Nonmalignant variants of systemic mastocytosis:
(1) indolent systemic mastocytosis
(2) bone marrow mastocytosis
(3) smouldering systemic mastocytosis
(4) systemic mastocytosis with associated clonal hematological non-mast cell lineage disease (SM-AHNMD)
(5) aggressive systemic mastocytosis
(6) lymphadenopathic mastocytosis with eosinophilia
General features for these variants:
(1) meet WHO criteria for systemic mastocytosis
(2) no evidence of mast cell leukemia
If there is evidence of a clonal hematological disorder or malignancy, then the process is termed SM-AHNMD. This may include:
(1) myelodysplasia
(2) chronic myeloproliferative disorder
(3) AML
(4) malignant lymphoma
(5) any other hematological neoplasm
If there is no evidence of a clonal hematological disorder or malignancy, then the patient can be classified by the pattern of B and C clinical findings:
|
"B" Findings
|
"C" Findings
|
Diagnosis
|
|
0
|
0
|
indolent
|
|
1
|
0
|
not given in table
|
|
>= 2
|
0
|
smouldering
|
|
NA
|
>=1
|
aggressive
|
Special types:
(1) bone marrow mastocytosis: indolent systemic mastocytosis with bone marrow involvement but no skin lesion
(2) lymphadenopathic mastocytosis with eosinophilia: aggressive systemic mastocytosis with progressive lymphadenopathy and peripheral eosinophilia, often with extensive bone involvement but usually without skin involvement
