Granularity in disease classification impacts survival prediction in advanced systemic mastocytosis: A single institution study of 329 informative cases

The World Health Organization (WHO) classification system categorizes advanced systemic mastocytosis (SM‐Adv) into aggressive SM (ASM), mast cell leukemia (MCL), and SM with associated hematological neoplasm (SM‐AHN). By contrast, the International Consensus Classification (ICC) requires “immature”...

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Bibliographic Details
Published in:American journal of hematology 2024-01, Vol.99 (1), p.21-27
Main Authors: Tefferi, Ayalew, Abdelmagid, Maymona, Al‐Kali, Aref, Patnaik, Mrinal, Hogan, William J., Begna, Kebede, Gangat, Naseema, Orazi, Attilio, Chen, Dong, Reichard, Kaaren K., Pardanani, Animesh
Format: Article
Language:English
Subjects:
Adolescent
Adult
Aged
Aged, 80 and over
Alkaline phosphatase
Classification
Hematologic Neoplasms - diagnosis
Hematology
Humans
Leukemia, Mast-Cell
Male
Mast cell leukemia
Mast Cells
Mastocytosis
Mastocytosis - diagnosis
Mastocytosis, Systemic - diagnosis
Middle Aged
Prognosis
Risk Factors
Thrombocytopenia
Tumors
Young Adult
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Summary:The World Health Organization (WHO) classification system categorizes advanced systemic mastocytosis (SM‐Adv) into aggressive SM (ASM), mast cell leukemia (MCL), and SM with associated hematological neoplasm (SM‐AHN). By contrast, the International Consensus Classification (ICC) requires “immature” MC cytomorphology for the diagnosis of MCL and limits SM‐AHN to myeloid neoplasms (SM‐AMN). The current study includes 329 patients with SM‐Adv (median age 65 years, range 18–88; males 58%): WHO subcategories SM‐AHN (N = 212; 64%), ASM (N = 99; 30%), and MCL (N = 18; 6%); ICC subcategories SM‐AMN (N = 190; 64%), ASM (N = 99; 33%), and MCL (N = 9; 3%); WHO‐defined MCL with “mature” MC cytomorphology and SM‐AHN associated with lymphoid neoplasms were operationally labeled as “MCL‐mature” (N = 9) and SM‐ALN (N = 22), respectively, and distinguished from ICC‐defined MCL and SM‐AMN. Multivariable analysis that included the Mayo alliance risk factors for survival in SM (age >60 years, anemia, thrombocytopenia, increased alkaline phosphatase) revealed more accurate survival prediction with the ICC versus WHO classification order: (i) survival was significantly worse with MCL‐immature versus MCL‐mature (hazard ratio [HR] 15; p 
ISSN:0361-8609
1096-8652
1096-8652
DOI:10.1002/ajh.27113