Newman - Non-neoplastic WBC Disorders in Pediatrics Flashcards Preview

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Flashcards in Newman - Non-neoplastic WBC Disorders in Pediatrics Deck (37):
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)

16

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.

30

Cyclic neutropenia

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

31

***Schwachman-Diamond syndrome is what and what inheritance?**

***AR**
***Triad of (1) neutropenia, (2) exocrine pancreas insufficiency, and (3) skeletal abnormalities**
-Inc risk of MDS or leukemia

32

Fanconi anemia

perfect example of COMPLETE BM failure.

33

Partial oculocutaneous albinism - think what?

Chediak Higashi syndrome
AR

34

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

chronic granulomatous disease

35

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

chronic granulomatous disease

36

common viruses causing neutropenia

-**common other cause of neutropenia

EBV

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

37

DNAAACP - causes of eosinophila

-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)