[PAGID] Delayed post-BMT lung disease

[Richard Wasserman]

Complete pulmonary function testing showed increased residual volume as the
only abnormality not picked up by standard spirometry (coordinate decrease
in FEV1 and FVC. DLCO was normal. Pulmonologist (part of the lung transplant
group) believes that this is a recurrence of bronchiolitis obliterans
despite the negative biopsy. Her last episode, which occurred one year post
transplant in 2001, did not respond to prednisone 40mg bid for two week but
did respond to solumedrol 10mg/kg once a week. Are there other suggestions?
Note that the patient has had somewhat difficult to control hypertension.
Thanks for your input.
Richard Wasserman
Dallas

On Fri, Jul 9, 2010 at 5:38 PM, Sergio Rosenzweig <
srosenzweig at garrahan.gov.ar> wrote:


> Hi Richard,

> Is the patient AIRE mutation positive? (bronchiolitis obliterans has been

> described in APECED patients, probably autoimmune), is she fully grafted or

> a mixed chimera?

> Sergio

>

> Sergio D. Rosenzweig, MD, PhD

> Chief, Infectious Diseases Susceptibility Unit

> Laboratory of Host Defenses, NIAID, NIH

> 10 Center Dr., Bldg. 10, CRC 5W-3888

> Bethesda, MD 20892-1456

> Phone (301) 451 8971

> Fax (301) 451 7901

> Cell (240) 361 7617

> Pager 102 10678

> srosenzweig at niaid.nih.gov

>

> Disclaimer: The information in this e-mail and any of its attachments is

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> >>> Richard Wasserman <drrichwasserman at gmail.com> 07/08/10 8:01 PM >>>

> 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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