Certain clinical and laboratory findings can help distinguish different types of systemic mastocytosis.
Parameters:
(1) mast cell involvement in bone marrow
(2) myelodysplasia and/or myeloproliferative changes
(3) hepatosplenomegaly
(4) lymphadenopathy
(5) skeletal involvement
(6) gastrointestinal involvement
|
Parameter |
"B" Findings |
"C" Findings |
|
bone marrow |
> 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 |
|
hepatomegaly |
palpable, but with no impairment of liver function |
palpable with impaired liver function, ascites and/or portal hypertension |
|
splenomegaly |
palpable but without hypersplenism |
palpable with hypersplenism |
|
lymphadenopathy |
palpable or visceral |
|
|
skeletal involvement |
|
present with osteolysis and/or pathological fractures |
|
gastrointestinal |
|
mast cell infiltrates with malabsorption and weight loss |
where:
• 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).
Specialty: Hematology Oncology, Clinical Laboratory
ICD-10: ,
