Risk stratification for Splenic Marginal Zone Lymphoma based on haemoglobin concentration, platelet count, high lactate dehydrogenase level and extrahilar lymphadenopathy: development and validation on 593 cases

Abstract

SummaryThis international retrospective study of 593 Splenic Marginal Zone Lymphoma (SMZL) patients aimed to identify factors that determine treatment initiation and influence lymphoma-specific survival (LSS). Logistic regression was used to identify the factors associated with treatment. A Cox regression was used to analyse LSS in a derivation cohort of 366 patients. This produced a prognostic index (PI) and enabled the identification of three risk groups. The resulting stratification was validated in another cohort of 227 patients and compared with the Interguppo Italiano Linfomi (IIL) score in the group of 450 patients for whom all the required data were available using an extension of the net reclassification improvement. Haemoglobin concentration (Hb), extrahilar lymphadenopathy and hepatitis C virus status were associated with the initiation of treatment. Hb, platelet count, high lactate dehydrogenase level and extrahilar lymphadenopathy were independently associated with LSS. Three risk groups with significantly different five-year LSS (94%, 78% and 69%, respectively) were identified. This stratification (named HPLL on the basis of determinant factors) had a better discriminative power than the IIL score. This system is useful for stratifying SMZL patients into risk groups and may help in the selection of risk-tailored treatment approaches.

Efficacy of bendamustine and rituximab in splenic marginal zone lymphoma: results from the phase II BRISMA/IELSG36 study

“…The estimated 3‐year PFS, EFS and OS rates were 90% (95% CI: 77–96), 80% (95% CI, 65–89) and 96% (95% CI: 84–98), respectively (Fig ). The limited number of events did not enable conclusions to be drawn regarding PFS stratified by risk group (Intergruppo Italiano Linfomi [IIL] score, P = 0·70; Haemoglobin‐Platelet‐LDH‐extra‐hilar‐Lymphadenopathy [HPLL] score, P = 0·11; Arcaini et al , ; Montalbán et al , ). The estimated 3‐year PFS and OS of the 10 patients who did not complete the scheduled protocol treatment cycles was 63% and 90%, respectively.…”

“…Splenic marginal zone lymphoma (SMZL) (Piris et al , ) is a rare indolent B‐cell lymphoma, involving the spleen, bone marrow (BM) and, frequently, the blood. The disease is often asymptomatic, indolent and a minority of patients never need treatment (Montalbán et al , ); however, approximately 70% of cases eventually requires treatment. Because of the rarity of the disease, no randomized trials are available for SMZL, and information on therapeutic options are mostly based on retrospective series (Iannitto et al , ; Else et al , ; Cervetti et al , ; Kalpadakis et al , ; Xing et al , ; Perrone et al , ).…”

Splenic marginal zone lymphoma: a literature review of diagnostic and therapeutic challenges

“…Patients were stratified in three groups as shown in Table 1. The criteria of the IIL were applied to the same population but the stratification power for SES of the HPLL score were better,43, 44 so this seems to be the most suitable score so far.…”

Prognostic Assessment in Patients with Indolent B-Cell Lymphomas

“…Applying specific cut-points low-, intermediate- and high-risk groups with significantly different 5-year LSS of 94%, 78%, and 67%, respectively, were identified. In the validation set the system also separated 3 groups with significantly different 5-year LSS [43]. …”