Bjarne Christensen

Diffuse large B-cell lymphoma: clinical implications of extranodal versus nodal presentation – a population-based study of 1575 cases

Differences in genetic origin between nodal and extranodal diffuse large B‐cell lymphomas (DLBCL) exist. Using population‐based data from the registry of the Danish Lymphoma Group, the present study is the first to analyse clinical implications of nodal versus extranodal presentation of DLBCL. Of 4786 newly diagnosed non‐Hodgkins lymphoma patients in a 16‐year period, 1575 (33%) had DLBCL. The annual incidence rate was 2·9 per 100 000; 40% were extranodal. The clinical profile of patients with extranodal DLBCL was different from the nodal DLBCL patients. Extranodal DLBCL was associated with older age and poorer performance score, but also lower tumour burden. In extranodal DLBCL, 51% of the cases were stage I and 36% were stage IV, whereas the patients were relatively equally distributed between the four stages in nodal DLBCL. For stage I patients, extranodal DLBCL was independently associated with poor survival (P = 0·003). In contrast, among stage IV patients those with extranodal DLBCL survived longer (P = 0·009). We conclude that there are important clinical differences between nodal and extranodal DLBCL. The addition of these clinical results to the existing aetiological and genetic data suggests that the distinction between nodal and extranodal DLBCL is not only pathogenetically but also clinically important.

Mantle cell lymphoma: prognostic capacity of the Follicular Lymphoma International Prognostic Index

The International Prognostic Index (IPI) is the most commonly used prognostic model for mantle cell lymphoma (MCL). However, the prognostic value of the IPI is limited. The recently published Follicular Lymphoma International Prognostic Index (FLIPI) is built on variables, which are pertinent to MCL. This study was conducted to evaluate the prognostic value of FLIPI in a population‐based series of 93 patients with MCL diagnosed in a 7‐year period. End points of the study were response to therapy, overall survival, and disease‐free survival (DFS) according to the IPI and FLIPI. Applied to the whole series, the FLIPI identified three risk groups with markedly different outcome with 5‐year overall survival rates of 65%, 42%, and 8% respectively. Notably, the high‐risk group comprised 53% of patients. In contrast, the IPI only allocated 16% of cases to the high‐risk group and had a lower overall predictive capacity. When both the FLIPI and IPI were included in a multivariate analysis, only the FLIPI was related to survival. Multivariate analysis of DFS also identified the FLIPI, and not the IPI, as independently significant. Thus, in the present study, the FLIPI was superior as a prognostic model compared with the IPI and can therefore be recommended as a clinical prognostic index for MCL.

Conditional survival of patients with diffuse large B-cell lymphoma†‡

Prognosis of lymphoma patients is usually estimated at the time of diagnosis and the estimates are guided by the International Prognostic Index (IPI). However, conditional survival estimates are more informative clinically, as they consider those patients only who have already survived a period of time after treatment. Conditional survival data have not been reported for lymphoma patients.

Prognosis of localized diffuse large B‐cell lymphoma in younger patients

The International Prognostic Index (IPI) is widely used as a predictive model in diffuse large B‐cell lymphoma (DLBCL) patients of all ages and stages. To determine the optimal IPI‐based prognostic system at the time of diagnosis in younger patients with limited‐stage DLBCL, the authors evaluated the age‐adjusted IPI and the recently proposed stage‐adjusted IPI, and constructed an IPI‐based model adjusted for both age and stage.

Re‐emergence in remission of primary clone in acute myelogenous leukaemias with multiple chromosomal aberrations at diagnosis

Summary. We describe the clinical, haematological and cytogenetic features of three patients who had acute myelogenous leukaemia (AMD with complex bone marrow karyotypes when first cytogenetically examined. Induction chemotherapy led to remission from the acute leukaemia. However, neither clinically nor morphologically did this remission mean a return to normal haematopoiesis. The two patients who displayed myelodysplastic features before and when AML was diagnosed, again developed myelodysplasia. and the third patient, who had a long history of polycythaemia vera, returned to this myeloproliferative condition. Nor was cytogenetic normalization achieved: instead, abnormal cell clones were found in which all but one of the karyotypic aberrations present at acute leukaemia diagnosis had disappeared. The solitary anomalies that were detected in these reemerging clones must correspond to the primary cytogenetic aberrations of the patients pre‐leukaemic diseases. They were del(5) (q11q33) and del(17) (p11) in the two myelodysplastic cases, and der(18)t(9;18) (p11;p11) in the patient with long‐standing polycythaemia vera. The other, secondary, aberrations were probably the leukaemogenic changes, and with the eradication or reduction of the subclones containing them, the leukaemic phenotype disappeared. The three cases add cytogenetic evidence to the growing understanding that the remission obtained in some AMLs is actually a return to a preleukaemic, myeloproliferative or myelodysplastic. syndrome.