Table 7.

Updated Cologne clinicopathological criteria for the diagnosis of idiopathic myelofibrosis (IMF).21,*

*The combination of A1 + B1 establishes IMF—any other criterion confirms IMF.
 A1 + A2, B1 + MF0 is consistent with initial (prefibrotic) IMF.
 A1 + A3, B1 + MF1, MF2 is consistent with early manifestation of IMF.
 A1 + A4, B1 + MF3 is consistent with end stage (full-blown) IMF. 
† Adverse signs: Age > 70 years, hemoglobin < 10 g/dL, myeloblasts > 2% in peripheral blood, > 2% erythro-normoblats in PB, leukocytosis > 20 × 109/L, thrombocytopenia < 300 × 109/L, severe constitutional symptoms, massive splenomegaly, cytogenetic abnormalities. 
Clinical Criteria
 A1 No preceding or allied subtype of myeloproliferative disorders CML or MDS 
A2 Early clinical stages

  • - Normal hemoglobin or anemia, grade I: hemoglobin ≥ 12 g/dL

  • - Slight or moderate splenomegaly on palpation or > 11 cm on ultrasound scan or CT

  • - Thrombocythemia, platelet > 400 × 109/L

 
A3 Intermediate clinical stage

  • - Anemia grade II (hemoglobin ≥ 10 g/dL

  • - Definitive leuko-erythroblastic blood picture and/or tear drop erythrocytes

  • - Splenomegaly

  • - No adverse signs†

 
A4 Advanced clinical stage

  • - Anemia grade III: hemoglobin < 10 g/dL

  • - 1 or more adverse signs†

 
Pathological Criteria
 B1 Megakaryocytic and granulocytic myeloproliferation and relative reduction of erythroid precursors. Abnormal clustering and increase in atypical giant-sized megakaryocytes containing clumsy (cloud-like) lobulated nuclei and definitive maturation defects. 
Staging of idiopathic myelofibrosis (IMF)

  • MF0 prefibrotic stage IMF: no reticulin fibrosis MF1

  • MF1 early IMF: slight reticulin fibrosis

  • MF2 manifest IMF: marked increase in reticulin and/or collagen fibrosis

  • MF3 overt IMF: advanced collagen fibrosis-osteosclerosis (endophytic bone formation)

 
*The combination of A1 + B1 establishes IMF—any other criterion confirms IMF.
 A1 + A2, B1 + MF0 is consistent with initial (prefibrotic) IMF.
 A1 + A3, B1 + MF1, MF2 is consistent with early manifestation of IMF.
 A1 + A4, B1 + MF3 is consistent with end stage (full-blown) IMF. 
† Adverse signs: Age > 70 years, hemoglobin < 10 g/dL, myeloblasts > 2% in peripheral blood, > 2% erythro-normoblats in PB, leukocytosis > 20 × 109/L, thrombocytopenia < 300 × 109/L, severe constitutional symptoms, massive splenomegaly, cytogenetic abnormalities. 
Clinical Criteria
 A1 No preceding or allied subtype of myeloproliferative disorders CML or MDS 
A2 Early clinical stages

  • - Normal hemoglobin or anemia, grade I: hemoglobin ≥ 12 g/dL

  • - Slight or moderate splenomegaly on palpation or > 11 cm on ultrasound scan or CT

  • - Thrombocythemia, platelet > 400 × 109/L

 
A3 Intermediate clinical stage

  • - Anemia grade II (hemoglobin ≥ 10 g/dL

  • - Definitive leuko-erythroblastic blood picture and/or tear drop erythrocytes

  • - Splenomegaly

  • - No adverse signs†

 
A4 Advanced clinical stage

  • - Anemia grade III: hemoglobin < 10 g/dL

  • - 1 or more adverse signs†

 
Pathological Criteria
 B1 Megakaryocytic and granulocytic myeloproliferation and relative reduction of erythroid precursors. Abnormal clustering and increase in atypical giant-sized megakaryocytes containing clumsy (cloud-like) lobulated nuclei and definitive maturation defects. 
Staging of idiopathic myelofibrosis (IMF)

  • MF0 prefibrotic stage IMF: no reticulin fibrosis MF1

  • MF1 early IMF: slight reticulin fibrosis

  • MF2 manifest IMF: marked increase in reticulin and/or collagen fibrosis

  • MF3 overt IMF: advanced collagen fibrosis-osteosclerosis (endophytic bone formation)

 
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