Successful treatment of multiple relapsed–refractory Langerhans cell histiocytosis with cyclosporine and review of the literature

Langerhans cell histiocytosis is a rare hematologic disorder and patients who fail first-line treatment have a poor prognosis, and require more intensive treatment. We present an infant diagnosed with multisystem Langerhans cell histiocytosis refractory to multimodal therapy who was successfully treated with cyclosporine. Cyclosporine might be an effective alternative drug as nonmyelosuppressive rescue therapy for multiple relapsed-refractory Langerhans cell histiocytosis that has not achieved remission with cladribine and cytarabine therapy.

Download Full-text

Comparison of vindesine and prednisone and cyclophosphamide, etoposide, vindesine, and prednisone as first-line treatment for adult Langerhans cell histiocytosis: A single-center retrospective study

Leukemia Research ◽

10.1016/j.leukres.2016.01.012 ◽

2016 ◽

Vol 42 ◽

pp. 43-46 ◽

Cited By ~ 10

Author(s):

M.-H. Duan ◽

X. Han ◽

J. Li ◽

W. Zhang ◽

T.-N. Zhu ◽

...

Keyword(s):

Retrospective Study ◽

Langerhans Cell Histiocytosis ◽

Langerhans Cell ◽

Line Treatment ◽

Single Center ◽

First Line ◽

First Line Treatment

Download Full-text

Case Report: Early Association of Vemurafenib to Standard Chemotherapy in Multisystem Langerhans Cell Histiocytosis in a Newborn: Taking a Chance for a Better Outcome?

Frontiers in Oncology ◽

10.3389/fonc.2021.794498 ◽

2021 ◽

Vol 11 ◽

Author(s):

Stefania Gaspari ◽

Valentina Di Ruscio ◽

Francesca Stocchi ◽

Roberto Carta ◽

Marco Becilli ◽

...

Keyword(s):

Langerhans Cell Histiocytosis ◽

Laboratory Data ◽

Langerhans Cell ◽

Braf V600e ◽

Line Treatment ◽

First Line ◽

Standard Chemotherapy ◽

Multisystem Disease ◽

Response To Chemotherapy ◽

First Line Treatment

Langerhans cell histiocytosis (LCH) is due to aberrant monoclonal proliferation and accumulation of dendritic cells, ranging from a self-limiting local condition to a rapidly progressive multisystem disease with poor prognosis. Pathogenic cells originate from a myeloid-derived precursor characterized by an activation of the MAPK/ERK signaling pathway in about 70% of cases. In particular, BRAF V600E mutation is usually associated with a more severe clinical course and poor response to chemotherapy. We report on a newborn with multisystem LCH in life-threatening medical conditions. At diagnosis, the patient was successfully treated with the early association of BRAF inhibitor Vemurafenib to standard chemotherapy representing a new approach in first-line treatment. A rapid clinical improvement with a prompt fever regression from day 2 and complete resolution of skin lesions by week 2 were observed; laboratory data normalized as well. Vemurafenib was discontinued after 12 months of treatment. No signs of relapse occurred after 12 months of discontinuation. This case indicates that early combination of target therapy with standard treatment may induce rapid response and prolonged disease remission without significant toxicities in infants. This approach represents a valid and safe option as first-line treatment in multisystem disease, especially in high-risk patients.

Download Full-text

First-Line Treatment of Pediatric Langerhans Cell Histiocytosis

Histiocytic Disorders ◽

10.1007/978-3-319-59632-7_5 ◽

2017 ◽

pp. 103-118

Author(s):

Carlos Rodriguez-Galindo ◽

Cor van den Bos

Keyword(s):

Langerhans Cell Histiocytosis ◽

Langerhans Cell ◽

Line Treatment ◽

First Line ◽

First Line Treatment

Download Full-text

Delayed leukoencephalopathy from suspected polymer embolism after neuroendovascular procedures

The Neuroradiology Journal ◽

10.1177/19714009211029172 ◽

2021 ◽

pp. 197140092110291

Author(s):

Thomas Mellemkjær ◽

Ronil V Chandra ◽

Lasse Speiser ◽

Benedicte P Ulhøi ◽

Claus Z Simonsen

Keyword(s):

Polymer Material ◽

Endovascular Procedures ◽

Rare Complication ◽

Diagnostic Process ◽

Line Treatment ◽

Review Of The Literature ◽

First Line ◽

Stroke Treatment ◽

Oral Glucocorticoids ◽

First Line Treatment

As the neurointervention field grows, a new side effect emerges. Delayed leukoencephalopathy (DL) is believed to be an inflammatory or allergic reaction to polymer material that is shed from catheters during endovascular procedures. We present four cases of DL after aneurysm treatment in two patients, endovascular stroke treatment and diagnostic arteriography. We present our diagnostic process, including biopsy results in two patients, our anti-inflammatory treatment and outcomes together with a review of the literature. In our series, prognosis was variable with ongoing seizures in two patients. Our literature review reveals that asymptomatic shedding of polymer material is common, occurring in a third of endovascular stroke procedures, whereas symptomatic DL occurs in <0.5% of therapeutic neuroendovascular procedures. Clinicians should be aware of this rare complication, and oral glucocorticoids seem to be a reasonable first-line treatment strategy.

Download Full-text

Liver Transplantation for Langerhans Cell Histiocytosis: A U.S. Population‐Based Analysis and Systematic Review of the Literature

Liver Transplantation ◽

10.1002/lt.25995 ◽

2021 ◽

Author(s):

Ioannis A. Ziogas ◽

Christos D. Kakos ◽

W. Kelly Wu ◽

Martin I. Montenovo ◽

Lea K. Matsuoka ◽

...

Keyword(s):

Systematic Review ◽

Liver Transplantation ◽

Langerhans Cell Histiocytosis ◽

Population Based ◽

Langerhans Cell ◽

Review Of The Literature

Download Full-text

Unifocal Granuloma of Femur due to Langerhans' Cell Histiocytosis: A Case Report and Review of the Literature

Case Reports in Medicine ◽

10.1155/2010/686031 ◽

2010 ◽

Vol 2010 ◽

pp. 1-3 ◽

Cited By ~ 2

Author(s):

Harpreet Singh ◽

Satnam Kaur ◽

P. Yuvarajan ◽

Nishant Jain ◽

Lalit Maini

Keyword(s):

Case Report ◽

Clinical Data ◽

Case History ◽

Langerhans Cell Histiocytosis ◽

Eosinophilic Granuloma ◽

Pediatric Population ◽

Langerhans Cell ◽

Long Bones ◽

Review Of The Literature ◽

Osteolytic Lesions

The radiological diagnosis of osteolytic lesions of the long bones in pediatric population constitutes a challenge when the case history and clinical data are uncharacteristic. We believe that the description of few clinically and histologically proven cases to verify the existence of radiological signs useful for diagnosis may be of interest. Here, we describe a case of Langerhans' cell histiocytosis (LCH) presenting as unifocal eosinophilic granuloma of femur along with a brief review of the literature.

Download Full-text

Maintenance intervention to improve survival in patients with metastatic nasopharyngeal carcinoma who benefit from first-line treatment: A prospective multicenter randomized controlled clinical study.

Journal of Clinical Oncology ◽

10.1200/jco.2021.39.15_suppl.6035 ◽

2021 ◽

Vol 39 (15_suppl) ◽

pp. 6035-6035

Author(s):

Ying Lu ◽

Haixin Huang ◽

Hui Yang ◽

Xiaohua Hu ◽

Xianbing Feng ◽

...

Keyword(s):

Nasopharyngeal Carcinoma ◽

Maintenance Therapy ◽

Poor Prognosis ◽

Observation Group ◽

First Line ◽

Metastatic Nasopharyngeal Carcinoma ◽

Ebv Dna ◽

Maintenance Intervention ◽

First Line Treatment

6035 Background: The role of drug maintenance intervention in improving survival outcomes remains controversial.To investigate the safety and effect of Tegafur(S1) maintenance intervention in patients with metastatic nasopharyngeal carcinoma who benefit from the first-line treatment in a multicenter randomized controlled study, and to identify the related biological prognostic factors and guide the individualized treatment choice. Methods: Patients with metastatic nasopharyngeal carcinoma in the Fourth Affiliated Hospital of Guangxi Medical University and other cancer centers who met the inclusion criteria were randomly divided into maintenance therapy group: S1 maintenance therapy until disease progression or intolerance; Observation group: follow-up to disease progression. PFS, overall survival (OS) and adverse reactions of S1 maintenance therapy were compared between the two groups. The correlation between EBV-DNA, human serum amyloid A (SAA) and prognosis was evaluated. Results: Follow-up was conducted to May 2020, with a median follow-up of 19.8 months (6.1-51.3 months), 183 cases were evaluable (88 cases in S1 maintenance treatment group, 95 cases in observation group). Compared with the observation group, the S1 maintenance treatment group significantly increased patients' median PFS (16.2 months vs. 8.7 months, P < 0.001) and median OS (32.1 months vs. 18.2 months, P < 0.001). Reduced the risk of poor prognosis for PFS and OS (PFS: HR 0.305, 95%CI 0.211-0.441, < 0.001; OS: HR 0.363, 95%CI 0.238-0.553, P < 0.001). In the maintenance treatment group, the median S1 treatment lasted for 14 courses (4-58 courses), and the main adverse reactions were grade 1 skin pigmentation, oral mucositis, hand-foot syndrome, nausea, etc. No grade 4 toxic reaction occurred, and it was well tolerated. Compared with observation patients with negative EBV-DNA, observation patients with positive EBV-DNA had a higher risk of poor prognosis for PFS (HR 1.764, 95%CI 1.078-2.887, P = 0.024). The risk of poor prognosis in patients with positive EBV-NDA was significantly reduced by 61.1% ( < 0.001) for PFS and 65.5% (P = 0.001) for OS (P = 0.001). Compared with the observation group with stable SAA expression, S1 maintenance therapy significantly improved the prognosis of patients. Patients with continuous decline in SAA had a 61.9% lower risk of poor prognosis in PFS (P < 0.001) and a 60.2% lower risk of poor prognosis in OS (P = 0.007). Conclusions: For patients with metastatic nasopharyngeal carcinoma who benefit from first-line treatment, maintenance therapy of S1 can significantly improve the survival prognosis and is well tolerated. Patients with positive EBV-DNA and continuous decline in SAA may benefit more from maintenance intervention. Clinical trial information: ChiCTR-IOR-16007939.

Download Full-text