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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Eosinophilic granuloma, a type of Langerhans cell histiocytosis, exhibits a classic vertebral collapse, which is called vertebra plana (Calve's disease) and it manifests as a solitary bony lesion. Vertebra plana can cause severe pain in patients. Bisphosphonates (clodronate, pamidronate and zoledronic acid) have been recently used to treat osteolytic bone lesions of LCH. Zoledronic acid has 100 times relative potency that of pamidronate. We report a case of a 10-year-old girl who had zoledronic acid treatment for severe back pain due to vertebra plana. X-ray photographs of the patient's body showed dense metaphyseal band sign, which can be found in lead poisoning, treated leukemia, healing rickets, recovery from scurvy, vitamin D hypervitaminosis, congenital hypothyroidism and hypoparathyroidism. Increased biological potent zoledronic acid deprived her of severe back pain due to vertebra plana and might cause dense metaphyseal band sign of her skeleton. Conclusion; We have cured the severe back pain of a 10-year-old girl case of eosinophilic granuloma with zoledronic acid. After that treatment, X-ray photographs of the patient's body showed dense metaphyseal band sign. There have been few such cases reported until now.
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PMID:Langerhans cell histiocytosis case with dense metaphyseal band sign. 2340 85

Eosinophilic granuloma is a rare disease which is difficult to diagnose clinically and radiographically. Localized Langerhans' cell histiocytosis, previously known as eosinophilic granuloma, mainly affects the skull, mandible, vertebrae, pelvis and ribs in children and the long bones of adults. We present a case report of a female who developed pain and swelling over the left mandibular region, and was later diagnosed as eosinophilic granuloma, which after administration of intralesional corticosteroid with surgical enucleation showed positive response. This disease is of importance to dental professionals because early clinical signs can occur in the jaw and can cause extensive destruction of the periodontal tissues and bone. The purpose of this case report is to describe a case of eosinophilic granuloma with emphasis on conservative approach for the treatment and the radiographic changes observed during and after the treatment.
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PMID:Management of polyostotic eosinophilic granuloma. 2355 66

A 39-year-old male was admitted to our clinic with symptoms of headache, dizziness, nausea, otalgia, otorrhea, tinnitus, and hearing loss in both ears for 3 weeks. Physical examination revealed edema in the tympanic membrane and external ear canal, and pain by palpation in the mastoid area bilaterally. There was no nystagmus, and the rest of the physical examination was otherwise normal. Temporal bone high resolution computed tomography (CT) showed a lesion causing erosion in the mastoid cortex, tegmen tympani, ossicles, and in the bone covering the sigmoid sinus bilaterally. There was also erosion in the superior semicircular canal and petrous bone on the left side. Cortical mastoidectomy was performed under general anesthesia. Histopathologic examination of the tissue revealed Langerhans cell histiocytosis (LCH). In this paper a case with LCH, presenting with bilateral mastoid involvement which has been rarely reported in the literature, is discussed with the existing literature.
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PMID:Langerhans cell histiocytosis in bilateral mastoid cavity. 2384 Oct 5

Eosinophilic granuloma is a rare disease belonging to the Langerhans' cell histiocytosis group. It accounts for only 1% of all tumor-like lesions of bone. A 6-year-old girl presented with proptosis, and a mass and pain in her right eye. Cranial computerized tomography and magnetic resonance imaging revealed a mass in her right orbital area. The patient was operated through a right frontotemporal craniotomy and orbitozygomatic osteotomy. The tumour was totally removed. The postoperative course was uneventful. Histopathological examination revealed a diagnosis of eosinophilic granuloma with typical Langerhans cells and eosinophylic leucocyte infiltration. We report an orbital eosinophilic granuloma case, which is rarely seen, and discuss its clinical and pathological features.
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PMID:Orbital eosinophilic granuloma in a child: a case report. 2410 Dec 88

Erdheim-Chester disease (ECD) is a rare, non-Langerhans cell histiocytosis with classic radiographic findings of patchy or diffuse osteosclerosis predominantly involving the long bones in a bilaterally symmetrical pattern. A 49-year-old woman presented with diffuse lymphadenopathy, painful skin lesions, and constitutional symptoms. Recent history was significant for a nontraumatic fracture of the tibia 3 weeks prior to admission. Physical examination and laboratory studies were notable for lower extremity pain and swelling, nodular lesions on the skin, and normocytic, normochromic anemia. Plain radiographs showed a lytic pattern of destruction with a superimposed fracture in the left proximal tibia. MRI showed focal bone marrow replacement extending from the subchondral bone to the tibial diaphysis. Excisional lymph node and skin biopsies of the lesions demonstrated a CD-68 positive, S-100 variable, and CD1a-negative histiocytic cell proliferation filling the dermis and completely replacing the sampled lymph node with an accompanying chronic inflammatory infiltrate and fibrosis, pathognomonic for ECD. We report an unusual case of ECD presenting initially as diffuse, painful lymphadenopathy, and subsequently demonstrating a lytic lesion of the tibia underlying a nontraumatic fracture.
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PMID:Erdheim-Chester disease: an unusual presentation of an uncommon disease. 2436 32

Langerhans Cell Histiocytosis (LCH) is a rare neoplasm characterized by abnormal proliferation of histiocytic cells. In this case report, we describe a unique case of a 13-year-old boy who presented to the clinic with an insidious onset of mid-clavicular pain. The provisional radiologic diagnosis of Langerhans Cell Histiocytosis of the clavicle was confirmed by an incisional biopsy of the left mid-clavicle lesion. The patient's lesion was treated by curettage, bone grafting, and internal fixation, due to the presence of pathologic fracture. At the 2-year followup, the patient was asymptomatic, and the lesion showed complete resolution without recurrence. The case report highlights the characteristic features of Langerhans Cell Histiocytosis in an unusual location, the knowledge of which would help avoid delayed or missed diagnosis in the future.
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PMID:Langerhans cell histiocytosis of the clavicle in a 13-year-old boy. 2451 64

We report two cases of eosinophilic granuloma of the spine in which the first manifestation of the disease was an associated neural deficit. In addition, we review the literature for cases of histiocytosis X in which the initial diagnosis was based only upon the discovery of spinal lesions. Thirty-nine reported cases of what we designated as "symptomatic eosinophilic granuloma of the spine" were found. A review of all 41 cases revealed that most patients present with pain, muscular stiffness and/or neurologic symptoms, the disorder most often affects the cervical vertebrae, and a high incidence of osteolytic lesions (as opposed to vertebral collapse) are found. These findings vary from those of radiologic and clinical evaluations of classical, multifocal, histiocytosis X that involves the spine.
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PMID:Symptomatic eosinophilic granuloma of the spine: report of two cases and review of the literature. 2482 12

Langerhans cell histiocytosis is a rare neoplastic proliferative disorder of the Langerhans cells. The clinical course is variable, ranging from a low symptomatic single bone lesion to fatal multiple organ involvement. Rarely, the sternum can be the first and single location of the disease. We report on a 12-year-old boy who presented with an aggressive lytic lesion of the proximal sternum associated with local pain and afternoon fever. Histopathological analysis of the closed biopsy specimen indicated eosinophilic granuloma of bone/Langerhans cell histiocytosis. Soon after the biopsy procedure the pain and fever subsided. Computed tomography at 2 months showed healing of the lytic lesion. The patient received no other type of treatment. At 2 year follow up he was symptom and disease free.
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PMID:Eosinophilic granuloma of the sternum in a child treated with closed biopsy. 2489 27

Burkitt's lymphoma is the most frequent subtype of non-Hodgkin's lymphoma in childhood. Radiographic findings are protean and can often overlap with other neoplastic and nonneoplastic processes. We present an unusual case of Burkitt's lymphoma in a 12-year-old boy presenting with a one-week history of urinary retention, dysuria, and "tailbone pain," as well as a 4-week history of jaw pain, initially treated as a dental abscess. On dental radiography, the patient was found to have resorption of alveolar bone adjacent to the lower first molars bilaterally, in keeping with "floating teeth," classically associated with Langerhans cell histiocytosis. Additionally, a large, eccentric, prostatic mass was noted, prompting the inclusion of rhabdomyosarcoma on the differential diagnosis, with subsequent definitive diagnosis of Burkitt's lymphoma on tissue and bone marrow biopsy. This case highlights the imaging overlap of these childhood neoplasms with an unusual lymphomatous prostate mass. It is important that the radiologists and pediatricians be aware of this potential overlap and the unusual presentation of Burkitt's lymphoma.
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PMID:A Rare and Unusual Case of Burkitt's Lymphoma Presenting with a Prostate Mass in a 12-Year-Old Boy. 2495 75

This study evaluated the outcome of patients with symptomatic bone Langerhans cell histiocytosis (LCH) treated with indomethacin alone, either at diagnosis or after reactivation (after recurrence with previous therapies). We evaluated the nonrandomized use of oral indomethacin (2 mg/kg/d) in patients with symptomatic single-system bone LCH. From 1997 to 2012, 38 sequential patients were treated for a median of 4 months. Criteria of nonactive disease (NAD) after initial treatment (8 wk) were: no pain, no soft tissue involvement, no increase of size, or no new bone lesions. Twenty-two patients were treated at diagnosis: 18 showed NAD after initial treatment (2 patients who had bone reactivations were retreated with indomethacin and remain with NAD). Three patients improved and they are with NAD after treatment with indomethacin, steroids, or radiotherapy. One patient developed progressive bone disease and he is with NAD after treatment with steroids and chemotherapy. Sixteen patients were treated after reactivation, and all were with NAD after initial treatment: 5 reactivated and 4 remain with NAD after retreatment with indomethacin. Toxicity was not significant. We conclude that indomethacin is a well tolerated and active drug in patients with symptomatic bone disease. The results support the concept that chemotherapy may not be necessary for limited bone disease.
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PMID:Symptomatic bone langerhans cell histiocytosis treated at diagnosis or after reactivation with indomethacin alone. 2497 2


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