[PAGID] Delayed post-BMT lung disease

[Richard Wasserman]

No.

On Thu, Jul 8, 2010 at 8:00 PM, Church, Joseph <JChurch at chla.usc.edu> wrote:


>  Do you have arterial blood gas, diffusion capacity, residual volume?

>

>

>

> Joe Church

>

> Childrens Hospital LA

>

>

>  ------------------------------

>

> *From:* pagid-bounces at list.clinimmsoc.org [mailto:

> pagid-bounces at list.clinimmsoc.org] *On Behalf Of *Richard Wasserman

> *Sent:* Thursday, July 08, 2010 4:00 PM

> *To:* PAGID

> *Subject:* [PAGID] Delayed post-BMT lung disease

>

>

>

> A now 27 year old woman with chronic mucocutaneous candidiasis and multiple

> endocrinopathy (thyroid, parathyroid, adrenal, ovary?) received a match

> sibling transplant in 1997 and now has dyspnea and abnormal pulmonary

> function tests.

>

>

>

> She has RSV during the transplant (first identified prior to marrow

> infusion) but did quite well. Approximately nine months post-transplant she

> developed Zoster that responded well to treatment. One year and a few days

> after transplant she developed respiratory symptoms and a significant fall

> in FEV1 and FVC. CT report showed:

>

> *Ct chest  4/21/99*

>

> *History: possible interstitial lung disease. Bone marrow transplant

> patient.*

>

> *Technique: helical 7-mm images are obtained through the cheat at a 1-to-1

> pitch without intravenous contrast. Then, an expiratory and an inspiratory

> image was obtained at 2-cm increments 1-mm high-resolution cuts to judge

> areas of air trapping or interstitial disease.*

>

> *Findings: no definite adenopathy can be seen on the soft tissue windows.

> Standard 7-mm lung windows show no focal infiltrate, edema, pleural

> thickening, or effusion. There is mild prominence of peribronchial lining.

> *

>

> *High-resolution images, inspiratory and expiratory films, fail to reveal

> focal or localized areas of air trapping. There is peribronchial thickening

> with some mild suggested bronchiectasis in the right upper lung, superior

> segment of the lower lobes. There is slight granular haze of the lungs

> without discrete reticular nodular pattern which is nonspecific. There is no

> "beading" or discrete retraction or fibrosis noted. The mild bronchiectatic

> change appears more prominent compared to the study of february 1997.

> Peribronchial cuffing was noted at that time as well, no central

> intraluminal mucous plug can be seen.*

>

> *Impression:*

>

> *1. Nonspecific pzribronchial cuffing with wild suggestion of

> bronchiectasis can be associated with asthma or reactive airway disease but

> is otherwise nonspecific. High-resolution images suggest some slightly gray

> parenchymal changes diffusely without nodularity also nonspecific. Focal

> nodular deposits of suspected graft-versus-host disease cannot be

> conclusively demonstrated on this ct study.*

>

> *She was bronchoscoped:*

>

> *transbronchial biopsies obtained on the day prior to admission

> demonstrated bronchiolitis obliterans with active interstitial pneumonitis.

> No viral inclusions, acid-fast bacilli, fungi, or malignant cells were

> identified. Immunostains for Pneumocystis carinii and cytomegalovirus were

> negative. Viral, bacterial, fungal and AFB cultures were negative.*

>

>

>

> She was treated with pulse Solumedrol and IVIG and resolved with return of

> FEV1 and FVC to her premorbid levels and clearing of her chest CT with no

> residual abnormality.

>

>

>

> In the intervening years she has had mild persistent asthma but has not

> needed oral steroids. Antibody production and mitogen and antigen responses

> have been normal. There has been normal recovery from respiratory viral

> infection (she's a school teacher), no candida and rare need for

> antibiotics. She has had hypertension. In March, 2010 she presented with

> shortness of breath.

>

>

>

> December 08 - FEV1 106% of predicted, FVC 97% of predicted

>

> December 09 - FEV1 94% of predicted, FVC 85% of predicted

>

> March 10 - FEV1 68% of predicted, FVC 64% of predicted

>

> June 10 - FEV1 72% of predicted, FVC 64% of predicted

>

>

>

> *Repeat bronchoscopy in March 2010:*

>

> *MICROSCOPIC DIAGNOSES: Transbronchial biopsies of lung: Multiple biopsies

> are present containing adequate bronchial and alveolar parenchymal tissue

> for evaluation; minimal subacute bronchitis is present unaccompanied by

> granulomas or viral inclusions; the alveolar tissues are histologically

> unremarkable; a histologic explanation for the patient's worsening

> lung function is not identified; special stains for acid-fast bacilli and

> fungi are negative, as are *immunostains for Cytomegalovirus and

> Pneumocystis carinii; a special stain for amyloid was, likewise, negative.

> *

>

>

>

> *Right bronchial washings (cell block and cytospin preparations, Pap and

> Wright stains): Benign respiratory columnar cells and pulmonary macrophages

> are present within a clean inflammatory background; no viral inclusions or

> malignant cells are identified; special stains for acid-fast bacilli and

> fungi are negative, as are *immunostains for*

>

> *Cytomegalovirus and Pneumocystis carinii; all stains exhibit

> appropriately reactive controls.*

>

>

>

> *Culture for bacteria, virus, fungus and AFB grew only alpha hemolytic

> streptococci. She was treated with minocycline without benefit.*

>

>

>

> At this time she is mildly dyspnea at rest but has no exercise tolerance.

> Pulmonology has no suggestions for further diagnosis or treatment.

>

>

>

> I would appreciate any thoughts on this patient.

>

>

>

> Richard L. Wasserman, MD, PhD

> DallasAllergyImmunology

> 7777 Forest Lane, Suite B-332

> Dallas, Texas 75230

> Office (972) 566-7788

> Fax (972) 566-8837

> Cell (214) 697-7211

>




-- 
Richard L. Wasserman, MD, PhD
DallasAllergyImmunology
7777 Forest Lane, Suite B-332
Dallas, Texas 75230
Office (972) 566-7788
Fax (972) 566-8837
Cell (214) 697-7211
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