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Skin, Lymph, Blood > Dr. Newmann - Red/White Cell Disorders Pediatrics > Flashcards

Dr. Newmann - Red/White Cell Disorders Pediatrics Flashcards

1
Q

When does the infant have the lowest amount of hemoglobin

A

2mos

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2
Q

2,3-DPG concentration does what to O2 distribution curve

A

Shifted to the right (lower affinity of O2, since tissues signal they need O2)

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3
Q

Anemia does what to the heart

A

Increase CO

Murmur can be heard due to low viscosity

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4
Q

history that can point to anemia

A

Infection , travel, exposures, chronic illness, anemia in family, splenomegally, jaundice, early onset gallstones

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5
Q

Anemia SX that are common to see on someone

A

Pale, irritability, sleepiness, low exercise tolerance, bloody stools, LAD, fever, viral Sx, heavy menses, SOB, cough

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6
Q

Diamond-Blackfan Syndrome

A

Congenital only RBC aplasia

  • presents in infancy (erythroid precursor apoptosis)
  • low reticular count
  • macrocytic anemia
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7
Q

Fanconi Anemia

A

Inherited aplastic anemia (most common)**

  • macrocytic anemia
  • LOW reticular count
  • leukopenia + thrombocytopenia (low RBC, plt, WBC)= apoptosis in BM
  • usually young up to 10yo when DX**
  • can become panocytopenia
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8
Q

IDA in pediatric patients

A
  • usually from not eating enough Fe
  • microcytic, hypochromic high RDW
  • HIGH transferrin, LOW ferritin and FE sat
  • SX : pale, and big cow milk drinker (you get Fe from mothers breast milk)**
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9
Q

Mentzer Index

A

When pt had mild microcytic anemia : MCV/RBC

  1. <13 = IDA
  2. > 13 = B-thalassemia trait
  3. = 13 = indeterminate
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10
Q

How to calculate ANC (Absolute Neutrophil Count)

A

ANC = (%N +%Bands) (WBC) / 100
Mild : 1000-1500
Moderate : 500-1000
Severe neutropenia : 500

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11
Q

What drug causes Neutropenia

A

Chemotherapy

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12
Q

Kostmann Syndrome

A

(Severe Congenital Neutropenia) AR

  • Life threatening pyogenic infections early in life
  • Impaired myeloid differentiation caused by maturation arrest of N precursors
  • ANC <200
  • high risk of AML
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13
Q

Cyclic Neutropenia

A

Cyclic Fever, oral ulcers, gingivitis, periodontal disease, bacterial infections **
- stem cell regulatory defect resulting in defective maturation **
- ANC <200 for 3-7 days every 15-35 days **
(NO RISK for any AML)**

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14
Q

Schwachman- Diamond Syndrome

A

TRIAD**
1. Neutropenia
2. Exocrine pancreas insufficiency
3. skeletal abnormalities
= X N mobility, migration, chemotaxis, count
- (higher risk to get MDS or other leukemia)

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15
Q

Fanconi Anemia SX and when do you usually see it, and in risk of what

A

BM cant make any cells lines normally (aplastic anemia) = can become panocytopenia

  1. GU + Skeletal anomalies, increased chr fragility
  2. First 10 years of life
  3. AML, Brain tumors, Wilms tumor
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16
Q

Leukocyte adhesion deficiency
SX
Why
Risks

A

Rare

    • Delayed separation of umbilical cord stump (3weeks)**
    • bacteria and fungal infections with NO PUS**
    • poor wound healing
  2. N can adhere to cross BV
  3. No risks
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17
Q

Hyper-immunoglobulin E syndrome (Job syndrome)
What
Why
Risk

A
  • TRIAD **
    1. Severe eczema
    2. Recurrent staph skin infection (or other bacteria)
    3. Recurrent pulmonary infections (bacteria/fungal)
  • no N chemotaxis and high IgE
  • Hodgkin lymphoma risk
18
Q

Chediak-Higashi Syndrome
SX
Why
Risks

A
  1. Oculocutaneous albinism, neuropathies, recurrent pyogenic infections
  2. No N chemotaxis/degranulation, X granulopoisis (die dry age 20yo)LYSOSOME BREAKDOWN PROBLEM
  3. No risks
19
Q

Chronic granulomatous disease
SX
Why
Risks

A
  1. Recurrent purulent infections (fungal/bacterial), CATALASE +
    (Usually infancy, chronic inflammatory granulomas)
  2. X oxidative metabolism, X superoxide in N
    (X-linked)
  3. No risks
20
Q

Parovirus B19

A

5th disease of childhood
- bright red cheeks, diffuse lacy red rash)
Anemia/ ( can cause thrombocytopenia +neutropenia), I think aplastic anemia

21
Q

Digeorges Syndrome

What is it and causes what

A

X thymus

Causes T-cell immunodeficiency

22
Q

Leading cause of death in infancy in US

Leading cause of death in due to disease

A
  1. Unintentional injuries (accidents)

2. Brain Cancer and Leukemia

23
Q

Most common malignancy in 15-19yo and risk factors to this

A
  1. Hodgkin Lymphoma

2. HHV-6, cytomegalovirus, EBV

24
Q

B signs for Hodgkin’s lymphoma

A
  1. Unexplained fever (102.2F, 39C)
  2. Wt loss > 10% over 6 months
  3. Drenching night sweats
25
Q

When should I do a chest X-ray in a pt with lymphadenopathy

A
  1. Any pt with unexplained LA, with no association to inflammation or infection process (rule out mediastinal mass*)
  2. Any pt with persistent LAD and with cough, SOB, CP
  3. Pt with lymphoma
26
Q

Most common type of lymphoma in children and adolescents

A

Non-Hodgkin lymphoma

27
Q

Wiscott-Aldrich syndrome

A
  • X-linked**
  • TRIAD**
    1. Ear infections/ sino-pulmonary infections
    2. Severe atopic dermatitis
    3. Thrombocytopenia (bleeding)
  • RISK TO GET NON-HODGKIN LYMPHOMA
28
Q

3 types of Burkitts lymphoma

A
  1. Sporadic : abd disease, EBV, Africa
  2. Endemic Type : head,neck, Africa, EBV/ Malaria
  3. Immunodeficiency- restated: immunosuppressive drug use
29
Q

Number 1 malignancy in children is what

A

Leukemia (ALL is 97%)

30
Q

Which genetic condition can one be born with that is associated with AML and ALL

A

Trisomy 21

31
Q

Plt counts normal
Severe
Life threatening

A

150,000-450,000
20,000
10,000

32
Q

Decreased Plt production caused by what 3 causes

A
  1. VIT B12, folate, FE deficiency
  2. BM problem : AA, leukemia, lymphoma
  3. Genetic problem : Wisconsin-Aldrich, Fanconi, schwachman-diamond, thrombocytopenia-absent-radius syndrome (TAR)
33
Q

Causes for increased plt destruction and consumption

A
  1. ITP
  2. HUS/DIC
  3. TTP
  4. Kasabach-Merritt phenomenon
34
Q

Reasons for plt sequestration

A

Hypersplenism

Von-Willebrand disease

35
Q

ITP

A

Immune thrombocytopenia purpura
ANTI-plt antibodies
(50% filled by viral infection lasting 1-3 weeks)
<20,000plt
- normal PT and PTT
- normal other cell lines (otherwise check BM)
SX : usually normal, bleeding

36
Q

Kasabach- Merritt phenomenal

A

Thrombocytopenia + hypo-fibrinogenemia + GIANT HEMANGIOMAS + intravascular coagulation

37
Q

3 red flag sx in thrombocytopenia pt

A
  1. Panocytopenia sign
  2. Raised LAD (cancer can be it)
  3. Renal impairment
38
Q

Acute vs chronic ITP

ACUTE

A 
ACUTE ITP : 
2-6yo
1-3 week infection before
<20,000 plt
High lymphocytes 
80% spontaneous remission (2-6 weeks)
39
Q

Acute vs chronic ITP

Chronic

A 
20-40yo
Female 
No infection before
30,000 - 80,000plt
Low lymphocytes 
Mo-years (no spontaneous remission usually)
40
Q

HUS

A

Hemolytic uremic syndrome

  • after acute gastroenteritis
  • TRIAD
    1. Microangiopathic hemolytic anemia
    2. Thrombocytopenia
    3. Acute renal failure
  • blood in urine, if proteinuria then worse prognosis
41
Q

Which organism is usually the cause of gastroenteritis cussing HUS

A

E. coli 0157:H7

Skin, Lymph, Blood (9 decks)

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