PEDIATRICS Vol. 19 No. 6 June 1957, pp. 1033-1042
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MAST CELL LEUKEMIA PRESENTING AS URTICARIA PIGMENTOSA

Report of a Case

William J. Waters M.D.1 and Perpetua S. Lacson M.D.1

1 Department of Pediatrics, State University of New York Medical Center at Syracuse

The concept of "urticaria pigmentosa" as a benign dermatologic syndrome needs revision. Generalized organ involvement may be present and in this case, with demonstration of the tissue mast cells in the peripheral blood and bone marrow, we chose to classify it as a form of leukemia.

The differentiation between tissue mast cells and blood basophils is emphasized.

Post-mortem examination revealed generalized infiltration of the body organs with tissue mast cells.

"Heparin" and histamine determinations on frozen, post-mortem specimens of liver showed a concentration of approximately 100-times normal.

Tissue mast cells were demonstrated in the peripheral blood.

The factors associated with the hemorhagic diathesis are reviewed.

Submitted on September 25, 1956
Accepted on November 26, 1956