scholarly journals Langerhans Cell Histiocytosis in an Infant Mimicking a Lymphoma at Presentation

2015 ◽  
Vol 2015 ◽  
pp. 1-4 ◽  
Author(s):  
Anjan Madasu ◽  
Asim Noor Rana ◽  
Saleh Banat ◽  
Hani Humad ◽  
Rashid Mustafa ◽  
...  
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Mediastinal Mass ◽  
Clinical Presentation ◽  
Empirical Therapy ◽  
Lymphoblastic Lymphoma ◽  
Langerhans Cell ◽  
Severe Respiratory Distress ◽  
Appropriate Therapy ◽  
Generalized Lymphadenopathy ◽  
Superior Mediastinal Syndrome

Langerhans cell histiocytosis (LCH) is a rare disorder characterized by proliferation and accumulation of clonal dendritic cells with varied clinical presentation and an unpredictable course. We report a 5-month-old infant with LCH who presented with severe respiratory distress, a large mediastinal mass, significant generalized lymphadenopathy, and hepatosplenomegaly. Lymphoma, especially T cell lymphoblastic lymphoma, can present with superior mediastinal syndrome needing urgent empirical therapy without biopsy. However, lack of response prompted a biopsy which confirmed it to be a case of LCH and that leads to appropriate therapy and survival. There have been reports of LCH presenting with isolated mediastinal mass or with generalized lymphadenopathy, but the combined presentation of generalized lymphadenopathy with large mediastinal mass, hepatosplenomegaly, and fever in an infant has rarely been reported.Conclusion. LCH should also be considered in the differential diagnosis of an infant presenting with generalized lymphadenopathy, mediastinal mass, hepatosplenomegaly, and fever.

CD79a+ T-Cell Lymphoblastic Lymphoma With Coexisting Langerhans Cell Histiocytosis

2001 ◽  
Vol 125 (7) ◽  
pp. 958-960
Author(s):  
Shiyong Li ◽  
Michael J. Borowitz
Keyword(s):  
T Cell ◽  
Langerhans Cell Histiocytosis ◽  
Close Association ◽  
Cervical Lymphadenopathy ◽  
Lymphoblastic Lymphoma ◽  
Langerhans Cell ◽  
Terminal Deoxynucleotidyl Transferase ◽  
Simultaneous Occurrence ◽  
Cell Phenotype ◽  
Malignant Neoplasms

Abstract Although there is a close association between Langerhans cell histiocytosis and malignant neoplasms, simultaneous occurrence of lymphoblastic lymphoma and Langerhans cell histiocytosis in the same lymph node is an extremely rare finding. Herein, we describe such a case in a 26-year-old woman who presented with progressive cervical lymphadenopathy. The lymphoma cells have an immature T-cell phenotype (terminal deoxynucleotidyl transferase+, HLA-DR+, CD34+, CD38+, and CD7+) with expression of both CD3 and CD79a on immunohistochemical stain. The Langerhans cells are present focally with the characteristic morphologic features and immunophenotype (CD1a+ and S100+). The significance of CD79a coexpression in T-cell lymphoblastic lymphoma and the association between lymphoblastic lymphoma and Langerhans cell histiocytosis are discussed.

Mediastinal Mass and Paraparesis as the Sole Presenting Features of Langerhans Cell Histiocytosis

2010 ◽  
Vol 32 (4) ◽  
pp. 336-337
Author(s):  
Prasanth Ganesan ◽  
Seema Kaushal ◽  
Sanjay Thulkar ◽  
Sameer Bakhshi
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Mediastinal Mass ◽  
Langerhans Cell

scholarly journals Langerhans Cell Histiocytosis as Cause of Central Diabetes Insipidus: Case Report

2018 ◽  
Vol 37 (01) ◽  
pp. 76-79 ◽  
Author(s):  
Diana Nascimento ◽  
Manoel Teixeira ◽  
Eberval Figueiredo
Keyword(s):  
Diabetes Insipidus ◽  
Langerhans Cell Histiocytosis ◽  
Clinical Presentation ◽  
Bone Lesion ◽  
Langerhans Cell ◽  
Vital Functions ◽  
Hypothalamic Pituitary Axis ◽  
The Individual ◽  
Single Bone ◽  
Made In

AbstractLangerhans cell histiocytosis (LCH) is a rare disease of the monocyte-macrophage system, characterized by the aberrant proliferation of specific dendritic cells. The clinical presentation ranges from a single bone lesion to widespread multiorgan involvement. This disease is usually considered to be a disease of childhood; however, the diagnosis is frequently made in adulthood. The course of the disease is fairly unpredictable and varies from spontaneous resolution to progress into a debilitating form, which compromises the vital functions with occasional fatal consequences. Langerhans cell histiocytosis exhibits a predilection for the hypothalamic-pituitary-axis, with diabetes insipidus being the most common endocrine consequence related to the disease, which may be prior to diagnosis or develop at any time during the course of the disease. The diagnosis of LCH should be based on histologic and immunophenotypic examination of a lesional biopsy, although other testing may be done, depending on the symptoms. There is no established, widely agreed-upon treatment of LCH, in general. The treatment depends upon the individual patient and the extent and areas of involvement. The present article aims to describe the case of a 26-year-old male patient whose symptoms started with a headache and occipital bone lesion that progressed later with diabetes insipidus.

Acral, Pure Cutaneous, Self-Healing, Late-Onset, Cellulitis-Like Langerhans Cell Histiocytosis

2009 ◽  
Vol 13 (1) ◽  
pp. 43-47
Author(s):  
Prafulla Kumar Sharma ◽  
Sunil Sabhnani ◽  
Minakshi Bhardwaj ◽  
Hemanta Kumar Kar
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Clinical Presentation ◽  
Late Onset ◽  
Early Adulthood ◽  
Langerhans Cell ◽  
Self Healing ◽  
Diagnostic Dilemma

Background: An unusual and not yet reported clinical presentation of pure cutaneous Langerhans cell histiocytosis (LCH) that was a diagnostic dilemma. Objective: To bring to light the newer presentation of this disease. Method: The case was managed on clinical grounds initially as cellulitis. However, after the investigations and confirmation of the diagnosis, she was managed symptomatically. Results: Pure cutaneous LCH may manifest in early adulthood with features of cellulitis, erythematous edematous vesicular plaques, and nodules distributed in the flexural acral areas. Conclusion: Pure cutaneous LCH may have an acute late onset with unusual morphology as cellulitis, inverse acral distribution, and self-healing.

scholarly journals FNAC of Bacillus- Calmette- Guerin lymphadenitis masquerading as Langerhans cell histiocytosis

CytoJournal ◽  
2004 ◽  
Vol 1 ◽  
pp. 6 ◽  
Author(s):  
Nalini Gupta ◽  
Vijay Kumar ◽  
Raje Nijhawan ◽  
Radhika Srinivasan ◽  
Arvind Rajwanshi
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Index Case ◽  
Needle Aspiration ◽  
Langerhans Cell ◽  
Bacillus Calmette Guerin ◽  
Diagnostic Challenge ◽  
Aspiration Cytology ◽  
Needle Aspiration Cytology ◽  
Generalized Lymphadenopathy ◽  
Foamy Macrophages

Bacillus Calmette Guerin (BCG) lymphadenitis is a well known entity. Disseminated BCG infection usually presents as generalized lymphadenopathy, skin rash and hepatosplenomegaly and at times, can pose a diagnostic challenge to clinicians. There are only a few published studies on the cytological findings of BCG lymphadenitis. In this letter we report the fine needle aspiration cytology (FNAC) of BCG lymphadenitis clinically masquerading as Langerhans cell histiocytosis (LCH). FNA smears showed sheets of foamy macrophages and many polymorphs in a dirty necrotic background with many macrophages as well as polymorphs showing negatively stained rod like structures within their cytoplasm. Zeihl Neelson stain revealed that these cells were heavily loaded with acid fast bacilli (AFB). In the index case, AFB were also seen within the cytoplasm of polymorphs, which has not been documented earlier in the literature.

scholarly journals A Painful Shoulder Revealing Langerhans Cell Histiocytosis

2020 ◽  
pp. 24-27
Author(s):  
N. A. R. Ranaivo ◽  
M. L. Rakotomahefa Narison ◽  
M. Bemena ◽  
S. H. Raobijaona
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Clinical Presentation ◽  
Bone Lesion ◽  
Langerhans Cell ◽  
Irregular Boundary ◽  
X Ray ◽  
Multidisciplinary Consultation ◽  
Life Threatening ◽  
Spontaneous Improvement ◽  
The Right

Introduction: Langerhans cell histiocytosis is a systemic proliferative disease. It is a rare disease that can affects all tissues. Evolution can be spontaneously favorable. Multi-organ involvement may be life-threatening. We report the case of an toddler with bone lesion and issues. Case Report: It was a 33-month-old infant with right shoulder pain. Clinically, she had a swelling in the front side of the right shoulder with a limited abduction. X-ray of the right shoulder showed osteolysis with an irregular boundary of the right humeral head. The blood work was normal. In view of the painful swelling of the right shoulder, a biopsy was performed. Histological examination confirmed the diagnosis of Langerhans histiocytosis. A conservative treatment was decided after a multidisciplinary consultation meeting. After six months, spontaneous improvement was noted. Conclusion: Langerhans cell histiocytosis is a proliferative pathology that can affect one or more organs. The clinical presentation is polymorphic according to the affected organ. An extension assessment is fundamental in the management in order to determine the treatment.

scholarly journals Langerhans cell histiocytosis of liver

2020 ◽  
Vol 8 (8) ◽  
pp. 3079
Author(s):  
Swapna Samudrala ◽  
Anuradha Sekaran ◽  
Bharat Patodiya ◽  
Nageshwar Reddy Duvuru
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Clinical Presentation ◽  
Survival Rates ◽  
Langerhans Cell ◽  
Unknown Etiology ◽  
Hepatic Involvement ◽  
Organ Systems ◽  
Impact On Survival ◽  
Considerable Impact ◽  
Single Organ

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.

ENDOSCOPIC INTRAVENTRICULAR BIOPSY OF INFUNDIBULAR LANGERHANS CELL HISTIOCYTOSIS

Neurosurgery ◽  
2009 ◽  
Vol 65 (1) ◽  
pp. E214-E215 ◽  
Author(s):  
Brendan D. Killory ◽  
Francisco A. Ponce ◽  
Scott D. Wait ◽  
Stephen W. Coons ◽  
Harold L. Rekate
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Clinical Presentation ◽  
Third Ventricle ◽  
Langerhans Cell ◽  
Pathological Examination ◽  
Surgical Biopsy ◽  
Early Therapy ◽  
The Third ◽  
Small Risk ◽  
Extracranial Disease

Abstract OBJECTIVE Surgical biopsy of isolated infundibular lesions is indicated in patients who are symptomatic or whose imaging suggests a treatable lesion. Early therapy can prevent irreversible neurological or endocrinological damage and, potentially, metastasis. These considerations justify the small risk associated with surgery. CLINICAL PRESENTATION A 5-year-old boy presented with diabetes insipidus and an enhancing lesion of the pituitary stalk without evidence of extracranial disease. INTERVENTION The lesion was biopsied via an endoscopic intraventricular approach without complications. Pathological examination revealed Langerhans cell histiocytosis. CONCLUSION Endoscopic intraventricular biopsy can be considered as a less invasive alternative to craniotomy in patients with infundibular lesions protruding superiorly into the third ventricle.

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