Certain clinical and laboratory findings can help distinguish different types of systemic mastocytosis.
(1) mast cell involvement in bone marrow
(2) myelodysplasia and/or myeloproliferative changes
(5) skeletal involvement
(6) gastrointestinal involvement
> 30% mast cells, with focal, dense aggregates OR serum total tryptase level > 200 ng/mL
myelodysplasia and/or myeloproliferative changes in non-mast cell lines
present, but insufficient for definitive diagnosis of haematopoietic neoplasm; blood counts normal or only slightly abnormal
present but insufficient for definitive diagnosis of a haematopoietic neoplasm; blood counts show one or more cytopenias
palpable, but with no impairment of liver function
palpable with impaired liver function, ascites and/or portal hypertension
palpable but without hypersplenism
palpable with hypersplenism
palpable or visceral
present with osteolysis and/or pathological fractures
mast cell infiltrates with malabsorption and weight loss
• Cytopenias: hemoglobin < 10 g/dL, platelet count < 100,000 per µL, absolute neutrophil count < 1,000 per µL
• If there is evidence for a definitive diagnosis of haematopoietic neoplasm (myelodyplastic syndrome, chronic myeloproliferative syndrome, acute leukemia, malignant lymphoma, etc.), then the patient is classed as having systemic mastocytosis with associated clonal haematological non-mast cell lineage disease (SM-AHNMD).
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Specialty: Hematology Oncology, Clinical Laboratory