Blood Dyscrasias Flashcards
Sickle Cell Anemia (SCA)
norman adult hemoglobin (HgbS) is partly or completely replaced by abnormal sickle hemoglobin S (HbS)
SCA Etiology
hereditary autosomal recessive disorder
primarily in africans/ eastern mediterrranean descent
SCA Dx
Prenatal: Amniocentesis Newborn screening: sickledex hemoglobin electrophoresis -differentiates trait from disease Transcranial Doppler (TCD) assess intracranial vascular flow ID CVA annually done ages 2-16
SCA Pathophysiology
RBS sickling > blood viscosity - blood flow obstruction - tissue hypoxia - tissue ischemia - ischemia causes pain > RBC destruction: chronic anemia
SCA s/s
start around 6 mos SOB/ fatigue tachy pallor impaired healing/ freq infections delayed physical growth systolic heart murmur & heart failure
3 conditions causing RBCs to sickle
low oxygen concentrations
acidosis
dehydration
SCA: Vaso occlusive crisis
Acute: -severe pain (bones, joints, abd) -swollen joints, hands, feet -anorexia, vomiting, fever -hematuria -obstructive jaundice -visual dsiturbances CVA
SCA: Vaso-occlusive chronic s/s
resp inf retinal detachment/ blindness systolic murmurs renal failure and enuresis liver enlargement/ failure spleen enlargement/ > inf seizures skeletal deformities
SCA Management
bed rest hydration pain management electrolyte replacement (corrective acidosis) blood products ABX (oral prophylactic penicillin) heat & passive ROM oxygen (short term)
SCA complication Prevention
Transfusion Program -q 4-5- weeks -maintaing Hgb 9-10 hydroxyureaw (PO med) - > fetal hgb which doesn't sickle BMT
SCA Pt & family education
freq rest periods no contact sports if spleen enlarged avoid infections avoid low O2 environments adequate hydration enuresis is a S/E of hydration heat to affected area maintain vaccination schedule
Hemophilia
A group of bleeding disorders from a congenital deficiency of coagulation proteins
most are X linked recessive factor VIII
mother is carrier and transmits to children
Hemophilia Dx
Females: DNA testing for trait
factor specific assays to ID deficiency
prolonged PTT
Hemophilia Pathophysiology
Liver decreased clotting factor production - factor assay <25% injury response remains intact -vasoconstriction -platelet aggregation
Hemophilia s/s
prolonged bleeding hemorrhage excessive bruising spontaneous hematuria hemathrosis (bleeding into joint cavity) hematomas (swelling, pain, limited ROM)
Hemophilia Complication Prevention
Primary: regular schedule of receiving factor VIII
Secondary: factor infusion after joint bleed
Mild Cases: DDAVP (desmopression which > Factor VIII)
Antifibrinolytic agents (Amicar) inhibits breakdown of fibrin use for mouthwash or trauma surgery
Hemophilia Management
Blood transfusions for severe bleeding RICE: rest, ice, compression, elevation severe bleeding may need aspiration pain management (No NSAIDS) bleeding precautions Active ROM (not passive)
Idiopathic/ Immune Thrombocytopenia Purpura (ITP)
immune mediated platelet destruction cased by platelet antibodies
Autoimmune process
ITP etiology
viral infections
drugs (heparin)
Collage vascular diseases (lupus)
ITP types
Acute
- majority of cases
- self-limiting
- resolves in days/weeks
Chronic
-duration > 6 mos
ITP Dx
No difinitive test
Platelet count < 20,000
ITP Management
restrict activity Anti-D antibody platelet replacement IVIG Prednisone Spenectomy for chronic severe form
Aplastic Anemia
All formed elements of the blood are depressed
- RBCs
- WBCs
- platelets
Aplastic Anemia Etiology
Bone Marrow Depression
systemic diseases interfering w/ bone marrow activity
70% ideopathic
Aplastic Anemia Dx
Bone Marrow Aspiration
Labs - < RBC
- < WBC
- < Platelets
Aplastic Anemia Management
immunosupressive therapy
colony stimulating factors
BMT
Iron Deficienct Anemia
Physiologic Anemia -normal process -onset 2-3 mos Iron Deficiency Anemia (9mos) -< iron intake -< iron stores -> iron loss -poor utilization of iron by body
Iron Deficient Anemia Assessment
Overweight milk baby
pallor, pale mucous membranes
content, tired, fatigued
Ages Seen: 6-24mos, toddlers, female teens
Iron Deficient Dx
< Hgb
TIBC totat iron binding capacity
Aplastic Anemia s/s
petechia ecchymosis pallor epistaxis fatigue/ weakness tachycardia anorexia infection
Iron Deficiency Tx
Infant: iron formula, iron supp, iron enriched cereal & food, limit milk intake to < 32 oz/day
Older child: > 12 mo. 24 oz milk max/day, may need to limit activities, need rest, iron supp
Iron Supplement Education
give on empty stomach use dropper to avoid teeth stains stools may be tarry/ > constipation don't give w/ dairy max effect: give in 3 divided doses/ day O.D. can be fatal- child proof vitamins over 6 months of age- give w/ citrus to > absorbtion
