TY  - JOUR
AU  - Al-Toma, Abdul
AU  - Koene, Harry R.
PY  - 2020
DA  - 2020/02/18
TI  - Hematopoietic Stem Cell Transplantation in Refractory Celiac Disease: An Overview with Focus on Infectious Complications
JO  - OBM Transplantation
SP  - 101
VL  - 04
IS  - 01
AB  - Refractory celiac disease (RCD) is a rare condition in which a known celiac patient, usually an adult, suffers from persistence or recurrence of gluten-related symptomatology, laboratory abnormalities, and inflammatory enteropathy despite following an optimal dietary therapy with gluten-free diet (GFD). Arbitrarily, a duration of at least 12 months of GFD has been recommended prior to establishing such a diagnosis. Furthermore, exclusion of the other possible causes of non-celiac villous atrophy, particularly enteropathy associated T-cell lymphoma (EATL), is a prerequisite for establishing a diagnosis of RCD. RCD is subdivided into two types, depending on the percentage of immunophenotypically aberrant intraepithelial lymphocytes (IEL). The refractory patients having a high percentage of abnormal ‘aberrant’ IEL (RCD-II) are regarded as having pre-lymphoma due to the high probability of developing EATL. In addition, they are at high risk for infection owing to the impaired immunity resulting from malnutrition, bacterial overgrowth and translocation in the small intestine, and the presence of hyposplenism (functional asplenia). The RCD-II patients are generally non-responsive to the currently available pharmacological treatments. However, both clinical and histopathological remissions have been achieved using the purine analog cladribine (2-CDA). Autologous hematopoietic stem cell transplantation (auto-HSCT) appears to be an effective therapy for these patients as it is well tolerated and has a low risk of post-transplant infections or other complications. The present review provides an overview of the application of auto-HSCT for the treatment of patients with RCD-II, which is a classic example of an autoimmune disorder. The focus is particularly on the infectious complications developing after the application of auto-HSCT.
SN  - 2577-5820
UR  - https://doi.org/10.21926/obm.transplant.2001101
DO  - 10.21926/obm.transplant.2001101
ID  - Al-Toma2020
ER  -