Encapsulating peritoneal sclerosis in a patient after allogeneic hematopoietic stem cell transplantation: a case report

Abstract

BackgroundEncapsulating peritoneal sclerosis (EPS) is a chronic clinical syndrome of acute or subacute gastrointestinal obstruction seen mainly in patients undergoing peritoneal dialysis. Although there are a few reports on EPS developing in non-peritoneal dialysis patients, it has not been reported in patients undergoing allogeneic haematopoietic stem cell transplantation (HSCT). Here, we report a case of EPS after a second HSCT.Case presentationA 46-year-old man with myelodysplastic syndrome showed relapse after HSCT and received a second HSCT. The patient was diagnosed with chronic graft-versus-host disease (cGVHD)-associated serositis because of persistent ascites. His ascites improved gradually and disappeared without immunosuppressive therapy. He presented with nausea, weight loss, and constipation 1 year after improvement of ascites. Computed tomography revealed no organic obstruction, but did reveal dilated, thickened, and adhered small bowel loops with a mass-like appearance. He was diagnosed with EPS on the basis of clinical symptoms and image findings. He received corticosteroid therapy (20 mg/body) without any improvement in symptoms. He developed recurrence of myelodysplastic syndrome at 1 month after initiation of corticosteroid therapy. This progressed into acute myeloid leukaemia after 3 months. He died 31 months after the second HSCT. At autopsy, the small and large intestines had formed extensive adhesions and showed signs of progressive fibrosis with peritoneal sclerosis, fibroblast swelling, fibrin deposition, and inflammatory cell infiltration, which confirmed the diagnosis of EPS.ConclusionThis case suggests that EPS may complicate patients with cGVHD-associated serositis. Although the mechanism of EPS development is not clear, clinicians should be aware of this eventuality.

Causes of Polyserositis: A Systematic Review

“…Twenty patients (17.5%) had a known previous history of cancer. Hematological neoplasia (7; 35%) and lung cancer (7; 35%), which were identified in an equal number of patients, were the most common types [ 1 , 9 , 10 , 11 , 12 ]. Two of the six remainders had a previous history of breast or ovarian carcinoma [ 13 , 14 ], while in Losada’s study the other types of neoplasia were not mentioned [ 1 ].…”

“…One patient, with relap dysplastic syndrome after allogenic hematopoietic stem cell transplantation, nosed with chronic graft-versus-host-disease-associated serositis (bilateral pl sion and ascites), which improved after a second allogenic hematopoietic stem Other causes were identified in 14 cases (12.3%). One patient, with relapsed myelodysplastic syndrome after allogenic hematopoietic stem cell transplantation, was diagnosed with chronic graft-versus-host-disease-associated serositis (bilateral pleural effusion and ascites), which improved after a second allogenic hematopoietic stem cell transplantation [10], and one woman with ovarian hyperstimulation syndrome developed a large amount of bilateral pleural effusion and a slight amount of ascites, with improvement after drainage [16]. In another CR, the first systemic inflammatory reaction was presented to the 13-valent pneumococcal conjugate vaccine in a patient without a previous autoimmune disease, who progressively developed polyarthralgia, myalgias, and finally marked serositis of the pleura and pericardium [14].…”

“…The exclusion criteria included any of the following: (1) papers without evidence of PS, (2) papers involving people under the age of 18, (3) papers not involving humans, (4) papers not mentioning if the effusions were transudates or exudates, (5) papers not mentioning which criteria, or if any criteria were fulfilled in order to affirm that the effusions were exudates, (6) papers labeling the effusions as exudates according to the tests performed on previous presentations, unrelated to the actual acute event, (7) papers labeling the effusions as PS, without acknowledging if the effusions were present in at least two cavities at the same time, (8) narrative reviews and systematic reviews, (9) study protocols, (10) papers which did not have the full text available, and (11) publications in languages other than those mentioned above.…”

Encapsulating Peritoneal Sclerosis in Systemic Lupus Erythematosus, Rheumatoid Arthritis, and Systemic Sclerosis

“…Encapsulating peritoneal sclerosis (EPS) is a rare clinical syndrome characterized by an inflammatory fibrocollagenous membrane encasing the small and large intestines, resulting in recurrent intestinal obstruction ( 1 - 3 ). EPS often occurs as refractory ascites retention due to chronic peritonitis ( 2 - 6 ). Eltringham et al first reported EPS as a side effect of β-blockers in 1977 ( 7 ); however, most EPS cases have been described as secondary to peritoneal dialysis ( 1 - 3 ) since this phenomenon was first described by Gandhi et al in 1980 ( 8 ).…”