[PAGID] Delayed post-BMT lung disease

[Berger, Melvin]

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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