Case Report: 52 Year-Old Male 11 Months after MUD for Angionimmunoblastic T Cell Lymphoma Developed Acute Fibrinous Organizing Pneumonitis Successfully Treated with Etanercept Suggesting TNF Alpha in the Pathogenesis in This Sub-Type of Pulmonary GVHD

Title: A Case Report of a 52 year-old almost one year after MUD for angionimmunoblastic T cell

lymphoma developed acute fibrinous organizing pneumonitis successfully treated with

etanercept suggesting TNF alpha in the pathogenesis in this sub-type of pulmonary GVHD.

Introduction: We describe a case of 52 year-old man with history of refractory

Angioimmunoblastic T-cell lymphoma 11 months after matched unrelated allogeneic

hematopoietic cell transplant with acute fibrinous organizing pneumonitis successfully

treated with high dose steroids, tacrolimus, and 8 doses of etanercept.

Case presentation: A 52 year-old Caucasian male with a history of refractory stage IV

angioimmunoblastic T-cell lymphoma treated with CHOP, ICE and Romidepsin followed by

ATG/TBI matched unrelated allogeneic hematopoietic cell transplant (HCT) in March 2013.

He had an uncomplicated course with the exception of mild classic chronic GVHD of skin

treated with topical steroids. Eleven months after HCT, he was off immunosuppression and

receiving his vaccinations per our institutions protocol when he presented to clinic with dry

cough, dyspnea, and oxygen saturations in mid 60% on room air. A chest CT

demonstrated significant multifocal nodular opacities and ground glass in all lung fields

(figure 1) as well as enlargement of axilla, sub pectoral and mediastinal lymph nodes. He

underwent a non-diagnostic bronchoscopy followed by VATS left upper lobe and left lower

lobe wedge resections. The pathology revealed acute organizing fibrinous pneumonitis

(AFOP) (figure 2). He was intubated in the intensive care unit for 2 days after VATS wedge

resection procedure. Therapy for AFOP was initiated methylprednisolone 1000mg daily in

divided doses along with tacrolimus. Several days later etanercept 25mg SQ twice weekly

was initiated because patient was not improving his oxygenation. He received a total of 8

doses of etanercept. His recovery was complicated by prolonged pneumothorax and

pneumomediastium requiring a pigtail catheter. He returned to the operating room for

decortication, mechanical pleurodesis and chest tube placement. Eight days postoperatively

the chest tube was removed. He was weaned of oxygen in seven days. Repeat

chest CT demonstrated improved multifocal airspace disease (figure 1) and he is still

without oxygen as of September 2014.

Discussion: To the best of our knowledge this is the first case demonstrating the successful

treatment of AFOP, a sub-type of pulmonary chronic GVHD, with Etanercept. Etanercept is

an inhibitor of tumor necrosis factor-alpha (TNF- alpha). Therefore,

Figure 1: Biopsy wedge resection left upper lobe consistent with acute organizing fibrinous

pneumonitis (AFOP)

Figure 2: CT images before and after etanercept

 

         January 31, 2014                          March 11, 2014