Pneumatoza pęcherzykowa jelit - jedno z powikłań choroby przeszczep przeciw gospodarzowi. Opis dwóch przypadków.

Abstract

SummaryBackground:Pneumatosis cystoides intestinalis (PCI) is a rare disorder characterized by the presence of multiple gas collections in the subserosal or submucosal intestinal wall of the large or small intestine. We report two cases of PCI in the course of chronic graft-versus-host disease.Case Report:A 5-year-old girl was treated for acute lymphoblastic leukemia. Twenty-four months after the hematopoietic stem cell transplantation, in the course of graft-versus-host disease, she developed subcutaneous emphysema of the right inguinal and pudendal region. PCI was diagnosed based on a CT examination. A 3-year-old boy was treated for juvenile myelomonocytic leukemia. Fourteen months after the hematopoietic stem cell transplantation he presented with an increased severity of intestinal symptoms, including intermittent bleeding from large intestine. PCI was diagnosed based on a CT exam and was confirmed by a colonoscopy.Conclusions:Pneumatosis cystoides interstitialis in the course of chronic graft-versus-host disease has a heterogeneous clinical presentation that does not correlate with results of imaging. CT is a method of choice to diagnose PCI. In patients with PCI, the presence of free air in the peritoneal cavity does not confirm an intestinal perforation.

Spontaneous free perforation of the small intestine in adults

“…After stem-cell transplantation, intestinal perforation has also been recorded with thrombotic microangiopathy [28] and post-transplant lymphoproliferative disorder [29] . Of note, after bone marrow or hematopoietic stem cell transplantation and subsequent development of GVHD, free air appearing within the peritoneal cavity, caused by pneumatosis cystoides intestinalis, has also been reported to mimic an intestinal perforation [30,31] .…”

Pneumatosis Intestinalis in a Patient with Acute Promyelocytic Leukemia

“…Furthermore, it has been proposed that long-term administration of corticosteroids in patients undergoing chemotherapy, postallogeneic stem cell transplant patients, or patients with chronic COPD may possibly induce erosion of the intestinal mucosa, leading to lymphoid depletion and mucosal disturbances causing penetration of gas into the submucosal layer [ 9 – 11 ]. In these patients, the depletion of Peyer's patches in the distal ileum and colon has also been hypothesized as a cause of benign PI [ 10 , 12 – 14 ].…”