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Presented by Peter Illei, M.D. and prepared by Robert E. LeBlanc, M.D.
Case 2: 74-year-old man with history of transformed Waldenstrom’s macroglobulinemia, s/p non-myeloablative haploidentical transplant on protocol 16 month ago.
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1. Question
Week 523: Case 2
74-year-old man with history of transformed Waldenstrom’s macroglobulinemia, s/p non-myeloablative haploidentical transplant on protocol 16 month ago complicated by skin GVHD and colitis as well as reactivation HBV. Patient now presented with productive cough, fever and shortness of breath x 1 week. Multiple studies for infectious workup including cultures, histoplasma and legionella antigens were negative. Bronchoscopy was performed and the airways did not demonstrate significant inflammation or friability. He did have somewhat bloody return from the periphery which raised the specter of alveolar hemorrhage. BAL cultures and pneumocystis stains were negative. Patient underwent intubation and VATS biopsy.images/S12-42520 DAD & BOOP 40x.jpg
images/S12-42520 DAD 4x.jpg
images/S12-42520 froathy ex 40Xb.jpg
images/S12-42520 HM 40Xb.jpg
images/S12-42520 pneum atyp40x.jpgCorrect
Answer: Pneumocystis jiroveci pneumonia with DAD
Histology: Sections show acute and organizing diffuse alveolar damage (DAD) with numerous hyaline membranes, reactive pneumocyte atypia, interstitial cellular fibrosis, partial parenchymal collapse and focal intraalveolar frothy exudate. Focal organizing pneumonia with intraalveolar fibroplasias is also noted. There is no evidence of constrictive bronchitis/bronchiolitis indicative of graft versus host disease. A fungal stain (GMS) highlights numerous clusters of pneumocystis organisms that are also seen on an immunostain for pneumocystis. A Movats stain highlights the intraalveolar fibroblast plugs ant the cellular interstitial fibrosis. A cytokeratin (AE1/AE3) stain shows lack of pneumocytes lining the alveolar septae that are covered by hyaline membranes, a feature that can be helpful in small biopsies to confirm the diagnosis of acute DAD. All viral cultures and PCR testing were negative.
Discussion: Pneumocystis pneumonia is an important infection in immunocompromised patients that can present with a variety of histologic pictures. The classic, most common form typically seen in AIDS patients shows frothy/foamy intraalveolar exudate with an associated interstitial pneumonitis. DAD with prominent hyaline membrane formation is the second most common form and is more common in non-AIDS patients. Other forms include granulomatous inflammation and/or BOOP-like reaction.
Incorrect
Answer: Pneumocystis jiroveci pneumonia with DAD
Histology: Sections show acute and organizing diffuse alveolar damage (DAD) with numerous hyaline membranes, reactive pneumocyte atypia, interstitial cellular fibrosis, partial parenchymal collapse and focal intraalveolar frothy exudate. Focal organizing pneumonia with intraalveolar fibroplasias is also noted. There is no evidence of constrictive bronchitis/bronchiolitis indicative of graft versus host disease. A fungal stain (GMS) highlights numerous clusters of pneumocystis organisms that are also seen on an immunostain for pneumocystis. A Movats stain highlights the intraalveolar fibroblast plugs ant the cellular interstitial fibrosis. A cytokeratin (AE1/AE3) stain shows lack of pneumocytes lining the alveolar septae that are covered by hyaline membranes, a feature that can be helpful in small biopsies to confirm the diagnosis of acute DAD. All viral cultures and PCR testing were negative.
Discussion: Pneumocystis pneumonia is an important infection in immunocompromised patients that can present with a variety of histologic pictures. The classic, most common form typically seen in AIDS patients shows frothy/foamy intraalveolar exudate with an associated interstitial pneumonitis. DAD with prominent hyaline membrane formation is the second most common form and is more common in non-AIDS patients. Other forms include granulomatous inflammation and/or BOOP-like reaction.
