Aggressive systemic mastocytosis with multiple organ involvement: a case report.
Effat IranijamMaryam SalimiMohammad NegareshNima JavanshirPublished in: Oxford medical case reports (2023)
Systemic mastocytosis is a rare malignancy whose main diagnostic finding is the abnormal proliferation of clonal mast cells. In this report, a 63-year-old woman is presented who was referred to the emergency department with lower back pain. Due to the hypereosinophilia in blood tests, a bone marrow biopsy was performed, and except for the presence of a large number of mastocytes, no other pathologic findings were seen. Furthermore, the immunohistochemistry test showed positive CD117 and CD25 markers, and the patient's platelet-derived growth factor receptor alpha test was positive. Hence, the patient was diagnosed with aggressive systemic mastocytosis. Treatment was initiated with the Cladribine regimen, but unfortunately, in the third course, the patient experienced bradycardia and loss of consciousness and expired. Systemic mastocytosis can manifest itself with non-cutaneous symptoms. Non-cutaneous symptoms do not rule out systemic mastocytosis as a differential diagnosis in patients with hypereosinophilia.