lecture 11 [hematologic & immunologic dysfunctions] Flashcards
what is the definition of hemostasis?
the process that stops bleeding when a blood vessel is injured
what blood components does hemostasis require?
- vascular clotting factors
- plasma clotting factors
- platelets
what is the definition of fibrinolysis?
clot breakdown
what is hemophilia?
a group of bleeding disorders characterized by difficulty controlling bleeding due to lack of clotting factors
what are the types of hemophilia?
- Hemophilia A (classic)
- Hemophilia B
- Von Willebrand Disease
which clotting factor does Hemophilia A lack?
factor VIII
which clotting factor does Hemophilia B lack?
factor IX
which is the most common type of hemophilia?
Von Willbrand disease
what is Von Willebrand hemophilia?
the inherited lack of the von willebrand factor protein characterized by inability of platelets to aggregate
both Hemophilia A & B are male dominant (x-linked recessive)
true
Von Willebrand disease affects both males & females
true
how is hemophilia diagnosed?
- hx of bleeding episodes
- evidence of x-linked inheritance
- PTT
- factor assays
- DNA testing
what are the medications & treatment for hemophilia?
(6)
- synthetic vasopressin (DDAVP)
- steroids
- factor VIII products
- pooled plasma
- recombinant products
- NSAIDs
what is the MOA of DDAVP (Desmopressin)?
increases plasma factor VIII (only for Hemophilia A)
what is the nursing care for hemophilia?
(4)
- monitor urine, stool, & NG fluid for occult blood
- administer Acetaminophen instead of aspirin
- administer injections via subQ only
- venipunctures are preferred for blood sampling
what is idiopathic thrombocytopenia purpura?
a decrease in platelet count manifested by discoloration caused by petechia beneath the skin
acute idiopathic thrombocytopenia purpura is most often seen after acquiring these diseases:
- MMR
- chickenpox
- parovirus B19 (Fifth’s disease)
what are the clinical manifestations of ITP?
- easy bruising
- bleeding from mucous membranes
what is considered a low platelet count?
less than 20,000
what is the function of the spleen?
controls the level of blood cells (RBC, WBC, PLT, etc) by elimination
what is often a therapeutic procedure for ITP?
splenectomy
what are the medications for ITP?
- prednisone
- IVIG
what are the neoplastic disorders?
- leukemia
- lymphoma
what are the types of leukemia in children?
- acute lymphoblastic leukemia
- acute myelogenous leukemia
acute lymphoblastic leukemia is more common than acute myelogenous
true
what are the s/s of leukemia?
- fever
- bone/ joint pain
- petechiae
- anemia
- bleeding
- pallor
what is the most common form of childhood cancer?
leukemia
what is leukemia?
unrestricted proliferation of immature WBCs
what is the diagnostic procedure for leukemia?
bone marrow aspiration
what are the stages of chemotherapy?
- induction
- CNS prophylaxis
- intensification
- maintenance
what is the goal of induction?
stage of chemotherapy
to achieve complete remission or reduce leukemic cells in bone marrow by 5% or less
what is the goal of CNS prophylaxis?
stage of chemotherapy
prevents leukemic cells from invading CNS
what is the goal of intensification (consolidation)?
stage of chemotherapy
to eradicate residual leukemia cells
what is the goal of maintenance?
stage of chemotherapy
maintain remission
what are the effects of late stage leukemia?
- cardiomyopathy
- cognitive delays & deficits
what are the complications of myelosuppression?
- infection
- anemia
- thrombocytopenia
what is considered an emergency for patients who are immunosuppressed?
fever
what are the lab tests & diagnostic procedures for immunocompromised patients?
- blood cultures
- urine cultures
- CXR
what are the prognostic factors for leukemia?
- initial WBC count
- Karyotype analysis
- type of cell involved
what are common side effects of chemotherapy?
- nausea
- vomiting
- hemorrhagic cystitis
- alopecia
- moon face
what is lymphoma?
a group of neoplastic diseases that arise from the lymphoid & hematopoietic system
what are the types of lymphoma?
- Hodgkin lymphoma
- Non-hodgkin lymphoma
Hodgkin lymphoma primarily affects which age group?
patients aged 15-19
Non-Hodgkin lymphoma primarily affects which age group?
children younger than 14 years old
what are the diagnostic tests for lymphoma?
(2)
- bone scan
- lymph node biopsy
what are the clinical manifestations of lymphoma?
- fever
- weight loss
- night sweats
- anorexia
- enlarged lymph nodes
- pruritus
what is the therapeutic management for lymphoma?
- radiation
- chemotherapy
what is the immunologic deficiency disorder in this lecture?
HIV/ AIDS
HIV is usually transmitted by lymphocytes & monocytes
true
in which bodily fluids is HIV found?
- blood
- semen
- vaginal secretions
- breast milk
what is vertical transmission of HIV?
mother passes it to infant during pregnancy
what is horizontal transmission of HIV?
through sexual contact or exposure to infected body fluids
what are the diagnostic tests for HIV?
- ELISA
- Western blot test
what is the treatment & prevention for HIV?
- antiretroviral drugs
- immunizations
what are the treatments for hematologic & immunologic deficiencies?
- blood transfusions
- hematopoietic stem cell transplant
what is the most severe reaction to a blood transfusion?
hemolytic rxn
what are the most common rxns to a blood transfusion?
- febrile
- allergic
what is intensive ablative therapy?
deplete entire bone marrow to prevent rejection of new stem cells w/ chemo or radiation
what are the steps to hematopoietic stem cell transplant?
- intensive ablative therapy
- stem cells infusion by IV
- engrafting
definition
engrafting
body accepts new bone marrow & proliferates
definition
autologous
HSCT
use of the patient’s own bone marrow cells
definition
allogenic
HSCT
use of matching histocompatible donor with a recipient
what is the goal of treating WBC disorders?
repair or replace damaged WBCs while stopping the proliferation of immature or damaged WBCs
what is the normal range for Hgb?
11.5 - 15.5g/dL
what is the normal range for Hct?
35 - 45%
what is a reticulocyte?
an immature RBC
in which condition are reticulocytes elevated?
chronic hemolytic anemia
purpose
neutrophils
primary defense of bacterial infection
purpose
eosinophils
defends against parasites, allergies, & neoplasms
purpose
basophils
increases blood flow to injured tissues
purpose
lymphocytes
(antibodies)
fights off antigens & delays hypersensitivity
purpose
monocytes
large phagocytic cells involved in the early stages of inflammatory rxns
definition
Mean Corpuscular Volume
- calculates svg size & volume of RBCs
- determines the type of anemia
definition
Mean Corpuscular Hemoglobin
calculates the avg amount of Hgb in (1) RBC
what is the normal range for platelets?
150-400
what is the main function of PLT?
helps clot blood
what are the types of anemias?
- iron deficiency
- sickle cell
- B-Thalassemia(Cooley)
- aplastic
what are the various reasons for anemia?
- decreased RBC production
- increased loss of RBC (from bleeding)
- increased hemolysis
what are common clinical manifestations of anemia?
- fatigue
- pallor
- decrease in Hgb
what are the main reasons for decreased RBC production?
- nutritional deficiency
- bone marrow failure
what are the reasons for increased hemolysis?
- membrane deficiencies (spherocytosis)
- Thalassemia
- chemotherapy
- radiation
what are the causes for iron deficiency anemia?
- decreased supply of iron
- malabsorption of iron
- change in Hgb synthesis
milk decreases absorption of iron
true
what does the nurse tell parents when giving their child iron supplements?
- give 2x a day in between meals
- give with Vit C
- iron turns stools tarry green
- it can stain teeth
- do not take with milk
what is the normal type of Hgb?
Hgb A
patients with sickle cell anemia have what type of Hgb?
Hgb S
predominantly; it replaces Hgb A
which diagnostic procedure is used for sickle cell anemia?
- hemoglobin electrophoresis
- sickledex finger stick
sickle cell anemia is an autosomal recessive disorder
true
what are the nursing actions for a sickle cell crisis?
- adequate oxygenation
- hydration
- transfusion
- ABX
- analgesics
- FEB
what are the s/s of vasooculsive crisis in sickle cell anemia?
- pain
- priapism
- acute chest syndrome
- stroke
what are the s/s of sequestration crisis in sickle cell anemia?
- spleenic sequestration
- hepatic sequestration
definition
sequestration
trapping of cells and debris in the spleen, causing a decrease in Hgb
what is the prevention for sepsis in sickle cell anemia?
prophylactic Penicillin
until 5 years old
what are the clinical manifestations of Cooley anemia?
B-Thalassemia
- small stature
- splenomegaly
- hepatomegaly
- decreased H&H
- abnormal blood smear
how does the patient present with untreated Cooley anemia?
- enlarged head due to prominent parietal & frontal bossing
- prominent malar eminences
- enlarged maxilla
- freckled & bronzed complexion
what is the therapeutic management for cooley anemia?
- chronic blood transfusions
- iron chelation essential
- splenectomy
what are the guidelines & parameters of chronic blood transfusion for Cooley anemia?
- required every 3-5 weeks
- maintain Hgb > 9.5
what is aplastic anemia?
a bone marrow failure condition in which formed elements of the blood are simultaneously depressed
what are the conditions associated with aplastic anemia?
- anemia
- leukopenia
- thrombocytopenia
what does hypoplastic anemia entail?
only the RBCs are decreased
how is aplastic anemia managed?
- Cyclosporin (immunosuppressive therapy)
- steroids
- HSCT
