Flashcards in Newman - Non-neoplastic WBC Disorders in Pediatrics Deck (37)
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1
WBC numbers
Most = Never > Let > Monkeys (monos) > Eat > Bananas = Least
2
Three criteria for "Worrisome" recurrent infections (start thinking about immunodeficiencies).
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.
3
Three clinical features suggestive of a primary immunodeficiency
1. FH of immunodeficiency or unexplained death *
2. Failure to gain wt or grow normally
3. IV antibiotics and/or hospitalization to clear infections
4
Condition with complete T cell deficiency
SCID - severe combined immunodeficiency
5
Three conditions with partial T cell deficiency
1. DiGeorge
2. **Ataxia telangiectasia (combined B and T cell)
3. Wiskott-Aldrich syndrome (combined B and T cell)
6
Main pathogens in T cell deficiency infections
1. Intracellular organisms (HSV, mycobacterium, listeria)
2. Fungi
7
Primary immunodeficiencies are... congenital or acquired?
congenital
Secondary - meds
8
Five pertinent things about BIRTH HISTORY in someone with immunodeficiency
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)
9
***Chediak Higashi syndrome
***Immunodeficiency + shows progressive NEUROLOGIC dysfunction in young adults + albinism
10
Amish people associated with ___. This is AR or AD?
Ataxia telangiectasia, AR
11
***What is Leukocyte Adhesion Defect? High count in what leukocyte?***
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.
12
***Between 2yo-6yo with recurrent URI, think what main deficiency?
***IgA deficiency
13
URI that cause/signs of immunodeficiencies
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
14
RECURRENT BACTERIAL MENINGITIS and SEPSIS blood and brain infection) suggest.
An antibody deficiency or COMPLEMENT DEFECT.
15
Signature organisms in: B cell abnormalities
recurrent sinopulmonary infections
with encapsulated organisms (pneumococcus, H.
influenzae type b)
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Signature organisms in: Agammaglobulinemia or complement defect
recurrent pneumococcal disease
17
Signature organisms in: SCID and other primary or secondary T cell immunodeficiencies (like HIV).
Looks like what on CXR?
Pneumocystis jiroveci (carinii) pneumonia
CXR = ground glass
18
Signature organisms in: Phagocytic disorders or profound antibody or T cell immunodeficiency
pseudomonas sepsis, fungus
19
Signature organisms in: X-linked agammaglobulinemia (XLA) -->
Recurrent bacterial meningits, is either:
enteroviral meningoencephalitis
1. XLA, no immunoglobulins
2. complement
20
Signature organisms in: CGD (Chronic Granulomatous Disease)
abscesses or soft tissue infections secondary to
Aspergillus, Staphylococcus aureus, coagulase-negative
staphylococcus, Serratiamarcescens, Chromobacterium
violaceum
21
Signature organisms in: Hyperimmunoglogulin E syndrome
recurrent staphylococcal
skin infections, abscesses, lung cysts, or pneumonia
22
Signature organisms in: Abnormal T cell immunity
prolonged and severe candidiasis
involving the buccal mucosa, tongue, and palate may be the earliest sign
23
Signature organisms in: Deficiencies of the late components of complement (C5 to
C9)
invasive infection with Neisseria species (meningitidis,
gonorrhoeae)
24
Signature organisms in: PID
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
Clinical patterns suggestive of immunodeficiency in what: Ataxia, telangiectasia, and developmental delay
in ataxiatelangiectasia
26
***Clinical patterns suggestive of immunodeficiency in what: Congenital heart disease, developmental delay,
dysmorphic facies with low-set ears, hypertelorism,
downturning eyes and micrognathia **
in DiGeorge syndrome
27
Clinical patterns suggestive of immunodeficiency in what: Extensive warts or **molluscum contagiosum**
in T cell disorders, innate immune defects, or the **warts** hypogammmaglobulinemia infections myelokathexis (WHIM) syndrome
28
**ANC less than what has serious risk of infection?
**less than 500
29
Kostmann Syndrome
AR. Severe neutropenia , ELA-2 mutations, **increased risk of AML.**
Manifests as life threatening pyogenic infections in infancy.
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