Cphon : Section 9 Hematology

Front 1

Autoimmune Hemolytic Anemia is a group of disorders characterized by

Back 1

- malfunction of the immune system where antibodies are produced against antigens on the surface of RBSs, resulting in hemolysis

Front 2

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.

Back 2

True

Front 3

Intravascular Hemolysis is

Back 3

- 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

Front 4

Extravascular hemolysis is

Back 4

- 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

Front 5

Causes of AIHA (4)

Back 5

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)

Front 6

Incidence of AIHA

Back 6

1 case per 80,000 persons

Front 7

Clinical Symptoms of Severe AIHA

Back 7

- pallor
- jaundice
- fatigue
- tachycardia
-hypoxia --> organ damage
- splenomagaly

Front 8

What labs should be ordered if AIHA is suspected?

Back 8

cbc d/p, retic, peripheral smear, Coombs test, bilirubin, LDH, and haptoglobin

Front 9

Labs findings that suggest AIHA is possible:

Back 9

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

Front 10

Possible parts of the Treatment Plan of AIHA

Back 10

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)

Front 11

What are the three types of cytoxic agents that can be used for AIHA?

Back 11

1. Antimetabolites (6-mecaptupurine, azathioprine)
2. alkylating agents (cyclophosphamide)
3. Mitotic agents (vincristine, vinblastine)

Front 12

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.

Back 12

False. 75% of cases are warm body.

Front 13

Pathophysiology of Warm Body AIHA

Back 13

IgG 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

Front 14

T/F

50% of cases if warm anitbody AIHA are idiopathic (primary)

Back 14

True

Front 15

Prognosis of AIHA

Back 15

- Usually transient
- less than 3 months
- usually resolve spontaneously

Front 16

Cold Antibody AIHA is most common in children when

Back 16

- secondary to infection
- IgM or IgG cold reacting antibodies that cross react with the ABO antigens on the surface of RBCs are produced.

Front 17

What organ is the main site of hemolysis in cold antibody AIHA?

Back 17

Liver

Front 18

Primary cold agglutinin disease is :
chronic or transient

Back 18

chronic

Front 19

Most common infection causing secondary cold antibody AIHA is:

Back 19

Mycoplasma pneumoniae

but also viral (measles, mumps, flu, EBV, Adeno, VZV, CMV) and bacterial (syphilis and HIB)

Front 20

T/F
Blood products should be washed and warmed before transfusion for persons with Cold Antibody AIHA

Back 20

True

Front 21

6 nursing assessment and interventions for AIHA

Back 21

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

Front 22

Definition of Sickle Cell Disease

Back 22

-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)

Front 23

Who first described SCD?

Back 23

James Herrick (1904)

Front 24

SCD pathophysiology

Back 24

- 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

Front 25

Why do SCD pt have chronic anemia?

Back 25

decrease life span of RBC due to friable nature, which leads to chronic state of anemia
- usually 10-20 days

Front 26

Conditions which increase chances of hypoxia or acidosis in SCD: (4)

Back 26

infection,
1. fever
2. exposure to extreme 3. temperatures
4. dehydration.

Front 27

Hx of SCD:

Back 27

- evolved in W. Africa as a genetic mutation in response to malaria
- Increase SCD where increase Malaria

Front 28

Incidence of SCD

Back 28

- most common inherited disorder in the US
72,000 people
1 in 500 AA
1 in 1000-14000 Hispanic Americans

Front 29

SCD identified

Back 29

- universal newborn screeening which is performed in almost every state.

Front 30

T/F
SCD is an autosomal recessive disease

Back 30

True

Front 31

Acute Complications of SCD are: (6)

Back 31

1. Fever and infection
2. Pain Crisis
3. Acute Chest Syndrome
4. Splenic Sequestration
5. Aplastic Crisis
6. Cerebral Vascular Accident of Stroke

Front 32

What is the leading cause of death in SCD?

Back 32

Streptococcus Pneumococcus Sepsis

functionally asplenic > specific IgG antibodies to the polysaccharide encapsulated oraganisms

Front 33

SCD pains is caused by (3)

Back 33

1. Ischemia > occlusion of blodd vessels by sickled RBCs
2. Damage to the vascular endothelium
3. Inflammation

Front 34

Dactylilitis (hand and foot syndrome) is caused by :

Back 34

vaso-occlusive crisis that occurs in SCD pt babies and toddlers, usually first presenting symptom.

Front 35

Signs of Acute Chest Syndrome (occlusion of vessels to the lungs) are :

Back 35

- rapid deteritionation in respiratory function
- fever
- increase O2 demand
- infiltrate on Chest Xray

Front 36

Nursing interventions for Acute Chest Syndrome are: (4)

Back 36

1. frequent eval of resp status
2. monitoring pulse oximetry
3. encourage incentive spirometry
4. ambulation

Front 37

Acute Chest is treated with: (4)

Back 37

1. abx
2. pain management
3. increased oxygenation
4. transfusions (RBC or exchange)

Rare- mechanical ventilation

Front 38

What possible lab values will you see with Splenic Sequstration? (3)

Back 38

- severe drop in Hbg
- rise in retic
- drop in platelets

Front 39

What is autotransfusion in splenie sequestration?

Back 39

when transfused pRBCs cause the spleen to release the trapped RBCs back into circulation, potentially increasing the blood's viscosity

Front 40

Aplastic Crisis is most common associated with what virus in SCD?

Back 40

parvo B19 infection ( usually fifth disease)

Front 41

What labs are effected in aplastic crisis?

Back 41

decrease hbg
extremely low retic count

Front 42

Chronic Complications with SCD (8) :

Back 42

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

Front 43

Retinopathy and sickle cell is mostly commonly found in pts with :

Back 43

Hbg SC

Front 44

Chronic burden on the heart of anemia often results in (2):

Back 44

- cardiomegaly
- EKG changes

Front 45

Hyposthenuria

Back 45

The inability to conc urine

resulting at time in nocturnal enuresis/ further dehydration

Front 46

Leg ulcers happen in SCD pt as a results of:

Back 46

poor perfusion of the skin

usually lower legs
usually start as small abrasion

Front 47

SCD is diagnosis by and subtype determined by:

Back 47

hemoglobin electrophoresis

Front 48

Preventive interventions for SCD are:

Back 48

- penicillin prophylactic
- immunization with 23 valent pneumococcal vaccine
- education ( esp regarding s/s of sepsis)
- start hydrouxyrea if indicated

Front 49

Hydroxyurea MOA and SE

Back 49

-raise fetal hemoglobin level
-decreases leukocytosis, platelets, and retic counts
- myelosuppressive

Front 50

Average life span of person with SCD:

Back 50

45-65 years

Front 51

Poor prognostic factors associate with SCD: (3)

Back 51

1. dactylitis before 1 y/o
2. consistently elevated WBC
3. Hbg of 7gm/dL or less

Front 52

Acute nursing goals for pt with SCD crisis:

Back 52

- Assess
- PE
- Review Labs
- ABX with fever
-aggressive hydration
- pain management
- pRBC as indictated
- cultures
- chest xray

Front 53

Hb AS

Back 53

Carrier state

Front 54

Hb SS

Back 54

Usually severe SCD

Front 55

HB SS with increased Hbg F

Back 55

moderate to severe

Front 56

Hb Sc

Back 56

moderate to severe

Front 57

Hb Sbeta0 Thalassemia

Back 57

usually severe

Front 58

Hb Sbeta+ Thalassemia

Back 58

usually mild to moderate

Front 59

Hb SO - Arab, SD - Punjab, SE

Back 59

usually mild to moderate but may be severe

Front 60

Def of Thalassemia

Back 60

- 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

Front 61

Four alpha globin genes are located on what chromosome?

Back 61

16

Front 62

Alpha thalassemia :

Back 62

- 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

Front 63

T/F
Iron supplementation will correct the anemia in Alpha Thalassemia

Back 63

False

Front 64

Diagnosis of Beta Thalassemia and clinical presenting symptoms :

Back 64

- usually newborn screening
- significant anemia
- failure to thrive by 6 months of age

Front 65

What bone changes can occur if transfusion needs are not met in patients with Beta Thalassemia?

Back 65

- expanding marrow
- bossing of forehead/skull
- malocclusion of teeth
- expansion of cheeks
- paraspinal deformities

Front 66

Extramedullary erythropoesis is:

Back 66

a process whereby blood is produced outside of the marrow to compensate for severe anemia

Front 67

Clinical finding of extramedullary erythropoesis:

Back 67

enlarged spleen
hypersplenism is hard to correct
begins to destroy RBCs that are being transfused

Front 68

T/F
All forms of iron overload in Beta Thalassemia pt are related to pRBC transfusions

Back 68

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

Front 69

Hemoglobin E is common in :

Back 69

Southeast Asia
1 in 12 people are carriers

Front 70

Diagnostic Tests associated with B- Thalassemia :

Back 70

cbc d/p, hemoglobin electrophoresis , gene mapping , ferritin, endocrine fxn test, EKG, echo, bone density, liver biopsy, HLA, Audio, optho exam

Front 71

Tx of Beta thalassemia;

Back 71

- chronic transfusions (2-4 wks)
- folic acid supplementation
- Chelation therapy (deferoxiamine)
- splenectomy
- hydroxyurea
- BMT

Front 72

Most significant prognostic factor in Beta Thalassemia is :

Back 72

degree of iron overload

70% of all deaths- leading to cardiac issues

Front 73

Nursing assessments and intervention for extramedullary erythropoesis:

Back 73

- review labs
- monitor for facial or skeletal changes
- transfuse pRBC if between 9-10 gm/dl
- splenectomy if needed

Front 74

Nursing assessments and intervention for chelation/iron overload:

Back 74

- labs
- s/s growth delay
- s/s pubertal delay
- assess cardiac
- liver biopsy, SQUID, T2 MRI
- Audiogram
- chelation therapy
- minimize dietary iron

Front 75

Def of Glucose 6 Phosphate Dehydrogenase Deficiency:

Back 75

- inherited, sex linked ( band Xq28)
- metabolic disorber of RBCs
- enzyme defect that causes hemolysis of RBCs

Front 76

Pathophysiology of G6PD

Back 76

- 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

Front 77

What is the most common metabolic disorder of the RBCs?

Back 77

G6PDD

35 million worldwide
- tropical and subtropics of the Eastern Hemisphere

Front 78

G6PDD is expressed in ________ males and __________ females

Back 78

hemizygous males (98 % )
homozygous females

Front 79

What is the most classic manifestation of G6PDD?

Back 79

acute hemolytic anemia

Front 80

T/F

Fava beans can cause acute hemolysis in pt with G6PDD

Back 80

True

and medications: antimalarials, analgesics, aulfonamides, aulfones, anthelminthics, aitrofurans, probenecid, dimercaprol, vitamin K analogues, and rasburicase

Front 81

Lab findings for G6PDD:

Back 81

- moderate to severe anemia
- wide RBC distribution width
- retic elevated
- unconj bili elevated
- WBC increased C increased granulocytes

Front 83

Nursing Assessments for G6PDD:

Back 83

- monitor labs
- monitor for anemia
- Transfuse pRBC prn
- Assess for fluid overload
- hydration (prevent risk of renal complications)

Front 84

Hereditary Spherocytosis is

Back 84

- inherited
- hemolytic anema involving cell membrane alteration that results in fragile RBC trapped in spleen therefore short life span

Front 85

Pathophysiology of hereditary spherocytosis:

Back 85

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

Front 86

Clinical presentation of Hereditary spherocytosis :

Back 86

- Anemia
- Jaundice
- Splenomegaly

@ any age- usually hemolytic anemia and hyperbilirubinemia

Front 87

Dx of Hereditary spherocytosis:

Back 87

- family genetic evaluation
- blood smear with spherocytes
- elevated retic
- indirect hyperbilirubenemia
- + osmotic fragility test

Front 88

TX of hereditary spherocytosis:

Back 88

- epogen
- if severe, blood transfusion (RARE)
- folic acid supplementation ( if severe)
- splenectomy ( sometimes partial)

Front 89

Splenectomy in herediatry spherocytosis is performed so that:

Back 89

RBCs can have a longer life cycle
It is not a cure.

Front 90

Nursing Assessment in Hereditary Spherocytosis: (4)

Back 90

1. assess for anemia
2. check for splenomegaly
3. Labs
4. Monitor pain

Front 91

Bone Marrow Failure happens as a results of (3)

Back 91

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

Front 92

Peak ages for Bone Marrow Failure are:

Back 92

15-25 y/o and then older than 60 y/o

Front 93

What chemical is strongly linked to genetic events that lead fo marrow failure?

Back 93

Benezene

Front 94

Drugs that can contribute to bone marrow failure:

Back 94

- NSAIDs
- neuroleptics
- sulfonamides
- corticosteriods
- psychotropics

Front 95

Tx for bone marrow failure:

Back 95

- BMT
- Or immune suppression with antithymocyte globin and cyclosporine

Front 96

Clinical signs of aplastic anemia: (6)

Back 96

- petechia
- ecchymosis
- anemia
- pallor
- fatigue
- fever

Front 97

T/F
With bone marrow failure, the marrow is often replaced by fatty cells

Back 97

True

Front 98

What is the ANC count of someone with severe aplastic anemia?

Back 98

200 cells/mm 3

Front 99

Congenital aplastic anemia (2)

Back 99

Faconi Anemia
Dyskeratosis Congenita

Front 100

The most common congenital aplastic anemia is :

Back 100

faconi

Front 101

What is the age that Faconi's usually presents clinically?

Back 101

2 - 15 y/o

can present at birth

Front 102

Faconi Anemia is an autosomal _________ disorder

Back 102

recessive

Front 103

There are ___ genes assocaiated with Fanconi anemia. These affect :

Back 103

11

cell apoptosis, interference with tumor necrosis factor, propensity to malignancy

BRCA2 is associated

Front 104

Classic symptoms of Faconi Anemia:

Back 104

- 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

Front 105

Lab findings for Faconi Anemia:

Back 105

- macrocytic anemia
- thrombocytopenia (early sign)
- neutropenia
- hypocelluar bone marrow and fatty

Front 106

T/F
All FA patients will develop MDS or AML

Back 106

False

but the risk is high 50% develop MDS or AML

Front 107

Diagnostic Test for FA

Back 107

- chromosome break analysis using dieposybutan or mitomycin-C

Front 108

FA patients are at high risk for developing malignanies of the (3)

Back 108

- head
- neck
- gynecological

Front 109

Nursing interventions for FA:

Back 109

- 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.

Front 110

Dyskeratosis Congenita is:

Back 110

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

Front 111

Dyskeratosis Congenita genetics:

Back 111

DKC gene - DKC1 at chromosome Xq28

decrease telomerase activity which affect rapidly growing cells (skin, mucosa, bone marrow)

Front 112

T/F
Females tend to have more severe symptoms with DKC due to skewed X chromosome pattern.

Back 112

false

Front 113

DKC hyperpigmentation is most commonly found in:

Back 113

- face
- neck
-shoulders
- trunk

Front 114

Clinica presentation of DKC:

Back 114

- hyperpigmentation of skin
- abnormalities of eyes
- abnormalities of dentition
- osteoporosis
- short stature
-urethral stenosis
- hypospadias
- hypoplastic testes
- leukoplakia
- thrombocytopenia

Front 115

Diamon Blackfan anemia is what kind of inherited form of aplasia?

Back 115

pure red cell

Front 116

T/F
The majority of acquired red cell aplasias occur in childhood and are almost always transient.

Back 116

False

Adult is more common and they are chronic

Front 117

Transient Erythroblastopenia of Childhood is :

Back 117

- an acquired red cell aplasia
- acute
- self limiting

Front 118

Diamond Blackfan anemia is :

Back 118

- rare
- chronic
- pure red cell aplasia
- presents in the first 9 months of life
- early death of pRBCs resulting in severe anemia

Front 119

Incidence of Diamond Blackfan Anemia is:

Back 119

-400 cases per year world wide
- more common in caucasians
- equal between male and female, except recessive is more common in males

Front 120

Diamond Blackfan Anemia: what are the three modes of transmission hypothesized?

Back 120

1. autosomal dominant
2. X linked pattern ( for recessive inheritance)
3. a new sporadic mutation

Front 121

What gene is mutated for Diamond Blackfan Anemia and what chromosome is it located on?

Back 121

- ribosomal protein gene RPS19 defect
- chromosome 19q13.2

Front 122

What are the clinical findings with Diamond Blackfan Anemia?

Back 122

- 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

Front 123

What are the lab findings for a Diamond Blackfan Anemia ?

Back 123

- macrocytic anemia
- decreased or absent reticulocytes
- increased platelets >400k
- increased fetal hbg
- BM bx/a shows erthroid hypoplasia or aplasia

Front 124

Dx of Diamond Blackfan Anemia is by:

Back 124

BM bx/a : anemia, reticulocytopenia and absence of erythriod percursors

Front 125

T/F

Most patients with Diamond Blackfan Anemia need a short course of steriods to correct the issues.

Back 125

False

Chronic steriods; maintained on low dose steriods

Front 126

Pts is Diamond Blackfan Anemia have an increase risk of ?

Back 126

- leukemia
- MDS

Front 127

Transient Erthroblastopenia of childhood is commonly found :

Back 127

- between ages 1-3 (peak @ 23 months)
- with recent hx of viral illness or vaccination

Front 128

Clinical presentation of Transient Erthroblastopenia of childhood is :

Back 128

- anemia
- recent hx of viral illness
- normocytic anemia ( w/o any other cytopenias of familial hx of anemia)

Front 129

Tx of Transient Erthroblastopenia of childhood :

Back 129

- observation
- single transfusion if anemia with flow murmur or tachycardia

Front 130

Prognosis of Transient Erthroblastopenia of childhood :

Back 130

4-6 weeks spontaneously
usually without relapse

Front 131

Schwachman-Diamond Syndrome is:

Back 131

- rare autosomal recessive disease
- characterized by pancreatic insufficiency, failure to thrive, skeletal abnomalities, and BM dysfunction

Front 132

The pathophysiology of Schwachman-Diamond Syndrome:

Back 132

- 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

Front 133

Schwachman-Diamond Syndrome genetic defect is believed to be on chromosome?

Back 133

7

Front 134

Incidence of Schwachman-Diamond Syndrome is:

Back 134

1 in 20,000 births
incr'd in male

Front 135

What is the clinical presentation of Schwachman-Diamond Syndrome?

Back 135

- 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

Front 136

Tx of Schwachman-Diamond Syndrome:

Back 136

-pancreatic enzyme replacement
- fat soluble vitamins
- proph abx

Front 137

Specific Dx Test for Schwachman-Diamond Syndrome:

Back 137

- 72 hour fevel fat test
- sweat test
- pancreatic stimulation testing
- serum immunoreactive trypsinogen
- complete metabolic profile

Front 138

Neutropenia is:

Back 138

- ANC < 1500 cells/mm3

mild (ANC 1000 - 1500)
moderate (500-1000)
severe (<500)

Front 139

Neutrophils last ________ with a half life of _________.

Back 139

1-2 days
8 hours

Front 140

Chronic neutropenia predisposes children to what other complications (3) ?

Back 140

- oral (gingivitis and periodontal disease)
- Orthopedic ( bone demineralization)
- and a subgroup have incr'd risk for MDS and AML