Hematology Flashcards
(119 cards)
1
Q
G6PD medications to avoid
A
Medications: sulfonamides, cipro, moxifloxacin, norfloxacin, ofloxacin, dapsone, septra, nalidixic acid, chloramphenicol, nitrofurantoin, primaquine, pamaquine, chloroquine, quinacrine, B-naphthol, niridazole, acetanilide, vitamin K analogs, methylene blue, toluide blue, probenecid, dimercaprol, ASA, rasburicase, phenazopyridine, benzene, naphthalene, diabetic acidosis, hepatitis, sepsis
2
Q
Inheritance G6PD
A
x-linked
3
Q
Pathophys G6PD
A
G6PD catalyzes conversion of glucose 6 phosphate to 6-phosphogluconic acid which produces NADPH which maintains GSH which provides protection against oxidant threat from certain drug and infections that would otherwise cause precipitation of hemoglobin (Heinz bodies) or damage RBC membrane
4
Q
ethnic groups for G6PD
A
- 13% of males of African descent have G6PD
Italians, Greeks, Mediterranean, middle eastern, African and Asian ethnic groups also have a high incidence ranging from 5-40%
5
Q
heniz bodies
A
G6PD
6
Q
Ethnicity fo hereditary aphserocytosis
A
Northern European
7
Q
Proteins involved in hereditary spherocytosis
A
Abnormalities of ankyrin or spectrin are the most common molecular defects
8
Q
Inheritance spherocytosis
A
AD
9
Q
Labs in HS
A
MCV is normal or een slightly increased, increased MCHC, low RDW
10
Q
Mgmt Hereditary spherocytosis
A
- Should have vaccinations, spleen size monitored, screening for gallbladder disease at age 4 and repeated every 3-5 years
- Folic acid supp if moderate or severe HS
Splenectomy is curative
- Folic acid supp if moderate or severe HS
11
Q
Pyruvate kinase deficiency inhertiance
A
AR
12
Q
Hemophilia inhetiance
A
x-linked
13
Q
Is PT abnormal inhemophilia
A
No
14
Q
Earliest joint involved in hemophilia
A
ankle
15
Q
What lab value is abnormla in hemophilia
A
PTT, usually 2-3 times uLN
16
Q
Severe hemophilia how much factor
A
<1%
17
Q
Fanconi anemia ethnicity
A
Higher in Ashkenazi jews and Afrikaners
18
Q
Rare, multisystem hereditary disorder results in the development of bone marrow failure in those affected and predisposition to malignancy including myelodysplasia and AML, epithelial cancers
A
Fanconi anemia
19
Q
Triad for fanconi anemia
A
TRIAD of bone marrow failure, congenital anomalies and elevated chromosome fragility
20
Q
Congen abnormalities in fanconia enmia
A
Common congenital anomalies are absent radii, hypoplastic thumbs, supernumerary, bifid or absent, anomalies of the feet, congenital hip dislocation and leg abnormalities can also be seen
21
Q
Cancers in Fanconi
A
- squamous cell carcinomas of head and neck, esophagus, vulva, anus or cervix
22
Q
Screening fanconi
A
- Should get ultrasound of abdomen and echo to rule out other abnormalities when diagnosed
- CBC every 3mo and bone marrow every year
- Annual TSH for hypothyroidism, glucose checks
Solid tumors annually with physical exam and inspection of skin, oral cavity and other organs
23
Q
Bloom Syndrome ethnicity
A
ashkenzi jews
24
Q
bloom inheritance
A
AR
25
bloom clinical fts
- Sensitivity to UV radiation leading to chromosomal breaks
- May have café au lait spots
- IGUR, short stature
- Intellect is normal to average
- Immunodeficiency
- GI malabsorption, GERD, hypogonadism is common
- Increased risk for solid tumors (esp of skin) and lymph malignancies
26
Schwachmann diamond inhertiance
AR
27
Schwachmann diamond CBC findings
Most common to have neutropenia, then anemia, only 21% have pancytopenia, 98% have exocrine pancreatic insufficiency
28
Schwachmann diamond clinicla ft
Exocrine pancreatic insufficiency and metaphyseal dysplasia
- Shor stature is common, usually normal growth velocity though
- Metaphyseal dysplasia, osteopenia, delayed appearance of secondary ossification centers, short or flared ribs and thoracic dystrophy
Some patients have hepatomegaly and elevations in liver enzymes
- Most patients have dental abnormalities
Many have neurocognitive problems and poor social skills
29
Risk in schwacmann diamond
25% develop leukemia by age 18
30
is neurodevelopment impairment reversible with iron supp
No
31
Most frequent event preceding ACS
VOC needing opiods for pain
32
Platelet lifespan
7-10 days
33
Neutrophil lifespan
< 24h
34
Causes of normocytic anemia
TEC, anemia of choronic disease, medications, infections, renal failure, bleeding, hemolysis, de
35
Macrocytic anemia causes
Vitamin B12, folate, liver disease, myelodysplastic syndrome, bone marrwo dailure syndromes, down syndrome
36
Signs/ Symptoms of IDA
pica, koilpnychia, breath holding spells
37
Red cell indices in IDA
RDW elevated, RBC is low or noaml, MCV very low
38
Peripheral smear in IDA
hypochromic microcytic pencil cells
39
Iron studies in IDA
elevated TIBC, decreased ferritin, decreased transferrin saturation
40
When to repeat bw after starting treatment for IDA
check CBC, retic, iron at 1month and 3 months
41
how much and how long to supplement LBW infants 2-2.5kg iron
1-2mg/kg/day for 0-6 months
42
How much and how long to supplement LBW infants < 2 kg
2-3mg/kg/day for 0-12mo
43
Is iron supp required for LBW infants fed preterm formulas
no, these formulas are higher in iron
44
Pathophys of anemia of chronic disease
involves hepcidin elevated levels that decrease GI absorption of iron and limit the expor of iron from amcrophages into red blood cells
45
TIBC in anemia of chronic disease
low/normal
46
HbA is made of
2 alpha, 2 beta chains
47
emoblogin A2 is made of
2 alpha, 2 delta chains
48
emoglobin F is made of
2 alpha, 2 gamma chains
49
Beta thal trait labs
Hb is typically normal or low normal, RBC elevated, RDW is low
50
Hb electropheresis of Heta thal trait
Hb A2 will be eelbated > 3.5%
51
Mentzer index formula
MCV/RBC
52
Mentzer > 13
IDA
53
Mentzer 13>
thallassemia
54
When does beta thal present
after 6 months (no longer making HbF)
55
alpha thal trait
microcytosis, asymptomatic, normal hemoglobin, can be cis/trans for genetics implications
56
HbH disease
decreased hemoglobin, typically not transfusion dependent, dont typically require chelation, present at birth, three alpha gene deletion
57
Hb Bart
Four alpha gene deletion, cannot make hemoglobin A, A2 or F, severe fetal anemia
58
How to diagnose alpha thal trait
diagnosed by genetic testing
59
Should you have sickle cells on smear in sickle cell trait
no
60
Complications of SCD
clinical stroke, silenet stroke, neurocog changes, proliferative retinopathy, asthma, OSA, acute chest, pulm hypertension, cncentrating defects, papillary necrosis, enuresis, liver of splenic sequestration, gallstones, priapism, VOC, aplasticc crisis, functionally asplenic
61
ACS diagnosis
fever or resp symptoms and new infiltrated on CXR
62
What does ACS put patients at riskf ro
stroke
63
What is clinical stroke in SCD an indication for
acute exchange tranfusion
64
Sickle cell surveillance
follow-up q6mo, monthyl CBC on hyroxyurea, pen prophylaxis age 2mo until 5 years, folic acid, all extra vaccines, BP at each visit, TCS- annually startni at age 2, at age 10 retinopathy screening, annual u/a and creatinine
65
How often to do PFT and echo in SCD
Newest guidelines only if symptomatic
66
Reasons for splenectomy in children
sickle cell disease after one episode of sevre splenic sequestration, certain inhertied hemolytic anemias with severe symptoms > 5 years old, transufion dependent thallassemia, chronic ITP refractory to all treatment
67
Complications post splenectomy
infection with encapsulated orgnaisms and certain parasites, thrombosis
68
Diamond Blackfan inheritance
AD
69
When does DBA present
First year of life < 6 mo usually
70
What type of anemia in DBA
macrocytic
71
Kids with DBA are at increased risk of
MDS/AML
72
ITP treatment with doses
prednisone 4mg/kg/day for four days or pred 2mg/kg/day for 1-2 weeks with tapering dose
IVIG 1g/kg
73
How many with ITP that repond to treatment will relapse
1/3
74
When is ITP considered chronic
> 12 mo
75
%neonates with NAIT with ICH
10-25%
76
When to do cranial ultrasound in NAIT
plt , 50000
77
Mgmt NAIT
HPA1a-ve platelets ASPA, rnadom donor platelets if infant is bleeding and no HPA1a-ve in region
78
iwskott Aldrich syndrome triad
small platelets, thrombocytopneia, eczema, immunodeficiency
79
Fanconi anemia buzzwords
anmeia,pancytopenia, absent or abnormal thumbs, short and small features, cafe au lait macules, VACTERL anomalies
80
Dyskeratosis congenita buzz words
dystrophic nails, lacy reticular rash, white tongue coating (leukoplakia), pancytopenia
81
Schwachman diamond syndrome buzz words
neutropenia, expocrine pacratic insuffiicnecy, bony abnomrlaities
82
CAMT
thrmbocytopenia, bo birth defects
83
Kassabach Meritt labs
thrombocytopenia, low fibronogen, elevated didimer, PTT may be minimally prolonged, usually not a MAHA
84
isoalted INR prolongation
factor 7
85
isolated pTT prolongation
factor 8, 9, 11, 12, lupus anticoagulant
86
PTT and INR normal, bleeding
factor 13 deficiency or not the clotting casacade (vWF, plt nnumber or function problem)
87
test for lupus anticoagulant
dilute russel viper venom time
88
vitamin K dependent factors
89
Vitamin K deficiency will cause?
elevated INR and PTT
90
What becomes abnomrla first in vit k deficiency
INR because of short half life of F7
91
vwf is a carrier for
f8
92
Normal vwf antigen/activity level
> .5
93
Type 1 vWF disease
AD, most commonl, botn antigen/activity will be proprtionally low, ratio >.7
94
Type 2 vWF disease
AD, ratio < .7
95
type 3 vwf disease
levels undetectable, AR, factor 8 severely low range < 1%
96
Treatment vwf type 3
vwf/VIII concentrate
97
labs in hemophilia
INR normal, PTT prolonged often > 60, fibrinogen normaml, vWF normal, factor 8 levels low < 40%
98
mild/mod/severe hemophilia factor levels
5-40, 1-5, < 1%
99
How does Factor 13 defieicny present
umbilical stump bleeding or ICH
100
Genetic disorders that increase risk for thrombosis
factor V leiden, prothrombin, antithrombin deficiency, protein C and S deficiency
Homocystinura, proteus
101
How does heparin work
binding to antithrombin 3 to inhibit factor 10
102
infections from transfusions
Hep B>> Hep C>> HIV
103
Management for VOE in SCD
analgesi within 30-60 minutes
avoid cold packs
regular tylenol and advil
acoid overhydration
incentive spirometry
104
ACS management
maintain O2 sat gretaer than 95
acoid over hydration
incentive spirometry
broad spectrum anitbiotics
cross match for possible transfusion
105
When are SCD patients highest risk for splenic sequenstration
before age 5
106
what are indications for simple RBC transfusion in SCD
splenic sequestration
aplasitc crisis
107
indication for exchange transfusion in SCD
stroke
108
age for TCD screening SCD patients
2-16 to identify high risk pateints
109
Most common human genetic disease
SCD
110
what do SCD kids need more vaccines against
pneumococcal, meningococcal and addition dose of Hib, Hep A nand B and annual flu vaccine
111
when to give prophylactic antibitooics SCD patients
age 2mo-5y, can be extended if splenectomy or hsitory of invasive bacterial infections
112
alternative of SCD prophylaxis if pen allergy
cotrimoxazole or erythromycin
113
when should hydroxyurea be offerred
all SCD kids over 9mo
114
oxygen sat target SCD patients
95 greater
115
ACS definition
new pulmonary infiltrate in the presence of fever and respiratory signs or symptoms
116
who is more likely to have splenic sequestration
HbSC and HbSB
117
most common cause pediatric stroke
SCD
118
when should use screen TCD in SCD patients
2-16, no evidence after age 16
119
Low risk criteria for SCD patient with fever
These patients can be managed as outpaitnet
fever < 40
> 6 months
WBC 5-20, plt > 100, HB not mroe than 20 below baseline
no resp distress or CSR abnormalitiy
no clinical findings of meningitis
no higostry of sepsis or meningitits
no signfiicant pain or dehydration
initial visit
ability to follow up closely
