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Sakai H, Ibe M, Takahashi H, Matsuo S, Okamoto K, Makino I, Oomori Y, Iizuka H	Satisfactory remission achieved by PUVA therapy in Langerhans cell hisiocytosis in an elderly patient.	The Journal of dermatology 1996, 23: 42
Langerhans cell histiocytosis is currently regarded as a reactive proliferative process of Langerhans cells rather than a malignancy. The disease is characterized by Langerhans cell infiltration of skin, lung, bone and other organs. We report a 74-year-old man with Langerhans cell histiocytosis who had generalized hemorrhagic and crusted papules. He also had diabetes insipidus. Because he did not have any severe constitutional symptoms or failure of vital organs, we applied topical PUVA treatment to his skin lesions, which responded well to the therapy. Diabetes insipidus, however, remained, in spite of X ray radiotherapy for the pituitary lesion.
Sander CS, Kaatz M, Elsner P	Successful treatment of cutaneous langerhans cell histiocytosis with thalidomide.	Dermatology (Basel, Switzerland) 2004, 208: 149
Langerhans cell histiocytosis (LCH) represents a group of rare histiocytic syndromes characterized by tissue infiltration with dendritic cells. The management of LCH is difficult as these disorders respond inconsistently to immunosuppressive and chemotherapeutic strategies. Thalidomide (N-phtalimidoglutarimide), initially used as a tranquilizer, has recently been used in the management of several inflammatory skin diseases. We describe the case of a 38-year-old male with mucocutaneous LCH. A treatment course with 6 cycles of 2-chlorodeoxyadenosin (cladribine) was initiated. This was well tolerated but withdrawn after 6 months to prevent secondary malignancy. A partial remission was seen. Subsequently, a treatment course with thalidomide 200 mg daily was started. This therapy resulted in a significant improvement of the mucocutaneous lesions within 4 weeks and complete healing was achieved after 3 months. Treatment was then successfully continued with daily doses of 100 mg to prevent relapse. In conclusion, thalidomide monotherapy represents an effective, safe and well-tolerated treatment option that should be considered as first-line therapy for mucocutaneous LCH.
Sartoris DJ, Parker BR	Histiocytosis X: rate and pattern of resolution of osseous lesions.	Radiology 1984, 152: 679
Resolution rate and qualitative characteristics of healing were studied in 71 osseous lesions of histiocytosis X. Rates of healing, as assessed planimetrically and by vertebral body-height measurement, did not differ significantly among lesions that were treated with chemotherapy alone, chemotherapy plus radiation, radiation alone, or those that received no treatment. Lesions of Letterer-Siwe disease, Hand-Schuller-Christian disease, multiple eosinophilic granuloma, and solitary eosinophilic granuloma resolved at comparable rates. A trend toward more rapid healing was noted in younger children. Lesions treated with radiation alone showed a greater tendency to resolve with sclerosis, but radiographic healing characteristics did not strictly depend upon specific mode of therapy. These results support the concept of osseous histiocytosis X as a benign self-limited disorder when systemic disease is absent, and they encourage therapeutic conservatism.
Saven A, Burian C	Cladribine activity in adult langerhans-cell histiocytosis.	Blood 1999 Jun 15; 93: 4125
Langerhans-cell histiocytosis (LCH) results from the accumulation of tissue histiocytes derived from the same progenitor cells as monocytes. Because cladribine is potently toxic to monocytes, we conducted a phase II trial of cladribine. Cladribine was administered to 13 LCH patients at 0.14 mg/kg per day by 2-hour intravenous infusion for 5 consecutive days, every 4 weeks for a maximum of six courses. Median age was 42 years (range, 19 to 72) and median pretreatment disease duration was 99 months (range, 6 to 252). One patient was untreated, one had received prior prednisone only, one prior radiation only, six prior radiation and chemotherapy, and four prior surgery, radiation, and chemotherapy. Seven patients had cutaneous involvement, six multifocal osseous, six pulmonary, two each with soft tissue and nodal involvement, and four had diabetes insipidus. Of 13 patients, 12 were evaluable for response and all for toxicity. After a median of three courses (range, 1 to 6), seven (58%) patients achieved complete responses (two pathologic and five clinical) and two (17%) patients achieved partial responses; overall response rate, 75%. Median response follow-up duration was 33 months (range, 1 to 65). Seven patients experienced grade 3 to 4 neutropenia. Only one patient had a documented infection, dermatomal herpes zoster. At a median follow-up of 42 months (range, 5 to 76), 12 patients remain alive and one patient has died. Thus, cladribine has major activity in adult LCH and warrants further investigation in both pediatric and adult LCH as a single agent and in combination with other drugs.
Schmahmann JD, Gardner R, MacMore J, Vangel MG	Development of a brief ataxia rating scale (BARS) based on a modified form of the ICARS.	Movement disorders : official journal of the Movement Disorder Society 2009 Sep 15; 24: 1820
To develop a brief ataxia rating scale (BARS) for use by movement disorder specialists and general neurologists. Current ataxia rating scales are cumbersome and not designed for clinical practice. We first modified the International Cooperative Ataxia Rating Scale (ICARS) by adding seven ataxia tests (modified ICARS, or MICARS), and observed only minimally increased scores. We then used the statistics package R to find a five-test subset in MICARS that would correlate best with the total MICARS score. This was accomplished first without constraints and then with the clinical constraint requiring one test each of Gait, Kinetic Function-Arm, Kinetic Function-Leg, Speech, and Eye Movements. We validated these clinical constraints by factor analysis. We then validated the results in a second cohort of patients; evaluated inter-rater reliability in a third cohort; and used the same data set to compare BARS with the Scale for the Assessment and Rating of Ataxia (SARA). Correlation of ICARS with the seven additional tests that when added to ICARS form MICARS was 0.88. There were 31,481 five-test subtests (48% of possible combinations) that had a correlation with total MICARS score of > or =0.90. The strongest correlation of an unconstrained five-test subset was 0.963. The clinically constrained subtest validated by factor analysis, BARS, had a correlation with MICARS-minus-BARS of 0.952. Cronbach alpha for BARS and SARA was 0.90 and 0.92 respectively; and inter-rater reliability (intraclass correlation coefficient) was 0.91 and 0.93 respectively. BARS is valid, reliable, and sufficiently fast and accurate for clinical purposes.
Sharma R, Maplethorpe R, Wilson G	Effect of pregnancy on lung function in adult pulmonary Langerhans cell histiocytosis.	The journal of maternal-fetal & neonatal medicine : the official journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians 2006, 19: 67
Adult pulmonary Langerhans cell histiocytosis (APLCH) is a rare lung disease. We report a case of APLCH and pregnancy with detailed lung function tests. There was no significant change in the lung function tests during pregnancy. It is apparent that pregnancy does not influence the evolution of pulmonary Langerhans cell histiocytosis.
Sheehan MP, Atherton DJ, Broadbent V, Pritchard J	Topical nitrogen mustard: an effective treatment for cutaneous Langerhans cell histiocytosis.	The Journal of pediatrics 1991, 119: 317
In 16 children with multisystem Langerhans cell histiocytosis (mean age 22 months, range 5 to 36 months) severe symptomatic skin involvement was treated with topical nitrogen mustard (mechlorethamine hydrochloride). In each case, rapid clinical improvement occurred within 10 days; subsequent complete healing was observed in 14 children, and partial healing in 2 others in whom treatment was a component of palliative care. Mean duration of treatment was 3.5 months (range 2 to 6 months). Systemic treatment was averted in 11 patients because response to topical therapy was so favorable, but bone marrow or respiratory failure led to a fatal outcome in 5 other patients. Adverse effects were minimal. One patient developed contact allergy to topical nitrogen mustard after 2 years of intermittent therapy, but was successfully desensitized and was then able to continue treatment. We conclude that the topical application of nitrogen mustard is an effective treatment for cutaneous Langerhans cell histiocytosis. Although adverse effects were minimal in the short term, there remains concern about the possibility of long-term cutaneous carcinogenicity.
Simko SJ, Tran HD, Jones J, Bilgi M, Beaupin LK, Coulter D, Garrington T, McCavit TL, Moore C, Rivera-Ortegón F, Shaffer L, Stork L, Turcotte L, Welsh EC, Hicks MJ, McClain KL, Allen CE	Clofarabine salvage therapy in refractory multifocal histiocytic disorders, including Langerhans cell histiocytosis, juvenile xanthogranuloma and Rosai-Dorfman disease.	Pediatric blood & cancer 2014, 61: 479
Existing therapies for recurrent or refractory histiocytoses, including Langerhans cell histiocytosis (LCH), juvenile xanthogranuloma (JXG), and Rosai-Dorfman disease (RDD), have limited effectiveness. We report our experience with using clofarabine as therapy in children with recurrent or refractory histiocytic disorders, including LCH (11 patients), systemic JXG (4 patients), and RDD (3 patients).
Singhi AD, Montgomery EA	Gastrointestinal tract langerhans cell histiocytosis: A clinicopathologic study of 12 patients.	The American journal of surgical pathology 2011, 35: 305
Gastrointestinal (GI) tract involvement by Langerhans cell histiocytosis (LCH) is a rare condition. It is typically noted in male patients with systemic disease and is associated with both poor prognosis and high morbidity. The incidence peaks in childhood. However, a limited number of cases have been reported in adults. To further characterize this disease process, we collected 24 cases of GI tract LCH from 12 patients. The patients included 2 children (4 mo and 2.3 y) and 10 adults (40 to 77 y; mean, 58.4 y), with a female predominance (9 of 12, 75%). Both children presented with failure to thrive, bloody diarrhea, and anemia. In contrast, 5 of 10 (50%) adults were asymptomatic and the rest had unrelated symptoms. Endoscopically, the pediatric patients showed the involvement of the duodenum and multiple colonic sites. However, 8 of 10 (80%) adults presented with a solitary polyp, primarily involving the colorectum (7 of 8, 88%). The lesions ranged in size from 0.1 to 0.8 cm (mean, 0.4 cm), and were predominantly intramucosal (18 of 24, 75%) with either a marginated (14 of 24, 58%) or infiltrative (10 of 24, 42%) growth pattern. Microscopic features were similar to those of LCH found elsewhere, although some cases differed by showing prominent lymphocytes (12 of 24, 50%) rather than eosinophils and large nucleoli (2 of 24, 8%). Reactive overlying mucosal and entrapped epithelial changes (10 of 24, 42%), mucosal ulceration (3 of 24, 13%), focal necrosis (1 of 24), and multinucleated giant cells (1 of 24) were also identified. Mitotic figures were absent. On immunohistochemistry, all lesions expressed the S-100 protein and CD1a. Follow-up information was available for 11 (92%) patients ranging from 2 months to 5.3 years (mean, 1.8 y). One pediatric patient was lost to follow-up. However, the other patient developed multisystem disease and died 1 year after the initial diagnosis. Two adult patients developed cutaneous disease, 2 months and 2 years after the initial diagnosis, 1 of whom had multifocal colonic disease. On the basis of this study, GI tract LCH lesions present in both children and adults with a female predominance. Consistent with earlier reports, pediatric cases are associated with systemic disease and poor prognosis. However, in adults, LCH is typically encountered as an incidental, solitary polyp. Rare cases of systemic disease may occur and, therefore, close follow-up may be warranted.
Slater JM, Swarm OJ	Eosinophilic granuloma of bone.	Medical and pediatric oncology 1980, 8: 151
Eosinophilic granuloma of bone is an uncommon granulomatous process for which therapy recommendations vary considerably. Consequently, a survey of the world literature from 1940 to 1974 was undertaken in an attempt to develop more concrete guidelines. This survey revealed that the disease manifests itself primarily in males under 20 years of age of all races. The most common symptom is pain, with or without swelling. Multiple sites in a single case are frequent. The clinical course is generally benign, unlike Letterer-Siwe disease or Hand-Schuller-Christian disease. A remission rate of 95% was noted, with relapses usually occurring as distant disease within the first year. Simple excision or curettage was the most common surgical procedure utilized, and radiotherapy doses varied widely. Excellent local control of the disease can be provided using conservative surgery, low-dose irradiation, or both. Morbidity secondary to pathologic fracture or to overly aggressive treatment are the major concerns, not failure to control the disease.
Sosman, MC	Xanthomatosis (Schüller's disease; Christian's syndrome). A report of three cases treated with roentgen rays.	American Journal of Roentgenology 1930, 23: 581
Steen AE, Steen KH, Bauer R, Bieber T	Successful treatment of cutaneous Langerhans cell histiocytosis with low-dose methotrexate.	The British journal of dermatology 2001, 145: 137
Langerhans cell histiocytosis (LCH) can be a difficult therapeutic problem. We present a 40-year-old woman with a 4-year history of LCH who was successfully treated with low-dose methotrexate (20 mg weekly).
Steiner M, Matthes-Martin S, Attarbaschi A, Minkov M, Grois N, Unger E, Holter W, Vormoor J, Wawer A, Ouachee M, Woessmann W, Gadner H	Improved outcome of treatment-resistant high-risk Langerhans cell histiocytosis after allogeneic stem cell transplantation with reduced-intensity conditioning.	Bone marrow transplantation 2005, 36: 215
Children with multisystem Langerhans cell histiocytosis (LCH) and risk organ involvement who fail to respond to conventional chemotherapy have an extremely poor prognosis. Myeloablative stem cell transplantation (SCT) as a possible salvage approach for these patients has been associated with a high risk of transplant-related mortality. Therefore, allogeneic stem cell transplantation following a reduced-intensity conditioning regimen (RIC-SCT) has recently been performed as an alternative salvage approach. We report on the experience with allogeneic RIC-SCT in nine pediatric high-risk LCH patients. Conditioning regimen included fludarabine in all patients, melphalan in eight patients, total lymphoid irradiation in six patients, total body irradiation in two, antithymocyte globulin in five, and Campath in four patients. RIC-SCT was well tolerated with regard to common procedure-related complications. Two patients died 50 and 69 days after RIC-SCT, respectively. Seven out of the nine patients survived and showed no signs of disease activity (including one with nonengraftment and full autologous hematopoietic recovery) after median follow-up of 390 days post-SCT. Based on this observation, we conclude that RIC-SCT is a feasible procedure with low transplant-related morbidity and mortality and a promising new salvage approach for high-risk LCH patients with resistant risk organ involvement.
Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Thiele J, Vardiman JW	WHO Classification of Tumors of Haematopoietic and Lymphoid Tissues.	IARC press, Lyon Fourth Edition. 252, pp.358
Szturz P, Adam Z, Rehák Z, Koukalová R, Kodet R, Nebeský T, Neubauer J, Moulis M, Smardová L, Mayer J	[Lymphoma-like course in aggressive adult multisystem Langerhans cell histiocytosis and the benefit of PET/CT imaging in evaluation of diffuse metabolic activity of lung parenchyma]. [Article in Czech].	Vnitr Lek 2010, 56: 1177-93.
The case report given here describes an unusual case of a 35-year-old otherwise healthy male diagnosed with aggressive form of Langerhans cell histiocytosis initially taking course under the form of lymphoma with expressed B symptoms (night sweats, fever and weight loss) and generalized peripheral lymphadenopathy. Also present were productive cough and perianal itching. The diagnosis was determined from lymph node and perianal skin biopsies. Furthermore, by a typical finding on HRCT (high-resolution computed tomography), pulmonary involvement was confirmed the gradual development of which we succeeded to document through a series of several HRCT and PET/CT scans from its initial florid phase characterized by disseminated nodularities up to the terminal phase with the decline of activity and development of cystic formations. After the collection of peripheral blood stem cells, the planned patient's therapy started which in all consisted of three monotherapy cycles with cladribine followed by three cycles of combined chemotherapy (cladribine + cyclophosphamide + methylprednisolone) and complemented with curative radiotherapy of the perianal area. This treatment put the disease into complete remission. However, in two months the initial B-symptoms occurred again, along with the pulmonary symptomatology, perianal pruritus and newly also hip bone pains. The suspected LCH relapse was proved histologically by lymph node biopsy and confirmed at a restaging PET/CT examination which also showed disease dissemination into the hip bones. Consequently, an aggressive form of the disease with early relapse had been the case, which was indicated for administering 4 cycles of CHOEP (cyclophosphamide + doxorubicin + vincristine + etoposide + prednisone) as salvage regimen completed in March 2010 with autologous peripheral blood stem cell transplantation after high-dose BEAM (carmustine + etoposide + cytarabine + melphalan) chemotherapy. Thus, the generalized involvement of nodes doesn't always need to be malignant lymphoma or metastatic dissemination of a tumour but also LCH may be the case. The presence of B symptoms may very likely stand for an aggressive form of the disease course. Histological evaluation of the proliferative characteristic (given by Ki-67 immunohistochemical proliferative index marker and also morphologically by the number of mitosis) may draw attention to an aggressive form of this disease. However, therapy with cladribine (2-chlorodeoxyadenosine) which proves beneficial in classic forms of LCH, in cases of highly aggressive forms of LCH doesn't need to have the same effect as in LCH with low proliferative activity, which conforms to the present experience in the treatment of indolent and aggressive lymphomas. In our study, the hybrid PET/CT imaging proved high sensitivity in evaluating the activity of the disease, including its early relapse. We are presenting here a new method for description and evaluation of diffuse increased activity of pulmonary parenchyma by means of PET/CT examination and for using this method within the framework of monitoring the curative response.
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