[CIS PIDD] [cis-pidd] a case of CVID and possible GLILD

[Hajjar, Joud]

Colleagues,



I would appreciate your input on this case below, regarding treatment recommendations for the patient summarized below with CVID and ILD.



He is 49 y old, and was diagnosed with CVID based on hypogam, recurrent sinopulmonary infections and started on IVIG in 2004. About 2 years ago he developed shortness of breath, shallow breathing and overall fatigue. CT scan of his chest and abdomen showed multiple granulomatous lesions and LAD, together with splenomegaly. At the time transbronchial lung bx was done and bx showed non-caseating granuloma, the exact description of cellular population in that bx were not described, but he was thought to have GLILD.
He was referred to me at that point to treat GLILD. I did send his bx for a second opinion, and the second opinion was “There is limited tissue for review as it usually is with transbronchial biopsy material. There is a non-necrotizing granuloma and focal lymphocytic infiltrate; otherwise, fragments of unremarkable bronchial tissue. If there is a clinical suspicion for LG or any other condition, additional tissue is needed, preferably an open biopsy”

He came back again, still feeling the same, rather than SOB he feels overall tired and in pain. His most recent CT scan read was “1. Scattered pulmonary nodules measuring up to 7 mm which are grossly stable to priors. 2. Stable findings of mediastinal and right hilar adenopathy. 3. No significant findings of interstitial lung disease.
His immune phenotyping is significant for elevated CD19+CD38-/LowCD21-/Low %15.3, and his CD4 counts is slightly low 478.
His PFTs showed normal volume loops but low DLCO 54% (this might be technical according to our pulmonary colleagues)-no O2 requirement.

We proceeded with an open lung biopsy, and the official pathology read the following:

DIAGNOSIS PART A RIGHT LUNG MIDDLE LOBE, WEDGE BIOPSY:
> LUNG PARENCHYMA WITH NON-CASEATING GRANULOMAS AND LYMPHOID AGGREGATES.
> NO TUMOR SEEN.
>
> PART B RIGHT LUNG LOWER LOBE, WEDGE BIOPSY:
> LUNG PARENCHYMA WITH NON-CASEATING GRANULOMAS AND LYMPHOID AGGREGATES.
> NO TUMOR SEEN.
>
> COMMENT This patient has a prior history of common variable immunodeficiency now presenting with pulmonary nodules as per the electronic medical records in Epic. Histological sections demonstrate portions of lung parenchyma with focal nodular lymphoid aggregates and non-caseating granuloma formation with focal calcification reminiscent of Schauman bodies. There are areas of consolidation and fibroblast plugging in the focal parts of the remaining lung parenchyma, however much of the remaining lung appears relatively normal in morphology. The overall features of this biopsy are non specific however possible differential diagnoses include primarily infection versus sarcoidosis in the appropriate clinical setting. Special stains for acid fast bacilli (AFB) and fungal organisms (GMS) performed on block A1 are negative. Immunohistochemical studies performed to assess the nature of the lymphoid aggregates demonstrate a mixed population of CD20 positive B cells and CD3/CD5 positive T cells. There is no co-expression of CD20 and CD5. Concurrent flow cytometry studies are negative (see reports F15-531, F15-532)
>
> In summary, there above findings are non-specific, however included in the differential is sarcoidosis and infections. Correlation with clinical findings, microbiology studies, and rheumatologic studies is recommended. There is no morphologic evidence of malignancy.
>

> FLOW:
>
> B LYMPHOCYTE ASSOCIATED:
> CD19 (Pan B Cell)
> CD20 (B Cell Mat.)
> CD22 (B Cell Mat.)
> CD10 (CALLA)
> CD5/CD19 (B-CLL)
> CD22/CD11c (HCL)
> CD23 (B Cell)
> FMC7 (B Cell)
> sIg KAPPA
> sIg LAMBDA
> CD38/CD20
>
> T LYMPHOCYTE ASSOCIATED:
> CD2 (E-rosette receptor)
> CD3 (Pan T Cell)
> CD5 (T Cell)
> CD16+56 (Natural Killer Cells)
> CD4 (T Helper Cells)
> CD8 (T Supressor Cells)
> CD7 (Pan T Cell)
>
> Flow Interpretation
> Phenotypic analysis was performed using the above listed markers. The lymphoid population accounts for 39% of analyzed events and are comprised of T cells (83% of lymphocytes) with a CD4;CD8 ratio of 1.5:1. Of note, within the T cell population there is a partial loss of CD2, however other T cell markers remain intact. Whether or not this represents an aberrant T cell population of a technical imperfection cannot be determined. B cells account for 5% of lymphocytes and are polytypic. Correlation with concurrent morphologic studies required for complete interpretation.







The pathologist's differential was sarcoidosis vs. infection. However, I do favor the diagnosis of GLILD considring his medical history and immune phenotyping.

My questions are:

-Baseed on the history and path above, would you agree on the diagnosis of GLILD?

-If yes, would you recommend to treat? and if treatment indicated would you use Rituximab as well as Azithromycin, considering he only has about 5% CD20 B cells?

-What follow up criteria would you use to assess response and length of treatment.





Thank you so much for your input

Most appreciatively,

Joud



Joud Hajjar MD

Assistant Professor in Medicine

Immuonology, allergy and rheumatology

Baylor college of medicine



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Hajjar, Joud
Thu 6/11/2015 3:28 PM
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Joud Hajjar MD
Assistant Professor
Texas Children's Hospital
Section of Immunology, Allergy & Rheumatology
joud.hajjar at bcm.edu<mailto:joud.hajjar at bcm.edu>


Texas Children's Hospital
1102 Bates FC 330
Houston, TX 77303
Office: 832-824-2595
Fax :   832-825-1260

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