Langerhans cell histiocytosis

Windebank, Kevin; Nanduri, Vasanta

doi:10.1136/adc.2007.125872

Cited by 77 publications

(88 citation statements)

References 56 publications

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“…Unifocal LCH can be monostotic or polyostotic. Multifocal unisystem variant is associated with fever, bone lesions, diffuse eruptions commonly on the scalp, ear canal and also causes involvement of pituitary stalk causing diabetes insipidus; as is also observed by the authors of previous studies [6]. Multifocal multisystem variant or letterer-siwe disease includes the involvement of bone, endocrine, ocular, central nervous system, spleen, lung and gastrointestinal systems; the last two organ systems were involved in our case scenario.…”

Section: Discussionsupporting

confidence: 51%

“…Multifocal multisystem variant or letterer-siwe disease includes the involvement of bone, endocrine, ocular, central nervous system, spleen, lung and gastrointestinal systems; the last two organ systems were involved in our case scenario. However, more than two-thirds of cases have single system involvement with bone and skin as the most commonly involved sites [5][6][7].…”

Section: Discussionmentioning

confidence: 99%

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Disseminated Langerhans Cell Histiocytosis Presenting as Cholestatic Jaundice

Kapoor

Loizides

Sachdeva

et al. 2015

JCDR

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A 2-year-old boy presented with progressive abdominal distention, right ear discharge and seborrhoeic dermatitis since six months of age. Prior to presentation, he also had a one month history of fever, jaundice and rash on his scalp for an uncertain duration. He was appropriate for age; all the anthropometric measurements were within the 50 th to 75 th centile according to the WHO standards. On admission to the hospital, the patient was febrile, icteric and deeply jaundiced. His urine was positive for bile and stool was acholic. Numerous infiltrative skin lesions were observed on his scalp and back.The lesions were raised, mobile, not attached to underlying structures, and non-tender. Ear examination revealed a dull tympanic membrane and non-foul smelling discharge with a central perforation.On abdominal examination, prominent veins were noted on the surface. Palpation of abdomen revealed smooth, non-tender, nonpulsatile hepatomegaly which was palpated 3cm below the left costal margin. Firm splenomegaly was noted. Shifting dullness was present. No lymphadenopathy was appreciated on examination of the whole patient.

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Section: Discussionsupporting

confidence: 51%

Section: Discussionmentioning

confidence: 99%

Disseminated Langerhans Cell Histiocytosis Presenting as Cholestatic Jaundice

Kapoor

Loizides

Sachdeva

et al. 2015

JCDR

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“…The diagnosis of LCH is confirmed by the characteristic histologic picture and the presence of CD1a or CD207 positive histiocytic cells. 1 Electron microscopy shows the presence of Birbeck granules in the cells but is rarely used currently to diagnose the disease.…”

Section: Discussionmentioning

confidence: 99%

Recurrent and rapidly occurring pericardial tamponade in Erdheim Chester disease

George

Subhendu

2012

Indian Heart Journal

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A B S T R A C TErdheim Chester disease is a very rare histiocytic disorder characterised by tissue infiltration by lipid laden histiocytes. The most common presentation is bone pains typically involving the long bones. Over time almost 50% of the patients develop extraosseous involvement. The prognosis depends on the extent and distribution of the extraskeletal manifestations. Cardiovascular involvement is seen in up to 40% of the patients and the most common manifestations are periaortic fibrosis and pericardial involvement. Respiratory distress, extensive pulmonary fibrosis, and cardiac failure are the most common causes of death in these patients. Cardiac tamponade has also been documented to cause death in these patients. We describe a patient of Erdheim Chester disease who presented with recurrent and very rapidly occurring cardiac tamponade in a short duration of time and benefited from timely recognition and management.

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“…Treatment of multisystem LCH normally benefits from systemic therapy, which usually reduces morbidity and mortality (3,9). Our patient received an initial 6-week course of therapy (prednisone 40 mg/m 2 /day orally, weekly reduction after week 4, vinblastine 6 mg/m 2 i.v.…”

Section: Discussionmentioning

confidence: 99%

Multisystem Langerhans cell histiocytosis with liver dysfunction as the first presentation: A case report

Liu

Zhang

2011

Oncology Letters

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Abstract. Langerhans cell histiocytosis (LCH) is a rare disease of unknown etiology characterized by oligoclonal proliferation of Langerhans cells. The diagnosis of LCH is complicated by the fact that it may involve multiple organ systems and its clinical presentation and course varies, ranging from an isolated to a multisystem disease. We report a 35-year-old male with LCH involving multiple systems, including the bones, lungs, spleen, liver and bile ducts, whose first clinical presentation was liver dysfunction. The patient was diagnosed following a skull biopsy that revealed infiltration of Langerhans cells. However, a liver biopsy revealed sclerosing cholangitis (SC) with no signs of Langerhans cell infiltration, and the clinical manifestations of the involved organs were atypical, leading to a delayed diagnosis. The patient was in partial remission following chemotherapy. In conclusion, findings of this case may aid our understanding of the pathophysiology of LCH and in improving its diagnosis and treatment. IntroductionLangerhans cell histiocytosis (LCH) is characterized by the proliferation of abnormal dendritic antigen-presenting histiocytes. LCH may involve almost any organ. The clinical presentation of LCH therefore varies and is frequently misdiagnosed (1-3). We report a patient who was initially admitted to the liver department with sclerosing cholangitis (SC), but was eventually diagnosed with multisystem LCH. Case reportA 35-year-old Chinese male was admitted to our hospital with a 2-year history of jaundice and pruritus. The patient's laboratory test results are shown in Table I. Magnetic resonance cholangiopancreatography (MRCP) and the pathology of a liver biopsy supported the diagnosis of SC by a local hospital. The patient received treatment with ursodeoxycholic acid (UDCA) at a dose of 750 mg/day, with no marked response. The patient had no history of smoking, alcohol consumption or blood transfusions, and no family history of similar symptoms. Physical examinations revealed mildly icteric sclera and skin, coarse breath sounds in the lungs and enlargement of the spleen accompanied by percussion pain. A 1.0x1.0-cm defect was detected in the patient's occipital bone, which had occasionally caused pain over the previous 8 months.The laboratory examination showed a normal complete blood count, positive urine bilirubin and urobilinogen, a normal stool test and an erythrocyte sedimentation rate (ESR) of 26 mm/h. Liver function test results are shown in Table I. Viral hepatitis markers were negative. Immunoglobulin (IgG) was 1,920.0 mg/dl. Antinuclear antibody (1:80) (normal <1:80) was positive. Anti-smooth muscle, anti-liver/kidney microsome, anti-soluble liver antigen and liver cytosol antigen type I antibody were all negative. Anti-mitochondrial antibody subtype-2, extractable nuclear antigen and double-stranded DNA (ds-DNA) were negative. α-fetoprotein, carcinoembryonic antigen and CA19-9 concentrations were also normal. Body mass index was normal.An X-ray examination showed multifocal osteolyti...

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Langerhans cell histiocytosis

Cited by 77 publications

References 56 publications

Disseminated Langerhans Cell Histiocytosis Presenting as Cholestatic Jaundice

Disseminated Langerhans Cell Histiocytosis Presenting as Cholestatic Jaundice

Recurrent and rapidly occurring pericardial tamponade in Erdheim Chester disease

Multisystem Langerhans cell histiocytosis with liver dysfunction as the first presentation: A case report

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