Late-onset pneumatosis cystoides intestinalis associated with non-infectious pulmonary complications after allogeneic hematopoietic stem cell transplantation
Late-onset pneumatosis cystoides intestinalis associated with non-infectious pulmonary complications after allogeneic hematopoietic stem cell transplantation
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DOI：
10.1007/s12185-008-0110-7
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发表时间：
2008-07-01
影响因子：
2.1
通讯作者：
Kurokawa, Mineo
中科院分区：
文献类型：
--
作者：
Suzuki, Hiroshi I.;Izutsu, Koji;Kurokawa, Mineo
Pneumatosis cystoides intestinalis (PCI) is a rare complication of allogeneic hematopoietic stem cell transplantation (HSCT), characterized by multiple gas accumulation within the bowel wall. PCI usually occurs in association with acute graft-versus-host disease (GVHD) and immunosuppressive medication during the early posttransplantation period [1–3]. Herein, we report a rare case of very late-onset PCI associated with severe late-onset non-infectious pulmonary complications (LONIPCs) after unrelated bone marrow transplantation (BMT) for chronic myeloid leukemia (CML). The potential association between PCI and LONIPCs is discussed. A 20-year-old Japanese woman with Philadelphia chromosome-positive CML in the accelerated phase underwent BMT from an HLA 5/6-matched unrelated donor following conditioning with cyclophosphamide and fractionated total body irradiation (12 Gy). The patient received GVHD prophylaxis with cyclosporine A and a short course of methotrexate. She developed grade 2 acute GVHD of the skin that resolved with corticosteroid. Six months after BMT, she developed bronchiolitis obliterans organizing pneumonia (BOOP) of the bilateral lungs, which was confirmed by transbronchial biopsy. Although pulmonary infiltrates showed partial improvement with an increased dose of corticosteroid, she developed exertional breathlessness 4 months later. She was diagnosed as having bronchiolitis obliterans (BO) complicated with BOOP based on the results of computed tomography (CT) scan of the chest and pulmonary function test. BO was refractory to immunosuppressive therapy, and cough and dyspnea persisted. Three years after BMT, she suffered from pneumothorax of the right chest and underwent thoracoscopic pleurodesis.Three years and 6 months after BMT, upon taking 10 mg of prednisolone and 40 mg of cyclosporine A for chronic GVHD and LONIPCs, she experienced diarrhea, abdominal pain, and fever following a transient exacerbation of dyspnea. An abdominal X-ray and CT scan revealed intramural gas collection extending from the cecum through the transverse colon and free intraperitoneal gas (Fig. 1), which were consistent with the diagnosis of PCI. A chest CT scan revealed diffuse hypoattenuations, bronchiolectasis, multiple ground-glass opacities, and residual pneumothorax, but no significant change was seen compared with scans taken 2 months earlier. Cultures and serological studies detected no infectious pathogens. She was managed conservatively with the discontinuation of oral intake and administration of antibiotics. A CT scan obtained 9 days later revealed the marked improvement of intramural gas and disappearance of free intraperitoneal gas; no recurrence of PCI has been noted since then. According to the literature, the intervals between HSCT and the onset of subsequent PCI are usually less than 6 months [1–3], although a few exceptions have been reported (9–16 months after HSCT)[3–6]. The present case developed PCI 3 years after HSCT, which is the most delayed onset among those reported to date. The pathogenesis of this disease is still poorly understood, but various underlying conditions have been postulated, including intestinal obstruction, necrotizing enterocolitis in
