Hodgkin M.; Hodgkin's disease; Hodgkin's sarcoma; Lymphogranuloma papulosum disseminatum; Lymphogranulomatosis; lymphogranulomatosis maligna; lymphogranulomatous ulcer; Paltauf Steinberg disease; Paltauf-Steinberg disease
HistoryThis section has been translated automatically.
Hodgkin, 1832; Paltauf, 1897; Sternberg, 1898; Reed, 1902
DefinitionThis section has been translated automatically.
A malignant disease of the lymphoreticular system histologically characterized by atypical mononuclear (Hodgkin cells) or polynuclear (Sternberg-Reed cells) atypical cells in a granulomatous tissue. Clinically unilocular onset, spread via adjacent lymph node stations and later hematogenic dissemination.
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ClassificationThis section has been translated automatically.
Four stages are distinguished depending on the spread or pattern of infestation:
- Stage 1: Infestation of a lymph node region (I) or localized extralymphatic focus (IIE).
- Stage 2: two or more regions equilateral to the diaphragm (II) or solitary extralymphatic focus and/or one or more lymph node regions equilateral to the diaphragm (IIIE).
- Stage 3: Infection on both sides of the diaphragm (III), spleen (IIIIS) or localized extralymphatic foci (IIIIE) or both (IIIISIE)
- Stage 4: Disseminated organ infestation.
Occurrence/EpidemiologyThis section has been translated automatically.
Incidence: 2-3/100.000 inhabitants/year.
EtiopathogenesisThis section has been translated automatically.
Unclear, possible virus infection (Epstein-Barr virus).
ManifestationThis section has been translated automatically.
Especially 3rd to 4th decade of life, possible at any age. Men are 1-2 times more frequently affected than women.
Clinical featuresThis section has been translated automatically.
- Specific skin lesions (0.5-3.5% of patients): Blurred, plate-like infiltrates. Also single, multiple or papulo-nodose exanthema, brownish to livid-red, cutaneous-subcutaneous nodules. Tendency to necrotizing ulceration: Ulcus lymphogranulomatosus, especially on the trunk, lower abdomen, inguinal and thigh region and head. Specific infiltrates in the lymphatic throat ring with a tendency to ulceration.
- Non-specific skin changes (in 30-50% of patients): agonizing, drug-induced pruritus with scratching effects, impetiginization, eczematization and lichenification, erythroderma, blistering, pyoderma. Diffuse, Addison's disease like hyperpigmentation on skin and mucous membranes. Prurigo symptomatica, Prurigo lymphogranulomatotica. Ichthyosis-like changes; zoster generalisatus.
- General symptoms: Enlargement of lymph nodes, especially in the neck and armpits, weight loss. In about one third of patients pain in the lymphogranulomatous foci a few minutes after the slightest alcohol consumption, intermittent lymphogranulomatous fever (Pel-Ebstein fever) with recurrent character. In the early stages: a few days of fever alternating with fever-free intervals. In the late stages: appearance of Pel-Ebstein fever with retroperitoneal lymphomas.
LaboratoryThis section has been translated automatically.
Absolute lymphocytopenia in 3/4 of the patients in the phase of generalization. SPA elevation, increase in γ globulins.
HistologyThis section has been translated automatically.
Depending on the cell infiltrate there are 5 different forms:
- Diffuse lymphocytic and/or histiocytic form (about 5% of cases)
- Nodular lymphocytic and/or histiocytic form (about 80% of cases)
- Mixed-cell form (approx. 10-15% of cases)
- Diffuse fibrosis (about 1% of cases)
- Low lymphocyte form (about 1% of cases)
DiagnosisThis section has been translated automatically.
Trial excision of an enlarged lymph node.
Differential diagnosisThis section has been translated automatically.
TherapyThis section has been translated automatically.
Polychemotherapy or irradiation by haematologists or radiotherapists.
Progression/forecastThis section has been translated automatically.
10-year survival rate of 60%. The prognosis will be less favourable one to five years.
LiteratureThis section has been translated automatically.
- Ambinder R (2003) Infection and lymphoma. N Engl J Med 349: 1309-1311
- Browne P et al (2003) The B-cell transcription factors BSAP, Oct-2, and BOB.1 and the pan-B-cell markers CD20, CD22, and CD79a are useful in the differential diagnosis of classic Hodgkin lymphoma. At J Clin Pathol 120: 767-777
- Hjalgrim H et al (2003) Characteristics of Hodgkin's lymphoma after infectious mononucleosis. N Engl J Med 349: 1324-1332
- Hodgkin T (1832) On some morbid appearances of the absorbent glands and spleen. Medico-Surgical Transactions (London) 17: 68-114
- Listinsky CM et al (2002) A practical approach to the diagnosis of Hodgkin lymphoma. Am J Clin Pathol 117S: S76-94
- Macaya A et al (2003) Cutaneous granulomas as the first manifestation of Hodgkin's disease. Eur J Dermatol 13: 299-301
- Paltauf R (1889) On the relationship of the thymus to sudden death. Vienna Klin Wschr 46: 877-881
- Paltauf R (1897) Lymphosarcoma (lymphosarcomatosis, pseuloleukemia, myeloma, chloroma). Results of general pathology and pathological anatomy of humans and animals 3: 652-691
- Reed D (1902) On the pathological changes in Hodgkin's disease, with especial reference to its relation to tuberculosis. Johns Hopkins Hospital Reports (Baltimore) 10: 133-196
- Sternberg C (1898) On a peculiar tuberculosis of the lumphatic apparatus, which develops in the form of pseudoleukemia. Journal of Medicine (Prague) 19: 21-90
- Winzer M, Müller W, Arnholdt H (1991) Specific skin infiltrates in Hodgkin's disease. dermatologist 42: 648-651
Incoming links (17)Brentuximab vedotine; Hodgkin, m.; Hodgkin's disease; Hodgkin's sarcoma; Ichthyosis, paraneoplastic; Lymphadenopathy, dermatopathic; Lymphogranuloma papulosum disseminatum; Lymphogranulomatosis maligna; Lymphogranulomatous ulcer; Lymphomatoids papulose; ... Show all
Outgoing links (8)Cutaneous lymphomas (overview); Cutaneous t-cell lymphomas (overview); Erythroderma; Hyperpigmentation; Lichenification; Pruritus; Pyoderma; Shingles;
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