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Neontal boards J > hematology > Flashcards

hematology Flashcards

1
Q

What is the incidence of thrombocytopenia

A

1-5%
increased risk with dec gestational age

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2
Q

Etiology of thrombocytopenia

A
  1. decreased production
  2. Increased destruction or consumption
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3
Q

Etiology of Neonatal Alloimmune thrombocytopenia

A
  • HPA incompatibility
  • Maternally derived antibodies against fetal and neonatal platelet
  • No impact on maternal platelet
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4
Q

Etiology of Neonatal Autoimmune thrombocytopenia

A
  • Maternal autoimmune d/o- - associated with SLE and ITP
  • Attack maternal and fetal platelet
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5
Q

Clinical presentation of NAIT

A
  1. mod to severe neonatal thrombocytopenia
  2. ICH- pre or postnatal
  3. prior child or close maternal relative with suspected NAIT (can happen even on 1st pregnancy)

Can be asymptomatic but high risk for symptomatic severe throbocytopenia

No hemolysis

Placental villa provide the exposure for immune response

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6
Q

Clinical presentation of Neonatal autoimmune thrombocytopenia

A
  1. neonatal thrombocytopenia in the setting of maternal thrombocytopenia or autoimmune d/o
  2. normal or mildly low plt count
  • Generally asymptomatic
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7
Q

how to diagnose NAIT

A

Parental and fetal HPA genotyping

Most common HPA-Ia, HPA-5b

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8
Q

Diagnosis of neonatal autoimmune thrombocytopenia

A

diagnosis of exclusion in setting of maternal and neonatal thrombocytopenia

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9
Q

Prenatal mgt of NAIT

A
  1. if known prenatally: IVIG and steroids
  2. CS if fetal plt if <100K

-Goal: prevent ICH

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10
Q

Prenatal mgt of neonatal autoimmune thrombocytopenia

A

Follow maternal treatment for autoimmune disease

  1. Prednisone (does not cross the placenta)
  2. IVIG

-Does not improve neonatal outcome

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11
Q

Postnatal mgt of NAIT

A
  1. stat HUS
  2. Platelet transfusion
  3. IVIG with platelet transfusion
  4. testing maternal aunt
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12
Q

Postnatal mgt of neonatal autoimmune thrombocytopenia

A
  1. IVIG
  2. platelet transfusion for symptomatic bleeding- not as efficient as in NAIT
  3. Methylprednisone 1mg/kg BID x 5days if does not recover
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13
Q

when is rhogam given

A

Single dose at 300 μg of anti-RhD IgG

  1. at 28 weeks’ gestation
  2. within 72 hours of delivery, if the newborn is RhD positive
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14
Q

A well appearing neonate with mild to moderate thrombocytopenia and leukopenia

What is the most probable etiology of the thrombocytopenia

A

placental insufficiency

  • maternal pregnancy-induced hypertension
  • intrauterine growth restriction.
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15
Q

The most common cause of severe thrombocytopenia in a healthy-appearing neonate

A

neonatal alloimmune thrombocytopenia.

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16
Q

What factor deficiency to consider if platelet, PT, PTT and INR are normal in a baby with history of bleeding

A

Factor XIII

-Autosomal recessive

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17
Q

How are the result of hemoglobin elctrophoresis reported

A

in order of decreasing amounts of the expressed hemoglobin molecules.

Example:

In patients with sickle cell trait, there is more hemoglobin A than hemoglobin S, as A is more stable than S, and so the correct result for patients with a sickle trait is FAS.

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18
Q

What is a sensitive marker for Vit K defieciency

A

Des-gamma-carboxyprothrombin (DCP) is a protein induced by vitamin K absence.

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19
Q

formula for exchange volume

A

[(obs hct-desired hct)/obs hct] × bby bld vol (BW*80 ml/kg)

desired hct 55-60%

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20
Q

Chromosome for coding

  1. alpha globulin
  2. beta globulin
A
  1. Chromosome 16
  2. Chromosome 11
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21
Q

Forms of hemoglobin based on gestational age

A
  • first 8 weeks: Gower 1, Portland, Gower 2
  • 8 weeks: Hgb F (alpha and gamma)
  • increasing after birth: Hgb A (alpha and beta)
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22
Q

Different alpha thalasemia

A
  1. Carrier: 1 defect- no clinical sx
  2. Trait: 2 defect- low MCV Low MCHC, anemia
  3. Hgb H: 3 defects- 4 beta
  4. HbH-CS- Constant spring
  5. HgBart- 4 gama- hydrops- No Alpha
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23
Q

2 mutation of alpha thalassemia trait

A
  1. Cis (sis)- same chromosome- higher chance with offspring severe
    * common in Asians (SE Asia)
  2. trans- opposite side
    * common in African American
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24
Q

What is alpha thal constant spring (CS)

A

Glutamine in place of stop code

Unstable

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25
Q

Chances of off spring with HgBart hydrops with Cis mutation

A

25%

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26
Q

What has caused an increase in alpha-thal in some areas of the world

A

Migration

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27
Q

Pathogenesis of Hg Bart and Hg H

A

Inc oxygen affinity (poor O2 delivery to tissues) → Hypoxia

  • HSM
  • dysmorphism bone: ineffective erythropoesis
  • Damage to RBC membrane→ Hyperbili, cholestasis, cholelithiasis, splenomegaly
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28
Q

Which alpha thalassemia is clinically similar to beta thalassemia major

A

Hemoglobin H-Constant spring

  • requires freq BT
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29
Q

Supplements for alpha thalasemia

A
  • Folic Acid
  • Avoid iron (inc Fe GI absorption resulting to iron overload)
  • not transfusion dependent
30
Q

Cause of death for Bart hydrops

A

High cardiac output failure

  • from chronic hypoxia and anemia
31
Q

Ethnicity associated with beta thal

A

Asian, Indian, Middle east, SE asia

32
Q

True or false

Beta-thal trait is transfusion dependent

A

False

  • inc HgB A2 (alph2, delta 2)
33
Q

Lab findings for beta thalasemia

A

mild microcytic hypochromic anemia, target cells on peripheral smear

34
Q

What is Cooley anemia

A
  • beta 0 no beta
  • severe hemolytic anemia
35
Q

What is Beta thal +

A
  • some production of B chain
  • transfusion INDEPENDENT except infection and puberty
36
Q

True or false

Beta Thal requires iron supplementation

A

False: risk for iron overload

37
Q

What is most common hemoglobinopathy in the US

A

Hemoglobin S

  • Africans, Americas, Middle east, Mediterranean, India
38
Q

Defect in Hgb S

A

point mutation of B-globin gene at codon 6 (glutamic acid replaced by valine)

39
Q

First manifestation of sickle cell disease

A

Dactylitis

  • not until months after birth d/t persistence of HgbF
40
Q

Most severe type of sickle cell disease

A
  1. sickle cell anemia (SS)
  2. sickle cell B0 thal
  • start prophylactic antibiotics ASAP
41
Q

What Hgb commonly west Africa

A

Homozygous Hgb C

Hgb SC- aseptic necrosis of femoral head and proliferative retinopathy

42
Q

Most prevalent HgB in Asia

A

Hgb E- B-globulin mutation

Homozygote risk for aplastic crisis

Hgb E- B thal: transfusion dependent

43
Q

When does bone marrow hematopoiesis begin

A

22 weeks gestation

44
Q

Reason neonates at risk for neutropenia

A
  1. quickly deplete reserves with time to accelerate progenitor granulocyte maturation
  2. smaller neutrophil reserve
  3. GCSF, GMCSF already at max rate (no further boost)
45
Q

When does neutrophil function improve in neonates

A

2-3 weeks

46
Q

what are the risk factors for neutrophilia (6)

A
  1. sepsis
  2. maternal HTN
  3. IUGR
  4. SGA
  5. asphyxia
  6. periventricular hgh
47
Q

What is idiopathic neutropenia of prematurity

A

Late onset neutropenia associate with anemia and reticulocytosis

  • occurs bet 6-8 weeks
48
Q

Duration of neutrophilia from SGA/IUGR/ maternal HTN

A

2-3 days

49
Q

What is Wiskott-Aldrich syndrome

A
  • x linked neutropenia and thrombocytopenia
  • risk for myelodysplastic syndrome
50
Q

What condition has:

  1. neutropenia
  2. exocrine pancreatic insufficiency,
  3. skeletal AbN
  4. failure to thrive
A

Schwamman-Diamond syndrome

51
Q

What are the vit K dependent factors

A

10, 9,7,2, protein C and S

52
Q

Newborn presents with

  • multiple hemangioma or inc hemangioma
  • dec plt
  • high cardiac output failure
  • DIC
A

Kasabach-Merritt (KM) syndrome

53
Q

What deficiency that presents with bleeding:

N platelet

N PTT

prolonged PT

A

Vitamin K

54
Q

What deficiency presents with bleeding:

N platelet

Inc PT and PTT

A

Factor 2, 5, 10

55
Q

What deficiency presents with bleeding:

N plt, N PT

Inc PTT

A

Von Willi, 8, 9

56
Q

What deficiency presents with bleeding:

N plt, PT, PTT

A

Factor 13

57
Q

What law explains symptoms of hyperviscosity syndrome

A

Poiseuille’s law

R= viscosity * length/ R4

Affects microcirculation

58
Q

What condition presents with hemolytic anemia secondary to:

Chromosomal fragility/ breakage

A

Aplastic/ Fanconi’s anemia

59
Q

What condition presents with anemia with finding of dec NADPH

A

G6PD

Mediterranean, African, and Asian descent,

60
Q

What presents with hemolytic anemia:

Bld smear: N chromic, N cytic, reticulocytosis

Hyperbili

N LFT

A

Disordered activity of pyruvate kinase

Ethnicity: Amish greatest risk

61
Q

3 day old newborn delivered at home present with bleeding from circumcision and umbilical stump.

Whats the probable cause

A

Vitamin K def (Classic)

  • presents 2-7 days
  • bleed from umb stump, GI, ICH, prolonged bleeding from circ
  • from dec store, intake (ie exclusive BF)
62
Q

Newborn delivered less than 24 hours presents with bleeding. Mom has seizure disorder on phenytoin. What is a possible treatment?

A

Vitamin K

Early vit K def:

  • present within 24 hours
  • assoc with anti seizure meds, cephalosporin and warfarin
63
Q

3 month old boy with biliary atresia presents with bleeding. Work showed N CBC and PTT, LFT was elevated, prolonged PT.

What is a possible treatment

A

Vitamin K

Late onset Vit K def

  • 2weeks-6 mos
  • Boys, in summer (?)
  • associated with poor PO intake and/or liver disease
64
Q

Newborn with trisomy 21 presents with hepatosplenomegaly, effusions, and bleeding or petechiae. Blood smear showed leukocytosis with neutrophilia, thrombocytopenia, and blasts. What is this?

A

Transient myeloproliferative disease, or transient leukemia,

  • occurs 5% to 10% of infants with T21
  • self-limited
  • resolving spontaneously by two months of age.
  • chemotherapy for: hyperviscosity or life-threatening bleeding from liver involvement.
  • does not prevent the occurrence of subsequent leukemia.
  • About 20% will develop myeloid leukemia
65
Q

Dose of phototherapy depend on (4)

A
  1. the light spectrum (wavelength of 425 to 475 nanometers is most effective),
  2. irradiance of light (i.e., power of electromagnetic radiation per unit area)
  3. distance of the infant from the light source,
  4. surface of skin exposed.
66
Q

Facts on Sacrococcygeal teratomas (SCT)

A
  • benign germ cell tumors
  • classified as mature (most neonatal and infant teratomas) or immature (high malignant potential).
  • more common in girls
  • occur sporadically.
  • may cause high output heart failure and non-immune hydrops.
  • involve the dura of the spinal cord: neurologic symptoms.
  • Treatment: complete surgical excision, including the coccyx, to prevent recurrence.
  • recurrence 10%,
  • risk of malignant transformation 15-20%.
  • followed with regular imaging and measurement of the AFP (elevated levels associated with endodermal sinus tumor) and BHCG (elevated levels associated with choriocarcinoma)
67
Q

Pathophysiology of Anemia associated with anti-K1

A
  • suppression of hematopoiesis and destruction of distorted red blood cell (RBC) progenitor cells by the reticuloendothelial system.
68
Q

How is ABO blood type inherited

A

A and B alleles are codominant

O allele is recessive.

Each child receives 1 allele from each parent

  • 6 possible genotypes
  • 4 possible phenotypes.
69
Q

Definition of anemia in neonates

A

Anemia: Hgb/Hct: 5th percentile lower reference interval
Moderately severe anemia: H/H between the 1st and 5th percentile
Severe anemia: H/H below the 1st percentile.

For memory estimate cut off at birth (p5)
<27 wks: 10/30
27-32 wks: 12/33
33-38 wks: 13/35
39-42 wks: 14/40

70
Q

Which of the laboratory test is typically the first to become abnormal in iron deficiency?

A

Reticulocyte hemoglobin content

reticulocyte life span: around 24 hours

71
Q

laboratory evaluation should raise the suspicion of hereditary spherocytosis?

A

A high MCHC/MCV ratio

72
Q

Its purpose is to decrease transmision of CMV via blood transfusion

A
  1. CMV negative blood donor
  2. leukocyte reduced
  3. filtering blood to remove WBC

Neontal boards J (19 decks)

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