREASED PLATELET CONSUMPTION (Heparin-induced thrombocytopenia (HIT), Idiopathic (immune) thrombocytopenia purpura (ITP), Thrombotic thrombocytopenia purpura, Disseminated intravascular coagulation (DIC))
Hemophilias
inherited bleeding disorder that results in decreased coagulation (a mutation in a gene that provides instructions for making clotting factors); A or B
X-LINKED RECESSIVE
may be inherited or caused by spontaneous mutation of factor gene (30%)
Hemophilia A
factor VIII deficiency; more common (1 in 5000 male births)
Hemophilia B
factor IX deficiency (Christmas disease); 1 in 30000 male births
Hemophilia Manifestations
bleeding or signs of bleeding (bruising, petechia, etc.)
may see s/s during infant circumcision or when infant starts crawling/walking
hemarthrosis
Hemarthrosis
bleeding into the joints
Von Willebrand Disease (VWD)
most common hereditary bleeding disorder, characterized by decreased platelet adhesion and aggregation; multiple types with different inheritance patterns and may be acquired or spontaneous
mutation on chromosome 12 that causes deficit of Von Willebrand factors which normally causes platelets to aggregate and stick to vessel wall when injured
MANIFESTATIONS: abnormal bleeding that is hard to stop, indications of bleeding (bruising, petechia, etc.)
Immune Thrombocytopenic Purpura (ITP)
hypocoagulation due to autoimmune destruction of platelets; acute (self-limiting and more common in children with sudden onset) and chronic (more common in women and adults age 20-50)
MANIFESTATIONS: abnormal bleeding or indications of bleeding (petechiae, mucosal bleeding, epistaxis, purpura)
ITP Causes
idiopathic
autoimmune disease
live vaccine immunization
immunodeficiency disorders (AIDS)
viral infections
