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Histiocytosis
Kinds of Histiocytosis Selected AbstractsLangerhans Cell Histiocytosis: A Review of the Current Recommendations of the Histiocyte SocietyPEDIATRIC DERMATOLOGY, Issue 3 2008Elizabeth K. Satter M.D., M.P.H. Historically, the nomenclature regarding this entity has been confusing because the disease had been subcategorized simply based upon the different clinical manifestations. In the following article, we summarize the current recommendation of the Histiocyte Society regarding the classification, evaluation, prognosis, and treatment of Langerhans cell histiocytosis. [source] Urticating Hashimoto,Pritzker Langerhans Cell HistiocytosisPEDIATRIC DERMATOLOGY, Issue 1 2001David F. Butler M.D. The Darier sign has been a reliable feature in the diagnosis of mastocytosis. However, the cutaneous infiltrate of Hashimoto,Pritzker Langerhans cell histiocytosis (LCH) may contain a large number of mast cells, leading to confusion both clinically and histologically. We report an infant who developed red-brown papules of Hashimoto,Pritzker LCH during the neonatal period and presented with a positive Darier sign and acute urticaria. [source] Smoking preceded pulmonary involvement in adults with Langerhans cell histiocytosis diagnosed in childhoodACTA PAEDIATRICA, Issue 11 2000C Bernstrand ABSTRACT. Two patients with childhood Langerhans cell histiocytosis (LCH) (aged 2 and 6 y at diagnosis) in whom pulmonary involvement was diagnosed in adulthood, 23 and 12 y later, respectively, are presented. In each patient, smoking preceded the diagnosis of pulmonary involvement by 3 y, providing further evidence that smoking is a risk factor in the development of pulmonary LCH. ,Langerhans cell histiocytosis, lungs, smoking [source] Langerhans cell histiocytosis in lymph nodes , cytomorphological diagnosis and pitfallsCYTOPATHOLOGY, Issue 6 2000S. Kakkar Background Langerhans cell histiocytosis (LCH) is a rare disorder of unknown aetiology that may present as a multisystem or unisystem disease. The Lymph nodes can be involved as part of disseminated disease, as a metastatic site draining a focus of LCH or may be a unisystem involvement. Paucity of literature on the cytomorphology of LCH in lymph nodes led us to undertake this study. Materials and methods Nine cases with a confirmed histological diagnosis of LCH and a prior lymph node aspirate were retrieved over a 12 year period (1988,1999). Five more cases were reviewed where the cytological diagnosis of LCH was rendered on a background of clinical and radiological findings. Papanicolaou and May Grunwald,Giemsa-stained smears were examined. S-100 protein staining was available in four cases. Results and conclusions Nine cases had multisystem involvement, while in five cases only lymph nodes were involved. There were eleven males and three females; age ranged from five months to 27 years. The cytological diagnosis of LCH had been rendered in six, suspected in four and missed in four. Of the latter, two were reclassified as LCH on review, one as reactive lymphadenitis and in one a necrotising lesion was suspected. The pathognomonic ,LCH cell' was identified in 12 of 14 cases along with varying numbers of eosinophils, polymorphs and lymphocytes. Giant cells were seen in only six cases. In conclusion lymph node involvement by LCH can be identified on aspirates. However, LCH must be differentiated from dermatopathic lymphadenitis, sinus histiocytosis with massive lymphadenopathy and Hodgkin's disease. [source] Fine-needle aspiration cytology of Rosai-Dorfman disease of boneDIAGNOSTIC CYTOPATHOLOGY, Issue 7 2008Xin Jing M.D. Abstract Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) is a rare, benign self-limiting condition of unknown etiology. Less than a quarter of cases have only extranodal involvement and a few cases of skeletal involvement of Rosai-Dorfman disease without associated lymphadenopathy have been reported in the literature. We herein report cytohistologic findings in a case of sole skeletal Rosai-Dorfman disease in a 51-year-old woman who presented with an expansile, heterogeneous lesion at T11 with cord compression and edema. A CT-guided fine-needle aspiration of T-11 lesion was performed and the sample was processed by ThinPrep technique. The ThinPrep smear showed characteristic features of Rosai-Dorfman disease including hypercellularity with moderate number of histiocytes in a background of lymphocytes, plasma cells, and neutrophils. The histiocytes possessed abundant, pale and vacuolated cytoplasm, rounded nuclei with smooth nuclear membranes, fine chromatin, and distinct nucleoli. The histiocytes showed emperipolesis of lymphocytes and neutrophils. The diagnosis was confirmed by concurrent biopsy with immunhistochemical study. Our case highlighted the role of fine-needle aspiration with ThinPrep technique in the diagnosis of Rosai-Dorfman disease. Diagn. Cytopathol. 2008;36:516,518. © 2008 Wiley-Liss, Inc. [source] Adult Langerhans cell histiocytosisEUROPEAN JOURNAL OF HAEMATOLOGY, Issue 5 2006Marcus Stockschlaeder Abstract:, Langerhans cell histiocytosis (LCH) is a proliferative histiocytic disorder of unknown cause originating from dendritic cells. The clinical presentation of LCH is highly variable. Although the features of this disease have been well described in children, they remain poorly defined in adults. Here, we review the current knowledge about adult LCH, focussing on clinical presentation, diagnosis, treatment, and prognosis. [source] A colonic polyp due to Langerhans cell histiocytosis: a lesion not to be confused with metastatic malignant melanomaHISTOPATHOLOGY, Issue 4 2006S Sharma No abstract is available for this article. [source] Langerhans cell histiocytosis: fascinating dynamics of the dendritic cell,macrophage lineageIMMUNOLOGICAL REVIEWS, Issue 1 2010R. Maarten Egeler Summary:, In its rare occurrence, Langerhans cell histiocytosis (LCH) is a dangerous but intriguing deviation of mononuclear phagocytes, especially dendritic cells (DCs). Clinically, the disease ranges from self-resolving or well manageable to severe and even fatal. LCH lesions in skin, bone, and other sites contain high numbers of cells with phenotypic features resembling LCs admixed with macrophages, T cells, eosinophils, and multinucleated giant cells. Here we review current progress in the LCH field based on two central questions: (i) are LCH cells intrinsically aberrant, and (ii) how does the lesion drive pathogenesis? We argue that LCH cells may originate from different sources, including epidermal LCs, tissue Langerin+ DCs, or mononuclear phagocyte precursors. Current and prospective in vitro and in vivo models are discussed. Finally, we discuss recent insights into plasticity of T-helper cell subsets in light of the lesion microenvironment. LCH continues to provide urgent clinical questions thereby inspiring innovative DC lineage research. [source] Unifocal Langerhans cell histiocytosis of the oral mucosaJOURNAL DER DEUTSCHEN DERMATOLOGISCHEN GESELLSCHAFT, Issue 7 2009Susanna Fistarol Summary A 24-year-old man was admitted for a painful gingival ulcer. Histology and immunohistochemistry of a lesional biopsy revealed the diagnosis of Langerhans cell histiocytosis (LCH). To rule out multifocal disease, a complete staging was performed. There was no evidence of bony lesions or any other organ involvement. The diagnosis of LCH restricted to the oral mucosa was established. The complete oral lesion was ablated by CO2 laser and subsequently treated topically with triamcinolone acetonide. The patient is still in remission after one year of follow-up. LCH confined to the oral mucosa is rare. It presents usually as an inflammatory or ulcerative lesion, easily leading to misinterpretation and delayed diagnosis. Patients with limited unifocal mucocutaneous disease, as in the present case, usually have an excellent prognosis. However, the oral lesion may represent an early sign of LCH, predating and progressing to an aggressive life-threatening multiorgan disease. [source] Fibroblastic rheumatism: fibromatosis rather than non-Langerhans cell histiocytosisJOURNAL OF CUTANEOUS PATHOLOGY, Issue 5 2010Nicolas Kluger Background: Fibroblastic rheumatism is a unique fibro-proliferative disease affecting the skin and joints. It is characterized by distinctive clinical and histological features related to benign spindle-shaped cells proliferation. Pediatric reports are scarce in the literature. Objective: We describe here a new case in a 10-year-old boy and discuss the potential origin of the cell proliferation. Methods: Clinical findings, radiology, microscopic examination and outcome are reviewed. Histopathology and immunochemistry studies were performed on skin biospies using CD68, CD163, desmin, factor XIIIa, CD34, smooth muscle actin, PS100, epithelial membrane antigen, and calponin. Results: Histological sections disclosed a rather circumscribed nonencapsulated nodular infiltrate, invading the dermis and the upper subcutaneous tissue, consisted of a proliferation of spindle or stellate-shaped cells and thickened collagen fibers. Orcein staining showed disappearance of the elastic network. Aponeurosis and muscle were normal. A mild perivascular lymphohistiocytic infiltrate was noted. Calponin-staining was less strongly expressed as SMA, and some of them but not all were CD68 positive, as well. On the other hand, all were CD34, CD163, FXIIIa, PS100, EMA and desmin-negative. Conclusion: The true origin of these cells remains unclear. Some authors have speculated a histiocytic origin. However, immuno-chemical staining in our case failed to confirm this hypothesis and instead supported a fibroblastic/myofibroblastic origin. Given the clinical course and the histological and immunohistochemical results, we suggest that FR should be added to the group of fibromatoses. Kluger N, Dumas-Tesici A, Hamel D, Brousse N, Fraitag S. Fibroblastic rheumatism: fibromatosis rather than non-Langerhans cell histiocytosis. [source] Intravascular histiocytosis presenting with extensive vulvar necrosisJOURNAL OF CUTANEOUS PATHOLOGY, Issue 2009Pedram Pouryazdanparast Intravascular histiocytosis (IVH) is a rare reactive cutaneous lesion of unknown pathogenesis. Most cases are reported in association with rheumatoid arthritis, and cutaneous eruptions typically occur near swollen joints. The skin changes have included erythematous and violaceous macules, papules, plaques and indurated patches with a livedo-like pattern of erythema. We report the first case of IVH presenting with florid vulvar necrosis in an 87-year-old patient without a history of rheumatoid arthritis. Physical examination revealed an edematous, exudative and diffusely necrotic vulva with erythema surrounding the areas of necrosis, extending out to the thighs. The debrided skin revealed an extensively necrotic epidermis and multiple clusters of markedly dilated blood vessels within the dermis. These vessels contained fibrin thrombi admixed with numerous CD68+ and CD163+ histiocytes. Her skin changes improved significantly after surgical debridement and treatment with antibiotics. Interestingly, our patient was also found to have a lupus anticoagulant with elevated anticardiolipin antibodies. This is the first report of IVH possibly related to a thrombogenic diathesis associated with a hypercoagulable state. A diagnosis of IVH is important and may necessitate further clinical evaluation to exclude the possibility of co-existent systemic disease. [source] Prominent Langerhans' cell migration in the arthropod bite reactions simulating Langerhans' cell histiocytosisJOURNAL OF CUTANEOUS PATHOLOGY, Issue 12 2007Se Hoon Kim Background:, Epidermal Langerhans' cells (LCs) play pivotal roles in cutaneous immune responses. An encounter with antigens or other stimuli causes the mobilization and migration of LCs. Therefore, some dermatoses, which originated from antigenic stimuli or trauma, can show LC migration. Recently, we experienced several cases of anthropod bites that showed marked inflammatory infiltrates with eosinophils and CD1a-positive LCs. It was difficult to differentiate these cases from Langerhans' cell histiocytosis (LCH). Methods:, The degree and pattern of LC infiltration in the skin of arthropod bite reaction was evaluated. The characteristics of CD1a immunohistochemical expression in the arthropod bite reactions were compared with those of LCH. Results:, A few arthropod bite cases (approximately 36%) showed extensive CD1a-positive LCs in the dermis, especially in the perivascular area. In addition, the CD1a expression patterns of LCs in the arthropod bite reactions were dendritic, whereas that of tumor cells in LCH were distinctly membranous and cytoplasmic. Conclusion:, Some arthropod bite reactions can show marked CD1a-positive LCs in the dermis, especially in the perivascular area. The dendritic CD1a immunohistochemical staining pattern in arthropod bite reactions is useful in helping to differentiate from LCH. [source] Lymphomatoid papulosis with CD1a+ dendritic cell hyperplasia, mimicking Langerhans cell histiocytosisJOURNAL OF CUTANEOUS PATHOLOGY, Issue 7 2007Chris H. Jokinen Although CD1a+ dendritic cells (DC) in cutaneous T-cell lymphomas (CTCL) have been well documented, the presence of large numbers of DC within lymphoid infiltrates can pose a diagnostic difficulty. We present a case of a 70-year-old man with a 3-year history of recurrent red papules and plaques on the extremities and trunk that was referred to our institution, with the diagnosis of Langerhans cell histiocytosis. Skin biopsies showed a wedge-shaped cellular infiltrate in the superficial and deep dermis consisting of two cell populations. Most prominent were clusters of epithelioid cells with grooved nuclei and abundant eosinophilic cytoplasm, which stained with antibodies to CD1a and S-100. A second, less prominent population of atypical lymphocytes, some with enlarged, hyperchromatic and convoluted nuclei, were intermixed. The latter were positive for CD30, CD3 and CD5 and negative for CD20, CD34, CD68, ALK-1 and TdT. T-cell receptor gene rearrangement studies confirmed a clonal T-cell population, which with the clinical history was consistent with the diagnosis of lymphomatoid papulosis. While previous studies have shown an increased density of dermal DC in CTCL, we believe that this represents the first report of an unusually florid DC proliferation mimicking Langerhans cell histiocytosis and masking a lymphoproliferative disorder. [source] Cutaneous Rosai-Dorfman DiseaseJOURNAL OF CUTANEOUS PATHOLOGY, Issue 1 2005L. Lin Rosai-Dorfman disease, or sinus histiocytosis with massive lymphadenopathy (SHML), is a rare benign disease of unknown etiology that involves the cervical lymph nodes. Histologic findings include emperiopolesis of lymphocytes and S-100 protein positivity. Extranodal involvement occurs in 30,40% of cases, often involving skin. However, cutaneous Rosai-Dorfman disease without lymph node involvement is uncommon. We describe a case of cutaneous Rosai-Dorfman disease and discuss its clinical and histopathologic findings. A 69-year-old Caucasian male presented with a two-year history of a slowly enlarging violaceous mass on the mid-upper abdomen. He had a history of renal cell carcinoma two-year ago and was asymptomatic without any systemic symptoms. Physical examination revealed a large, violaceous mass of mid-upper abdomen with several violaceous nodules surrounding the periphery. There was no palpable lymphadenopathy. Punch biopsy showed diffuse dermal infiltrates composed of central xanthomatous cells surrounded by lymphoplasmacytic cells. Focal germinal center was noted. Immunostaining revealed strong S-100 and CD68 reactivity of central xanthomatous cells and kappa and lambda polyclonal lymphoplasmacytic cells. Special stains for infectious disorder were negative. Cutaneous Rosai-Dorfman disease is a benign reactive process, often under-recognized due to lack of systemic symptoms and lymphadenopathy. A high index of diagnostic awareness is required for its recognition. [source] IL-1, and PGE2 levels are increased in the saliva of children with Langerhans cell histiocytosisJOURNAL OF ORAL PATHOLOGY & MEDICINE, Issue 9 2008Virginia F. Preliasco Langerhans cell histiocytosis (LCH) is a rare disorder mainly of children, whose pathogenesis is still unknown. Some studies have demonstrated that LCH lesions produce different cytokines abnormally that may be relevant to the pathogenesis of the disease. The purpose of this study was to investigate interleukin-1, (IL-1,) and prostaglandin E2 (PGE2) levels in saliva from children with different clinical subtypes of LCH. We studied 29 children with LCH: seven unifocal (Group I), seven multifocal (Group II), 15 multisystemic (Group III) and 12 healthy volunteers (Group IV). Salivary IL-1, and PGE2 levels were significantly higher in LCH than in normal children. A multi-comparison test showed significantly (P < 0.001) higher levels of both IL-1, and PGE2 in saliva from Group III compared with Groups II and I. A significant correlation (r = 0.05) between IL-1, and PGE2 concentrations in saliva from each group was determined. Our findings demonstrated an association between high concentrations of salivary IL-1, and PGE2 and advanced stages of the disease. This allows us to suggest that the abnormal amount of these factors in saliva may serve as a risk marker for disease progression. [source] Langerhans cell histiocytosis and human herpes virus 6 (HHV-6), an analysis by real-time polymerase chain reactionJOURNAL OF ORTHOPAEDIC RESEARCH, Issue 3 2006Michael P. Glotzbecker Abstract Patients with Langerhans cell histiocytosis (LCH) usually present to orthopedic surgeons because this disease most commonly affects bone. The pathogenesis of LCH is unknown, although roles for environmental, infectious, immunologic, and genetic causes have been postulated. More specifically, there is limited data suggesting that human herpes virus 6 (HHV-6) may be a potential etiologic agent. Frozen biopsy material was obtained from 13 patients with LCH and 20 patients without the disease. After ensuring histologic adequacy of the material, the tissue was tested for HHV-6 by qualitative and quantitative real-time TaqMan PCR. Four of 13 patients with LCH had evidence of HHV-6 DNA in their tissue while 7 of 20 control patients tested positive for HHV-6 genome. Viral loads are reported for the positive patients; no statistical difference was observed in the presence or quantity of HHV-6 DNA found in either population, suggesting that the prevalence of HHV-6 in the tissue of LCH patients is the same as that found in tissue from individuals without disease. © 2005 Orthopaedic Research Society. Published by Wiley Periodicals, Inc. J Orthop Res 24:313,320, 2006 [source] Expression of a newly defined tumor-rejection antigen SART3 in musculoskeletal tumors and induction of HLA class I-restricted cytotoxic T lymphocytes by SART3-derived peptidesJOURNAL OF ORTHOPAEDIC RESEARCH, Issue 3 2001Naotake Tsuda We recently reported that a SART3 tumor-rejection antigen possessing tumor epitopes is capable of inducing HLA class I-restricted and tumor-specific cytotoxic T lymphocytes in cancer patients. We studied the expression of the SART3 protein in musculoskeletal tumors to find a molecule for potential use in tumor-specific immunotherapy. The SART3 was detected at protein levels in 100% of the osteosarcoma cell lines (n = 20), in 50% of the musculoskeletal tumor tissue specimens (n = 32), and at notable levels in 67% of osteosarcoma tissues (n = 9) and malignant fibrous histiocytosis tissues (n = 9), respectively. SART3-derived peptides at positions 109-118 and 315-323 induced HLA-A24-restricted tumor-specific cytoxic T lymphocytes from peripheral blood mononuclear cells of patients with osteosarcoma or malignant fibrous histiocytosis. These peptide-induced cytotoxic T lymphocytes recognized HLA-A24+ SART3+ osteosarcoma cells but not HLA-A24, or SART3, cells. These results suggest that the SART3 protein and its derived peptides could be molecules appropriate for use in specific immunotherapies for approximately 60% of HLA-A24+ patients with osteosarcoma or malignant fibrous histiocytosis. © 2001 Orthopaedic Research Society. Published by Elsevier Science Ltd. All rights reserved. [source] Cutaneous sterile granulomas/pyogranulomas, leishmaniasis and mycobacterial infectionsJOURNAL OF SMALL ANIMAL PRACTICE, Issue 11 2008D. Santoro Cutaneous "sterile" granulomas represent a group of uncommon skin disorders of unknown aetiopathogenesis. Many diseases are included in this group (for example, sterile granuloma/pyogranuloma syndrome and reactive histiocytosis). The definition of sterile is based on the exclusion of other possible aetiological agents (for example, microorganisms or foreign body). Many techniques are used to rule out a microbial aetiology including cytology, histology, immunohistochemistry and culture. However, some organisms are "fastidious" and difficult to culture or to identify with routine methods, and molecular studies are necessary. This is particularly true for mycobacteria (for example, canine leproid granuloma syndrome) and Leishmania. Recently, studies in human and veterinary medicine have proved the presence of microorganisms (mycobacteria and Leishmania) using a polymerase chain reaction technique in specimens previously diagnosed as sterile. Therefore, it is very important, with the development of new technologies, to use a multidisciplinary diagnostic approach to definitively rule out any microorganism before declaring a disease sterile. [source] A Retrospective Study of the Incidence and the Classification of Bone Marrow Disorders in the Dog at a Veterinary Teaching Hospital (1996,2004)JOURNAL OF VETERINARY INTERNAL MEDICINE, Issue 4 2006Douglas J. Weiss Background: An 8-year retrospective study was conducted to evaluate the prevalence and the classification of canine bone marrow disorders in a clinical pathology service at a university referral hospital. Animals: Dogs evaluated for bone marrow disorders at a veterinary teaching hospital. Hypothesis: A better understanding of the spectrum and the prevalence of canine bone marrow disorders can be achieved with a multiyear retrospective study. Methods: Bone marrow aspirate smears, core biopsy specimens, and case records from 717 dogs were reviewed. Results: Bone marrow specimens were first categorized based on the presence or the absence of a primary bone marrow disorder. Nondysplastic and nonmalignant pathologic changes were placed into 14 subcategories. Frequently observed pathologic disorders included nonregenerative immune-mediated anemia, pure red cell aplasia, bone marrow necrosis, myelofibrosis, and hemophagocytic syndrome. Dysmyelopoiesis (n = 61) was subcategorized into myelodysplastic syndromes (n = 27), and congenital (n = 1) and secondary (n = 33) dysmyelopoiesis. One hundred twenty-six cases of neoplasia were divided into acute leukemia (n = 46), chronic leukemia (n = 7), stage 5 malignant lymphoma (n = 28), multiple myeloma (n = 25), malignant histiocytosis (n = 11), metastatic mast-cell tumor (n = 3), sarcoma (n = 5), and carcinoma (n = 1). Conclusions and Clinical Importance: This study provides a general indication of the spectrum and the prevalence of canine bone marrow disorders at a referral center in North America. [source] Langerhans cell histiocytosis: oral/periodontal involvement in adult patientsORAL DISEASES, Issue 8 2009S Annibali Objective:, Langerhans cell histiocytosis (LCH) is a clonal proliferative multisystem disease. Although bone and mucosae have been classified as non-risk organs, their involvement may increase the risk of disease progression. Oral and periodontal lesions are burdened with a significant impairment of quality of life for associated signs, symptoms and loss of function. Most of information regards paediatric disease; the disease in adults has received limited attention. Subjects and Methods:, A total of 31 adult patients affected by immuno-histopathology confirmed LCH have been prospectively examined; attention was paid to the occurrence and characterization of oral lesions. Results:, Twelve patients developed oral lesions. Posterior regions of jawbones were always affected; the involvement of anterior regions was not constant. Unifocal oral involvement was significantly associated with multisystemic disease while multifocal lesions were associated with unisystemic disease. Oral disease presented with soft tissue ulcers (50% of cases), gingival bleeding (66.7%), pain (83.4%), periodontal damage (50%), tooth mobility (16.7%), non-healing extraction socket (8.3%); 41.6% of patients complained of negative outcomes on quality of life. Oral lesions were easily handled with local measures. Conclusions:, Posterior regions require attention; single oral lesions may be part of multisystemic disease; oral and periodontal lesions may be early signs of disease reactivation. [source] Crystal-storing histiocytosis in plasma cell myeloma,AMERICAN JOURNAL OF HEMATOLOGY, Issue 6 2010Yusong Yang No abstract is available for this article. [source] Langerhans cell histiocytosis with digestive tract involvement,PEDIATRIC BLOOD & CANCER, Issue 4 2010Satya P. Yadav DCH Abstract Gastrointestinal tract (GIT) involvement in Langerhans cell histiocytosis (LCH) is not commonly described. We present two children presenting with GIT involvement with LCH, one successfully treated on standard protocol and other being treated on a protocol for relapsed disease. A review of literature showed almost 95% children were less than 2 years of age and 62% were females. Vomiting, abdominal pain, constipation, intractable diarrhea, malabsorption, bloody stools, protein-losing enteropathy, and even intestinal perforation are some of the reported symptoms. More than 50% patients died within 18 months from diagnosis. Pediatr Blood Cancer. 2010;55:748,753. © 2010 Wiley-Liss, Inc. [source] Langerhans cell histiocytosis following acute leukemia in an adult,AMERICAN JOURNAL OF HEMATOLOGY, Issue 10 2009Rebecca Hirsh No abstract is available for this article. [source] An infant with self-healing cutaneous Langerhans cell histiocytosis followed by isolated thymic relapsePEDIATRIC BLOOD & CANCER, Issue 2 2009Naoki Hatakeyama MD Abstract Thymic involvement with Langerhans cell histiocytosis (LCH) typically occurs in children as part of multi-system (M-S) LCH. Patients who develop skin-only LCH during infancy may either follow a self-healing course with spontaneous regression or may progress to M-S involvement. We describe a male infant who developed isolated thymic LCH after spontaneous complete regression of isolated cutaneous lesions. His erythrocyte sedimentation rate and C-reactive protein increased temporarily during the skin-only stage of LCH, and increased again considerably during the thymic relapse. Even for patients with skin-only LCH, these laboratory data might indicate possible relapse or late progression of the disease. Pediatr Blood Cancer 2009;53:229,231. © 2009 Wiley-Liss, Inc. [source] Isolated pulmonary Langerhans cell histiocytosis with recurrent bilateral pneumothoraces treated with chemotherapy and chemical pleurodesisPEDIATRIC BLOOD & CANCER, Issue 1 2009Lubbna Valliani MD No abstract is available for this article. [source] Central nervous system-related permanent consequences in patients with Langerhans cell histiocytosis,PEDIATRIC BLOOD & CANCER, Issue 1 2007Edda Mittheisz MD Abstract Background Permanent consequences in Langerhans cell histiocytosis (LCH) are irreversible late sequelae related to the disease that may severely impair the quality of life of survivors. The frequency and pattern of permanent consequences affecting the central nervous system (CNS) remains to be determined. Procedure In this single center study, 25 LCH patients observed for a median time of 10 years 3 months underwent a uniform thorough follow-up program including neuropsychological testing and electrophysiological evaluation. Results Overall permanent consequences were seen in 9 of 25 patients. Intracranial abnormalities were the most frequent including diabetes insipidus (DI) in seven patients, anterior pituitary deficiencies in five patients, and neurodegenerative CNS disease in five patients. No patient had overt neurological symptoms upon neurological evaluation, but psychological testing revealed subtle deficits in short-term auditory memory (STAM) in 14 patients. Brain stem evoked potentials showed abnormalities in four of nine tested patients, all of these four had neurodegeneration on MRI. Conclusion Psychoneuroendocrine sequelae were found in an unexpectedly high number of patients in this single center study. Long-term follow-up focusing on such sequelae are important in LCH survivors, in order to detect early deficits, to monitor the evolution of the disease, and to provide specific support. Pediatr Blood Cancer 2007;48:50,56. © 2006 Wiley-Liss, Inc. [source] Langerhans cell histiocytosis affecting the eyesPEDIATRIC BLOOD & CANCER, Issue 5 2006Giulio J. D'Angio MD No abstract is available for this article. [source] Langerhans cell histiocytosis: Too many cytokines, not enough gene regulation?PEDIATRIC BLOOD & CANCER, Issue 2 2006Maurizio AricòArticle first published online: 12 JAN 200 No abstract is available for this article. [source] Langerhans Cell Histiocytosis: A Review of the Current Recommendations of the Histiocyte SocietyPEDIATRIC DERMATOLOGY, Issue 3 2008Elizabeth K. Satter M.D., M.P.H. Historically, the nomenclature regarding this entity has been confusing because the disease had been subcategorized simply based upon the different clinical manifestations. In the following article, we summarize the current recommendation of the Histiocyte Society regarding the classification, evaluation, prognosis, and treatment of Langerhans cell histiocytosis. [source] Urticating Hashimoto,Pritzker Langerhans Cell HistiocytosisPEDIATRIC DERMATOLOGY, Issue 1 2001David F. Butler M.D. The Darier sign has been a reliable feature in the diagnosis of mastocytosis. However, the cutaneous infiltrate of Hashimoto,Pritzker Langerhans cell histiocytosis (LCH) may contain a large number of mast cells, leading to confusion both clinically and histologically. We report an infant who developed red-brown papules of Hashimoto,Pritzker LCH during the neonatal period and presented with a positive Darier sign and acute urticaria. [source] | ||||||||||||||||||||||||||||||||||