Pneumocystis cystoides intestinalis with pneumoperitoneum and pneumoretroperitoneum in a patient with extensive chronic graft-versus-host disease

Abstract

Summary:Pneumatosis cystoides intestinalis is a rare finding of intramural gasfilled cysts in the bowel wall and sometimes free air in the abdomen. A few conditions are reported to cause this disease, one of them being immunosuppression. We describe a 50-year-old Caucasian male with extensive chronic graft-versus-host disease (GVHD) of the gut and skin who developed PCI with pneumoperitoneum and pneumoretroperitoneum. To our knowledge, this is the first report of PCI occurring in a patient with active chronic GVHD which resolved spontaneously. Keywords: Pneumatosis cystoides intestinalis; chronic graft-versus-host disease Pneumatosis cystoides intestinalis (PCI) is a rare disorder consisting of multiple intramural gas collections in the bowel wall. Patients are either asymptomatic or present with diarrhea, pain, tenderness, vomiting and flatulence. The pathogenesis of this abnormality is still unknown. It is associated with chronic obstructive pulmonary disease, necrotizing enterocolitis in premature infants, intestinal obstruction, ischemic bowel disorders, bacterial and viral infections 1 and drug therapy. 2 In contrast to other gastrointestinal diseases PCI is a benign finding and usually resolves spontaneously. It responds well to conservative therapy. Rarely, PCI is observed after allogeneic bone marrow transplantation (BMT) where fatalities have been reported in patients receiving immunosuppression.3,4 We describe a patient with myelodysplastic syndrome given an allogeneic marrow graft. He experienced extensive chronic graft-versus-host disease (GVHD) and developed PCI 8 months after BMT. Case reportA 50-year-old Caucasian male who was diagnosed with myelodysplasia, subtype chronic myelomonocytic leukemia, underwent BMT after conditioning with cyclophosphamide and fractionated total body irradiation (12 Gy) at our institution. He received 3.2 × 10 8 nucleated bone marrow cells/kg body weight from his HLA-identical sibling. GVHD prophylaxis consisted of cyclosporin A and a short course of methotrexate according to the Seattle protocol. 5During aplasia, he experienced fever and Staphylococcus aureus bacteremia. On day +16 the patient developed acute GVHD grade II of the skin that resolved with corticosteroids (2 mg/kg/day). Two months after transplantation, he received pre-emptive gancyclovir therapy because of cytomegalovirus (CMV) reactivation. Five weeks later extensive GVHD of skin required treatment with steroids. Seven months after BMT, herpes encephalitis was diagnosed which resolved with acyclovir therapy. Another course of pre-emptive gancyclovir for CMV reactivation was also administered.One month later the patient presented with diarrhea, slight diffuse abdominal pain and flatulence. Parenteral nutrition and electrolyte and fluid infusions were started. Colonoscopy showed signs of diffuse mucosal irritation. On histological examination biopsy specimens from two regions of the colon showed individual crypt cell necrosis, crypt abscesses with cell flattening and degeneration consistent with GVHD gr...

Pneumatosis intestinalis following Cytotoxic or Immunosuppressive Treatment

“…In accordance with results obtained in previous studies [15, 16, 17], the clinical courses of our patients indicate that in the absence of secondary complications a conservative approach should be the treatment of choice for PI following antineoplastic chemotherapy. Since our patients suffered from severe immunosuppression, we chose treatment with empiric broad-spectrum antibiotics including metronidazole additionally to parenteral nutrition in all cases.…”

Medical management of pneumatosis intestinalis in patients undergoing allogeneic blood and marrow transplantation

“…30 Conservative management includes broad spectrum antibiotics, administration of oxygen at high concentrations, bowel rest, TPN followed by diet of low-flatulence producing carbohydrates when oral feeding is tolerated. 11,31 Galm et al reported five cases of PI following cytotoxic chemotherapy. Patients did not demonstrate signs of peritonitis and they were treated conservatively with metronidazole, piperacillin, tobramycin and TPN.…”

“…The diagnosis is established by endoscopy or radiography and is manifested by multiple thin-walled cysts that develop in the colonic submucosa or subserosa. 1 The secondary form accounts for the majority of cases and is associated with: celiac disease, 2 connective tissue disorders, 3 including systemic lupus erythematosus, 4 infectious enteritis, 5 chronic obstructive pulmonary disease, 6 primary immunodeficiency, 7 acquired immunodeficiency syndrome, 8 leukemia, 9 organ transplantation, 10 bone marrow transplantation 11 and in patients receiving glucocorticoids 12 and chemotherapy. 13 PI traditionally has been managed surgically with a high mortality rate (33-44%).…”

Is pneumatosis intestinalis a contraindication to allogeneic hemopoietic stem cell transplantation?

“…Thereafter, PI worsened after FEAM conditioning for the auto-HSCT. The decision to proceed to the second transplant with an unrelated donor was of particular concern due to the risk of a further worsening of the PI, especially in the possible case of an intestinal GVHD or of infectious complications [5][6][7]12,13]. However, considering the poor prognostic likelihood of our patient affected by DLBCL resistant to several lines of therapy, we decided to proceed with an allo-HSCT, sharing the decision with the patient and her relatives.…”

“…It has been associated with congenital disorders, collagen vascular diseases, acquired immunodeficiency, infection, endoscopic procedures, steroid use and chemotherapy [4][5][6][7][8]. PI is also found in association with organ transplantation and HSCT due to pre-transplant chemotherapy, infections and GVHD after HSCT [9][10][11][12][13]. It is hypothesized that when a pathogenic noxa causes a GI wall disruption necrosis, an increased wall permeability can occur, leading to gas infiltration.…”