Quantified bleeding history is a cornerstone of haematological assessment in patients presenting with abnormal bleeding. Bleeding scores systematically capture the severity and number of bleeding symptoms to distinguish normal variation from a true bleeding disorder. The ISTH Bleeding Assessment Tool (ISTH-BAT) was developed by the International Society on Thrombosis and Haemostasis to standardise bleeding history collection and replace inconsistent previous tools. It assigns severity scores (0-4) for 14 bleeding categories and has been validated in von Willebrand disease, platelet function disorders, and other inherited bleeding conditions.
Hematology9 results
The PLASMIC Score was developed by Bendapudi and colleagues at Massachusetts General Hospital in 2017 from a derivation cohort of 112 patients with thrombotic microangiopathy (TMA), then externally validated in 63 patients. It was designed to rapidly identify patients with severe ADAMTS13 deficiency (the hallmark of thrombotic thrombocytopenic purpura) before ADAMTS13 results return — which typically takes 2-5 days. Given that TTP mortality without plasma exchange exceeds 90%, earlier identification and treatment is life-saving. The seven PLASMIC criteria can be evaluated at the bedside from routine blood tests available within hours of presentation.
ITP Response Assessment
The International Working Group (IWG) criteria for ITP response assessment were established by Rodeghiero and colleagues in 2009 to standardise the terminology and outcome definitions used in ITP clinical trials and clinical practice — replacing a confusing patchwork of 14 different response criteria used previously. The 2009 IWG guidelines defined complete remission, response, sustained response, and no response using objective platelet count thresholds and treatment-free periods. Immune Thrombocytopenia (ITP) affects approximately 3-5 per 100,000 adults annually, with clinical manifestations ranging from incidental thrombocytopaenia to life-threatening haemorrhage.
The International Prognostic Scoring System (IPSS) for Myelodysplastic Syndromes was developed by Greenberg and colleagues in 1997 from a pooled analysis of 816 MDS patients from seven clinical trials. It uses three variables — cytogenetics, bone marrow blast percentage, and number of cytopenias — to stratify patients into four risk groups (Low, Intermediate-1, Intermediate-2, High) with dramatically different survival and AML transformation rates. The IPSS was revised in 2012 (IPSS-R) with improved cytogenetic risk grouping and continuous scoring to provide better prognostic discrimination, particularly in the lower-risk groups. MDS is a clonal stem cell disorder with annual incidence of ~4 per 100,000, predominantly affecting elderly patients.
The Revised International Prognostic Index (R-IPI) for Diffuse Large B-Cell Lymphoma was developed by Sehn and colleagues at the British Columbia Cancer Agency in 2007, in response to the observation that the original IPI (developed in the pre-rituximab era) lost its ability to distinguish four separate risk groups when applied to patients treated with R-CHOP. In the R-CHOP era, original IPI "low-intermediate" and "high-intermediate" risk groups merged into a single outcome group. The R-IPI collapses the five original IPI categories into three clinically meaningful groups (Very Good, Good, Poor) with substantially different 4-year OS and PFS estimates, providing better guidance for treatment intensification decisions.
The Revised International Prognostic Index (R-IPI) for Diffuse Large B-Cell Lymphoma was developed by Sehn and colleagues at the British Columbia Cancer Agency in 2007, in response to the observation that the original IPI (developed in the pre-rituximab era) lost its ability to distinguish four separate risk groups when applied to patients treated with R-CHOP. In the R-CHOP era, original IPI "low-intermediate" and "high-intermediate" risk groups merged into a single outcome group. The R-IPI collapses the five original IPI categories into three clinically meaningful groups (Very Good, Good, Poor) with substantially different 4-year OS and PFS estimates, providing better guidance for treatment intensification decisions.
Validated In
Derivation cohort 365 R-CHOP-treated DLBCL patients; validated across multiple rituximab-era cohorts
Laurie H. Sehn and colleagues, BC Cancer Agency, Vancouver, Canada (2007)
Same 5 variables as original IPI
Age >60, LDH >ULN, ECOG ≥2, Stage III-IV, >1 extranodal site (1 point each)
R-IPI Very Good (score 0)
4-year OS 94%, PFS 94%. No intensification needed.
R-IPI Good (score 1-2)
4-year OS 79%, PFS 67%. Standard R-CHOP × 6.
R-IPI Poor (score 3-5)
4-year OS 55%, PFS 53%. Consider Pola-R-CHP or dose-dense regimens.
The R-IPI was derived from 365 DLBCL patients treated with R-CHOP and demonstrated superior discriminatory ability over the original 4-group IPI in the rituximab era.
Pola-R-CHP (polatuzumab vedotin antibody-drug conjugate + R-CHP) vs R-CHOP: POLARIX trial demonstrated superior 2-year PFS (76.7% vs 70.2%) in previously untreated DLBCL — FDA-approved 2023.
Double-hit lymphoma (MYC rearrangement + BCL2 and/or BCL6): Poor outcomes with R-CHOP regardless of IPI — prefer DA-EPOCH-R (dose-adjusted).
Circulating tumour DNA (ctDNA) monitoring: Emerging tool for assessing early treatment response and predicting relapse in DLBCL — more sensitive than conventional CT imaging for residual disease detection.
All DLBCL patients require COO (cell-of-origin) testing by IHC (Hans algorithm) or gene expression profiling — GCB vs non-GCB/ABC has prognostic and treatment implications.
CAR-T cell therapy as 2nd-line consolidation (ZUMA-7, TRANSFORM trials): Axi-cel superior to salvage chemo + ASCT for early relapsed/refractory DLBCL — now preferred 2nd-line for eligible patients.
Sehn LH, et al. The revised International Prognostic Index (R-IPI) is a better predictor of outcome than the standard IPI for patients with diffuse large B-cell lymphoma treated with R-CHOP. Blood. 2007;109(5):1857-1861.
Tilly H, et al. Polatuzumab vedotin in previously untreated diffuse large B-cell lymphoma. N Engl J Med. 2022;386(4):351-363. (POLARIX)
Locke FL, et al. Axicabtagene Ciloleucel as Second-Line Therapy for Large B-Cell Lymphoma. N Engl J Med. 2022;386(7):640-654. (ZUMA-7)
The Sokal Score for Chronic Myeloid Leukaemia was developed by John Sokal and colleagues in 1984 from a collaborative analysis of 813 CML patients treated with busulphan or hydroxyurea (the pre-TKI era). Despite being developed over 40 years ago, the Sokal score retains prognostic value in the TKI era and is recommended by the European LeukemiaNet (ELN) 2020 guidelines for initial risk stratification of newly diagnosed CML. Patients with high Sokal scores achieve lower rates of deep molecular response (MR4/MR4.5) with imatinib and are more likely to require 2nd-generation TKIs. The Sokal formula uses age, spleen size, platelet count, and peripheral blood blast percentage.
The Dynamic International Prognostic Scoring System (DIPSS) for myelofibrosis was developed by Passamonti and colleagues in 2010 as a dynamic improvement over the original IPSS (which only used presentation data). DIPSS uses the same five risk factors as IPSS but weights anaemia (Hgb <10 g/dL) twice, reflecting its stronger prognostic impact discovered in follow-up analyses. DIPSS can be applied at any time during the disease course, not just at diagnosis — making it "dynamic." DIPSS-Plus (2011) added three additional independent adverse factors (platelet count <100, unfavourable karyotype, transfusion need) for further refinement. Primary myelofibrosis (PMF) has an annual incidence of ~0.5-1.5 per 100,000, predominantly affecting patients aged 60-70.
The ISTH Bleeding Assessment Tool (ISTH-BAT) was developed in 2010 by the Scientific and Standardisation Committee (SSC) of the International Society on Thrombosis and Haemostasis to provide a standardised, validated instrument for quantifying bleeding history in patients suspected of having inherited bleeding disorders. Before the ISTH-BAT, at least five different bleeding questionnaires were in use, making it impossible to compare data across centres and studies. The ISTH-BAT assigns severity scores (0-4) for each of 14 bleeding symptoms and calculates a total bleeding score (BS). An abnormal BS (>3 in males or >5 in females) has been validated as predictive of von Willebrand disease, platelet function disorders, and other inherited bleeding conditions.
Antiphospholipid Syndrome (APS) is an acquired autoimmune thrombophilia characterised by persistent antiphospholipid antibodies (aPL) causing arterial/venous thrombosis and/or obstetric complications. The Sapporo criteria (1998), revised as the Sydney criteria (2006), established the international consensus definition requiring at least one clinical criterion (thrombosis or pregnancy morbidity) and one laboratory criterion (LA, aCL, or anti-β2GPI positive on two occasions ≥12 weeks apart). The triple-positive profile (all three antibodies positive) confers the highest thrombotic recurrence risk and is a key driver of treatment decisions — warranting warfarin over DOACs based on multiple randomised trials demonstrating DOAC inferiority in this group.