CME in Minutes: Education in Dermatology
May 5, 2026 · 14 min · 12 segments
Please visit answersincme.com/ZTY860 to participate, download slides and supporting materials, complete the post test, and get a certificate. Presented by Daniel J. DeAngelo, MD, PhD. In this…
Daniel D'AngeloGuest
I'm Chief of the Division of Leukemia at Dana-Farber Cancer Institute in Boston.
The goal of this program is to convert the growing body of TKI clinical data into practical guidance for everyday clinical use.
Let's begin by reviewing the physical and psychosocial burden of patients with indolent systemic mastocytosis.
When mast cells degranulate, they can release a lot of cytokines, histamine, interleukins, TNF, tryptase, that cause a wide range of systemic manifestations, including cardiovascular problems, hypotension, resulting sometimes in syncope or lightheadedness, tachycardia.
They can manifest in cutaneous issues such as flushing, pruritus, uticaria or hives, angioedema, gastrointestinal problems, including gastrointestinal abdominal cramping, diarrhea, sometimes esophageal reflux, leading to nausea, vomiting.
Patients can describe musculoskeletal problems such as aches and bone pain, osteopenia, or even osteoporosis.
Other problems including neurologic with complaints of anxiety or depression, decreased ability to concentrate, memory lapses, insomnia.
Some patients even have migranous headaches, respiratory complaints such as nasal congestion, pruritus, shortness of breath or wheezing, and then, of course, just generalized systemic fatigue, malaise, or weight loss.
Long-term complications really influences their quality of life, and so early recognition and diagnosis can lead to early treatment and therefore, hopefully, resolution of symptoms and improvement in quality of life.
There's a diagnostic criteria based on the WHO 2022 with multifocal dense aggregates as a major finding or minor findings, including the presence of abnormal or atypical morphologic features of mast cells, hit mutation, elevated tryptase, or a barren expression of CD25 or CD2, differentiating indolent systemic mastocytosis from more advanced advanced disease would require not only bone marrow examination, but also the absence of any definable need for cytotoxic reduction, such as organ damage, what we call C-findings.
Once a diagnosis is made and there's no evidence of organ damage, then a patient has indolent systemic mastocytosis.
Although there are possibility of patients progressing from non-advanced or indolent disease to more advanced disease.
There is about 6% of the general population that may have hereditary alpha-tryptasemia.
And if you look into the patients with indolent systemic mastocytosis, somewhere between 15% and 18% will have hereditary disease.
I'm Chief of the Division of Leukemia at Dana-Farber Cancer Institute in Boston.
The goal of this program is to convert the growing body of TKI clinical data into practical guidance for everyday clinical use.
Let's begin by reviewing the physical and psychosocial burden of patients with indolent systemic mastocytosis.
When mast cells degranulate, they can release a lot of cytokines, histamine, interleukins, TNF, tryptase, that cause a wide range of systemic manifestations, including cardiovascular problems, hypotension, resulting sometimes in syncope or lightheadedness, tachycardia.
They can manifest in cutaneous issues such as flushing, pruritus, uticaria or hives, angioedema, gastrointestinal problems, including gastrointestinal abdominal cramping, diarrhea, sometimes esophageal reflux, leading to nausea, vomiting.
Patients can describe musculoskeletal problems such as aches and bone pain, osteopenia, or even osteoporosis.
Other problems including neurologic with complaints of anxiety or depression, decreased ability to concentrate, memory lapses, insomnia.
Some patients even have migranous headaches, respiratory complaints such as nasal congestion, pruritus, shortness of breath or wheezing, and then, of course, just generalized systemic fatigue, malaise, or weight loss.
Long-term complications really influences their quality of life, and so early recognition and diagnosis can lead to early treatment and therefore, hopefully, resolution of symptoms and improvement in quality of life.
There's a diagnostic criteria based on the WHO 2022 with multifocal dense aggregates as a major finding or minor findings, including the presence of abnormal or atypical morphologic features of mast cells, hit mutation, elevated tryptase, or a barren expression of CD25 or CD2, differentiating indolent systemic mastocytosis from more advanced advanced disease would require not only bone marrow examination, but also the absence of any definable need for cytotoxic reduction, such as organ damage, what we call C-findings.
Once a diagnosis is made and there's no evidence of organ damage, then a patient has indolent systemic mastocytosis.
Although there are possibility of patients progressing from non-advanced or indolent disease to more advanced disease.
There is about 6% of the general population that may have hereditary alpha-tryptasemia.
And if you look into the patients with indolent systemic mastocytosis, somewhere between 15% and 18% will have hereditary disease.
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