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Table 2 World Health Organization classification of mastocytosis

From: Critical care management of systemic mastocytosis: when every wasp is a killer bee

Variant

Subvariants

1. Cutaneous mastocytosis

 

- Urticaria pigmentosa

- Diffuse cutaneous mastocytosis

- Mastocytoma of skin

2. Systemic mastocytosis

Indolent systemic mastocytosis

- Smouldering systemic mastocytosis Isolated bone marrow mastocytosis

Aggressive systemic mastocytosis

- Lymphadenopathic systemic mastocytosis with eosinophilia

Systemic mastocytosis with an associated non-mast cell lineage disorder

- Systemic mastocytosis with myelodysplastic syndrome

- Systemic mastocytosis with myeloproliferative disorder

- Systemic mastocytosis with chronic myelomonocytic leukaemia

- Systemic mastocytosis with non-Hodgkin’s lymphoma

- Systemic mastocytosis with hypereosinophilic syndrome

Mast cell leukaemia

- Aleukaemic mast cell leukaemia

3. Mast cell neoplasms

Mast cell sarcoma

 

Extracutaneous mastocytoma

 
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Critical Care

ISSN: 1364-8535