PMC9950552_01
Timeline and case progress with relevant data.
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The timeline of events and case progress with relevant data are presented in  Figure 1 .
Photographs showing (A) the chronic urticarial rash, (B) facial flushing and (C) profound hypotension during an anaphylaxis episode in our patient (Courtesy of K.T.; written informed consent was obtained from the individual shown).
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CT scans of the brain (to exclude an intracranial hemorrhage), chest and abdomen (to exclude a dissecting aortic aneurysm) were also unremarkable. Clinical examination revealed mild hepatomegaly and multiple, monomorphic, reddish brown macules of the trunk and extremities ( Figure 2A ). Figure 2B ), tachycardia, lightheadedness and hypotension ( Figure 2C ) in the past, which he could link with emotional stress. Further inquiry about preceding events revealed a fairly consistent pattern of ingestion of certain foods (nuts, legumes, chocolate) or alcoholic beverages, together with emotional stress prior to the onset of the episodes.
Immunohistochemical staining of bone marrow biopsy showing large clusters of mast cells (red arrows), expressing (A) CD25 and (B) mast cell tryptase. (C) A hematoxylin and eosin (H&E)-stained section of bone marrow core shows areas of paratrabecular fibrosis, in which mast cells can be identified (yellow oval circles).
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Table 1 ), establishing the diagnosis of SM. The observed mast cells had oval or elongated nuclei and were mostly located around bone marrow trabeculae ( Figure 3C ) and, to a lesser extent, blood vessels. The overall BM cellularity was normal (normal representation of the lymphoid lineage, mild elevation of the erythroid lineage and lower than normal myeloid lineage).