Affiliations: Department of Pathology, Celal Bayar University
Faculty of Medicine, Manisa, Turkey | Department of Hematology, Ege University Faculty of
Medicine, Izmir, Turkey | Department of Pathology, Ege University Faculty of
Medicine, Izmir, Turkey
Note: [] Corresponding author: Nalan Neşe. Postal address: Celal
Bayar University Faculty of Medicine, Department of Pathology, Manisa, Turkey
45010. Tel.: +90 5332433133; +90 236 2323133 (302); Fax: +90 236 23701213;
E-mail: nalannese@hotmail.com
Abstract: Systemic mastocytosis is a disease characterized by multifocal mast
cell proliferation in the bone marrow or other extracutaneous organs. Because
of loosely scattered and hypo-/agranular mast cells, the diagnosis is sometimes
very difficult. In the bone marrow, mast cell infiltration may be associated
with prominent lymphoid infiltration leading to a misdiagnosis of a low grade
non-Hodgkin lymphoma. A 49-year-old woman presented with right arm and leg
pain, psychiatric symptoms, and diarrhea for four years. Physical examination
and laboratory investigation revealed hepatosplenomegaly, anemia, mild
thrombocytosis, mild leucocytosis and lymphocytosis. In the bone marrow biopsy,
there was a prominent B lymphocyte proliferation reminiscent of a low grade
non-Hodgkin lymphoma/leukemia and there were some spindle cells aggregates in
paratrabecular location. The consecutive bone marrow biopsies were similar to
the first. The subsequent splenectomy specimen exhibited striking fibrosis. In
the lymph node sections, there was marginal zone hyperplasia. Multifocal
accumulations of mast cells were strongly positive with mast cell tryptase and
CD117 on immunohistochemical staining, though no metachromasia was identified
in Giemsa and Toluidine Blue stained aspirates and tissue sections, probably
due to hypo-/agranulation of mast cells. The case was presented to emphasize
the importance of the antibody to mast cell tryptase in the diagnosis of
mastocytosis and to discuss problems of differential diagnosis of systemic
mastocytosis.
