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Clinical References

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Clinical Pearls

Hematology9 results

Bleeding Risk Assessment (ISTH-BAT)

HematologyISTH Scientific and Standardisation Committee
Open

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.

7 criteria6 pearls3 refsValidated

PLASMIC Score (TTP)

HematologyPavan K. Bendapudi and colleagues
Open

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.

8 criteria6 pearls3 refsValidated

ITP Response Assessment

HematologyFrancesco Rodeghiero and the International Working Group on ITP
Open

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.

5 criteria6 pearls3 refsValidated

IPSS / IPSS-R (MDS)

HematologyPeter Greenberg
Open

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.

7 criteria6 pearls3 refsValidated

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.

Validated In

Pooled analysis of 816 MDS patients from 7 cooperative groups/institutions

Peter Greenberg, Christine Cox, Maria M. LeBeau, and the IPSS Group (1997)

1

Cytogenetics — Favourable (0 pts)

Normal, −Y alone, del(5q), del(20q)

2

Cytogenetics — Intermediate (0.5 pts)

All other abnormalities

3

Cytogenetics — Poor (1 pt)

Complex (≥3 abnormalities) or chromosome 7 anomalies

4

Bone Marrow Blasts — <5% (0 pts)

Lower blast % correlates with better survival

5

Bone Marrow Blasts — 5-10% (0.5 pts)

Intermediate blast burden

6

Bone Marrow Blasts — 11-20% (1.5 pts) / 21-30% (2 pts)

Higher blasts = closer to AML threshold (≥20% = AML per WHO 2022)

7

Cytopenias — 0-1 (0 pts) / 2-3 (0.5 pts)

Hgb <10, ANC <1.8, Platelets <100

IPSS-R (Revised IPSS, 2012) provides better prognostic discrimination than original IPSS and is now preferred for clinical and research use. Uses continuous score rather than discrete categories.

Lower-risk MDS (IPSS Low/INT-1): Treat symptoms. ESAs (darbepoetin, epoetin) for transfusion-dependent anaemia with EPO <500 — response rate 30-60%. Lenalidomide for del(5q) — 70% transfusion independence.

Higher-risk MDS (IPSS INT-2/High): Azacitidine is standard of care — OS benefit over conventional care regimens (AZA-001 trial: azacitidine 24.5 vs 15 months median OS).

TP53-mutated MDS: Decitabine + cedazuridine (oral HMA) or venetoclax combinations under investigation. Poor response to azacitidine.

Enasidenib (IDH2) and ivosidenib (IDH1) inhibitors: FDA-approved for IDH-mutated MDS — test for IDH1/2 in all higher-risk MDS.

Allogeneic SCT: Only curative treatment for MDS. Aim to reduce blast burden to <5% with HMA before transplant. Reduced-intensity conditioning for older patients.

[1]

Greenberg P, et al. International scoring system for evaluating prognosis in myelodysplastic syndromes. Blood. 1997;89(6):2079-2088.

[2]

Greenberg PL, et al. Revised International Prognostic Scoring System for Myelodysplastic Syndromes. Blood. 2012;120(12):2454-2465. (IPSS-R)

[3]

Fenaux P, et al. Efficacy of azacitidine compared with that of conventional care regimens in the treatment of higher-risk myelodysplastic syndromes. Lancet Oncol. 2009;10(3):223-232. (AZA-001)

R-IPI (DLBCL)

HematologyLaurie H. Sehn and colleagues
Open

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.

4 criteria6 pearls3 refsValidated

Sokal Score (CML)

HematologyJohn E. Sokal and colleagues
Open

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.

4 criteria6 pearls3 refsValidated

DIPSS (Myelofibrosis)

HematologyFrancesco Passamonti and the IWG-MRT
Open

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.

7 criteria6 pearls3 refsValidated

ISTH-BAT Bleeding Score

HematologyISTH Scientific and Standardisation Committee
Open

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.

6 criteria6 pearls3 refsValidated

Antiphospholipid Syndrome Risk

HematologySapporo Criteria
Open

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.

5 criteria6 pearls3 refsValidated

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