current lymphoma classification: new concepts and practical applications-triumphs and woes

The World Health Organization [WHO] classification of lymphomas updated in 2008 represents an international consensus for diagnosis of lymphoid neoplasms based on the recognition of distinct disease entities by applying a constellation of clinical and laboratory features. The 2008 classification has refined and clarified the definitions of well-recognized diseases, identified new entities and variants, and incorporated emerging concepts in the understanding of lymphoid neoplasms. Rather than being a theoretical scheme this classification has used data from published literature. Recent knowledge of molecular pathways has led to identification and development of new diagnostic tools, like gene expression profiling, which could complement existing technologies. However, some questions remain unresolved, such as the extent to which specific genetic or molecular alterations define certain tumors. In general, practical considerations and economics preclude a heavily molecular and genetic approach. The significance of early or precursor lesions and the identification of certain lymphoid neoplasms is less clear at present, but understanding is evolving. The borderline categories having overlapping features with large B-cell lymphomas, as well as some of the provisional entities, are subject to debate and lack consensus in management. Lastly, the sheer number of entities may be overwhelming, especially, for the diagnosing pathologist, who do not see enough of these on a regular basis

Successful treatment of steroid-refractory autoimmune thrombocytopenia associated with Castleman disease with anti-CD-20 antibody [rituximab]

Multicentric Castleman disease [MCD] is a lymphoproliferative disorder of incompletely understood etiology and with various clinical presentations. The best therapeutic option for this disease is not well established. MCD is known to be associated with autoimmune phenomena. A 70-year-old female patient of MCD with progressive nodal disease associated with autoimmune thrombocytopenia failed steroid treatment and showed a transient response to intravenous immunoglobulin. The patient achieved complete recovery of her platelet count and a very good response in nodal disease after 3 weekly doses of anti-CD-20 antibody [rituximab]. Anti-CD20 antibody treatment could be a good therapeutic option for MCD, mainly when associated with immune-related disorders

Improved survival with combined chemo-radiotherapy in primary central nervous system lymphoma

Primary CNS lymphoma [PCNSL] is an aggressive primary brain tumor. Cranial irradiation alone rarely results in long term disease control or prolonged survival. We retrospectively analyzed data on the effect of adding high-dose methotrexate [HDMTX] prior to whole brain irradiation [WBI]. All patients with PCNSL diagnosed and managed during 1991-2004 were identified and demographic characteristics, prognostic factors, treatment and outcome were reviewed. Of 62 patients, 10 were excluded [4 had WBI < 40 Gy and 6 had no treatment]. Radiation alone was considered curative with a dose > 40 Gy. Combined modality therapy included 3-4 cycles of HDMTX [3g/m2] followed by WBI. Of 52 patients analyzed for outcome, 36 had WBI [dose > 40 Gy], 16 received 3-4 cycles of HDMTX followed by WBI [combined modality therapy [CMT]]. Median age was 48.2 years; 42 years in the CMT group, 51 years in WBI. Patient characteristics were comparable between two groups except for higher multifocal tumor in the CMT group [92% vs. x22%, P=.029]. Median follow up was 12.83 +/- 6.4 months. The hazard ration for an event was 0.64 [95% CI, 0.52-0.98] and for death 0.58 [95% CI, 0.48-0.92], both in favor of CMT. Univariate regression analysis using one-way analyses of variance [ANOVA] and multivariate Cox regression analysis for prognostic factors including age [< 60 vs. >60], ECOG PS [0-2 vs. 3-4], extent of surgery [biopsy vs. debulking], solitary vs multifocal tumor and dose of radiation therapy [>50Gy vs. >50 Gy] failed to identify any prognostic factor. This retrospecitive comparison supports phase II trial results that indicate that high-dose methotrexate followed by WBI in PCNSL improves outcome

Lung cancer

Lung cancer is the leading cause of cancer-related mortality. Since tobacco smoking is the cause in vast majority of cases, the incidence of lung cancer is expected to rise in those countries with high or rising incidence of tobacco smoking. Even though populations at risk of developing lung cancer are easily identified, mass screening for lung cancer is not supported by currently available evidence. In the case of non-small cell lung cancer, a cure may be possible with surgical resection followed by post-operative chemotherapy in those diagnosed at an early stage. A small minority of patients who present with locally advanced disease may also benefit from pre-operative chemotherapy and/or radiation therapy to down stage the tumor to render it potentially operable. In a vast majority of patients, however, lung cancer presents at an advanced stage and a cure is not possible with currently available therapeutic strategies. Similarly, small cell lung cancer confined to one hemi-thorax may be curable with a combination of chemotherapy and thoracic irradiation followed by prophylactic cranial irradiation, if complete remission is achieved at the primary site. Small cell lung cancer that is spread beyond the confines of one hemi-thorax is, however, considered incurable. In this era of molecular targeted therapies, new agents are constantly undergoing pre-clinical and clinical testing with the aim of targeting the molecular pathways thought be involved in etiology and pathogenesis of lung cancer