A recent case report published in Leukemia Research Reports details a patient who developed mast cell leukemia (MCL), a rare subtype of systemic mastocytosis (SM).
MCL makes up fewer than 5% of all cases of SM and has a poor prognosis. “Therapeutic options for MCL are limited, and no standard therapy exists due to the disease’s rarity,” the report’s authors said.
The case report describes a 74-year-old woman who presented with fever, shortness of breath and bone pain for 50 days. She did not respond to antibiotic treatment.
Analysis of her bone marrow revealed an elevated proportion of abnormal, immature mast cells. The mast cells also expressed CD117 and CD30, two proteins that are associated with MCL. Based on these findings, the patient was diagnosed with primary acute MCL, meaning she had no prior history of SM and experienced rapid disease progression.
Read more about SM testing and diagnosis
The patient was first administered dexamethosone, a corticosteroid, for five days. During this time, her symptoms eased but did not fully resolve. She then switched to methylprednisolone, a different corticosteroid, in addition to chemotherapy treatment with dasatinib. After seven days, she experienced significant improvement in her symptoms.
To evaluate whether the symptom reduction could be attributed to methylprednisolone, the authors reintroduced dexamethasone, which resulted in symptom reoccurrence. The patient was then placed back on methylprednisolone, once again experiencing symptom relief.
Following discharge from the hospital, the patient continued dasatinib therapy and discontinued corticosteroid treatment. Her symptoms returned within 24 hours, and she passed away 12 days after discharge as a result of heart failure and respiratory distress.
“This case underscores the importance of personalized treatment approaches in MCL, considering the distinct genetic profile and differential therapeutic responses to corticosteroids,” the study concluded.
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