Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0019829 (Hodgkin's disease)
 30,247 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Clinical, pathological, and immunological analysis of 20 patients with ocular adnexal lymphoid disease has demonstrated several parameters which are useful for distinguishing malignant from benign lesions. Patients in the fourth or fifth decade of life presenting with an acute history of pain, oedema, epiphora, double vision, and ptosis, with a mass localised in the lacrimal gland area, are more likely to have a pseudolymphoma or a chronic inflammatory lesion than a true non-Hodgkin lymphoma (NHL). It is not possible to obtain a definite diagnosis without surgical intervention, because only three out of nine patients with orbital NHL had evidence of a monoclonal B cell population in peripheral blood on admission to the Orbital Centre. Furthermore it was confirmed that the identification of the various orbital lymphoid infiltrates becomes more distinct when immunological techniques are added to the clinical and histopathological methods of investigation. Multidisciplinary cooperation leads to further improvement of diagnosis and treatment of ocular adnexal lymphoproliferative disease.
 ...
PMID:Lymphoid proliferations in the orbit: malignant or benign? 639 35

We report on a boy with Rapp-Hodgkin syndrome (RHS) or Rapp-Hodgkin ectodermal dysplasia. He had sparse, wiry, slow growing and uncombable hair, but no pili torti or pili canaliculi characteristic of RHS. He also had sparse eyelashes and eyebrows, and obstructed lacrimal puncta and epiphora. Bilateral bony external auditory canal stenosis led to hearing loss. The mouth was small with repaired bilateral cleft lip and palate. Oral manifestations included hypodontia, microdontia, unerupted mandibular premolars with well formed roots, large dental pulp spaces, enamel hypoplasia, multiple caries, glossy tongue, and congenital absence of lingual frenum and of sublingual caruncles including submandibular and sublingual salivary duct openings. Palmo-plantar keratoderma, unerupted premolars, congenital absence of lingual frenum, sublingual caruncles, glossy tongue, and pili canaliculi seen in the patient are newly recognized findings of this syndrome. Overlapping findings of RHS ectrodactyly-ectodermal dysplasia-clefting syndrome (EEC), and ankyloblepharon-ectodermal defects-cleft lip and palate syndrome (AEC) are discussed.
 ...
PMID:Rapp-Hodgkin syndrome with palmoplantar keratoderma, glossy tongue, congenital absence of lingual frenum and of sublingual caruncles: newly recognized findings. 977 99

Malignant tumors of the lacrimal sac are uncommon, and primary non-Hodgkin lymphomas are rare. We report the case of an 80-year-old woman presenting with epiphora and swelling of the left lacrimal area. A dacryocystorhinostomy was programmed, but within few weeks a painful tumor appeared with cervical nodes. Pathological studies of the mass revealed diffuse malignant lymphoma with large B cells. The lymphoma was classified as stage IIE using Ann Arbor staging after clinical, biological and radiological examination. Six CHOP systemic chemotherapy were proposed. After 12 months and only 4 cures because of hematological complications and cardiac toxicity, the patient was disease-free. Primary non-Hodgkin lymphoma of the lacrimal sac is extremely rare. Collaboration between the ophthalmologist, anatomopathologist and oncologist allowed us to determine the type and the extension and to propose an aggressive but successful treatment in this highly malignant and very localized lymphoma.
 ...
PMID:[A case of primary non-Hodgkin lymphoma of the lacrimal sac]. 1251 39

Rapp-Hodgkin syndrome (RHS) is an autosomal dominant disorder characterized by ectodermal dysplasia and cleft lip/cleft palate. Very recently, mutations in p63 have been identified as a cause of RHS; to date five such mutations have been identified. We describe a Thai girl with RHS. She had short stature, ectodermal dysplasia, epiphora, cleft lip, cleft palate, and normal development. Mutation analysis for the entire coding region of p63 identified a novel and de novo mutation, 1622C--> A (S541Y), in the SAM domain, predicting an abnormal alpha tail of the p63alpha protein isotypes. This observation supports that majority of patients with RHS are caused by mutations affecting the tail of p63alpha, a region that also contains most of the pathogenic mutations in ankyloblepharon-ectodermal dysplasia-clefting (AEC) syndrome.
 ...
PMID:De novo missense mutation, S541Y, in the p63 gene underlying Rapp-Hodgkin ectodermal dysplasia syndrome. 1580 90

The purpose is to report an unusual case of orbital non-Hodgkin lymphoma. A 75-year-old man presented with bilateral chronic epiphora complaint and inferior eyelid tumors, axial proptosis, without previous systemic manifestation. The patient was submitted to bilateral endonasal dacryocystorhinostomy twice and the epiphora complaint persisted. The inferior eyelid and bone marrow biopsy revealed non-Hodgkin lymphoma. The patient was treated with systemic chemotherapy and dacryocystorhinostomy with good resolution. The precise diagnosis and the treatment were very important to reach a good resolution of the bilateral epiphora complaint.
 ...
PMID:[Non-Hodgkin orbital lymphoma: case report]. 1851 34

We report a case of primary non-Hodgkins lymphoma of the lacrimal sac in a 60-year-old Asian lady, who presented with persistent epiphora and recurrent medial canthal swelling. Primary lymphoma of the lacrimal sac is rare and it can be easily misdiagnosed. Delayed in diagnosis may be related to mortality. To minimize the risk of overlooking specific pathology it is important to assess the appearance of the lacrimal sac and its surrounding structures intraoperatively. Biopsy of the lacrimal sac is required in cases where specific pathology is suspected.
 ...
PMID:Primary non-Hodgkins lymphoma of the lacrimal sac: mortality-related epiphora. 1987 26

Rapp-Hodgkin syndrome is a rare form of ectodermal dysplasia involving the hair, eyes, sweat glands, nails, teeth and palate. This syndrome is characterized by stiff sparse hair with the appearance of steel wool, sparse eyebrows and lashes, cleft palate, absence of lacrimal punctae, epiphora, a decreased number of sweat glands, and dystrophic nails. Cleft palate, hypodontia, abnormal tooth shape, multiple caries, delayed eruption of teeth are the main oral manifestations. In this paper we describe the clinical and dental findings of this syndrome in a 7 year old girl referred to the dental clinic because of severe tooth ache. The dental treatment included root canal treatment, composite restorations, extractions and removable prostheses. This paper highlights features of Rapp-Hodgkin Syndrome and its dental rehabilitation.
 ...
PMID:Rapp-Hodgkin syndrome: clinical and dental findings. 1995 14

Primary diffuse large B-cell lymphoma of the lacrimal sac is rare. Herein we report a 55-year-old female presented with epiphora in the right eye. Distention of the lacrimal sac secondary to nasolacrimal duct obstruction was observed. She was scheduled for external dacryocystorinostomy for the next month. When she came for surgery, a growing mass was recognised over the lacrimal sac region. On computer tomography scan, a subdermal mass causing nasal bone destruction was detected. Excisional biopsy of the mass was performed. Histopathologic and immunohistochemical evaluations revealed primary diffuse large B-cell non-Hodgkin lymphoma of the lacrimal sac. She was treated with cyclophospamide, vincristine, adriablastine and prednisone for eight courses combined with rituximab for 6 months. During a follow-up period of 25 months, patient is stable with no systemic disease. Although rare, lacrimal sac tumors can mimic dacryocystitis and must be considered in differential diagnosis. In suspicious cases incisional biopsy is recommended.
 ...
PMID:Primary diffuse large B-cell lymphoma of the lacrimal sac simulating chronic dacryocystitis. 2135 9

Midline Destructive Lesions (MDL) are well known to cause nasal problems. There is a long differential diagnosis of such lesions. However, in the pediatric population, the 2 main diseases to be aware of are Non-Hodgkin's T-cell lymphoma and granulomatosis with polyangiitis (previously known as Wegener's granulomatosis). The authors present the report of a 15-year-old boy who presented with epiphora, chemosis, and limitation of left abduction. CT scan of his orbits suggested a destructive lesion of the ethmoid sinuses. His laboratory investigations revealed a positive ANCA. The patient underwent endoscopic sinus surgery, and this was characteristic for granulomatosis with polyangiitis. He was treated with systemic steroids and then maintained on cyclophosphamide, which controlled his disease activity. This case highlights the need for ophthalmologists to have a high index of suspicion for MDL and concomitant orbital disease.
...
PMID:Orbital presentation of a nasal midline destructive lesion in a young boy. 2479 18

Primary non-Hodgkin lymphoma (NHL) of lacrimal drainage system (LDS) is quite rare in children, but it is important to expedite early diagnosis in an effort to alter possible life-threatening disease since they are usually misdiagnosed as chronic dacryocystitis. In the literature, there are few examples of tumors of LDS in children. The authors herein report two pediatric cases of extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) originating from lacrimal sac in an attempt to increase the knowledge about the clinical course of NHL of LDS. Considerable care must be taken since tumors of lacrimal drainage can mimic dacryocystitis clinically and macroscopically. Two patients both attended with painless swelling in the left lacrimal sac region and epiphora of the left eye. Orbital magnetic resonance imaging showed a tumoral lesion in the left lacrimal sac region and histopathological examination of excisional biopsy specimen demonstrated MALT lymphoma in both patients. The treatment regimen comprises lacrimal sac excision within the tumor, canalicular dacryocystorhinostomy (DCR) with bicanalicular silicone intubation (BSI) combined with chemotherapy and regional radiotherapy in one case, whereas the second case received only radiotherapy after canalicular DCR with BSI. Both of them maintained clinical remission along follow-up.
 ...
PMID:Primary mucosa-associated lymphoid tissue (MALT) lymphoma of the lacrimal drainage system in two pediatric patients. 3086 84


1