CPHON EXAM 139 Questions with Verified Answers
Autoimmune Hemolytic Anemia is a group of disorders characterized by - CORRECT ANSWER malfunction of the
... [Show More] immune system where antibodies are produced against antigens on the surface of RBSs, resulting in hemolysis
T/F RBC survival is proportional to the amount of antibody on the RBC surface; therefore the greater the amount of antibody the more rapidly the RBC is destroyed. - CORRECT ANSWER True
Intravascular Hemolysis is - - CORRECT ANSWER RBC lysis in circulation - Antibodies bind to the RBC membrane therefore activating the complement cascade - damaged membrane causes increased osmotic pressure within cell and the cell bursts
Extravascular hemolysis is - CORRECT ANSWER occurs when complement fixation to RBC fails to activate complement cascade - the complement on the RBC surface interacts with receptors in macrophages in the lungs, liver, and spleen -> RBC phagocytosis
Causes of AIHA (4) - CORRECT ANSWER 1. Autoimmune disorders (LUPUS) 2. Infections (hepatitis, EBV, myco pneu) 3. Drugs (peni and quinine) 4. Hematologic disorders(Evan's syndrome and paroxysmal nocturnal hemoglobinuria)
Incidence of AIHA - CORRECT ANSWER 1 case per 80,000 persons
Clinical Symptoms of Severe AIHA - - CORRECT ANSWER - pallor - jaundice - fatigue - tachycardia -hypoxia --> organ damage - splenomagaly
What labs should be ordered if AIHA is suspected? - CORRECT ANSWER cbc d/p, retic, peripheral smear, Coombs test, bilirubin, LDH, and haptoglobin
Labs findings that suggest AIHA is possible: - CORRECT ANSWER 1. Coombs direct (DAT) : + which indicates antibodies against the RBC 2. Low Hemoglobin 3. Increased Retic 4. Spherocytes, schistocytes, or erythrocyte agglutination on blood smear 5. Increased LDH 6. Decreased haptoglobin 7. Hemoglobinuria 8. Increased Unconj bilirubin
Possible parts of the Treatment Plan of AIHA - CORRECT ANSWER 1. Stop medication if suspected as cause 2. Prednisone 2-4 mg/kg/day 3. High Dose IVIG 4.Splenectomy 5. pRBC transfusion 6. Folic Acid supplementation 7. Plasmapheresis (b/c IgM is confind to the intravascular space) 8. Cytotoxic agents 9. Immunosuppressive agents (Cyclosporine) 10. Hormonal therapy (danazol)
What are the three types of cytoxic agents that can be used for AIHA? - CORRECT ANSWER 1. Antimetabolites (6-mecaptupurine, azathioprine) 2. alkylating agents (cyclophosphamide) 3. Mitotic agents (vincristine, vinblastine)
T/F Cold Antibody AIHA is most common AIHA, in whcih the autoantibodies become most active and attack RBCs usually at temperatures well below normal. . - CORRECT ANSWER False. 75% of cases are warm body
Pathophysiology of Warm Body AIHA IgG - CORRECT ANSWER is the most common antibody > attaches to RBC > recognized by monocytes and macrophages in the spleen > destroy RBC membrane> RBC changes shape and singled out for destruction
T/F 50% of cases if warm anitbody AIHA are idiopathic (primary) - CORRECT ANSWER True
Prognosis of AIHA - - CORRECT ANSWER Usually transient - less than 3 months - usually resolve spontaneously
Cold Antibody AIHA is most common in children when - . - CORRECT ANSWER secondary to infection - IgM or IgG cold reacting antibodies that cross react with the ABO antigens on the surface of RBCs are produced
What organ is the main site of hemolysis in cold antibody AIHA? - CORRECT ANSWER Liver
Primary cold agglutinin disease is : - CORRECT ANSWER chronic or transient chronic
Most common infection causing secondary cold antibody AIHA is: - CORRECT ANSWER Mycoplasma pneumoniae but also viral (measles, mumps, flu, EBV, Adeno, VZV, CMV) and bacterial (syphilis and HIB)
T/F Blood products should be washed and warmed before transfusion for persons with Cold Antibody AIHA - CORRECT ANSWER True
6 nursing assessment and interventions for AIHA - CORRECT ANSWER 1. Read labs for decreased hemoglobin 2. Monitor for anemia 3. Monitor for transfusion complications (rxn and fluid overload) 4. Maximize child's physical tolerance 5. Teach family about AIHA 6. Teach aout post slenectomy care prn
Definition of Sickle Cell Disease - - CORRECT ANSWER a hemogloinopathy -DNA mutation for hbg production - normal hemoglobin or hgb A is absent, but hbg S is present (alone or in combination with another form of abnormal hemoglobin)
Who first described SCD? - CORRECT ANSWER James Herrick (1904
SCD pathophysiology - - CORRECT ANSWER Hbg two pairs of polypeptide chains (alpha and beta) > on 6th position on the Beta chain in hbg A - glutamic acid is replaced by valine > decreasing pliability and changing RBC biconcave nature > cells then sickle due to polymerization, forming microtubules or stiff rods within the cell they get clogged in vasculature> leading to tissue ischemia
Why do SCD pt have chronic anemia? - CORRECT ANSWER decrease life span of RBC due to friable nature, which leads to chronic state of anemia - usually 10-20 days
Conditions which increase chances of hypoxia or acidosis in SCD: (4) - CORRECT ANSWER infection, 1. fever 2. exposure to extreme 3. temperatures 4. dehydration.
Hx of SCD: - - CORRECT ANSWER evolved in W. Africa as a genetic mutation in response to malaria - Increase SCD where increase Malaria
Incidence of SCD - - CORRECT ANSWER most common inherited disorder in the US 72,000 people 1 in 500 AA 1 in 1000-14000 Hispanic Americans
SCD identified -. - CORRECT ANSWER universal newborn screeening which is performed in almost every state
T/F SCD is an autosomal recessive disease - CORRECT ANSWER True
Acute Complications of SCD are: (6) - CORRECT ANSWER 1. Fever and infection 2. Pain Crisis 3. Acute Chest Syndrome 4. Splenic Sequestration 5. Aplastic Crisis 6. Cerebral Vascular Accident of Stroke
What is the leading cause of death in SCD? - CORRECT ANSWER Streptococcus Pneumococcus Sepsis functionally asplenic > specific IgG antibodies to the polysaccharide encapsulated oraganisms
SCD pains is caused by (3) - CORRECT ANSWER 1. Ischemia > occlusion of blodd vessels by sickled RBCs 2. Damage to the vascular endothelium 3. Inflammation
Dactylilitis (hand and foot syndrome) is caused by : . - CORRECT ANSWER vaso-occlusive crisis that occurs in SCD pt babies and toddlers, usually first presenting symptom
Signs of Acute Chest Syndrome (occlusion of vessels to the lungs) are : - - CORRECT ANSWER rapid deteritionation in respiratory function - fever - increase O2 demand - infiltrate on Chest Xray
Nursing interventions for Acute Chest Syndrome are: (4) - CORRECT ANSWER 1. frequent eval of resp status 2. monitoring pulse oximetry 3. encourage incentive spirometry 4. ambulation
Acute Chest is treated with: (4) - CORRECT ANSWER 1. abx 2. pain management 3. increased oxygenation 4. transfusions (RBC or exchange) Rare- mechanical ventilation
What possible lab values will you see with Splenic Sequstration? (3) - - CORRECT ANSWER severe drop in Hbg - rise in retic - drop in platelets
What is autotransfusion in splenie sequestration? - CORRECT ANSWER when transfused pRBCs cause the spleen to release the trapped RBCs back into circulation, potentially increasing the blood's viscosity
Aplastic Crisis is most common associated with what virus in SCD? - CORRECT ANSWER parvo B19 infection ( usually fifth disease)
What labs are effected in aplastic crisis? - CORRECT ANSWER decrease hbg extremely low retic count
Chronic Complications with SCD (8) : - CORRECT ANSWER 1. Retinopathy 2. Cardiac and pulmonary changes 3. cholelithiasis 4. Avascular necrosis 5. Renal Impairment 6. Leg Ulcers 7. Delayed Growth and maturation 8. Impaired Cognition
Retinopathy and sickle cell is mostly commonly found in pts with : - CORRECT ANSWER Hbg SC
Chronic burden on the heart of anemia often results in (2): - - CORRECT ANSWER cardiomegaly - EKG changes
Hyposthenuria - CORRECT ANSWER The inability to conc urine resulting at time in nocturnal enuresis/ further dehydration
Leg ulcers happen in SCD pt as a results of: - CORRECT ANSWER poor perfusion of the skin usually lower legs usually start as small abrasion
SCD is diagnosis by and subtype determined by: - CORRECT ANSWER hemoglobin electrophoresis
Preventive interventions for SCD are: - - CORRECT ANSWER penicillin prophylactic - immunization with 23 valent pneumococcal vaccine - education ( esp regarding s/s of sepsis) - start hydrouxyrea if indicated
Hydroxyurea MOA and SE - - CORRECT ANSWER raise fetal hemoglobin level -decreases leukocytosis, platelets, and retic counts - myelosuppressive
Average life span of person with SCD: - CORRECT ANSWER 45-65 years
Poor prognostic factors associate with SCD: (3) - CORRECT ANSWER 1. dactylitis before 1 y/o 2. consistently elevated WBC 3. Hbg of 7gm/dL or less
Acute nursing goals for pt with SCD crisis: - - CORRECT ANSWER Assess - PE - Review Labs - ABX with fever -aggressive hydration - pain management - pRBC as indictated - cultures - chest xray
Hb AS - CORRECT ANSWER Carrier state
Hb SS - CORRECT ANSWER Usually severe SCD
HB SS with increased Hbg F - CORRECT ANSWER moderate to severe
Hb Sc - CORRECT ANSWER moderate to severe
Hb Sbeta0 Thalassemia - CORRECT ANSWER usually severe
Hb Sbeta+ Thalassemia - CORRECT ANSWER usually mild to moderate
Hb SO - Arab, SD - Punjab, - CORRECT ANSWER SE usually mild to moderate but may be severe
Def of Thalassemia - - CORRECT ANSWER a group of inherited disorders that affect the RBCs -Hbg A is abnormal in thalassemia pts - alpha and beta types - severity depends on the # of genes affected
Four alpha globin genes are located on what chromosome? - CORRECT ANSWER 16
Alpha thalassemia : - - CORRECT ANSWER two gene abnormality or greater - mild microcytic and hypochromic anema c 3 gene deletions = Hemoglobin H - moderate to severe c 4 gene deletions = Hydrops Fetalis - not compatible with postnatal life
T/F Iron supplementation will correct the anemia in Alpha Thalassemia - CORRECT ANSWER False
Diagnosis of Beta Thalassemia and clinical presenting symptoms : - CORRECT ANSWER - usually newborn screening - significant anemia - failure to thrive by 6 months of age
What bone changes can occur if transfusion needs are not met in patients with Beta Thalassemia? - - CORRECT ANSWER expanding marrow - bossing of forehead/skull - malocclusion of teeth - expansion of cheeks - paraspinal deformities
Extramedullary erythropoesis is: - CORRECT ANSWER a process whereby blood is produced outside of the marrow to compensate for severe anemia
Clinical finding of extramedullary erythropoesis: - CORRECT ANSWER enlarged spleen hypersplenism is hard to correct begins to destroy RBCs that are being transfused
T/F All forms of iron overload in Beta Thalassemia pt are related to pRBC transfusions - CORRECT ANSWER False It is the primary reason. However there is intermedia iron overload- increase absorption of dietary iron as the body tries to compensate for ineffective RBC production
Hemoglobin E is common in : - CORRECT ANSWER Southeast Asia 1 in 12 people are carriers
Diagnostic Tests associated with B- Thalassemia : - CORRECT ANSWER cbc d/p, hemoglobin electrophoresis , gene mapping , ferritin, endocrine fxn test, EKG, echo, bone density, liver biopsy, HLA, Audio, optho exam
Tx of Beta thalassemia; - - CORRECT ANSWER chronic transfusions (2-4 wks) - folic acid supplementation - Chelation therapy (deferoxiamine) - splenectomy - hydroxyurea - BMT
Most significant prognostic factor in Beta Thalassemia is : - CORRECT ANSWER degree of iron overload 70% of all deaths- leading to cardiac issues
Nursing assessments and intervention for extramedullary erythropoesis: - - CORRECT ANSWER review labs - monitor for facial or skeletal changes - transfuse pRBC if between 9-10 gm/dl - splenectomy if needed
Nursing assessments and intervention for chelation/iron overload: - - CORRECT ANSWER labs - s/s growth delay - s/s pubertal delay - assess cardiac - liver biopsy, SQUID, T2 MRI - Audiogram - chelation therapy - minimize dietary iron
Def of Glucose 6 Phosphate Dehydrogenase Deficiency: - - CORRECT ANSWER inherited, sex linked ( band Xq28) - metabolic disorber of RBCs - enzyme defect that causes hemolysis of RBCs
Pathophysiology of G6PD - - CORRECT ANSWER 1st enzyme in the pentose phosphate pathway of glucose metabolism deficiency- metabolizes gluthathione - Gluthathione is an antioxidant crucial for protection of RBC hbg and membrane - If gluthathione is too low, O2 will not bind and cell wall will break down > hemolysis
What is the most common metabolic disorder of the RBCs? - CORRECT ANSWER G6PDD 35 million worldwide - tropical and subtropics of the Eastern Hemisphere
G6PDD is expressed in ________ males and __________ females - CORRECT ANSWER hemizygous males (98 % ) homozygous females
What is the most classic manifestation of G6PDD? - CORRECT ANSWER acute hemolytic anemia
T/F Fava beans can cause acute hemolysis in pt with G6PDD - CORRECT ANSWER True and medications: antimalarials, analgesics, aulfonamides, aulfones, anthelminthics, aitrofurans, probenecid, dimercaprol, vitamin K analogues, and rasburicase
Lab findings for G6PDD: - - CORRECT ANSWER moderate to severe anemia - wide RBC distribution width - retic elevated - unconj bili elevated - WBC increased C increased granulocytes
Nursing Assessments for G6PDD: - - CORRECT ANSWER monitor labs - monitor for anemia - Transfuse pRBC prn - Assess for fluid overload - hydration (prevent risk of renal complications)
Hereditary Spherocytosis is - - CORRECT ANSWER inherited - hemolytic anema involving cell membrane alteration that results in fragile RBC trapped in spleen therefore short life span
Pathophysiology of hereditary spherocytosis: - CORRECT ANSWER RBC is smaller in diameter and more rigid > increased fragility and inability to pass through certain organs> destroys spleen > decreased life span of RBC 10-30 dyas
Clinical presentation of Hereditary spherocytosis : - - CORRECT ANSWER Anemia - Jaundice - Splenomegaly @ any age- usually hemolytic anemia and hyperbilirubinemia
Dx of Hereditary spherocytosis: - CORRECT ANSWER - family genetic evaluation - blood smear with spherocytes - elevated retic - indirect hyperbilirubenemia - + osmotic fragility test
TX of hereditary spherocytosis: - - CORRECT ANSWER epogen - if severe, blood transfusion (RARE) - folic acid supplementation ( if severe) - splenectomy ( sometimes partial)
Splenectomy in herediatry spherocytosis is performed so that: - CORRECT ANSWER RBCs can have a longer life cycle It is not a cure.
Nursing Assessment in Hereditary Spherocytosis: (4) - CORRECT ANSWER 1. assess for anemia 2. check for splenomegaly 3. Labs 4. Monitor pain
Bone Marrow Failure happens as a results of (3) - CORRECT ANSWER 1. deceas or damage to stem cells and their microenviroment ( hypoplatic or aplastic) 2. matruation defects ( deficiency of Vit B 12 or folate) 3. differentation defects, such as myelodysplasia
Peak ages for Bone Marrow Failure are: - CORRECT ANSWER 15-25 y/o and then older than 60 y/o
What chemical is strongly linked to genetic events that lead fo marrow failure? - CORRECT ANSWER Benezene
Drugs that can contribute to bone marrow failure: - - CORRECT ANSWER NSAIDs - neuroleptics - sulfonamides - corticosteriods - psychotropics
Tx for bone marrow failure: - - CORRECT ANSWER BMT - Or immune suppression with antithymocyte globin and cyclosporine
Clinical signs of aplastic anemia: (6) - - CORRECT ANSWER petechia - ecchymosis - anemia - pallor - fatigue - fever
T/F With bone marrow failure, the marrow is often replaced by fatty cells - CORRECT ANSWER True
What is the ANC count of someone with severe aplastic anemia? - CORRECT ANSWER 200 cells/mm 3
Congenital aplastic anemia (2) - CORRECT ANSWER Faconi Anemia Dyskeratosis Congenita
The most common congenital aplastic anemia is : - CORRECT ANSWER faconi
What is the age that Faconi's usually presents clinically? - CORRECT ANSWER 2 - 15 y/o can present at birth
Faconi Anemia is an autosomal _________ disorder - CORRECT ANSWER recessive
There are ___ genes assocaiated with Fanconi anemia. - CORRECT ANSWER These affect : 11 cell apoptosis, interference with tumor necrosis factor, propensity to malignancy BRCA2 is associated
Classic symptoms of Faconi Anemia: - - - CORRECT ANSWER skin pigment changes: darkened area of skin, cafe au lait spots, vitiligo - short stature - limbs anomalies - small testicles, genital changes - skeletal anomalies: hip, spine, rib - microcephaly - eye and eyelid abnormalities - ear abnorm; deafness broadened nose - kidney malformation: absent or horseshoe or hypoplastic - GI and cardio malformation - FTT/ low birth weight - mental retardation - an affected sibling
Lab findings for Faconi Anemia: - - CORRECT ANSWER macrocytic anemia - thrombocytopenia (early sign) - neutropenia - hypocelluar bone marrow and fatty
T/F All FA patients will develop MDS or AML - CORRECT ANSWER False but the risk is high 50% develop MDS or AML
Diagnostic Test for FA - - CORRECT ANSWER chromosome break analysis using dieposybutan or mitomycin-C
FA patients are at high risk for developing malignanies of the (3) - - CORRECT ANSWER head - neck - gynecological
Nursing interventions for FA: - - CORRECT ANSWER remind families children are more sensitive to carcinogens - decrease the amounts of even low dose radiation of Xray or CT scans to decrease chance of chromosome breakage.
Dyskeratosis Congenita is: - CORRECT ANSWER a rare inherited disorder- can be both autosomal recessive and dominant - progressive bone marrow failure - reticulated skin hyperpigmentation, nail dystrophy, and leukplakia - telomerase dysfunction, ribosome deficiency, and protein synthesis dysfunction
Dyskeratosis Congenita genetics: - CORRECT ANSWER DKC gene - DKC1 at chromosome Xq28 decrease telomerase activity which affect rapidly growing cells (skin, mucosa, bone marrow)
T/F Females tend to have more severe symptoms with DKC due to skewed X chromosome pattern. - CORRECT ANSWER false
DKC hyperpigmentation is most commonly found in: - - CORRECT ANSWER face - neck -shoulders - trunk
Clinica presentation of DKC: - - CORRECT ANSWER hyperpigmentation of skin - abnormalities of eyes - abnormalities of dentition - osteoporosis - short stature -urethral stenosis - hypospadias - hypoplastic testes - leukoplakia - thrombocytopenia
Diamon Blackfan anemia is what kind of inherited form of aplasia? - CORRECT ANSWER pure red cell
T/F The majority of acquired red cell aplasias occur in childhood and are almost always transient. - CORRECT ANSWER False Adult is more common and they are chronic
Transient Erythroblastopenia of Childhood is : - - CORRECT ANSWER an acquired red cell aplasia - acute - self limiting
Diamond Blackfan anemia is : - - CORRECT ANSWER rare - chronic - pure red cell aplasia - presents in the first 9 months of life - early death of pRBCs resulting in severe anemia
Incidence of Diamond Blackfan Anemia is: - - CORRECT ANSWER 400 cases per year world wide - more common in caucasians - equal between male and female, except recessive is more common in males
Diamond Blackfan Anemia: what are the three modes of transmission hypothesized? - CORRECT ANSWER 1. autosomal dominant 2. X linked pattern ( for recessive inheritance) 3. a new sporadic mutation
What gene is mutated for Diamond Blackfan Anemia and what chromosome is it located on? - - CORRECT ANSWER ribosomal protein gene RPS19 defect - chromosome 19q13.2
What are the clinical findings with Diamond Blackfan Anemia? - - CORRECT ANSWER Anemia ( most common) The following in <50% - Cathie face - very light blond heair, snub nose, wide set eyes, thick upper lip, "intelligent looking" , cleft palate or lips - Thumb abnormalities - Short stature with LBW - Cardiac defects - hypoplastic fenitalia, duplicate ureters, horseshoe kidneys
What are the lab findings for a Diamond Blackfan Anemia ? - - CORRECT ANSWER macrocytic anemia - decreased or absent reticulocytes - increased platelets >400k - increased fetal hbg - BM bx/a shows erthroid hypoplasia or aplasia
Dx of Diamond Blackfan Anemia is by: - CORRECT ANSWER BM bx/a : anemia, reticulocytopenia and absence of erythriod percursors
T/F Most patients with Diamond Blackfan Anemia need a short course of steriods to correct the issues. - CORRECT ANSWER False Chronic steriods; maintained on low dose steriods
Pts is Diamond Blackfan Anemia have an increase risk of ? - CORRECT ANSWER - leukemia - MDS
Transient Erthroblastopenia of childhood is commonly found : - - CORRECT ANSWER between ages 1-3 (peak @ 23 months) - with recent hx of viral illness or vaccination
Clinical presentation of Transient Erthroblastopenia of childhood is : - - CORRECT ANSWER anemia - recent hx of viral illness - normocytic anemia ( w/o any other cytopenias of familial hx of anemia)
Tx of Transient Erthroblastopenia of childhood : - observation - - CORRECT ANSWER single transfusion if anemia with flow murmur or tachycardia
Prognosis of Transient Erthroblastopenia of childhood : - CORRECT ANSWER 4-6 weeks spontaneously usually without relapse
Schwachman-Diamond Syndrome is: - - CORRECT ANSWER rare autosomal recessive disease - characterized by pancreatic insufficiency, failure to thrive, skeletal abnomalities, and BM dysfunction
The pathophysiology of Schwachman-Diamond Syndrome: - - CORRECT ANSWER exocrine pancreatic insufficiency - ancini cells do not develop and become replaced by fatty tissue & its digestive enzymes can not reach the GI tract to assit with digestion > malabsorption & malnutrition > fatyy, foul smelling stools, stomach pain, and cramping ( usually resolved around age 4) BM dysfxn: life long, chronic neutropenia. Have neutrophils but defective. Defects in B and T cells, and immunoglobins. 1/3 convert to AML or MDS
Schwachman-Diamond Syndrome genetic defect is believed to be on chromosome? - CORRECT ANSWER 7
Incidence of Schwachman-Diamond Syndrome is: - CORRECT ANSWER 1 in 20,000 births incr'd in male
What is the clinical presentation of Schwachman-Diamond Syndrome? - - CORRECT ANSWER FTT - unexplained weight loss - diarrhea - steatorrhea - eczema - frequent bacterial infections - heme issues: easy bruising, petechiae, bloody emesis, bloody stool - webbed toes or fingers may be present
Tx of Schwachman-Diamond Syndrome: - - CORRECT ANSWER pancreatic enzyme replacement - fat soluble vitamins - proph abx
Specific Dx Test for Schwachman-Diamond Syndrome: - - CORRECT ANSWER 72 hour fevel fat test - sweat test - pancreatic stimulation testing - serum immunoreactive trypsinogen - complete metabolic profile
Neutropenia is: - - CORRECT ANSWER ANC < 1500 cells/mm3 mild (ANC 1000 - 1500) moderate (500-1000) severe (<500)
Neutrophils last ________ with a half life of _________. - CORRECT ANSWER 1-2 days 8 hours
Chronic neutropenia predisposes children to what other complications (3) ? - - CORRECT ANSWER oral (gingivitis and periodontal disease) - Orthopedic ( bone demineralization) - and a subgroup have incr'd risk for MDS and AML [Show Less]