scholarly journals Neonatal Systemic Juvenile Xanthogranuloma with an Ominous Presentation and Successful Treatment

2011 ◽  
Vol 5 ◽  
pp. CMO.S6686 ◽  
Author(s):  
Rong Fan ◽  
Jihong Sun

This case report originated from a case of neonatal multisystemic juvenile xanthogranuloma (JXG). The patient presented with blue muffin rush, cervical mass, bone destruction, lung nodule, hepatosplenomegaly, and coagulopathy and was successfully treated with Langerhans cell histiocytosis (LCH) based chemotherapy treatment. Similar cases in literature were reviewed and it seems that JXG, a relatively benign entity, when presented in its systemic form with liver involvement, could have an aggressive course and portend quite poor prognosis. Challenges and special consideration of the diagnosis, treatment, and future case observation are discussed.

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.


2017 ◽  
Vol 21 (3) ◽  
pp. 267-269 ◽  
Author(s):  
Catherine Vignault ◽  
Émilie Bourgeault ◽  
Éric Gagné ◽  
Janie Bujold

Juvenile xanthogranuloma (JXG) is the most frequent form of non–Langerhans cell histiocytosis. We present a case of giant congenital JXG in a 7-week-old boy, who had a firm and incompressible lesion, measuring 3 × 4 cm in diameter, on his right flank. The clinical appearance of the lesion and the ultrasound results suggested a vascular tumor, such as a hemangioma. Histology confirmed a JXG, although there was an absence of Touton cells, which are usually pathognomonic of JXG. In light of these findings, it would be important to include JXG in the differential diagnosis of congenital tumours, particularly vascular lesions.


2004 ◽  
Vol 26 (6) ◽  
pp. 371-374 ◽  
Author(s):  
Takuya Nakatani ◽  
Akira Morimoto ◽  
Ryuichi Kato ◽  
Sachiko Tokuda ◽  
Tohru Sugimoto ◽  
...  

2017 ◽  
Vol 34 (4) ◽  
pp. 765-770 ◽  
Author(s):  
Tae-Kyu Lee ◽  
Tae-Young Jung ◽  
Hee-Jo Baek ◽  
Seul-Kee Kim ◽  
Kyung-Hwa Lee ◽  
...  

2014 ◽  
Vol 68 (2) ◽  
pp. 104-106
Author(s):  
Aleksandra Pivkova Veljanovska ◽  
Sonja Genadieva Stavrik ◽  
Zlate Stojanoski ◽  
Lazar Cadievski ◽  
Adela Stefanija ◽  
...  

Abstract The article presents a case with diagnosed Hodgkin disease (HD) during pregnancy. The aim of this case study was to present diagnostic possibilities in determining HD stage during pregnancy and therapeutic dilemmas. The incidence of HD during pregnancy is 3.2% of all cases with this malignant hematological disorder. The treatment of this disease during pregnancy depends on disease-related factors, pregnancy-related factors, as well as possible implications for fetal morbidity and mortality. The need of analysis of the nature of the disease during pregnancy indicates examination of a larger series of pregnant women with HD and the drawn conclusions affect the decision whether chemotherapy treatment should start immediately or it should be postponed for after delivery.


2016 ◽  
Vol 0 (1-2.30-31) ◽  
pp. 78
Author(s):  
V.I. Desiateryk ◽  
O.V. Kotov ◽  
O.V. Brovko ◽  
K.P. Alimov ◽  
O.A. Hul

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