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1. Böhm P, Kunz W, Horny HP, Einsele H: Adult Gaucher disease in association with primary malignant bone tumors. Cancer; 2001 Feb 1;91(3):457-62
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Adult Gaucher disease in association with primary malignant bone tumors.
  • BACKGROUND: Malignant neoplastic disorders are more common in patients with Gaucher disease (GD) than in the general population.
  • Very few cases of primary malignant bone tumors in association with GD have been reported to date.
  • Thus, the recommendations for an adequate therapy are often based on limited professional experience.
  • METHODS: The clinical, radiologic, and histologic data of the authors' two patients who had GD and primary malignant bone tumor are presented.
  • Epidemiologic data, clinical data, and treatment results from published reports of 18 patients who had GD and various malignancies and the authors' 2 patients were compared and evaluated.
  • In both cases, the bone marrow architecture was partially effaced by sheets of large histiocytic cells with striated or fibrillary cytoplasm.
  • In both patients, chemotherapy was performed.
  • Whereas the patient who had the leiomyosarcoma showed poor recovery of the bone marrow that necessitated withdrawal of aggressive chemotherapy, the patient who had non-Hodgkin lymphoma and enzyme therapy tolerated the chemotherapy well.
  • In spite of local control after preoperative radiotherapy and hemipelvectomy, the first patient developed lung metastases and finally died.
  • The second patient was continuously free of disease at a follow-up examination 32 months after chemotherapy and radiotherapy.
  • In a total of 20 patients who had malignant disorders and GD, the numbers of males and females were equivalent, and the mean age was 55 years.
  • Approximately 33% presented with a cancer originating in the bone.
  • Of these, after a mean follow-up of 36 months (range, few days-108 mos), only 2 patients were continuously free of disease, and one patient was alive with disease.
  • The other patients had died of disease or hemorrhagic complications of GD.
  • CONCLUSIONS: Because there is a relatively high incidence of malignant disorders of the bone, the study suggested that malignant disorders have to be included in the differential diagnosis of painful lytic lesions in patients who have GD.
  • Evaluation of the expense and value of enzyme therapy to patients who have GD should be undertaken with regard to the incidence of malignant disorders and patient survival.
  • [MeSH-major] Bone Neoplasms / complications. Gaucher Disease / complications
  • [MeSH-minor] Adult. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bleomycin / therapeutic use. Cyclophosphamide / therapeutic use. Doxorubicin / therapeutic use. Etoposide / therapeutic use. Female. Humans. Ifosfamide / therapeutic use. Male. Prednisone / therapeutic use. Vincristine / therapeutic use

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  • [Copyright] Copyright 2001 American Cancer Society.
  • (PMID = 11169926.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 11056-06-7 / Bleomycin; 5J49Q6B70F / Vincristine; 6PLQ3CP4P3 / Etoposide; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide; UM20QQM95Y / Ifosfamide; VB0R961HZT / Prednisone; VACOP-B protocol
  • [Number-of-references] 22
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2. Wüstenberg EG, Theissig F, Offergeld C, Hüttenbrink KB: [Necrotizing lymphadenitis (Kikuchi-Fujimoto disease) as a rare cause of cervical lymphadenopathies. Diagnosis and differential diagnosis]. Laryngorhinootologie; 2000 Feb;79(2):93-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Necrotizing lymphadenitis (Kikuchi-Fujimoto disease) as a rare cause of cervical lymphadenopathies. Diagnosis and differential diagnosis].
  • We report on three cases of Kikuchi-Fujimoto disease as a benign self-limiting lymphadenopathy of unknown etiology, which is usually found in young women between 20 and 30 years of age.
  • The correct diagnosis requires the histologic examination of the lymph node.
  • Even for the experienced pathologist the differential diagnosis of malignant systemic disorders can be difficult.
  • PATIENTS: Three female patients suffering from Kikuchi-Fujimoto disease were treated in our department between September 1997 and March 1998.
  • In two of the three cases, histologic lymph node examination revealed T-cell lymphoma and Hodgkin's disease and in one case led to initial chemotherapeutic treatment.
  • As a result of a second histologic examination, both diagnoses were reviewed and Kikuchi-Fujimoto disease was diagnosed.
  • Chemotherapy was discontinued.
  • CONCLUSIONS: Kikuchi-Fujimoto disease is a benign, self-limiting lymphadenopathy which is usually diagnosed in young women.
  • Distinguishing Kikuchi-Fujimoto disease from malignant systemic disorders may even be difficult for pathologists using histological examination techniques and requires close cooperation between the ENT specialist and the pathologist.
  • In order to minimize the risk of misdiagnosis and incorrect treatment, the ENT specialist should inform the pathologist about the differential diagnosis of Kikuchi-Fujimoto disease in such cases.
  • [MeSH-major] Histiocytic Necrotizing Lymphadenitis / diagnosis. Lymph Nodes / pathology
  • [MeSH-minor] Adolescent. Adult. Cell Division / physiology. Diagnosis, Differential. Female. Humans. Hyperplasia. T-Lymphocytes / pathology

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  • (PMID = 10738716.001).
  • [ISSN] 0935-8943
  • [Journal-full-title] Laryngo- rhino- otologie
  • [ISO-abbreviation] Laryngorhinootologie
  • [Language] ger
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] GERMANY
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3. Fluri S, Gebbers JO: [Langerhans cell histiocytoses: 50 years to histiocytosis X]. Praxis (Bern 1994); 2004 Mar 31;93(14):559-65
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Histiocytoses encompass a heterogeneous group of histiocytic disorders that may be separated in a group of variable biological behaviour and in a clearly malignant group.
  • The histopathologic diagnosis of all types of histiocytoses is always mandatory.
  • It has a wide range of manifestations: self-healing cutaneous, benign solitary and malignant systemic manifestations require the staging in order to evaluate a possibly necessary therapy.
  • Chemotherapy should be performed within international protocols.
  • [MeSH-minor] Anti-Bacterial Agents / therapeutic use. Antineoplastic Agents / therapeutic use. Combined Modality Therapy. Histocytochemistry. Humans. Immunosuppressive Agents / therapeutic use. Microscopy, Electron. Prognosis. Radiotherapy Dosage

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  • (PMID = 15125070.001).
  • [ISSN] 1661-8157
  • [Journal-full-title] Praxis
  • [ISO-abbreviation] Praxis (Bern 1994)
  • [Language] ger
  • [Publication-type] Comparative Study; English Abstract; Journal Article; Review
  • [Publication-country] Switzerland
  • [Chemical-registry-number] 0 / Anti-Bacterial Agents; 0 / Antineoplastic Agents; 0 / Immunosuppressive Agents
  • [Number-of-references] 0
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