Newman - Non-neoplastic WBC Disorders in Pediatrics Flashcards

1
Q

WBC numbers

A

Most = Never > Let > Monkeys (monos) > Eat > Bananas = Least

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Three criteria for “Worrisome” recurrent infections (start thinking about immunodeficiencies).

A
  1. Two or more severe infections in one year
  2. Three or more respiratory infections in one year (that not URI’s, like bad pneumonias)
  3. The need for antibiotics for 60 days per year.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Three clinical features suggestive of a primary immunodeficiency

A
  1. FH of immunodeficiency or unexplained death *
  2. Failure to gain wt or grow normally
  3. IV antibiotics and/or hospitalization to clear infections
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Condition with complete T cell deficiency

A

SCID - severe combined immunodeficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Three conditions with partial T cell deficiency

A
  1. DiGeorge
  2. **Ataxia telangiectasia (combined B and T cell)
  3. Wiskott-Aldrich syndrome (combined B and T cell)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Main pathogens in T cell deficiency infections

A
  1. Intracellular organisms (HSV, mycobacterium, listeria)

2. Fungi

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Primary immunodeficiencies are… congenital or acquired?

A

congenital

Secondary - meds

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Five pertinent things about BIRTH HISTORY in someone with immunodeficiency

A
  1. Maternal illness (HIV, CMV, drugs, alcohol)
  2. Neonatal history
  3. Feeding history
  4. Duration of breast feeding
  5. WHEN THE CORD FELL OFF (leukocyte adhesion defect)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

***Chediak Higashi syndrome

A

***Immunodeficiency + shows progressive NEUROLOGIC dysfunction in young adults + albinism

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Amish people associated with ___. This is AR or AD?

A

Ataxia telangiectasia, AR

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What is Leukocyte Adhesion Defect? High count in what leukocyte?

A

Rare autosomal recessive disorder of leukocyte function (1/10,000,000).
Cord doesn’t fall off. Recurrent bacterial and fungal infections without pus accumulation. Depressed inflammatory response despite striking blood neutrophils.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

***Between 2yo-6yo with recurrent URI, think what main deficiency?

A

***IgA deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

URI that cause/signs of immunodeficiencies

A

nystatin-resistant or recurrent candidiasis (after 3 months of age), gingivitis, stomatitis, oral ulcerations occur in T cell and phagocytic cell disorders

fungal infection=T ells, HIV

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

RECURRENT BACTERIAL MENINGITIS and SEPSIS blood and brain infection) suggest.

A

An antibody deficiency or COMPLEMENT DEFECT.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Signature organisms in: B cell abnormalities

A

recurrent sinopulmonary infections
with encapsulated organisms (pneumococcus, H.
influenzae type b)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Signature organisms in: Agammaglobulinemia or complement defect

A

recurrent pneumococcal disease

17
Q

Signature organisms in: SCID and other primary or secondary T cell immunodeficiencies (like HIV).

Looks like what on CXR?

A

Pneumocystis jiroveci (carinii) pneumonia

CXR = ground glass

18
Q

Signature organisms in: Phagocytic disorders or profound antibody or T cell immunodeficiency

A

pseudomonas sepsis, fungus

19
Q

Signature organisms in: X-linked agammaglobulinemia (XLA) –>

Recurrent bacterial meningits, is either:

A

enteroviral meningoencephalitis

  1. XLA, no immunoglobulins
  2. complement
20
Q

Signature organisms in: CGD (Chronic Granulomatous Disease)

A

abscesses or soft tissue infections secondary to
Aspergillus, Staphylococcus aureus, coagulase-negative
staphylococcus, Serratiamarcescens, Chromobacterium
violaceum

21
Q

Signature organisms in: Hyperimmunoglogulin E syndrome

A

recurrent staphylococcal

skin infections, abscesses, lung cysts, or pneumonia

22
Q

Signature organisms in: Abnormal T cell immunity

A

prolonged and severe candidiasis

involving the buccal mucosa, tongue, and palate may be the earliest sign

23
Q

Signature organisms in: Deficiencies of the late components of complement (C5 to
C9)

A

invasive infection with Neisseria species (meningitidis,

gonorrhoeae)

24
Q

Signature organisms in: PID

A

Primary Immunodeficiency suggested with vaccine failure or with infection with
vaccine strains following the administering of LIVE VACCINES (oral rotavirus, oral MMR –> live vaccines) polio, measles, varicella, BCG)

25
Q

Clinical patterns suggestive of immunodeficiency in what: Ataxia, telangiectasia, and developmental delay

A

in ataxiatelangiectasia

26
Q

***Clinical patterns suggestive of immunodeficiency in what: Congenital heart disease, developmental delay,
dysmorphic facies with low-set ears, hypertelorism,
downturning eyes and micrognathia **

A

in DiGeorge syndrome

27
Q

Clinical patterns suggestive of immunodeficiency in what: Extensive warts or molluscum contagiosum

A

in T cell disorders, innate immune defects, or the warts hypogammmaglobulinemia infections myelokathexis (WHIM) syndrome

28
Q

**ANC less than what has serious risk of infection?

A

**less than 500

29
Q

Kostmann Syndrome

A

AR. Severe neutropenia , ELA-2 mutations, increased risk of AML.
Manifests as life threatening pyogenic infections in infancy.

30
Q

Cyclic neutropenia

A

Neutropenia count less than 200 for 3-7 days every 3 weeks (15-35 days).

31
Q

*Schwachman-Diamond syndrome is what and what inheritance?

A
  • AR
  • Triad of (1) neutropenia, (2) exocrine pancreas insufficiency, and (3) skeletal abnormalities
  • Inc risk of MDS or leukemia
32
Q

Fanconi anemia

A

perfect example of COMPLETE BM failure.

33
Q

Partial oculocutaneous albinism - think what?

A

Chediak Higashi syndrome

AR

34
Q

Recurrent purulent infections with fungal or bacterial catalase-positive organisms, usually starting in infancy, chronic inflammatory granulomas - this is what?

A

chronic granulomatous disease

35
Q

Nitroblue tetrazolium test and absence of generation of superoxide is what disease?

A

chronic granulomatous disease

36
Q

common viruses causing neutropenia

-**common other cause of neutropenia

A

EBV

other - DRUG INDUCED NEUTROPENIA - cytotoxic agents for treatment of malignancy

37
Q

DNAAACP - causes of eosinophila

A
  • Drugs
  • Neoplasm
  • Atopic Disease - allergy, asthma, eczema
  • Addison disease - primary adrenal insufficiency
  • AIN - acute interstitial nephritis
  • Collagen Vascular disease - -SLE, Churg-Strauss vasculitis
  • Parasites - ascaris (loeffler eosinophilic pneumonitis)