[PAGID] Delayed post-BMT lung disease

[Nathaniel D. Hare]

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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