Transplantation-associated thrombotic microangiopathy after steroid pulse therapy for polyserositis related to graft-versus-host disease

Abstract

Transplantation-associated thrombotic microangiopathy (TA-TMA) is a rare but devastating syndrome that occurs in allogeneic hematopoietic stem cell transplant recipients, and is associated with a variety of transplantation-related factors, including conditioning regimens, immunosuppressive agents, graft-versus-host disease (GVHD) and opportunistic infections. TA-TMA has an unfavorable prognosis and responds poorly to conventional treatment including plasma exchange (PE). We present a case of a 37-year-old man with membranous nephropathy (MN) and polyserositis caused by GVHD after hematopoietic stem cell transplantation. He developed TA-TMA after steroid pulse therapy for polyserositis. We treated the patient with PE and mycophenolate mofetil (MMF) after which the TA-TMA successfully improved and the MN underwent complete remission. The present case suggests that corticosteroids with severe GVHD might increase the risk of TA-TMA, and that PE in combination with MMF may be a valuable therapy to improve the prognosis.

Transplant-associated thrombotic microangiopathy: theoretical considerations and a practical approach to an unrefined diagnosis

“…Traditionally, significant cardiac and pulmonary vascular endothelial toxicities after HSCT were attributed to chronic GVHD or chemotherapy and radiation effects [66,67]. However, polyserositis has been described in TA-TMA, presenting as refractory pericardial effusion, pleural effusion, and ascites without overall generalized edema [49].…”

A new paradigm: Diagnosis and management of HSCT-associated thrombotic microangiopathy as multi-system endothelial injury

“…It often presents as refractory pericardial effusion, pleural effusion, and ascites without overall generalized edema even in patients with nephrotic range protein-uria. Polyserositis after allogeneic HSCT is often thought to be a presentation of chronic GVHD [15,49]. TA-TMA should be included in the differential diagnosis, especially in patients who do not exhibit other symptoms of GVHD, like chronic skin or mucosal changes and in patients with polyserositis after autologous HSCT.…”

Polyserositis as a Manifestation of Graft-Versus-Host Disease

“…Ishikawa et al [2] reported a case of polyserositis as an isolated finding of cGVHD 21 months after bone marrow transplant (BMT) and 4 months after developing skin GVHD. In our cases, the effusions occurred before day +100, without acute GVHD.…”