[CIS PIDD] [cis-pidd] Histiocytosis and persistant disseminated atypical mycobacteria

[Nacho Gonzalez]

Dear colleagues,

I would like to hear any input regarding diagnosis and treatment for a 3
1/2  yo male with LCH and disseminated M. genavense infection.

At 6 mo of age weight < P3 & height P10. Multisystemic Langerhans
histiocytosis (LCH)  treated with vinblastin, steroids and clofarabin (this
drug from Nov 2012 to 22 june 2013, 8 courses) finished in April 2013.
Maintenance chemo (MTX+MCP & steroids) stopped 8 months ago. LCH is
considered as non-active disease after several reevaluations.

Disseminated atypical mycobacteria was diagnosed in Jan 2014. After 1 month
under iv etambutol+levo+azithro treatment and 1 1/2  months po, fever
relapsed and bone marrow was again full of atypical mycobacteria. Then we
used iv linezolid, amikacin, ethambutol, azithromycin and levo. In June PCR
revealed NTM was M. genavense. Since then, he is under iv levo +
clarithromycin + ethambutol + rifampicin.
For the last 2 1/2 months fever subsided and night sweating dissapeared.
However in the last 10 days he has daily fever and night sweating.
Extensive infectious disease work up is negative.
He is TPN dependent because of chronic diarrhea, malnutrition and protein
losing enteropathy (PLE has resolved lately). Two gut endoscopies revealed
NTM within macrophages covering the lamina propria. Microbiologists cannot
ensure they are not viable. All cultures (BM, gut) have been negative for
NTM. Abdominal MRI (March 2013) suggested sclerosing mesenteritis. A trial
of colchicine turned to be ineffective. Follow up ultrasounds show
improvement of this.
In the past he had prolonged viral shedding with viral infections (but not
clinically severe) after Paraflu, Flu, noro and rota infections in the last
two years w or w/o chemo.
The patient had myelodisplasia in the last two BM samples. He has no
cytopenias now.

IFNg/IL12 axis assessment is normal, with normal production of TNFa and
IFNg after PBMC stimulation).
Lymph subsets: ALC 1300 - 45 00/uL CD3 (216 - 432 CD4, 1000 - 3000 CD8+.
Almost all of them have memory phenotype. Decreased thymic output, CD31+
10% ),  40 - 176 /uL CD19 with normal IgG levels. Decreased T cell
proliferation with PHA, PMA/iono, antiCD3/CD28. (see follow-up
immunological tests *attached*)
​​
Genes sequenced: IFNgR1, RAG1/2,GATA2. All wt

Questions:
Is this PID primary or secondary ? Is there any way to know it?
How to balance benefit/risk ratio of sc IFNg, as LCH eventually may recurr
? Anyone has experience on this?
HSCT? When?

Any input will be welcomed.
Best regards,

Luis Ignacio Gonzalez-Granado
Immunodeficiencies Division
Hematology & Oncology Division
Pediatrics. Hospital 12 octubre. Madrid. Spain

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