Systemic mastocytosis

Case contributed by Dr Alexandra Stanislavsky

Patient Data

Age: 65
Gender: Male
Modality: X-ray

Radiographs demonstrate diffuse sclerosis of the axial skeleton. The limbs and skull were not involved (x-rays not included here).

Modality: CT

CT of the abdomen demonstrates ascites, hepatosplenomegaly and upper abdominal lymphadenopathy. Windowing to bone confirms the diffuse sclerosis seen on the plain films.

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 : There is an excess of mast cells which are noted 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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Case Information

rID: 11026
Case created: 10th Oct 2010
Last edited: 15th May 2016
Inclusion in quiz mode: Included

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