HOMO SAPIENS DISEASES - HODGKIN'S DISEASE (HD) OR LYMPHOMA (HL) / MALIGNANT LYMPHOGRANULOMA / GRANULOMATOUS LYMPHOMA : a form of malignant lymphoma first described by Thomas Hodgkin in 1832 (On some morbid appearances of the absorbent glands and spleen. Med. Chir.Trans. (London) 17:68-114)

Table of contents :

  • Epidemiology
  • Aetiology
  • Pathogenesis
  • Symptoms & signs
  • Laboratory examinations
  • Differential diagnosis
  • Therapy 
  • Prognosis
  • Web resources

  • Epidemiology : 1% of all malignant tumors of adults; 25% of all lymphomas; incidence : 3 new cases every 100,000/yr. It affects twice as many males as females and has onset at age 20-30 or > 55. Whereas the incidence of NHL has been rising since World War II, the incidence of HL has been flat. 2 age-incidence peaks have long been recognized in North America and Western Europe: young adult and older adult. In developing countries and in parts of Asia, the young adult peak is much less prominent or even absent. Several epidemiologic studies suggested an association with small family size and other factors that might result in delayed exposure to common viral infections.
    Aetiology : HL can be divided into 4 entities on the basis of EBV status and age at presentation, with 3 groups of EBV-associated cases and a single group of EBV-. The aetiology of the latter cases is obscure although involvement of an infectious agent(s) is suspectedref.

    Tumorigenic cell types : HD is a B cell lymphoma originating from germinal centre B cellsref. The explanation for the difficulty in identifying the tumor may be attributed to two of its defining characteristics: the tumor cells are rare in the mass of the tumor despite lymph nodal architecture is destroyed and fail to express many B cell markers—most notably Ig.
    Lymph node histology and pathology : Symptoms & signs : Staging : the major purposes of staging are to determine prognosis and to ascertain which patients can be treated with radiotherapy. In many centers, patients with stage IA and IIA disease are treated with radiotherapy alone and patients with stage IIB, IIIB and IV disease receive chemotherapy. There has been variable practice in stage IB (rare) and IIIA disease. Staging laparotomy with splenectomy introduced at Stanford in the late 1960s has ceased to be widely used, in part because of improved imaging techniques, but largely because of the realization that although surgically staged patients had an improved disease-free survival, the overall survival was not significantly improved. This conclusion was arrived at in a matched pairs analysis of the British National Lymphoma Investigation (BNLI) databaseVaughan Hudson B, 212 and in modeling of the results from the Princess Margaret Hospital in Torontoref. In the European Organization for Research and Treatment of Cancer (EORTC) H2 trial a randomization to staging laparotomy or splenic irradiation was carried out and revealed only a marginal advantage in relapse-free survival for the laparotomy arm and no survival advantageref. The later EORTC H6 study randomized good risk CS I and II patients to laparotomy or not with more extensive irradiation in the non-laparotomized patients, and although there was a significant improvement in freedom from relapse in the laparotomy arm there was no difference in survivalref. This reflects the success of chemotherapy salvage in patients who have failed radiotherapy. Laboratory examinations : [then ... Differential diagnosis : Lennert's lymphoma / lymphoepithelioid lymphoma
    Therapyref : together with NHL, HL comprises only 8% of all malignancies, but patients are young and potentially curable. In contrast to many solid tumors, lymphomas are highly sensitive to chemotherapy or radiation therapy (RT) and long-term cure rates > 80% for HL are achieved. However, the magnitude of late treatment-related morbidity and mortality, especially in early-stage HL patients treated with combination chemo-radiotherapy, as well as the fact that many patients with advanced disease cannot be cured with ABVD chemotherapy, has tempered initial enthusiasm. Accordingly, tailoring the intensity of the treatment to the individual patient has become very topical. Prognosis : complete remission in 80-90% of patients with stage III or IV; relapse within 3 years (=> complete remission after 3 years). 20 will die for HD-related affections; 5-year survival for patients with stage III or IV HD = 65-70%, 85% are cured (less after age 60), but secondary tumors are common  (acute secondary leukemia in 10-15% of those who underwent MOPP or mediastinic radiotherapy; secondary breast and lung cancers) There is a good case for eliminating unnecessary imaging in patients with nonbulky early-stage HL. Not only is the recurrence rate 10% or less with the best standard of care, but also approximately 80% of these recurrences are first identified by the patient or the examining physician without the use of imagingref1, ref2 (Folz LM, Song KW, Connors JM. Who actually detects relapse in Hodgkin's lymphoma: patient or physician. Blood 2004;104:853a-854a). This means that the addition of serial post-therapy imaging provides "earlier" disease detection in only about 2% of treated patients, with no proven benefit (Cheson B. The case against heavy PETing. J Clin Oncol 2009;11:1742-1743). Yet these patients, who are usually young, generally undergo 5 to 10 episodes of surveillance computed tomography (CT) of the chest, abdomen, or pelvis, often in addition to several episodes of positron-emission tomography (PET)–CT, within the initial 3 to 5 years of their treatment, resulting in an effective cumulative dose of over 50 mSv. Physicians should therefore question the use of imaging studies in these patients beyond those performed for assessment of a response or for clear-cut cases in which management decisions must be guided by imaging. At the most, yearly chest radiography, which is associated with a considerably lower radiation dose than CT or PET-CT, should be performed in patients with previous mediastinal disease. Any imaging beyond that is not only unjustified but also potentially harmfulref.

    Web resources : HD at LymphomaInfo

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