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ASH Image Bank (2008); doi:10.1182/ashimagebank-2008-8-00161
Copyright © 2008 by the American Society of Hematology.
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Atlas Image Set

Systemic mastocytosis involving the bone marrow

Shriram Vaidia Nath, M.D., D.Ped, D.M.(Oncology), Geoff Davey, FRCPA, Giles Kelsey, FRCPA, and Graeme Swinton, FRCPA


Figure 1
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Figure 1. A 79-year-old gentleman was admitted to the hospital following a reaction to a CT scan dye. He was having the CT scan because of a 2-week history of profuse diarrhoea and had become mildly anaemic and mildly neutropenic. (Hb-102 g/L, WBC-3.2*10^9/L, Neu 1.6*10^9/L, Platelet 292*10^9/L). Normal renal and liver function. S.Tryptase was greater than 200.0 ug/L (<15.0). The CT-Scan revealed multiple osteolytic lesions in the spinal region. A bone scan revealed diffuse with irregular uptake in the axial bones, humerus, and the femur along the long shaft, upper tibial shafts, and forearm bones. The scanner view of the trephine demonstrates areas of normal haemopoietic tissue along with areas of mast cells with marked fibrosis. The bony trabeculae are increased in volume with evidence of increased bony turnover.

 

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Figure 2. A high-power view of the trephine demonstrating whorls of mast cells associated with marked fibrosis, abutting against the bony trabeculae.

 

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Figure 3. High-power view of cytoplasmic staining of mast cells with tryptase. The cytogenetics revealed 46, XY [14]. While FISH did not identify any deletion of CHIC2 and hence there was no molecular cytogenetic evidence of the FIP1L1/PDGFRA fusion on 4q12. His diarrhoea settled in 2 weeks time. Because of his age and general status, he was on supportive care in the form of regular transfusion, hydroxyurea, and allopurinol. He died after 15 months of diagnosis.

 

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Related ASH-SAP Chapter:space logo
Chapter 7: Myeloid disorders

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Right arrow Articles by Swinton, G.
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PubMed
Right arrow Articles by Nath, S. V.
Right arrow Articles by Swinton, G.
Related Collections
Right arrow Mast Cell Disease
Right arrow Related ASH-SAP Chapter
Right arrowRelated Image Bank Image Sets
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