Are breath-holding spells always innocuous: A case report

The diverse clinical manifestations of Langerhans cell histiocytosis (LCH) present a dilemma to clinicians and pediatricians play an important role in its diagnosis and multidisciplinary approach. LCH, previously known as histiocytosis X, is an uncommon hematological disorder characterized by uncontrolled stimulation and proliferation of normal antigen-presenting cells, Langerhans cells. The purpose of this report is to describe the case of a 7-month-old female child with multisystem involvement who presented with breath-holding spells and to discuss the clinical, radiological, and histopathological features of LCH.

Progress in the diagnosis and treatment of ocular histiocytosis / 国际眼科杂志(Guoji Yanke Zazhi)

Ocular histiocytosis is a rare and heterogeneous group of disorders which can occur in children and adults. There is a great challenge in the diagnosis and treatment because of the atypical clinical and imaging manifestations. With insights into molecular mechanism of histiocytosis, the discovery of BRAFV600E mutations has changed the understanding of this disease and enabled targeted therapies in most patients. The gold standard of diagnosis has developed into histopathological biopsy combined with the testing for mutations. Surgery is not the only treatment for ocular histiocytosis and targeted therapy has become an effective treatment for patients with mutations in MAPK-ERK signal-regulated kinase pathway. However, the greatest challenge for ocular histiocytosis is establishing the early and correct diagnosis due to the diverse types and clinical manifestations. Therefore, this article reviews recent progress in diagnosing and treating ocular histiocytosis, summarizes their clinical and pathological features, and aims to improve the level of diagnosis and treatment among clinicians.

A Case of Senile Langerhans Cell Histiocytosis with Soft Tissue Mass as the Main Manifestation / 罕见病研究

Langerhans cell histiocytosis (LCH) is a rare disease characterized by the proliferation of Langerhans cells and the destruction of local tissue. LCH large occurs in children, whilst incidence of the elderly population is extremely low, and there are few related studies. LCH lesions can involve multiple organs and systems, including bone tissue, lymph nodes, skin, liver, and spleen. However, it is rare that multiple soft tissues are implicated for eldly patients with LCH and present with soft tissue mass as the main manifestation. Here is a report on the clinical features, treatment and prognosis of an elderly LCH with multiple soft tissue masses as the main manifestation, in order to provide clinical reference.

Clinical Characteristics and Diagnostic Experience of Adult Thyroid Langerhans Cell Histiocytosis with Diabetes Insipidus / 罕见病研究

  Objective  To analyze the clinical characteristics of thyroid LCH to enhance understanding of the disease.  Methods  We retrospectively studied the clinical data from six thyroid LCH patients who hospitalized in Huashan Hospital Affiliated to Fudan University from January 2015 to January 2022.We analyzed the ultrasound and 18F FDG-PET/CT imaging characteristics of thyroid LCH.  Results  The six patients diagnosed (2 males and 4 females) were between 18 and 58 years old.All patients had diabetes insipidus.MRI revealed thickened pituitary stalk.Two cases had central hypothyroidism, while four cases euthyroidism.Three cases tested positive for thyroid antibodies.Ultrasound showed thyroid nodules of TI-RADS 3 in three cases, TI-RADS 4 in two cases, and 1 with nodular goiter.Ultrasound showed that all sic cases indicated low echogenicity, 5 of which clear boundaries, 4 of which uneven echo distribution, 5 of which irregular shape, and noen has calcification.18F FDG-PET/CT indicated high uptake nodules with SUVmax values all above 10.4 cases were diagnosed by surgical excision and the other 2 by coarse-needle aspiration biopsy.When diagnosed, two cases had liver and thymus involvement respectively.One case had lung and bone involvement respectively.After treatment, 4 cases showed that nodular goiter shrank, while the other two with liver involvement progressed fast and no assessment made.  Conclusions  Thyroid LCH presented low echogenicity, clear boundaries, irregular shape, without calcification, and high uptake in 18F FDG-PET/CT.A definite diagnosis of pituitary stalk thicking accompanied by thyroid nodules, especially those with hypoechoic and irregular nodules, can be achieved by coarse-needle aspiration biopsy and langerin-specific pathological staining.

Langerhans Cell Histiocytosis in an Adult Female with Multisystem Involvement

Langerhans cell histiocytosis (LCH) also known as histiocytosis X, is a rare systemic disorder arising from the clonal proliferation of myeloid dendritic cells (histiocytes) with a tendency to involve single or multiple organ systems with variable clinical course and prognosis. Clinical presentation usually depends on the site of involvement. The organs commonly affected in adults by order of decreasing frequency include lungs, bone, skin, pituitary glands, lymph nodes, and the liver. Vulval and perianal involvement is extremely rare in adults. We describe the case of a 31-year-old non-smoker adult female with multisystemic LCH involving the vulva, perianal region, and lung. Probable involvement of other sites with LCH included mandibular bone, pituitary gland, skin, lymph nodes, liver, thyroid, and colon. She is undergoing systemic chemotherapy and has completed two cycles of cytarabine and steroids without any complications. Treatment is not standardized due to the very less incidence of the disease and inadequate knowledge regarding its pathophysiology. Langerhans cell histiocytosis remains a major concern for treating physicians because of its rarity with many faces and requires careful consideration for management.

Langerhans cell histiocytosis of thyroid: Case report with review of literature

Langerhans cell histiocytosis (LCH) is a rare monoclonal disease of antigen presenting cells. Involvement of thyroid gland by LCH is exceedingly rare. Herein, we present a case of LCH involving the thyroid in a 38-year-old woman. Our patient presented clinically as a case of primary thyroid neoplasm. Presence of elongated, epithelioid neoplastic cells with grooved nuclei along with presence of background eosinophils were seen on fine-needle aspiration cytology (FNAC) and histopathology. Positive staining for CD1a and S100 immunohistochemistry confirmed the diagnosis of LCH. Patient was given combination chemotherapy and has responded well to the same without any complaints for last 6 months. With this report, our goal is to expand awareness of this rare tumor in the thyroid. Consideration at the time of FNAC and its correct diagnosis on subsequent excision is imperative for patient management.thyroid

Research progress in neurodegenerative langerhans cell histiocytosis / 中华实用儿科临床杂志

Langerhans cell histiocytosis (LCH) is a rare myeloid tumor disease, which is characterized by CD1a + CD 207+ dendritic cell proliferation.The clinical manifestations of LCH vary greatly due to the different locations and different involved organs.Among them, neurodegenerative disease (ND) is one of the manifestations of central nervous system involvement in LCH.The pathogenesis of LCH-ND is unclear and it is mainly characterized by neurological disorders and progressive imaging changes.Due to its unclear etiologies and long progress of LCH-ND, the treatment of LCH-ND remains very challenging.Presently, the main modalities of treatment include intravenous immunoglobulin, chemotherapy and targeted therapy.Early treatment and timely intervention may be the key to halt the progression of LCH-ND, to stabilize the central nervous system function and to improve the quality of life.The pathogenesis, clinical manifestations, diagnosis, treatment and clinical evaluation of LCH-ND are briefly reviewed.

Progress in the Treatment of Adult Langerhans Cell Histiocytosis --Review / 中国实验血液学杂志

Langerhans cell histiocytosis (LCH) is a rare proliferative disease dominated by the proliferation of Langerhans cells, which is inflammatory myeloid neoplasms. Its clinical manifestations are variable, occurring at any age and at any site, and it is rarer in adults than in children. The gold standard for diagnosis is histopathological biopsy. Due to the rarity of adult LCH and the heterogeneity of this disease, treatment of adult LCH should be developed according to the extent of the disease and risk stratification. With the discovery of MAPK, PI3K and c-KIT signaling pathway activation, especially BRAF V600E and MAP2K1 mutations, targeted therapy has become a hot spot for therapeutic research. Meanwhile, the discovery of high expression of M2-polarized macrophages and Foxp3+ regulatory T cells (Treg) in LCH has provided an important basis for the immunotherapy. In this article, we will focus on reviewing the latest research progress in the treatment of adult LCH in recent years, and provide a reference for clinical research on the treatment of adult LCH patients.

Recurrent and Refractory Langerhans Cell Histiocytosis in Children Treated with the Combination of Cladribine and Cytarabine / 中国实验血液学杂志

OBJECTIVE@#To observe the efficacy and prognosis of cladribine (2-CdA) combined with cytarabine (Ara-C) regimen in the treatment of relapsed refractory Langerhans cell histiocytosis (LCH) in children.@*METHODS@#Nine patients with relapsed refractory LCH treated with the 2-CdA combined with Ara-C regimen in the Department of Hematology and Oncology of Wuhan Children's Hospital from July 2014 to February 2020 were retrospectively analyzed, and the efficacy and disease status were evaluated according to the Histiocyte Society Evaluation and Treatment Guidelines (2009) and the Disease Activity Score (DAS), the drug toxicity were evaluated according to the World Health Organization（WHO） grading criteria for chemotherapy. All patients were followed up for survival status and disease-related sequelae.@*RESULTS@#Before the treatment combining 2-CdA and Ara-C, 7 of 9 patients were evaluated as active disease worse (ADW), and 2 as active disease stable (ADS) with a median disease activity score of 8 (4-15). Of 9 patients, 6 cases achieved non active disease (NAD) and 3 achieved active disease better (ADB) with a median disease activity score of 0 (0 to 5) after 2-6 courses of therapy. All 9 patients experienced WHO grade IV hematologic toxicity and 3 patients had hepatobiliary adverse effects (WHO grade I～II) after treatment. The median follow-up time was 31(1 to 50) months with all 9 patients survived, 3 of the 9 patients experienced sequelae to the disease with 2 combined liver cirrhosis as well as cholestatic hepatitis and 1 with oral desmopressin acetate tablets for diabetes insipidus.@*CONCLUSION@#2-CdA combined with Ara-C is an effective regimen for the treatment of recurrent refractory LCH in children, and the main adverse effect is hematologic toxicity, which is mostly tolerated in children. Early treatment with this regimen may be considered for patients with multisystem LCH with risky organ involvement who have failed first-line therapy and for patients with relapse.

Clinical analysis for 15 patients with pulmonary Langerhans cell histiocytosis and literature review / 中南大学学报(医学版)

OBJECTIVES@#Pulmonary Langerhans cell histiocytosis (PLCH) is a clonal disease, characterized by proliferation of Langerhans cells that derived from bone marrow infiltrating the lungs and other organs. Due to the rarity of the disease, the current understanding of the disease is insufficient, often misdiagnosed or missed diagnosis. This study aims to raise clinicians' awareness for this disease via summarizing the clinical characteristics, imaging features, and treatment of PLCH.@*METHODS@#We retrospectively analyzed clinical and follow-up data of 15 hospitalized cases of PLCH from September 2012 to June 2021 in the Second Xiangya Hospital of Central South University.@*RESULTS@#The age of 15 patients (9 men and 6 women, with a sex ratio of 3 to 2) was 21-52 (median 33) years. Among them, 8 had a history of smoking and 5 suffered spontaneous pneumothorax during disease course. There were 3 patients with single system PLCH and 12 patients with multi-system PLCH, including 7 patients with pituitary involvement, 7 patients with lymph node involvement, 6 patients with bone involvement, 5 patients with liver involvement, 2 patients with skin involvement, 2 patients with thyroid involvement, and 1 patients with thymus involvement. The clinical manifestations were varied but non-specific. Respiratory symptoms mainly included dry cough, sputum expectoration, chest pain, etc. Constitutional symptoms included fever and weight loss. Patients with multi-system involvement experienced symptoms such as polyuria-polydipsia, bone pain, and skin rash. All patients were confirmed by pathology, including 6 by lung biopsy, 3 by bone biopsy, 2 by lymph node biopsy, and 4 by liver, skin, suprasternal fossa tumor, or pituitary stalk biopsy. The most common CT findings from this cohort of patients were nodules and/or cysts and nodular and cystic shadows were found in 7 patients. Three patients presented simple multiple cystic shadows, 3 patients presented multiple nodules, and 2 patients presented with single nodules and mass shadows. Pulmonary function tests were performed in 4 patients, ventilation dysfunction was showed in 2 patients at the first visit. Pulmonary diffusion function tests were performed in 4 patients and showed a decrease in 3 patients. Smoking cessation was recommended to PLCH patients with smoking history. Ten patients received chemotherapy while 2 patients received oral glucocorticoid therapy. Among the 11 patients with the long-term follow-up, 9 were in stable condition.@*CONCLUSIONS@#PLCH is a neoplastic disease closely related to smoking. The clinical manifestations and laboratory examination are not specific. Pneumothorax could be the first symptom which is very suggestive of the disease. Definitive diagnosis relies on histology. There is no unified treatment plan for PLCH, and individualized treatment should be carried out according to organ involvement. Early smoking cessation is essential. Chemotherapy is the main treatment for rapidly progressing PLCH involved multiple organs. All diagnosed patients can be considered for the detection of BRAFV600E gene and relevant targeted therapies have been implemented recently.

A Patient with Sequential Diseases of Langerhans Cell Sarcoma, Langerhans Cell Histiocytosis, and Acute Lymphoblastic Leukemia / 罕见病研究

Langerhans cell histiocytosis(LCH)and Langerhans cell sarcoma(LCS)are characterized by clone proliferation of Langerhans-type cells, which may occur concurrently or sequentially with T-cell acute lymphoblastic leukemia (T-ALL) and other Lymphoid neoplasms. A 15-year old female patient diagnosed with T-ALL developed LCH involving multiple systems during maintenance chemotherapy of T-AL. After treated with chemotherapy with improved result, the patient showed progression of the illness and refractory to the second-line treatment. We found c.G35A (p.G12D)mutation in the KRAS gene and used the targeted drug Trametinib for treatment. The treatment proved effective, leading to partial remission within a week. Three months after Trametinib treatment, the patient developed new lymphadenopathy. Biopsy revealed the existence of LCS. The disease progressed quickly, and the patient died 7 days after diagnosis of LCS. The case of patients with T-ALL then developing LCH and LCS sequentially is extraordinarily rare. The causes of the case is unclear and may be related to cell transdifferentiation, clonal evolution, and chemotherapy. Targeted drugs can contain this disease for a short time.

Traversing the spectrum of non-Langerhans Cell Histiocytosis: A case of Rosai-Dorfman Disease with features of Necrobiotic Xanthogranuloma

Introduction@#Non-Langerhans cell histiocytoses (non-LCH) are a group of rare diseases with varied clinical manifestations and overlapping features seen among the subtypes. Here, we present a case of Rosai-Dorfman disease with features of necrobiotic xanthogranuloma. @*Case@#A 45-year-old female presented with a 10-year history of an enlarging neck mass with normal overlying skin accompanied by dysphagia and multiple asymptomatic pink to yellowish-brown papules, nodules, and plaques on the face, trunk and extremities. Biopsies of a skin nodule and plaque revealed granulomatous dermal infiltrates (lymphocytes, foamy histiocytes, and Touton giant cells), emperipolesis and areas of necrosis. CD1A and Fite-Faraco staining showed negative results while CD68 and S100 positively stained the tissues of interest. Histopathology of the neck mass paralleled these findings in addition to being negative for lymphoid markers. Patient had monoclonal gammopathy and thyromegaly with enlarged cervical lymph nodes on further tests and imaging. Intralesional and systemic steroids were given which led to flattening of skin lesions and improvement in dysphagia, respectively. @*Conclusion@#Diagnosis and classification of a particular type of non-LCH may be difficult due to similarities across its subtypes. Hence, it is our belief that these diseases may occur on a spectrum. Treatment involves a multidisciplinary approach for the best possible care.

Concomitant Langerhans cell histiocytosis of cervical lymph nodes in adult patients with papillary thyroid carcinoma: A report of two cases and review of the literature

Objective : Langerhans cell histiocytosis (LCH) is an uncommon entity of unknown etiology. It contains a wide range of clinical presentations. The discovery of oncogenic BRAF V600E mutation in LCH has provided additional evidence that LCH is a neoplasm. Papillary thyroid carcinoma is the most common cancer of the thyroid characterized by a high incidence of BRAF V600E mutations. LCH with concomitant PTC is rare, with few cases reported in the literature. Cases summary We identified two cases of LCH with concomitant papillary thyroid carcinoma in adult patients. The first was a 49-year-old female with a thyroid nodule diagnosed with papillary thyroid carcinoma. Later, the patient had a left neck mass; Ultrasound-guided lymph node FNA was diagnosed with Langerhans histiocytosis. Subsequently, a chest CT scan revealed signs of Langerhans cell histiocytosis in the lung. The second case refers to a 69-year-old male who presented with a left thyroid nodule diagnosed on FNA cytology as papillary thyroid carcinoma. The patient was found to have multiple bone lytic lesions. Biopsies revealed Langerhans cell histiocytosis. Later, the patient experienced LCH involvement of the bone marrow with associated secondary myelofibrosis. Conclusions LCH is rare in adults; the association with papillary thyroid carcinoma is reported and should be considered in the presence of Langerhans cell groups along with PTC, whether in the thyroid gland or cervical lymph nodes. Once LCH has been diagnosed, pulmonary involvement should also be investigated. This will direct treatment plans for patients with pulmonary or systemic disease involvement.

Histiocitosis de células de Langerhans, una afección pulmonar infradiagnosticada en fumadores: reporte de caso y revisión de la literatura / Langerhans cell histiocytosis, an underdiagnosed lung condition in smokers: case report and literature review

La histiocitosis de células de Langerhans (HCL) es una enfermedad que puede afectar a pacientes de cualquier edad, siendo en adultos un trastorno poco común de etiología desconocida, que ocurre predominantemente en fumadores jóvenes, sin diferencias en género. Aunque ciertas particularidades de la enfermedad pueden compartirse con las manifestaciones presentes en la población pediátrica, la proporción de casos con afectación pulmonar es mucho mayor en adultos. A menudo evoluciona a través de brotes sucesivos y su gravedad varía desde formas benignas hasta potencialmente mortales. Algunos pacientes desarrollan un importante deterioro funcional con repercusión psicosocial, que impacta en la calidad de vida y se asocia a discapacidad prolongada. La clave diagnóstica estará determinada por el antecedente de tabaquismo, la presencia de nódulos, nódulos cavitados y quistes de paredes gruesas y delgadas en la tomografía computarizada de tórax de alta resolución (TACAR). Sin embargo, el diagnóstico definitivo requiere la identificación de granulomas de células de Langerhans, que generalmente se logra mediante la realización de una biopsia pulmonar y su estudio histopatológico e inmunohistoquímico. En la actualidad, podríamos considerar a esta entidad como una enfermedad huérfana, de la cual aún no se tiene claridad del mecanismo patogénico, y que, por ende, aún no dispone de estrategias terapéuticasespecíficas. El objetivo de esta revisión está centrado en la aproximación diagnóstica y terapéutica de la histiocitosis de células de Langerhans en adultos,que permita facilitar su reconocimiento en etapas tempranas y mejorar el pronóstico en las personas que la padecen

Langerhans cell histiocytosis (LCH) is a disease that can affect patients of any age, but in adults it is a rare disorder of unknown etiology that occurs predominantly in young smokers, without differences in gender. Although certain peculiarities of the disease can be the same than in the pediatric population, the proportion of cases with pulmonary involvement is much higher in adults. It often evolves through successive flare-ups and its severity ranges from benign tolife-threatening. Some patients develop significant functional impairment with psychosocial repercussions, that impact the quality of life and are associated with prolonged disability. The diagnostic key will be determined by the history of smoking, and the presence of nodules, cavitated nodules, and thick and thin-walled cysts on high-resolution chest computed tomography (HRCT). However, the definitive diagnosis requires the identification of Langerhans cell granulomas, which is generally achieved by performing a lung biopsy and its histopathological and immunohistochemical study. Today, we could consider this a rare entity, of which there is no clear pathogenic mechanism, and therefore, does not have yet specific therapeutic strategies. The purpose of this review is centered on the diagnostic and therapeutic approach of Langerhans cell histiocytosis in adults, which allows its recognition in early stages and improve the prognosis in people who suffer from it

Erdheim-Chester disease presenting at the central nervous system

Erdheim-Chester disease is a rare non-Langerhans cell histiocytosis (LCH) that affects different body systems. It was recently recognized as a neoplastic disorder after identifying an activating mutation of the MAPK pathway. Neurological presentations of ECD are rare. We present a case of a 35-year-old male who presented to the emergency department with neck pain, headache and vomiting for 2 months; MRI showed multiple heterogeneous intracranial masses. Neurosurgery performed a suboccipital craniotomy, partially resected the cerebellar mass, and placed a parietal to frontal shunt catheter. Biopsy results from the cerebellar mass demonstrated cerebellar tissue involved by a diffuse proliferation of foamy histiocytes and spindle cells admixed with prominent lymphoplasmacytic infiltrate and positive for CD68, CD163, Factor XIIIa and Fascin. PET scan showed hypermetabolic uptake within the medullary portions of the diffuse abnormal lesions of the distal femurs, tibias, and fibulas, and cardiac MRI was nonsignificant. The patient was started on vemurafenib and continued to show improvement in a 3-month outpatient follow-up.

A retrospective analysis on Langerhans cell histiocytosis and the association between BRAF V600E mutation status and clinical features in children / 中华实用儿科临床杂志

Objective:To investigate the clinical features of patients with Langerhans cell histiocytosis (LCH), and analyze the association between BRAF V600E mutation status and clinical features. Methods:A retrospective analysis was carried out for the clinical data of 60 patients with LCH at the Department of Pediatric Oncology, Sun Yat-sen Memorial Hospital between April 2013 and December 2019.Among them, 39 patients undertook BRAF V600E mutation testing, which in paraffin-embedded tissue samples were detected by quantitative real-time PCR (qRT-PCR), and in peripheral blood and/or bone marrow were tested by high-throughput sequencing, for analyzing the correlation between BRAF V600E mutation and clinical characteristics of LCH. Results:(1)Clinical characteristics: the age of 60 LCH patients was (4.08±0.45) years, with 43 male cases and 17 female cases.Patients at young age (≤2 years) and with risk organ (RO+ ) and central nervous system (CNS) risk lesions involvement were concentrated in the multisystem involvement (MS) group ( P<0.05). (2)Therapeutic response after induction therapy: the response to induction therapy was achieved in 28 of 60 treated patients (41.7%) and 32 (53.3%) did not.After excluding stratification factors of treatment regimen, MS ( OR=6.855, 95% CI: 2.077-22.622, P=0.002) and the age≤2 years ( OR=4.944; 95% CI: 1.601-15.275; P=0.005) were risk factors in poor chemotherapy response.RO+ ( OR=8.250, 95% CI: 1.617-42.090, P=0.005) was a significant risk factor for a poor chemotherapy response in JLSG-02 treatment group.Differently, RO+ had no dramatic effect on chemotherapy response in CCHG-LCH-2019 treatment group.(3) BRAF V600E mutation: 39 patients were determined BRAF V600E status, with the positive rate of BRAF V600E mutation in paraffin-embedded tissue samples reaching 70.3%(26 cases). BRAF V600E mutation was not associated with early treatment response, age, sex, MS and RO+ ( P>0.05). However, the positive rate of BRAF V600E in children with MS and CNS risk lesions was higher than the controls, with 76.0% (19 cases) vs.57.1% (8 cases) and 74.1% (20 cases) vs.58.3% (7 cases), respectively.Totally, 3 of 8 cases were positive in bone marrow, with 2 cases of MS, and 1 case of multiple bone invasions, and 1 of 5 cases was positive in peripheral blood, with liver and spleen being involved. Conclusions:LCH patients with age≤2 years, MS and RO+ exhibited a poor response to initial treatment, required for more aggressive treatment strategy.Lesion with activating BRAF V600E mutations suggests that LCH is a clonal disorder.There may be great variability between BRAF V600E mutations and MS as well as CNS risk lesions.In the mutation dataset, part of patients had positive BRAF V600E mutations in bone marrow/peripheral blood.This might suggest a different pathogenesis in such patients, has a certain clinical sense in some aspect.

Cutaneous Rosai-Dorfman disease in a 40-year-old female: A case report

Introduction@#Rosai-Dorfman disease is a rare disease that manifests with painless cervical lymphadenopathy, fever, anemia, an elevated erythrocyte sedimentation rate (ESR), and hypergammaglobulinemia. Extranodal lesions occur in 1/3 of patients, and the skin is involved in more than 10% of cases. Purely cutaneous disease is uncommon and only about more than 100 cases have been reported. Cutaneous Rosai-Dorfman Disease (CRDD) appears to be a distinct entity with different age and race predilection from cases with lymph node involvement.@*Case report@#This is a case of a 40-year-old Filipino female who presented with multiple erythematous papules and plaques with pustules on the cheeks. Skin punch biopsy showed a dense dermal infiltrate of polygonal histiocytes with abundant cytoplasm and vesicular nuclei. Emperipolesis was also present. The histiocytes were highlighted by the immunohistochemical stains S-100 and CD68 and was CD1a negative. Complete blood count and ESR were normal. Cervical lymphadenopathy was absent. Findings were consistent with Cutaneous Rosai-Dorfman disease. The patient was started on methotrexate at 15mg/week with folic acid supplementation. Mild soap, benzoyl peroxide 5% gel and tretinoin 0.05% cream once daily were maintained during the treatment course. There was significant decrease in erythema and size of existing lesions after 2 months. The patient was referred to a hematologist for monitoring of possible future systemic involvement.@*Conclusion@#Because of its rarity, clinicopathological correlation is always mandatory to establish a diagnosis of CRDD. Immu- nohistochemical stains are required to differentiate this entity form other forms of Langerhans cell histiocytosis. Multidisci- plinary referral is required to rule out concomitant systemic involvement.

Langerhans cell histiocytosis of liver

Langerhans cell histiocytosis (LCH) is a rare disorder of unknown etiology caused by proliferation of Langerhans cells. It can involve single organ system to multi organ systems and clinical presentation is variable depending on the organ involved and have different prognosis. LCH is common in children when compared to adults. Hepatic involvement in adults is relatively rare. Liver involvement has considerable impact on survival rates. Histopathology and immunohistochemistry provide the definitive diagnosis. Authors report a case of Langerhans cell histiocytosis in a young adult with hepatic involvement.

Extranodal rosai dorfman disease: a case report of single soft tissue cystic lesion

Rosai-Dorfman disease (RDD) is an uncommon benign histiocytic disorder. It is found worldwide and slightly more common among in men (1.4:1), affecting individuals are with an average age of 20.6 years. Extranodal tissue involvement is documented in 25-43% of RDD patients. Here authors are discussing a case of 16-year-old male patient with the complaint of a single swelling over left forearm since one month with no lymphadenopathy. Radiology showed anechoic collection within the muscle and superficial to it. On microscopy, smear showed diffusely distributed histiocytes with cytoplasm showing intact lymphocytes with halo surrounding it (emperipolesis), without atypia. Based on cytomorphology diagnosis of Extranodal Rosai Dorfman Disease was made.

Retrospective analysis of clinical manifestations and treatment outcomes of patients diagnosed with langerhans cell histiocytosis from a tertiary cancer hospital in South India

Background: Langerhans cell histiocytosis (LCH) comprises a diverse group of disorders where pathologic Langerhans cells accumulate in a variety of organs. Aims and objectives of the study is to analyse the clinical manifestations and treatment outcomes of patients diagnosed with LCH in a tertiary cancer hospital in South India.Methods: Retrospective analysis of the case records of patients presenting with histological proven case of LCH over a period of 7 years from 2011 to 2018, being treated at Vydehi Institute of Medical Sciences and Research Centre.Results: 10 patients with biopsy proven LCH were included. The median age of diagnosis was 8 years (range 1 to 73 years) and 3 patients aged 18 years or older at the time of diagnosis. The male: female ratio was 3:2. Multisystem involvement was found in 4 patients (40%) and Single system Involvement in remaining 6 patients. Isolated bone lesions were found in 4 patients (40%), 1 patient had isolated Lymph node involvement; 1 patient had oral cavity lesion. None of the 4 patients with multisystem diseases had skin/mucosal involvement; 3 had bony involvement, 2 patients had lung involvement. One patients with multisystem disease expired while 5 patients were lost to follow-up. 4 out of the 10 patients are on regular follow-up and are in remission.Conclusions: Despite limitation by the retrospective nature, this descriptive study was done to provide further disease information regarding Indian population. Data from this study clearly confirms the known fact that most of the patients with Single System LCH have a very good response rate. Patients with multisystem disease have the highest risk of disease related mortality and morbidity as one among the 4 patients with multisystem disease died just after initiating treatment.