Scoring systems in primary myelofibrosis

Nataliia Lopina — Mon, 20 Dec 2021 16:11:01 +0000

Several scoring systems to determine the prognosis in primary myelofibrosis have been developed.

DIPSS-plus

The Dynamic International Prognostic Scoring System–plus (DIPSS-plus) for primary myelofibrosis uses the following eight adverse factors to predict survival:

Age older than 65 years
Hemoglobin level lower than 10 g/dL
Leukocyte count higher than 25 × 10 ⁹/L
Platelet count lower than 100 × 10 ⁹ /L
Circulating blasts of 1% or more
Constitutional symptoms
Red blood cell transfusion dependency
Unfavorable karyotype (ie, complex karyotype or sole or two abnormalities that include +8, -7/7q-, i(17q), inv(3), 5/5q-, 12p-, or 11q23 rearrangement)

DIPSS-plus classifications and median survival times are as follows:

Low risk (0 adverse points): 15.4 years
Intermediate-1 risk (1 adverse point): 6.5 years
Intermediate-2 risk (2-3 adverse points): 2.9 years
High risk (4-6 adverse points): 1.3 years

Risk factor	IPSS	DIPSS
Age >65 years	1	1
Constitutional symptoms*	1	1
Hgb < 10 g/dL	1	2
WBC >25×10⁹/L	1	1
Peripheral blasts ≥1 %	1	1

* Constitutional symptoms, defined as weight loss over 6 months, unexplained fever, and night sweats.
IPSS – International Prognostic Scoring System; DIPSS – Dynamic International Prognostic Scoring System; Hgb – hemoglobin; WBC – white blood cell.

DIPSS-plus

DIPSS intermediate-1	1
DIPSS intermediate-2	2
DIPSS high risk	3
RBC transfusion requirement	1
Platelet, 100×10⁹/L	1
Unfavorable karyotype*	1

* Unfavorable karyotype, defined as complex karyotype chromosomal abnormalities, or 1–2 abnormalities that include +8, -5/5q-, -7/7q-, i(17q), 12p-, inv(3), or 11q23 rearrangement

Median survival, years (points = years)

Risk group	IPSS	DIPSS	DIPSS-plus
Low	0 – 11,3	0 (not reached)	0 – 15,4
Intermediate-1	1 – 7,9	1–2 – 14,2	1 – 6,5
Intermediate-2	2 – 4	3–4 – 4	2–3 – 2,9
High	≥3 – 2,3	5–6 – 1,5	4–6 – 1,3

MIPSS70

The MIPSS70 (mutation-enhanced international prognostic scoring system for transplant-age patients) includes clinical risk variables and mutations; subsequent versions include karyotype (MIPSS70+ and MIPSS70+ version 2.0). This system was developed for transplant decision-making in patients age 70 years or younger.

MIPSS70 is based on three genetic variables and six clinical risk factors. The genetic variables are as follows:

Absence of CALR type 1/like mutation
Presence of any high molecular risk [HMR] mutation, specifically ASXL1, SRSF2, EZH2, IDH1, or IDH2
Presence of ≥2 HMR mutations)

The clinical risk factors are as follows:

Hemoglobin < 10 g/dL
Leukocytes >25 × 10 ⁹/L
Platelets < 100 × 10 ⁹/L
Circulating blasts ≥2%
Bone marrow fibrosis grade ≥2
Constitutional symptoms

MIPSS70+ added “unfavorable” karyotype as a fourth genetic variable, and reduced the number of clinical risk factors to four (hemoglobin < 10 g/dL, leukocyte count >25 × 10⁹/L, circulating blasts ≥2%, and constitutional symptoms). Those clinical and genetic risk factors were used to classify three risk categories for MIPSS70 (low, intermediate, and high) and four risk categories for MIPSS70+ (low, intermediate, high, and very high).

In the most recent version, MIPSS70+ 2.0, clinical risk variables and points are as follows:

Severe anemia (hemoglobin < 8 g/dL in women, < 9 g/dL in men): 2 points
Moderate anemia (hemoglobin 8–9.9 g/dL in women, 9–10.9 g/dL in men): 1 point
Circulating blasts ≥2%: 1 point
Constitutional symptoms: 2 points

Genetic variables and points are as follows:

Very high risk (VHR) karyotype (single/multiple abnormalities of −7, i(17q), inv(3)/3q21, 12p−/12p11.2, 11q−/11q23, +21, or other autosomal trisomies, not including +8/+9 [eg, +21, +19]): 4 points
Unfavorable karyotype (all other abnormalities): 3 points
≥2 HMR mutations: 3 points
One HMR mutation: 2 points
Absence of CALR type 1/like mutation: 2 points

Total score, risk factor level, and median survival, and estimated 10-year survival are as follows:

0 points – Very low ris; median not reached (92%)
1–2 points – Low risk; 16.4 years (56%)
3–4 points; Intermediate risk 7.7 years (37%)
5–8 points – High risk; 4.1 years (13%)
≥9 points – Very high risk; 1.8 years (< 5%)

Observation alone is advised for patiens whose MIPSS70+ version 2.0 scores are in the very low and low risk categories. Allogeneic stem cell transplant is the treatment of choice for high and very high risk disease. Patients with intermediate-risk disease are best served by participation in clinical trials.

GIPSS

The GIPSS (genetically inspired prognostic scoring system) is based exclusively on genetic markers: mutations and karyotype.Adverse factors and scores are as follows:

VHR karyotype – 2 points
Unfavorable karyotype – 1 point
Absence of type 1/like CALR mutation – 1 point
Presence of ASXL, SRSF2, and U2AF1Q157 mutations – 1 point

GIPSS risk categories and median survivals (5-year survival rate) are as follows:

Low (0 points): 26.4 years (94%)
Intermediate-1 risk (1 point): 8.0 years (73%)
intermediate-2 (2 points): 4.2 years (40%)
High (≥3 points): 2 years (14%)

Tefferi et al favor starting determination of prognosis with the GIPSS: patients with low-risk disease can receive long-term observation, while those with high-risk disease are candidates for allogeneic stem cell transplantation. MIPSS70+ version 2.0 can be considered for confirming the most appropriate treatment approach for an individual patient.

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Multiple Myeloma Diagnostic Criteria – Online Calculator

February 5, 2023

Email (indicate if you want the results to be sent to your email)

Date of assessment

≥1 of the following (A or B)

A. Clonal bone marrow plasma cells ≥10%

B. Biopsy-proven bony or extramedullary plasmacytoma

≥1 myeloma defining event (C1 or C2)

• C1. End organ damage attributable to underlying plasma cell disorder (≥1 of the following): *

o Hypercalcemia (Serum calcium >1 mg/dL (>0.25 mmol/L) higher than upper limit of normal or >11 mg/dL (>2.75 mmol/L))

o Renal insufficiency (Creatinine clearance<40 mL per min or serum creatinine >2 mg/dL (177 μmol/L))

o Anemia (Hemoglobin >2 g/dL (20 g/L) below lower limit of normal

o Bone lesions (≥1 osteolytic lesion on skeletal x-ray

• C2. ≥1 of the following biomarkers of malignancy: *

o Clonal bone marrow plasma cells ≥60%

o Involved:uninvolved sFLC ratio ≥100

o >1 focal lesion on MRI

Result

Diagnostic result

Negative. Not positive for multiple myeloma by IMWG criteria

Diagnostic result

Positive for multiple myeloma by IMWG criteria

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Сообщение Scoring systems in primary myelofibrosis появились сначала на ClinCaseQuest.

Rare diseases – ClinCaseQuest

Scoring systems in primary myelofibrosis

DIPSS-plus

MIPSS70

GIPSS

Multiple Myeloma Diagnostic Criteria – Online Calculator

≥1 of the following (A or B)

≥1 myeloma defining event (C1 or C2)

Revised Multiple Myeloma International Staging System (R-ISS) Online Calculator

Multiple Myeloma International Staging System (ISS) Online Calculator

SAVED VTE Score

IMPEDE VTE Score

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