Adult case of Langerhans cell histiocytosis

Shinmi, Kahori; Nagai, Yayoi; Matsushima, Yoichiro; Tamura, Atsushi; Ishikawa, Osamu

doi:10.1111/j.1346-8138.2007.00270.x

Cited by 7 publications

(7 citation statements)

References 18 publications

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“…It is rare and poorly defined in adults. Most retrospective studies in adult patients have shown that 69–72% of patients have involvement of only a single organ system, most often the bones (52%), lungs (40%) or, less frequently, the skin (7%) 5 . However, a multicenter retrospective study of 274 adults from 13 countries from the International Registry of the Histiocyte Society, contradicts previous reports, and showed that 188 patients (68.6%) had multisystem disease 6 .…”

Section: Discussionmentioning

confidence: 92%

Extensive cutaneous Langerhans cell histiocytosis in an elderly woman

Wang

Hong

et al. 2010

The Journal of Dermatology

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Langerhans cell histiocytosis (LCH), especially with involvement limited to the skin in adults is a rare entity. Primary cutaneous LCH in older patients usually follows a benign course and can regress spontaneously. We report a fatal case of disseminated cutaneous LCH in an elderly woman. A 64-year-old woman presented with confluenced erythematous macule and diffuse papuloscaling and papulocrusted lesions on the trunk that became erosive on the area of axillae and groins. Histopathological study of skin specimens showed extensive epidermotropic and folliculotropic, lichenoid infiltration of Langerhans' cells. Positive immunohistochemical staining for CD1a and S-100 protein in Langerhans' cells, and numerous typical Birbeck granules in the cytoplasm of Langerhans' cells by electron microscopy study confirmed the diagnosis of LCH. Chest X-rays and computed tomography scans showed mild interstitial pneumonia without a honeycomb appearance. The patient was diagnosed with LCH. Her general condition worsened rapidly and she died 1 month after diagnosis. Because extracutaneous involvement of LCH had been ruled out by laboratory and imaging investigations, we would like to believe this case should be classified as "malignant" LCH based on the clinical course.

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Section: Discussionmentioning

confidence: 92%

Extensive cutaneous Langerhans cell histiocytosis in an elderly woman

Wang

Hong

et al. 2010

The Journal of Dermatology

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“…Histologically, LCH is characterized by large cells with abundant cytoplasm within a background of lymphocytes and eosinophils. Findings of this type should prompt confirmation of the LCH diagnosis by means of electron microscopy, where Birbeck granules (cytoplasmic inclusions with inner serrations and occasional terminal dilations) are seen, or a positive immunohistochemistry for S100 protein and CD1a [1,5,6,15,16].…”

Section: Discussionmentioning

confidence: 98%

“…However, primary thyroid involvement is extremely rare [5,8]. Diagnosis is based on electronic microscopy (Birbeck granules) or immunohistochemistry (positivity for S100 protein and CD1a) [1,5,6,15,16]. The choice of treatment is based on the extent of the disease and the site of involvement [5,17].…”

Section: Introductionmentioning

confidence: 99%

Endocrine manifestations of Langerhans cell histiocytosis diagnosed in adults

et al. 2010

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Langerhans cell histiocytosis (LCH) is a rare granulomatous disease of unknown etiology. We retrospectively reviewed data from four patients (3 males and 1 female), mean age 33.5 years old (range: 21-40), with histopathological diagnosis of LCH. All of them presented with symptoms suggestive of endocrine involvement. The main complaint was goiter in two patients and polyuria and polydipsia in three. Before the LCH diagnosis, two patients had unevaluated symptoms of diabetes insipidus (DI) and hypogonadism. The mean time from symptoms onset to diagnosis was 6.25 years (range: 2-13). Histopathological diagnosis was established by total thyroidectomy (TT) biopsy in two patients, skin lesion biopsy in one, and pituitary stalk biopsy in the other. In the two-first patients, surgery was indicated after the fine-needle aspiration biopsy (FNAB) showed a false positive result of differentiated thyroid carcinoma and immunohistochemistry was used for diagnosis confirmation. Three cases were treated with chemotherapy; one of them had already received radiation therapy on the hypothalamic-pituitary region, developing post-radiation hypopituitarism.

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“…Langerhans cells are immunoreactive for both S100 and CD1a . These cutaneous manifestations of LCH may represent the earliest sign of the disease …”

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confidence: 99%

Coexistence of Langerhans cell histiocytosis, Rosai–Dorfman disease and splenic lymphoma with fatal outcome after rapid development of histiocytic sarcoma of the liver

et al. 2012

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The coexistence of skin-limited Langerhans cell histiocytosis (LCH) and Rosai-Dorfman disease (RDD) is an exceptional finding. The association of lymphomas and histiocytosis is also infrequent. We report the case of a 68-year-old man which presented an exceptional association of cutaneous LCH and RDD and splenic marginal zone lymphoma. He was stable for few years. Suddenly, the patient was admitted into Hematology Department with a remarkable enlargement of spleen and liver without enlargement of lymphadenopathies or skin lesions flare. He died 24 h later despite treatment with systemic chemotherapy combined with prednisone. Pre-mortem biopsy showed infiltration with histiocytic sarcoma. We think that a transdifferentiation phenomenon could explain our case, although we could not show a clonal relationship between the cutaneous and the liver diseases. We also want to pay attention to the fact that a fast transformation to a more aggressive disease can occur long time after the presentation of the first lesion, a problem that stresses the importance of performing a close and permanent follow-up of these patients.

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Adult case of Langerhans cell histiocytosis

Cited by 7 publications

References 18 publications

Extensive cutaneous Langerhans cell histiocytosis in an elderly woman

Extensive cutaneous Langerhans cell histiocytosis in an elderly woman

Endocrine manifestations of Langerhans cell histiocytosis diagnosed in adults

Coexistence of Langerhans cell histiocytosis, Rosai–Dorfman disease and splenic lymphoma with fatal outcome after rapid development of histiocytic sarcoma of the liver

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