Table 2.

Summary of clinical features and outcomes of type 1–like vs type 2–like CALR mutations

Type 1–like CALRType 2–like CALR
Most common mutation 52-bp deletion (L367fs*46) 5-bp insertion (K385fs*47) 
Prevalence More common in MF More common in ET 
Clinical (ET) Similar; lower platelet counts vs type 2 like Similar; higher platelet counts vs type 1 like 
Clinical (MF) Less splenomegaly, leukocytosis, anemia, and circulating blasts; lower DIPSS score; higher platelets (all vs type 2 like) More splenomegaly, leukocytosis, anemia, and circulating blasts; higher DIPSS score; lower platelets (all vs type 1 like). More similar to JAK2 V617F 
Post-ET MF Similar to/increased vs type 2 like Similar to/decreased vs type 1 like 
Overall prognosis (ET) Similar to type 2 like Similar to type 1 like 
Overall prognosis (MF) Improved vs type 2 like and JAK2 V617F Worsened vs type 1 like; more similar to JAK2 V617F 
Type 1–like CALRType 2–like CALR
Most common mutation 52-bp deletion (L367fs*46) 5-bp insertion (K385fs*47) 
Prevalence More common in MF More common in ET 
Clinical (ET) Similar; lower platelet counts vs type 2 like Similar; higher platelet counts vs type 1 like 
Clinical (MF) Less splenomegaly, leukocytosis, anemia, and circulating blasts; lower DIPSS score; higher platelets (all vs type 2 like) More splenomegaly, leukocytosis, anemia, and circulating blasts; higher DIPSS score; lower platelets (all vs type 1 like). More similar to JAK2 V617F 
Post-ET MF Similar to/increased vs type 2 like Similar to/decreased vs type 1 like 
Overall prognosis (ET) Similar to type 2 like Similar to type 1 like 
Overall prognosis (MF) Improved vs type 2 like and JAK2 V617F Worsened vs type 1 like; more similar to JAK2 V617F 
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