scholarly journals A Case Report of Pediatric Langerhans Cell Histiocytosis: Current Approach and Diagnostic Challenges for Dermatologist

2020 ◽  
Vol 12 (3) ◽  
pp. 79-86
Author(s):  
Irwan Junawanto ◽  
Khairuddin Djawad ◽  
Sri Rimayani ◽  
Farida Tabri ◽  
Nurelly N. Waspodo ◽  
...  
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Poor Prognosis ◽  
Clinical Manifestations ◽  
Current Approach ◽  
Myeloproliferative Disorder ◽  
Langerhans Cell ◽  
Histopathologic Examination ◽  
Chemotherapy Treatment ◽  
Cutaneous Manifestations ◽  
Patient Organ

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.

scholarly journals Langerhans cell histiocytosis misdiagnosed as cow protein allergy: a case report

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Xintong Lv ◽  
Libo Wang ◽  
Zhuang Pi ◽  
Chunyan Zhang
Keyword(s):  
Early Diagnosis ◽  
Langerhans Cell Histiocytosis ◽  
Poor Prognosis ◽  
Milk Protein ◽  
Chronic Diarrhea ◽  
Clinical Manifestations ◽  
Langerhans Cell ◽  
Cow Milk ◽  
Case Presentation ◽  
Cow Milk Protein Allergy

Abstract Background Langerhans cell histiocytosis (LCH) is a heterogeneous disease with diverse clinical manifestations. Abdominal organ involvement is rare. Early diagnosis and active treatment are needed. The purpose of this article is to enable readers to have a better knowledge of LCH and prevent misdiagnosis. Case presentation A 2-month-old boy had diarrhea, hematochezia, and a rash, and was diagnosed as having a cow milk protein allergy (CMPA). He was given an amino acid-based formula, but there was no sign of improvement in his condition. The patient then had a skin biopsy and was diagnosed as having multisystem Langerhans cell histiocytosis (MS-LCH). The general condition of the child deteriorated after the first two doses of chemotherapy, and the child died. Conclusions MS-LCH is a protracted and progressive condition with poor prognosis. Early diagnosis and treatment are essential for survival. If a child has chronic diarrhea and hematochezia in the presence of a characteristic rash, the pediatrician should consider the possibility of this disease to avoid misdiagnosis.

scholarly journals Recurrent pulmonary infections as the first presentation of Letterer Siwe disease

2021 ◽  
Vol 2021 (7) ◽  
Author(s):  
Leen Jamel Doya ◽  
Ghazal Dib ◽  
Fouz Hassan
Keyword(s):  
Rare Disease ◽  
Langerhans Cell Histiocytosis ◽  
Poor Prognosis ◽  
Langerhans Cell ◽  
Diagnostic Workup ◽  
Unknown Etiology ◽  
Pulmonary Infections

ABSTRACT Langerhans cell histiocytosis (LCH) is a rare disease of unknown etiology that affects several organs. The fatal type of Langerhans cell histiocytosis is called Letterer Siwe disease (LSD) which is multisystem with a poor prognosis. Herein, we report a 20-month-old male who was admitted for recurrent pulmonary infections at the age of 10 months. Diagnostic workup revealed a Letterer-Siwe disease. The patient was treated with a good response.

scholarly journals Biological and clinical significance of somatic mutations in Langerhans cell histiocytosis and related histiocytic neoplastic disorders

Hematology ◽  
2015 ◽  
Vol 2015 (1) ◽  
pp. 559-564 ◽  
Author(s):  
Carl E. Allen ◽  
D. Williams Parsons
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Somatic Mutations ◽  
Mapk Pathway ◽  
Clinical Manifestations ◽  
Langerhans Cell ◽  
Braf V600e ◽  
Common Mechanism ◽  
Recurrent Mutations ◽  
Wide Range ◽  
Neoplastic Disorders

AbstractLangerhans cell histiocytosis (LCH), juvenile xanthogranuloma (JXG), and Erdheim–Chester disease (ECD) represent histiocytic disorders with a wide range of clinical manifestations. Until recently, mechanisms of pathogenesis have been speculative and debate has focused on classification of these conditions as reactive versus neoplastic. Genomic studies have been challenged by scarce tissue specimens, as well as heterogeneous nature of the lesions with variable infiltration of pathologic histiocytes. Whole-exome sequencing recently revealed a very low frequency of somatic mutations in LCH, JXG, and ECD compared to other neoplastic disorders. However, at least in the cases of LCH and ECD, there is a very high frequency of activating mutations in MAPK pathway genes, most notably BRAF-V600E, as well as MAP2K1, in LCH and NRAS in ECD. In ECD, recurrent mutations in the PI3K pathway gene PIK3CA have also been described. The heterogeneous clinical manifestations of these disorders may therefore be the cumulative result of activation of MAPK mutations (along with modifying signals from other pathways) at distinct stages of myeloid differentiation. Implications of this model include redefinition of LCH, JXG, and ECD as a group of clinically diverse myeloid neoplastic disorders with a common mechanism of pathogenesis. This model supports refocusing therapeutic strategies for these diseases on a personalized approach based on specific mutations and the cell(s) of origin.

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 ◽  
...  
Keyword(s):  
Testicular Cancer ◽  
Langerhans Cell Histiocytosis ◽  
Clinical Laboratory ◽  
Salvage Chemotherapy ◽  
Lung Lesion ◽  
Langerhans Cell ◽  
Chemotherapy Treatment ◽  
Thoracic Ct ◽  
Pure Seminoma ◽  
Bep Chemotherapy

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

scholarly journals MR imaging features of orbital Langerhans cell Histiocytosis

2019 ◽  
Vol 19 (1) ◽  
Author(s):  
Chunnan Wu ◽  
Kuncheng Li ◽  
Yan Hei ◽  
Pengyu Lan ◽  
Xuetao Mu
Keyword(s):  
Mr Imaging ◽  
Diagnostic Accuracy ◽  
Langerhans Cell Histiocytosis ◽  
Clinical Manifestations ◽  
Langerhans Cell ◽  
Imaging Features ◽  
Mri Findings ◽  
Palpable Mass ◽  
Hyperintense Signal ◽  
Mri Features

Abstract Background To investigate the magnetic resonance imaging (MRI) features of orbital Langerhans cell histiocytosis (LCH) to improve diagnostic accuracy. Methods We retrospectively reviewed clinical manifestations and MRI findings of 23 patients with histopathology-confirmed LCH of the orbit. The findings were evaluated for the following: (a) symptoms, (b) disease duration, (c) location, (d) configuration, (e) margin, (f) MR imaging signal intensity and enhanced performance. Results Eighteen patients (78%) in our series were male, only five (22%) patients were female, and the mean age at presentation was 6.3 years. The common symptoms include swollen eyelids, exophthalmos, and a palpable mass. Fourteen patients presented with swollen eyelids and/or exophthalmos. Twenty-two cases involved unilateral orbits, and one case involved bilateral orbits. In our study, there was one patient with cough and expectoration, and one patient with polydipsia and polyuria. Lesions were located in the superior or superlateral orbital roof of seventeen patients (74%). Lesions formed masses or irregular shapes. The 12 out of 23 (52.2%) cases appeared heterogeneous isointense and 10 out of 23 (43.5%) cases showed iso-hypointense on T1-weighted imaging, there were 15 out of 23 (65.2%) cases showed hyper-hypointense mixed signals on T2-weighted imaging. 7 cases found patchy hyperintense signal on T1WI, and 11 cases showed markedly hyperintense signal near the edge of lesions on T2WI. After enhancement, 21 out of 23 (91.3%) cases lesions presented marked enhancement at the edges and the surrounding tissues, and with heterogeneous obvious enhancement of the lesion center. Besides, four cases lesions were surrounded by a low circular signal. Conclusion There were several characteristics MRI features that can provide crucial information for clinicians and improve our understanding and the diagnostic accuracy of the orbital LCH.

Langerhans Cell Histiocytosis: A Clinicopathologic Review and Molecular Pathogenetic Update

2015 ◽  
Vol 139 (10) ◽  
pp. 1211-1214 ◽  
Author(s):  
Charles M. Harmon ◽  
Noah Brown
Keyword(s):  
Risk Stratification ◽  
Langerhans Cell Histiocytosis ◽  
Wide Spectrum ◽  
Clinical Manifestations ◽  
Langerhans Cell ◽  
Molecular Pathogenesis ◽  
Molecular Characteristics ◽  
Reactive Process ◽  
Pathologic Features ◽  
Clinical Disorders

Langerhans cell histiocytosis (LCH) comprises a wide spectrum of clinical disorders that have in common a proliferation of Langerhans-type cells with characteristic morphologic, immunophenotypic, and ultrastructural features. In part because of the diverse clinical manifestations of LCH, there has long been controversy over whether LCH is best considered a reactive process or a neoplasm. Herein, we discuss the clinical and pathologic features of LCH, including recent advances in the understanding of the molecular pathogenesis of this disease that support its categorization as a neoplasm. We also review the implications that these recently described molecular characteristics may have on risk stratification and treatment of LCH.

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
Keyword(s):  
Case Report ◽  
Successful Treatment ◽  
Langerhans Cell Histiocytosis ◽  
Poor Prognosis ◽  
Lung Nodule ◽  
Bone Destruction ◽  
Juvenile Xanthogranuloma ◽  
Chemotherapy Treatment ◽  
Cervical Mass ◽  
Systemic Form

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.

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