Hodgkin lymphoma

Abstract

Hodgkin lymphoma (HL) is a curable malignancy which shows a bimodal curve in incidence in economically developed countries; there is a putative association with Epstein-Barr virus. The WHO 2008 classification schema recognises two histological types of HL: the nodular lymphocyte predominant and the "classic" HL. The latter encompasses four entities: nodular sclerosis, mixed cellularity, lymphocyte depletion, and lymphocyte-rich. Most patients with HL present with asymptomatic superficial lymphadenopathy. The commonest sites of disease are the cervical, supraclavicular and mediastinal lymph nodes, while sub-diaphragmatic presentations and bone marrow and hepatic involvement are less common. Splenic involvement is usually concomitant with hepatic disease and systemic symptoms; extranodal presentations are quite rare. Systemic symptoms are present in ∼35% of cases. The stage of disease is defined according to the Ann Arbor staging system or its Cotswolds variant, and staging work-up includes physical examination, chest X-rays, chest and abdominal CT scan, and bone marrow biopsy. (18)FDG-PET ((18)fluordeoxyglucose positron emission tomography) plays a central role in staging, response assessment and prognosis definition. Classic HL usually spreads by contiguity within the lymphatic tissue network, with a late extension to adjacent and distant viscera. Mortality from HL has been progressively decreasing, as confirmed by the most recent 5-year survival figure of 81%. The list of putative prognostic factors in HL has been increasing, but most factors still require prospective validation. Some of these variables are used to stratify early-stage disease into "favourable" and "unfavourable" categories, with "unfavourable early-stage" being intermediate between "favourable early-stage" and "advanced-stage". ABVD (adriamycin(doxorubicin), bleomycin, vinblastine, dacarbazine) combination chemotherapy followed by involved-field irradiation is the standard treatment for patients with early-stage HL, with a 5-year OS >95%. Several trials assessing less intensive approaches for patients with favourable early-stage HL are ongoing. More intensified combinations, such as the BEACOPP (bleomycin, etoposide, adriamycin, cyclophosphamide, vincristine (Oncovin), procarbazine, prednisone) regimen, are being investigated, usually in patients with unfavourable early-stage HL and interim PET+. ABVD is the standard chemotherapy treatment also for patients with advanced disease. Although some evidence suggests that more intensive combinations provide better disease control, the inevitable increased risk of relevant late toxicity worries investigators. Consequently, there has been a shift towards investigating the innovative strategy of a more aggressive schedule for patients with (18)FDG-PET positive results after the first 2 courses of ABVD. High-dose chemotherapy supported by ASCT (autologous stem cell transplantation) is considered the standard of care in patients with HL which has relapsed after, or is refractory to conventional chemoradio...

Improved Outcomes after Autologous Bone Marrow Transplantation for Children with Relapsed or Refractory Hodgkin Lymphoma: Twenty Years Experience at a Single Institution

“…Some of this decrease in TRM may be because of improvement in supportive care or in RT utilization, or because of changes in patient selection practices. Another factor that may contribute to the decrease in TRM, and perhaps also to overall improved outcomes, is that in efforts to decrease toxicity in more recent years, patients might have received less therapy before BMT and, thus, may be in a better position to respond to and to survive auBMT [26]. For example, some patients in the more recent cohort may have received less bleomycin than they would have in previous years and thus contributed to the decrease in pulmonary events seen in the more recently treated patients.…”

CD20 role in pathophysiology of Hodgkin’s disease

“…The most common drug therapy regimen is adriamycin/doxorubicin, bleomycin, vinblastine and dacarbazine (ABVD). 7 Most patients with classical HL have full remission after initial treatment; however, about 34% of patients with advanced disease and 15% of those with early disease experience a relapse after treatment. 8 …”

“…Also, HRS cells present hypermutation in variable regions of the immunoglobulin heavy chain gene. 7 Other mechanisms involved are deregulated expression of B cell molecules, down-regulation of B cell transcription factors and epigenetic silencing of B cell genes. EBV has potential to express LMP1 and LMP2A, which mimic key signals of growth and survival of B cells.…”

Hodgkin Lymphoma

“…The staging of disease is important and is usually achieved by workup including physical examination, chest X-ray, chest and abdominal CT scan, and bone marrow biopsy. Recently, it has been shown that 18-fluorodeoxyglucose positron emission tomography is useful for staging of Hodgkin lymphoma [7]. Staging of Hodgkin lymphoma includes the following: I Stage I: Involvement of a single lymph node region or lymphoid structure (e.g., Waldeyer ring, thymus, and spleen) I Stage II: Involvement of two or more lymph node regions on the same side of the diaphragm I Stage III: Involvement of lymph node regions or structures, both sides of the diaphragm I Stage IV: Diffuse or disseminated involvement of one or more extralymphatic organs, including any involvement of liver or bone marrow.…”