Sirolimus for the Treatment of Airway Obstruction due to Indolent T-Lymphoblastic Proliferation

Abstract

Introduction. Indolent T-lymphoblastic proliferation (iT-LBP) is a rare nonmalignant entity that presents as a proliferation of T-lymphoblasts. We report a first such case with a recurrent laryngeal obstruction presentation that was successfully controlled with Sirolimus. Case presentation. This is the case of a 29-year-old female who presented with a recurrent significant lymphoid hyperplasia in the adenoid and tongue base region as well as a right cervical lymph node. After repeated adenoidectomies and tonsillectomies, and based on pathological and clinical findings she was diagnosed with iT-LBP. Trials of radiotherapy and immunotherapy with cyclosporine and rituximab all failed to control the progression of the disease. Sirolimus was finally able to restrict the growth and improve her symptoms. Conclusion. While It-LBP does not usually require treatment, it is important to report cases in which treatment was crucial for the survival of the patient, and the effective role of Sirolimus in doing so, without any major adverse effects.

Indolent T‐lymphoblastic proliferation: A systematic review of the literature analyzing the epidemiologic, clinical, and pathologic features of 45 cases

“…As of January 2022, 25 papers involving iT‐LBP were identified in the literature 1–4,6,9–28 in addition to one older review 5 . All papers were included in the study 1–4,6,9–28 .…”

“…Multiple local recurrences were typical for patients with upper aerodigestive involvement; two of these patients lacked any evidence of associated disease, 1,22 two had associated MG and DM type 1. 4,19 The manifestations of autoimmune phenomena was temporally related in at least one case, 19 where symptoms of MG recurred together with the iT-LBP. It could not be determined if similar type of associations occurred in the other cases.…”

“…5 All papers were included in the study. [1][2][3][4]6,[9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28] All identified cases were reviewed by two of the authors (AS and RSO) for the diagnostic criteria initially put forth by Ohgami et al in 2013 (Table 1). Since re-review of pathologic slides of the cases identified was not possible the described cases have been described to be present in various conditions and tissues, especially in relation to Castleman disease.…”

“…[6][7][8][9]28 Three papers had incomplete data. These publications included one case report from a workshop on thymic tumors 29 and two papers that lacked histologic images and microscopic descriptions precluding assessment of diagnostic criteria 3,4 ; the case presented in the latter paper 4 had a follow-up of 204 months, which we believe excludes other differential diagnostic entities. We did not exclude these cases which did not appear to grossly affect overall cohort data.…”

“…The terminology “indolent T‐lymphoblastic proliferation” (iT‐LBP) was first coined by Velankar et al in 1999, in retrospect of a male patient with a T‐lymphoblastic proliferation in the upper aerodigestive tract which remained stable over 16 years 1 . Since then, several studies analyzing reactive T‐lymphoblasts and T‐lymphoblastic proliferations in tumors and secondary lymphoid organs have been reported, and many more cases of iT‐LBP have been described 1–29 . In particular, iT‐LBP has been found in association with entities including Castleman disease (CD), follicular dendritic cell tumor/sarcoma (FDCT/S), and peripheral T‐cell lymphoma (PTCL).…”