Hodgkin's lymphoma C81.9

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Hodgkin M.; Hodgkin's disease; Hodgkin's sarcoma; Lymphogranuloma papulosum disseminatum; Lymphogranulomatosis; lymphogranulomatosis maligna; lymphogranulomatous ulcer; Paltauf Steinberg disease; Paltauf-Steinberg disease

History
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Hodgkin, 1832; Paltauf, 1897; Sternberg, 1898; Reed, 1902

Definition
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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.

Classification
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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/Epidemiology
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Incidence: 2-3/100.000 inhabitants/year.

Etiopathogenesis
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Unclear, possible virus infection (Epstein-Barr virus).

Manifestation
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Especially 3rd to 4th decade of life, possible at any age. Men are 1-2 times more frequently affected than women.

Clinical features
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  • 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.

Laboratory
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Absolute lymphocytopenia in 3/4 of the patients in the phase of generalization. SPA elevation, increase in γ globulins.

Histology
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Depending on the cell infiltrate there are 5 different forms:
  1. Diffuse lymphocytic and/or histiocytic form (about 5% of cases)
  2. Nodular lymphocytic and/or histiocytic form (about 80% of cases)
  3. Mixed-cell form (approx. 10-15% of cases)
  4. Diffuse fibrosis (about 1% of cases)
  5. Low lymphocyte form (about 1% of cases)
In the early phase, the lymph nodes show reticulum cell proliferations, then an assertion with neutrophilic and eosinophilic granulocytes, scarring, mononuclear (Hodgkin cells) and polynuclear (Sternberg-Reed giant cells) as well as atypical reticular cells. Skin biopsies are often less characteristic. Hodgkin and Sternberg-Reed cells are CD30/Ki 1 positive.

Diagnosis
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Trial excision of an enlarged lymph node.

Differential diagnosis
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Therapy
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Polychemotherapy or irradiation by haematologists or radiotherapists.

Progression/forecast
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10-year survival rate of 60%. The prognosis will be less favourable one to five years.

Literature
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  1. Ambinder R (2003) Infection and lymphoma. N Engl J Med 349: 1309-1311
  2. 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
  3. Hjalgrim H et al (2003) Characteristics of Hodgkin's lymphoma after infectious mononucleosis. N Engl J Med 349: 1324-1332
  4. Hodgkin T (1832) On some morbid appearances of the absorbent glands and spleen. Medico-Surgical Transactions (London) 17: 68-114
  5. Listinsky CM et al (2002) A practical approach to the diagnosis of Hodgkin lymphoma. Am J Clin Pathol 117S: S76-94
  6. Macaya A et al (2003) Cutaneous granulomas as the first manifestation of Hodgkin's disease. Eur J Dermatol 13: 299-301
  7. Paltauf R (1889) On the relationship of the thymus to sudden death. Vienna Klin Wschr 46: 877-881
  8. Paltauf R (1897) Lymphosarcoma (lymphosarcomatosis, pseuloleukemia, myeloma, chloroma). Results of general pathology and pathological anatomy of humans and animals 3: 652-691
  9. 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
  10. 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
  11. Winzer M, Müller W, Arnholdt H (1991) Specific skin infiltrates in Hodgkin's disease. dermatologist 42: 648-651

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Last updated on: 29.10.2020