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Systemic mastocytosis is a rare and recalcitrant disorder with nonspecific clinical features. Hence, a high index of suspicion is required. Here, we report the case of a 64 years old male presenting with chronic diarrhoea that was evaluated at different centres and treated with multiple lines of therapy. The diagnosis of aggressive systemic mastocytosis was finally clinched following a holistic work up that included a Jejunal biopsy and a laparoscopic lymph node biopsy. Treatment of this disorder is difficult, responses are transient and most patients will eventually relapse, as illustrated by this case. Cladribine, Interferon α, steroids and imatinib have limited success in the management of this disease. The role of stem cell transplant is uncertain.

Systemic Mastocytosis with Associated Clonal Hematological Non-Mast Cell Lineage Disorder (MDS-RCMD): A Difficult Disease to Diagnose and Treat