Multinucleated giant cells in Langerhans cell histiocytosis

Abstract Pediatric Langerhans cell histiocytosis (LCH) clinical outcome in Hospital Civil de Guadalajara, México. Introduction : LCH results from clonal proliferation of functionally and immunophenotyped inmature round Langerhans cells along with eosinophiles, machrophages, lymphocytes and ocasionally multinucleated giant cells (1). Its incidence is 2-10 cases by million of children below 15 yr in US (2). Our objective was to describe the clinical characteristics and treatment outcome of patients with LCH at Departement of Hematology-Oncology of Hospital Civil de Guadalajara México. Methods: It was a retrospective design and 41 pediatric patients below 18 yr were included. The diagnosis was corroborated by pathology and immunohistochemistry. Variables as age, gender, localised vs systemic disease, risk organ commitment, global survival (GS) and event free survival (EFS) were analysed. We used descriptive and inferencial statistics with SPSS program. Results: There were included 41 patients from January 1st 2012 to December 31st 2017. Relation male:female was 1.1:1. Mean presentation was localised disease (58%). Bone was the principal affected structure (34%) and it was 71% to be combined with lung, lymph node and CNS compromise. Risk organ commitment was presented in 32%, being more frecquent bone marrow and liver in 22% each one. Time induction treatment was equal or below 12 weeks in 66% of patients. The 25% of patients had reactivation of LCH, with similar lesions to the beggining in 19.5%. We found statistically significant differences between dead patients (DP) (14.6%) and not dead patients (NDP) (85.4%) in clinical presentation: localised (0% in DP vs 69% in NDP) and systemic disease (100% vs 31%) (p=0,003) and risk organ commitment (100% in DP vs 20% in NDP) (p=0,000). Median age of 13 vs 24 months was for DP and NDP respectively. Conclusion: Dead patients were younger than 13 months old, with systemic disease, and risk organ commitment. We found a later asking of medical advice in DP (6 months) vs NDP (2 months). Keys words: langerhans cell histiocytosis, multisystem disease, risk organ Figure. Figure. Disclosures No relevant conflicts of interest to declare.

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Presence of osteoclast-like multinucleated giant cells in the bone and nonostotic lesions of Langerhans cell histiocytosis

Journal of Experimental Medicine ◽

10.1084/jem.20041785 ◽

2005 ◽

Vol 201 (5) ◽

pp. 687-693 ◽

Cited By ~ 75

Author(s):

Cristiana E.T. da Costa ◽

Nicola E. Annels ◽

Claudia M.J.M. Faaij ◽

Ramses G. Forsyth ◽

Pancras C.W. Hogendoorn ◽

...

Keyword(s):

Langerhans Cell Histiocytosis ◽

Myeloid Cell ◽

Cathepsin K ◽

Nuclear Factor Κb ◽

Giant Cells ◽

Langerhans Cell ◽

Bone Lesions ◽

Tartrate Resistant Acid Phosphatase ◽

Tissue Destruction ◽

Organ Systems

Langerhans cell histiocytosis (LCH) is a disease that can involve one or multiple organ systems characterized by an accumulation of CD1a+ Langerhans-like cells as well as several other myeloid cell types. The precise origin and role of one of these populations, the multinucleated giant cell (MGC), in this disease remains unknown. This work shows that in three different lesional tissues, bone, skin, and lymph node, the MGCs expressed the characteristic osteoclast markers, tartrate-resistant acid phosphatase and vitronectin receptor, as well as the enzymes cathepsin K and matrix metalloproteinase-9. Although, in bone lesions, the osteoclast-like MGCs were only CD68+, in the nonostotic sites, they coexpressed CD1a. The presence of osteoclast-like MGCs may be explained by the production of osteoclast-inducing cytokines such as receptor activator of nuclear factor κB ligand and macrophage colony-stimulating factor by both the CD1a+ LCH cells and T cells in these lesions. As osteoclast-derived enzymes play a major role in tissue destruction, the osteoclast-like nature of MGCs in all LCH lesions makes them a potential target for the treatment of this disease.

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Angiotensin-Converting Enzyme, Transforming Growth Factor β1, and Interleukin 11 in the Osteolytic Lesions of Langerhans Cell Histiocytosis

Archives of Pathology & Laboratory Medicine ◽

10.5858/2000-124-1287-acetgf ◽

2000 ◽

Vol 124 (9) ◽

pp. 1287-1290

Author(s):

Robert E. Brown

Keyword(s):

Growth Factor ◽

Angiotensin Converting Enzyme ◽

Langerhans Cell Histiocytosis ◽

Transforming Growth Factor ◽

Giant Cells ◽

Transforming Growth Factor Β1 ◽

Langerhans Cell ◽

Converting Enzyme ◽

Osteolytic Lesions ◽

Interleukin 11

Abstract Objective.—To assess the expression of potential osteoclastogenic and osteolytic factors in osteolytic lesions from patients with Langerhans cell histiocytosis. Design.—Paraffin-embedded biopsy sections from 5 such archival cases underwent immunohistochemical procedures with antibodies to detect the following antigens: CD1a, S100 protein, interleukin 11, the latency-associated peptide of transforming growth factor β1, and angiotensin-converting enzyme. Results.—Commonalities noted include (1) the presence of multinucleated osteoclast-like giant cells, (2) the expression of interleukin 11 and latency-associated peptide antigens in lesional Langerhans cells, and (3) plasmalemmal immunoreactivity for angiotensin-converting enzyme antigen on non–Langerhans cell histiocytes and, on occasion, osteoclast-like giant cells and endothelial cells. Conclusions.—These observations suggest a possible pathogenetic sequence for osteolysis in Langerhans cell histiocytosis that involves angiotensin II formation, leading to the activation of latent transforming growth factor β1 and, in turn, to the enhanced production of interleukin 11, resulting in both osteoclastogenesis and impaired remodeling of bone.

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Role of Fine Needle Aspiration in the Diagnosis of the Rare Disease of Langerhans Cell Histiocytosis in a Child

Case Reports in Pathology ◽

10.1155/2014/724895 ◽

2014 ◽

Vol 2014 ◽

pp. 1-3

Author(s):

Seema Lale ◽

Daniel Soto ◽

Patricia G. Wasserman

Keyword(s):

Langerhans Cell Histiocytosis ◽

Parasitic Infection ◽

Giant Cells ◽

Needle Aspiration ◽

Kimura’S Disease ◽

Cat Scratch Disease ◽

Multinucleated Giant Cells ◽

Dermatopathic Lymphadenitis ◽

Massive Lymphadenopathy

Langerhan’s cell histiocytosis (LCH) results from the proliferation of immunophenotypically and functionally immature, morphologically rounded Langerhan’s cells along with eosinophils, macrophages, lymphocytes, and, commonly, multinucleated giant cells. Here we report a case in a 6-year-old boy of differential diagnoses including dermatopathic lymphadenitis (DL), parasitic infection, Kimura’s disease, hypersensitivity reactions, cat-scratch disease, sinus histiocytosis with massive lymphadenopathy (SHML), hyperplasic lymph nodes, and lymphoma.

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Spontaneous regression of infantile dural-based non-Langerhans cell histiocytosis after surgery: case report

Journal of Neurosurgery Pediatrics ◽

10.3171/2014.10.peds14378 ◽

2015 ◽

Vol 15 (4) ◽

pp. 372-379 ◽

Cited By ~ 4

Author(s):

Yohei Miyake ◽

Susumu Ito ◽

Mio Tanaka ◽

Yukichi Tanaka

Keyword(s):

Langerhans Cell Histiocytosis ◽

Spontaneous Regression ◽

Histopathological Examination ◽

Residual Tumor ◽

Giant Cells ◽

Langerhans Cell ◽

Juvenile Xanthogranuloma ◽

Review Of The Literature ◽

Unique Case ◽

Postoperative Course

The authors report the case of a large left occipital mass lesion in an 8-month-old boy who presented with seizure. Neuroimaging demonstrated an approximately 5-cm extraaxial tumor, and the patient underwent partial resection. The tumor was strongly attached to the tentorium and falx. In the postoperative course the residual lesion regressed spontaneously, and after 5 years only a slight residual tumor remained along the tentorium. Histopathological examination of the tumor revealed non-Langerhans cell histiocytosis (non-LCH). However, the tumor was not diagnosed as juvenile xanthogranuloma (JXG) because it lacked Touton giant cells. Hence, the authors described this lesion as a fibroxanthogranuloma. Most intracraniospinal non-LCHs have been reported as JXG; however, several cases of xanthomatous tumors with histopathological features resembling those of JXG have been described as fibrous xanthoma, xanthoma, fibroxanthoma, and xanthogranuloma. Among JXG and the xanthomatous tumors, a review of the literature revealed several cases of dural-based tumors; these dural-based tumors have had favorable courses, including the case described in this report. In addition, the patient in the present case experienced spontaneous regression of the residual tumor. The authors report this unique case and review the literature on isolated intracraniospinal non-LCHs, especially in cases of dural-based lesion.

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