Lymphoproliferative disorder is a well-recognized complication of lung transplantation. Risk factors include Epstein-Barr virus infection and immunosuppression. The gastrointestinal manifestations of posttransplant lymphoproliferative disorder (PTLD) in lung transplant recipients have not been fully characterized.
Case presentation and review of all reported cases of PTLD with gastrointestinal involvement.
Patient ages ranged from 25 to 65 years (median 52 years). Median time form lung transplantation to onset of PTLD was 51 months (range 1-109 months); 65% of cases (11/17) occurred within 12 months; Eighty-eight percent of patients (15/17) had positive Epstein-Barr virus serology prior to transplantation. In five patients (29%), the PTLD also involved sites other than the gastrointestinal tract. The most common gastrointestinal site of PTLD was the colon, followed by the small intestine and stomach. Clinical features included abdominal pain, nausea, and bloody diarrhea. Diagnosis was based on typical pathological changes on gastrointestinal tract biopsy obtained mainly by colonoscopy. Treatment included a reduction in the immunosuppressive regimen in 15 of the 17 cases (88%) and surgical resection in 10 (59%). One patient was untreated. Seven of the 16 patients (44%) responded to treatment and 9 patients died. Median time from onset of PTLD to death was 70 days (range 10 to 85 days).CONCLUSIONS: PTLD with gastrointestinal involvement is a unique entity that should be considered in all Epstein-Barr-Virus-positive lung transplant recipients who present with abdominal symptoms. Although immunosuppressive modulation and resection can lead to remission, the risk of death is 50%.
PTLD with gastrointestinal involvement should be considered in lung transplant recipients who present with abdominal symptoms.
Characteristics of gastrointestinal involvement of PTLD following lung transplantation: review of 17 casesCaseSexAgeDiseaseTxEBV statusOnset (Months)GIT siteOther siteManagementOutcomeSurvival (Days)Cause of deathCommentsRef1M56COPDSL+36Transverse colon−Resection RISRemission360BOSHct 21% “Apple-core”12F52COPDNA+20Small bowel−NADiedNANA23M51COPDNA+8ColonLungNot treatedDiedNAPTLD24F31Bronchi- ectasisNA+4Colon−Resection RISDiedNAPTLDMultiple synchronous tumors25M28PVODSL+1Jejunum−Resection RIS IFNDied360Infection36NA25NANA+6ColonLungResection RISResolved289NA47M56COPDSL+33Colon−Resection RISResolvedNANA“Apple-core”58NA49A1ATBL+86GITLiverRISRemission1223NA69NA32PPHSL+70GITBMRIS ChemoDied85PTLD610NA32ASD/EDSLNA82GIT−Resection RISRemission50BOS, Pneumonia611NA40CFBL+109GIT−RIS ChemoDied10NA612NA52A1ATSLNA95GITOvaryResection RISDied70PTLD, Sepsis613NA52IPFSL+82GIT−Resection RISDied50NA614NA54PPHSL+51GIT−Resection RISRemission61BOS615NA59COPDSL+53GIT−RISDied20PTLD616NA60COPDSL+13GIT−Resection RISDied10PTLD, MOF617M65IPFSL+18Transverse colon−RISRemission360Bloody diarrhea; Hct 17%Present reportA1AT=alpha-one antitrypsin; ASD/ED=atrial septal defect with Eisenmenger syndrome: BL-bilateral lung transplantation; BOS-bronchiolitis obliterans syndrome; COPD=chronic obstructive pulmonary disease; CF=cystic fibrosis; GIT=gastrointestinal tract; EBV= Epstein-Barr virus; IPF=idiopathic pulmonary hypertension; PTLD=posttransplant lymphoproliferative disorder; PVOD=pulmonary veno-occlusive disorder; RIS=reduction of immunosuppression; SL=single-lung transplantation
S. David, None.