[PAGID] Delayed post-BMT lung disease

[Richard Wasserman]

Mel,
Her donor has allergic rhinitis and mild persistent asthma. She has minimal,
intermittent rhinitic symptoms and no response to bronchodilator. I have not
challenged her. I don't have a recent IgE. The onset of the current
deterioration was insidious; there has been no suggestion of an acute viral
process and no improvement over the past three months.
Richard

On Fri, Jul 9, 2010 at 10:38 AM, Berger, Melvin <
Melvin.Berger at uhhospitals.org> wrote:


> Richard- I realize no excess eos were seen, but what is her IgE and does

> she have any evidence of inhalanat allergy ?  Have you tried a cold-air,

> exercise or methacholine challenge. Maybe she over-reacts to mild viral

> infections.

>

>

>

> Melvin Berger, M.D., Ph.D.

> Adjunct Professor of Pediatrics and Pathology

> Case Western Reserve University

> Cleveland, OH 44106

>

> ________________________________

>

> From: pagid-bounces at list.clinimmsoc.org on behalf of Richard Wasserman

> Sent: Fri 7/9/2010 9:25 AM

> To: pagid at list.clinimmsoc.org

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

>

>

> Echo normal, followed closely by cardiology for the hypertension. PE ruled

> out by CT angio.

> Thanks,

> Richard

>

>

> On Fri, Jul 9, 2010 at 8:05 AM, Nathaniel D. Hare <NHare at cheshire-med.com>

> wrote:

>

>

>        Other questions:

>

>        Echocardiogram?  Pulmonary hypertension?  Pulmonary embolus ruled

> out?

>

>

>

>        Nathan Hare MD

>

>        Allergy/Immunology

>

>        Cheshire Medical Center - Dartmouth Hitchcock Keene

>

>        Keene, NH

>

>

>

>

> ________________________________

>

>

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

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

>        Sent: Thursday, July 08, 2010 7:00 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

>

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>

>

>

> --

> 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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-- 
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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