DEFECT
EXAMPLES
TYPICAL ORGANISMS
KEY POINT
X-linked agammaglobulinemia, CVID, selective IgA deficiency
Encapsulated bacteria (sinopulmonary), enteroviral meningoencephalitis, Giardia
No live vaccines in XLA · IVIG
Early complement (C1–C4, C3)
C2 deficiency (lupus-like), C3 deficiency
Encapsulated bacteria, recurrent pyogenic infections
C3b opsonization lost
Terminal complement (C5–C9)
Inherited, eculizumab
Recurrent Neisseria
Meningococcal vaccines
Splenectomy, sickle cell (autosplenectomy)
S pneumoniae, Hib, N meningitidis, Salmonella (osteomyelitis), Capnocytophaga (dog bite), Babesia
Howell-Jolly bodies · vaccines before elective splenectomy · standby antibiotics
Chemotherapy, aplastic anemia, drugs
S aureus, Pseudomonas and enteric gram-negatives, Candida, Aspergillus, Mucor
Febrile neutropenia → cefepime/piperacillin-tazobactam now
Chronic granulomatous disease
NADPH oxidase defect (X-linked most)
Catalase ⁺: S aureus, Serratia, Burkholderia, Nocardia, Aspergillus
Abnormal DHR test · TMP-SMX + itraconazole prophylaxis, IFN-γ
Leukocyte adhesion deficiency
CD18 integrin defect
Skin and mucosal bacterial infections without pus
Delayed umbilical cord separation, very high WBC
DiGeorge, SCID, HIV, steroids, calcineurin inhibitors
Viruses (CMV, VZV), Pneumocystis, Candida, Cryptococcus, mycobacteria, Listeria, Toxoplasma
No live vaccines
Receptor mutations
Disseminated mycobacteria (incl. BCG), Salmonella
IFN-γ therapy
Infliximab, adalimumab, etanercept
Reactivated TB, histoplasmosis, Listeria, HBV
Screen for latent TB and HBV first
< 1 month · 1–6 months · > 6 months
Surgical/nosocomial (MRSA, C diff, Candida) · CMV, EBV (PTLD), Pneumocystis, BK, Toxoplasma · community organisms
Prophylaxis: TMP-SMX, valganciclovir
Burns, IV catheters, Foley, ventilators, CF
Pseudomonas, S aureus, S epidermidis, Candida, Enterococcus
Remove devices
Neutrophil dysfunction, vascular disease, glycosuria
Mucor (DKA), malignant otitis externa (Pseudomonas), foot infections, emphysematous pyelonephritis, Candida
—
Hemochromatosis, deferoxamine
Yersinia, Vibrio vulnificus, Mucor, Listeria
Siderophilic organisms