NORMAL RESPONSE TO BLOOD BORNE ANTIGENS

Blood borne antigen splenic artery enters spleen white pulp phagocytosed by dendritic
cells dendritic cell present antigen via MHC-II to Th cells Th cells activated migrate to marginal
zone come in contact with B-cells in primary follicles B-cell activation secondary follicles and
plasma cell rich germinal centers forms produce antibodies Abs enter systemic circulation bind
antigen phagocytosis of pathogenic organism by opsonization

ASPLENIC PATIENTS

- Unable to mount this response as mentioned above

- High risk of overwhelming infection by encapsulated organisms like H. influenzae, S. pneumonia and
N meningitidis

ANGIODEMA

HEREDITARY ANGIODEMA

- Characterized by the following:

1. Rapid onset of

Non-inflammatory edema of the face, limbs, and genitalia

Laryngeal edema - can be life-threatening

Edema of the intestines resulting in colicky abdominal pain

2. No evidence of urticarial

- Angioedema can be hereditary or acquired.

- Pathology in both forms can involve C1 inhibitor deficiency, dysfunction, or destruction.

- A defect or deficiency of C1 inhibitor ↑ levels of the edema-producing factors C2b and bradykinin

- Hereditary angioedema typically presents in late childhood.

- Episodes usually follow an infection, dental procedure, or trauma.

- C1q levels are normal in hereditary angioedema and depressed in acquired forms, which usually
present much later in life.

- C4 levels are depressed in all forms of angioedema.

ACQUIRED ANGIOEDEMA

- Most common cause: ACE inhibitors (aka kininase)

- Mechanism: ACE breakdown of bradykinin. ACE inhibited by ACE inhibitor accumulation of

bradykinin. Bradykinin is responsible for edema, inflammation and sensation of pain

- C/F: Patient presents with edema of face, mouth, tongue, lips, glottis and larynx. Laryngeal edema
can cause airway obstruction and can be life threatening

- Time of presentation: typically within days to weeks of starting ACE inhibitors but can present at
ANYTIME, not just within weeks of starting.

- Management:

1. Check for airway compromise and vasomotor instability, which require subcutaneous epinephrine
administration if present.