Multicentre retrospective study of intravascular large B‐cell lymphoma treated at academic institutions within the United States

Peer Reviewedhttps://deepblue.lib.umich.edu/bitstream/2027.42/149677/1/bjh15923.pdfhttps://deepblue.lib.umich.edu/bitstream/2027.42/149677/2/bjh15923_am.pd

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 chemoradiotherapy, while allogeneic transplant is a suitable tool for patients with chemorefractory disease and patients failed after ASCT

A reappraisal of the diagnostic and therapeutic management of uncommon histologies of primary ocular adnexal lymphoma

Lymphoma is the most common malignancy arising in the ocular adnexa, which includes conjunctiva, lachrymal gland, lachrymal sac, eyelids, orbit soft tissue, and extraocular muscles. Ocular adnexal lymphoma (OAL) accounts for 1%-2% of non-Hodgkin lymphoma and 5%-15% of extranodal lymphoma. Histology, stage, and primary localizations are the most important variables influencing the natural history and therapeutic outcome of these malignancies. Among the various lymphoma variants that could arise in the ocular adnexa, marginal zone B-cell lymphoma (OA-MZL) is the most common one. Other types of lymphoma arise much more rarely in these anatomical sites; follicular lymphoma is the second most frequent histology, followed by diffuse large B-cell lymphoma and mantle cell lymphoma. Additional lymphoma entities, like T-cell/natural killer cell lymphomas and Burkitt lymphoma, only occasionally involve orbital structures. Because they are so rare, related literature mostly consists of anecdotal cases included within series focused on OA-MZL and sporadic case reports. This bias hampers a global approach to clinical and molecular properties of these types of lymphoma, with a low level of evidence supporting therapeutic options. This review covers the prevalence, clinical presentation, behavior, and histological and molecular features of uncommon forms of primary OAL and provides practical recommendations for therapeutic management