2. Fever for 1 month
Left SCJ arthritis
Multiple cervical lymphadenopathy
Anemia
Previous history of recurrent Salmonella group D
infection
3. periarticular osteolytic lesion of the left SC joint
(arrow)
hepatosplenomegaly, and multiple, varying in size,
hypodensed splenic lesions (2arrows)
4.
5.
6. Specimens from BM, Blood and SC joint
aerobic blood cultures were negative and
symptoms didn’t respond to Ceftriaxone :
Salmonella excluded
STAINING
9. Numerous acid-fast bacilli
After 3 weeks of antimicrobial treatment;
she was referred to another hospital for
further management.
10. Vital signs Finding Normal So What ?
Temp. 38.3⁰C (36.5-37.2)⁰C elevated
HR 102bt/min (60-100)B/ min normal
Respiratory Rate 16 B/min (15-20)B/ min normal
Blood pressure 92/54mmHg 120/80mmHg low
11. CBC and other tests Findings Normal SoWhat ?
HB
HCT
4.1g/dl
13%
F(12.0 - 15.0)g/dl
F(36 – 44) %
Sever anemia
WBC count
-Neutrophils
-lymphocytes
30,450/mm³
70%
15%
4,500 -10,000/mm³
54-62 %
24-44 %
Elevated; Infection
platelet count 80,000/ mm³ 100,000- 450,000/mm³
Low
direct Coombs’ test negative No Abs attached toRBCs
total bilirubin
direct bilirubin
1.8 mg/dl
1.6 mg/dl
0.1 - 1.2 mg/dl
0 - 0.3 mg/dl
Hyperbilirubinemia
BUN
creatinine
63.4 mg/dl
4.0 mg/dl
7 - 20 mg/dl
0.5-1.4 mg/dl
Elevated
azotemia
alkaline phosphatase level
(608 U/l) 33 - 131 IU/L elevated
12. Lung X-ray revealed Bilateral interstitial infiltrate
in lung
M.Tuberculosis suspected and treatment for
both TB and NTM was started.
13. Posterior-anterior CXR in Pneumocystis jiroveci pneumonia showing severe,
bilateral pulmonary interstitial infiltrates with pneumatocoeles
From the collection of Matthew Gingo, UPMC
14. Mycobacterium culture
RT-PCR ,reverse hybridization and
Innogenetics/auto-LiPA 48 -> M.avium
M.Tuberculosis excluded
MAC is responsible for septicemia
But why disseminated ?
15. Is patient Immunocompromised?
HIV antibody test -> negative
Immunosuppressive therapy -> no drugs engaged
dysfunctional intracellular pathogen clearance
pathway ?
1 -CD4 T-lymphocyte count 1,247 cell/μl
(normal)!
2 -IL-12 and IFN-gamma Receptors (normal)!
3 -PHA stimulated PBMN response (normal)!
16. Patient PBMC had the ability to produce normal
amount of IFN-gamma while the activity of IFN-
gamma was inhibited by patients Auto-
antibodies detected by ELISA.
Final Dx:
Anti-IFN-gamma auto-antibodies!
17. Intermittent, high grade fever reappeared, and an acute
generalized pustular eruption of the patient’s skin
developed during the third week of antimycobacterial
therapy.
Skin biopsy showed leukocytoclastic vasculitis (LCV) and was
negative for mycobacteria on tissue culture .
A consultant dermatologist postulated either the
clarithromycin or levofloxacin were possible causes of
the pustular eruption. The fever and skin lesions resolved
with discontinuation of clarithromycin and levofloxacin,
along with a 3-week course of intravenous dexamethasone
18. Previously called MAF; up-regulates FC-gamma-RI
-> enhances phagocytosis/positive feedback loop
Defective IFN-gamma pathway -> increased
susceptibility to intracellular pathogens.
It’s an autoimmune disease that causes
immunodeficiency .
19.
20.
21.
22.
23.
24. Patient was discharged home from the hospital after 8 weeks
of hospitalization
no curative treatement
Antimicrobial agents to control infection
MAY work ?
monoclonal anti-CD20 antibody like Rituximab ?
Anti-antibodies ?
