Table 1.
Non-HPV pathogens identified in patients with WHIM syndrome.
| Organism | Patient Designation | Site | Reference | Evidence | |
|---|---|---|---|---|---|
| Gram Negative Bacteria | *P. mirabilis | Patient 1 | Respiratory infection | 3 | Sputum culture |
| 5592 | Axillary abscess | 7 | Wound culture | ||
| 5446 | Pneumonia | 7 | Culture | ||
|
| |||||
| *H. influenzae | Pneumonia | 92 | Throat culture | ||
| Patient 1 | Respiratory infection | 3 | Sputum culture | ||
| Patient 2 | Pulmonary infection | 3 | Sputum culture | ||
| J.A. | Meningitis | 21 | CSF culture | ||
| Patient 1 | Respiratory infection | 30 | Sputum culture | ||
| Patient 2 | Respiratory infection | 30 | Sputum culture | ||
| 5592 | Sepsis | 7 | Culture | ||
| 5231 | Pneumonia | 7 | Culture | ||
| 5446 | Pneumonia | 7 | Culture | ||
|
| |||||
| *P. aeruginosa | P1 | Chronic airway colonization | 80 | Culture | |
| P3 | Chronic airway colonization | 80 | Culture | ||
| Septicemia | 93 | Culture | |||
| 5231 | Pneumonia, sepsis, gastrointestinal infection | 7 | Culture | ||
|
| |||||
| E. coli | Patient 2 | Cystitis | 3 | Culture | |
| 5546 | Cystitis | 7 | Culture | ||
| 5231 | Respiratory infection | 7 | Culture | ||
|
| |||||
| ˆM. morganii | 5446 | Pneumonia | 7 | Culture | |
|
| |||||
| ˆB. cartarrhalis | 5446 | Pneumonia | 7 | Culture | |
|
| |||||
| S. typhimurium | 5780 | Gastrointestinal infection | 7 | Culture | |
|
| |||||
| C. jejuni | 5231 | Gastrointestinal infection | 7 | Culture | |
|
| |||||
| Gram Positive Bacteria | *S. aureus | Respiratory infection | 31 | Epiglottic culture | |
| Patient 1 | Respiratory infection | 3 | Sputum culture | ||
| Patient 2 | Respiratory infection | 3 | Sputum culture | ||
| 5231 | Pneumonia, gastrointestinal, infection | 7 | Culture | ||
| 5446 | Pneumonia | 7 | Culture | ||
| 5449 | Adenophlegmon | 7 | Wound culture | ||
| 5780 | Osteoarthritis | 7 | Culture | ||
| WHIM-12 | Skin lesions | 24 | Wound culture | ||
|
| |||||
| *S. pneumoniae | Pneumonia | 92 | Sputum culture | ||
| Patient 1 | Respiratory infection | 30 | Sputum culture | ||
| Patient 2 | Respiratory infection | 30 | Sputum culture | ||
| 5592 | Sepsis | 7 | Blood culture | ||
| 5231 | Pneumonia | 7 | Culture | ||
| Pneumonia | 11 | Culture | |||
| 5446 | Pneumonia, pericarditis | 7 | Culture | ||
| Pneumonia, bacteremia | 13 | Blood culture | |||
|
| |||||
| S. pyogenes | Streptococcal pharyngitis | 92 | Throat culture | ||
|
| |||||
| C. perfringens | Septicemia | 93 | Blood culture | ||
|
| |||||
| Myco- bacteria | Nontypable | 5231 | Chronic skin granuloma | 7 | Skin biopsy |
|
| |||||
| M. gordonae | 5446 | Hepatitis, respiratory infection | 7 | Sputum culture | |
|
| |||||
| Fungi | A. glocus | 5592 | Sinusitis, mastoiditis | 7 | Culture |
|
| |||||
| C. albicans | 5231 | Respiratory infection, gastrointestinal infection | 7 | Cultures | |
| 5446 | Pneumonia | 7 | Culture | ||
|
| |||||
| Viruses | Rubella | Disseminated | 31 | Clinical impression | |
| S.S. | Disseminated | 25 | Clinical impression | ||
| P3 | Disseminated | 23 | Clinical impression | ||
|
| |||||
| Rubeola | Disseminated | 31 | Clinical impression | ||
|
| |||||
| Varicella zoster | Skin lesions | 92 | Clinical impression | ||
| Skin lesions | 31 | Clinical impression | |||
| S.S. | Skin lesions | 25 | Clinical impression | ||
| J.A. | Severe chickenpox | 21 | Clinical impression | ||
| Patient 2 | Severe chickenpox | 32 | Clinical impression | ||
| P10 | Skin lesions | 9 | Clinical impression | ||
| P3 | Dermatomal infection (left calf) | 80 | Polymerase chain reaction | ||
| Unidermatomal thoracic zoster | 13 | Clinical impression | |||
|
| |||||
| Herpes simplex | Herpes labialis | 31 | Clinical impression | ||
| Herpes labialis | 8 | Clinical impression | |||
| 5592 | Stomatitis (HSV1) | 7 | Clinical impression | ||
| 5446 | Stomatitis (HSV1) | 7 | Clinical impression | ||
| Recurrent cutaneous infections | 10 | Clinical impression | |||
| Severe perioral infection | 13 | Clinical impression | |||
| 5231 | Genital lesions (HSV2) | 7 | Clinical impression | ||
|
| |||||
| Epstein-Barr | B lymphoma | 26 | Polymerase chain reaction | ||
| Hemophagocytic lymphohistiocytosis, B lymphoma | 8 | In situ hybridization, immunohistochemistry | |||
|
| |||||
| Molluscum contagiosum | Skin lesions | 8 | Clinical impression | ||
| 5446 | Skin lesions | 7 | Clinical impression | ||
|
| |||||
| Cytomegalovirus | 5231 | Sepsis | 7 | Polymerase chain reaction | |
|
| |||||
| Influenza | P1 | Blood | 23 | Virologic analysis | |
| P2 | Blood | 23 | Virologic analysis | ||
Selected cases include patients with WHIM syndrome, defined as myelokathexis and/or CXCR4 C-tail mutations.
ˆ
Opportunist
*
Encapsulated organism