Patient Data
Age: 65 years
Gender: Male
From the case:
Systemic mastocytosis
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Radiographs demonstrate diffuse sclerosis of the axial skeleton. The limbs and skull were not involved (x-rays not included here).
From the case:
Systemic mastocytosis
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Ascites, hepatosplenomegaly and upper abdominal lymphadenopathy are seen. Diffuse bony sclerosis is confirmed.
Case Discussion
This patient went on to have a bone marrow aspirate.
Pathology
- cellularity: a markedly hypercellular marrow trephine with significant architectural distortion, paratrabecular fibrosis and marrow necrosis with areas of cellular ghosting
- bony trabeculae: markedly thickened
- erythropoiesis: markedly reduced in amount
- granulopoiesis: moderately increased in amount with evidence of progression to segmented forms.
- megakaryocytes: normal in number and morphologically normal.
- lymphocytes: normal in number with no significant aggregation or clustering.
- foreign cells: nil seen.
- other cells: excess of mast cells in a paratrabecular distribution in conjunction with the fibrosis. many of these appear round in nature and are noted to form clusters and aggregates. Additional cells are noted to be spindle-shaped in keeping with abnormal mast cells.
Conclusion:
A markedly hypercellular marrow trephine with significant paratrabecular fibrosis and an excess of abnormal mast cells. These features are in keeping with ongoing marrow involvement with mastocytosis.
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