scholarly journals Langerhans cell histiocytosis following treatment for testicular cancer. A case report and literature review

2015 ◽  
Vol 6 (1) ◽  
pp. 6-9
Author(s):  
Pistamaltzian Nikolaos ◽  
Nikolaidi Adamantia ◽  
Raftogiannis Maria ◽  
Economou Aggelos ◽  
Mourtzoukos Spryridon ◽  
...  

Abstract A 35 year old male patient received treatment for testicular cancer of pure seminoma histology. He underwent initially a right inguinal orchiectomy and afterwards he received 3 cycles of BEP chemotherapy, as his imaging studies showed enlarged para-aortic lymph nodes. Five months after completion of chemotherapy treatment, a thoracic CT revealed multiple micronodular lesions in both lungs. The patient was advised about the need of salvage chemotherapy, but he opted to undergo further investigation. A lung lesion biopsy was performed, and histology was compatible with diagnosis of Langerhans cell histiocytosis (LCH). LCH is quite an unusual clinical entity that affects usually -but not solely- the lungs of young adults. The hallmark of the disease, is the presence of the activated Langerhans cells. As its clinical, laboratory and radiological findings are nonspecific, a transbronchial biopsy is sometimes necessary to establish diagnosis. Treatment is generally symptomatic and reserved for patients with multisystem organ involvement. LCH’s relationship with cancer is complex and no certain correlation is evident. More troublesome can be its differential diagnosis from a cancer with metastatic spread to the lungs or other affected organs, and although rare, it has to be kept in mind when suspicious signs arise

2020 ◽  
Vol 12 (3) ◽  
pp. 79-86
Author(s):  
Irwan Junawanto ◽  
Khairuddin Djawad ◽  
Sri Rimayani ◽  
Farida Tabri ◽  
Nurelly N. Waspodo ◽  
...  

Abstract Langerhans Cell Histiocytosis (LCH) is a chronic and rare myeloproliferative disorder caused by disorders in Lang-erhans cell proliferation in various organs and tissues. LCH has a wide variety of clinical manifestations, making it difficult to diagnose. Cutaneous manifestations are polymorphic in the form of purpura, papule, vesicles and pustules. LCH can involve vital organs such as the liver and lungs as well as the hematopoiesis system that usually gives a poor prognosis. The prognosis is also influenced by the age of patient, organ dysfunction and response to the first 6 weeks of chemotherapy treatment. A 3-year-old girl reported a major complaint of an abscess-like lesion in the region of neck accompanied by an extensive purpura of scalp, neck and inguinal areas accompanied by vulvar erosions. The immunohistochemical and histopathologic examination support LCH and the clinical improvement after intravenous administration of intravenous 3 mg/m2 Vinblastine chemotherapy, 75 mg/m2 etoposide, oral 40 mg/m2 per prednisone. After the 6th cycle of chemotherapy, the patient died.


Author(s):  
Sebastian K Eder ◽  
Raphaela Schwentner ◽  
Philipp Ben Soussia ◽  
Giulo Abagnale ◽  
Andishe Attarbaschi ◽  
...  

Langerhans cell histiocytosis (LCH) is a neoplasm marked by the accumulation of CD1A+CD207+ cells. It is most commonly driven by a somatic, activating mutation in the BRAF serine-threonine kinase (BRAFV600E). Multisystem disease with risk-organ involvement requires myelotoxic chemotherapy, making BRAF-inhibitors an attractive treatment option. Here, we present a comprehensive analysis of the course of an LCH patient treated with the combination of vemurafenib and salvage chemotherapy who achieved sustained clinical and molecular remission. We show that there is no relationship between peripheral bloodBRAFV600E levels and clinical presentation during treatment with vemurafenib, but that vemurafenib leads to a fast, efficient, but reversible inhibition of clinical manifestations of systemic inflammation. In line, serum levels of inflammatory cytokines exactly mirror vemurafenib administration. Genotyping analysis identified the BRAFV600E mutation in multiple hematopoietic cell types, including NK cells and granulocytes. Single-cell transcriptome analyses of peripheral blood and bone marrow cells at time of diagnosis and during treatment indicate that RAF-inhibition abrogates the expression of inflammatory cytokines previously implicated in LCH such as IL1B and CXCL8. Together, our data suggest that while the CD1A+CD207+histiocytes are the hallmark of LCH, other BRAF-mutated cell populations may contribute significantly to morbidity in patients with multisystem LCH.


1998 ◽  
Vol 138 (5) ◽  
pp. 909-910 ◽  
Author(s):  
Marzano ◽  
Gasparini ◽  
Grammatica ◽  
De Juli ◽  
Caputo

2000 ◽  
Vol 111 (1) ◽  
pp. 258-262 ◽  
Author(s):  
Susi Scappaticci ◽  
Cesare Danesino ◽  
Elena Rossi ◽  
Catherine Klersy ◽  
Gian Mario Fiori ◽  
...  

1993 ◽  
Vol 29 (5) ◽  
pp. 973
Author(s):  
Jong Sung Kim ◽  
Duk Ja Bang ◽  
Hyun Chul Rhim ◽  
Seok Chol Jeon ◽  
Seung Ro Lee ◽  
...  

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