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Articles by Cem Akin
touchMDT Systemic mastocytosis: Making the right decisions at the right time
Overview & Learning Objectives Overview In this activity, expert faculty discuss strategies to recognize SM, apply current diagnostic and classification criteria, and collaborate within a multidisciplinary team to individualize management and make timely treatment decisions. This activity is jointly provided by USF Health and touchIME. touchIME is an EBAC® accredited provider.
Future Directions in ISM: Long-term Outcomes, Unanswered Questions, and Pediatric Considerations
Published on: Future directions in ISM, including evolving treatment goals, unanswered questions about osteoporosis, anaphylaxis, resistance, curative strategies, and the need to extend advances to pediatric populations. The integration of disease-targeted therapies into indolent systemic mastocytosis (ISM) care is shifting treatment goals from symptom palliation alone toward combined objectives of symptom control and disease modification.
Measuring Response in ISM: PRO Tools, Biomarkers, and Dose Optimization With Avapritinib
Published on: Use of symptom and quality-of-life tools alongside biomarkers in PIONEER, principles of dose titration from 25 mg to 50 mg, and practical recommendations for longitudinal safety monitoring. Assessment of therapeutic response in indolent systemic mastocytosis (ISM) requires integration of patient-centered outcomes with objective disease markers.
Targeted Therapy in ISM: Shared Decision-making, Safety, and Early PIONEER Data
Published on: Shared decision-making around initiating targeted therapy, key safety considerations for avapritinib, and core efficacy and safety findings from the 6‑month randomized phase of the PIONEER trial. Initiation of targeted therapy in indolent systemic mastocytosis (ISM) requires nuanced shared decision-making that incorporates symptom burden, prior treatment history, comorbidities, and patient preferences.
When Supportive Care Is Not Enough: Patient-Reported Outcomes and Escalation to Targeted Therapy
Published on: Role of patient-reported outcomes in trials and practice, criteria for escalation beyond supportive care, and the paradigm shift introduced by selective KIT D816V inhibition, including avapritinib. Symptom assessment tools play distinct but complementary roles in the clinical trial and routine practice settings for indolent systemic mastocytosis (ISM).
KIT D816V Biology and Longitudinal Symptom Assessment in Indolent Systemic Mastocytosis
Published on: Overview of KIT D816V–driven disease biology in ISM and practical approaches to longitudinal symptom monitoring, including emerging use of the Mastocytosis Control Test. Indolent systemic mastocytosis (ISM) is tightly linked to activating mutations in KIT, most commonly the D816V substitution in the intracellular domain, which drives mast cell survival, activation, and differentiation.
1 Reframing “Benign” Disease: The Modern Clinical Impact of Indolent Systemic Mastocytosis
Published on: Introduction to ISM with emphasis on evolving clinical perception, disease heterogeneity, and persistent unmet needs despite conventional symptomatic therapy. Indolent systemic mastocytosis (ISM) has historically been labeled a “benign” hematologic disorder on the basis of life expectancy, yet contemporary clinical experience underscores its substantial impact on morbidity and quality of life.
SUMMIT: Bezuclastinib Delivers Durable, Deepening Symptom Relief in NonAdvSM, With Cem Akin, MD
Bezuclastinib, an oral, highly selective type 1 KIT inhibitor, has demonstrated that clinically meaningful symptom improvement not only holds but continues to deepen out to 48 weeks of treatment in patients with nonadvanced systemic mastocytosis (NonAdvSM), with every assessed symptom domain showing progressive gains over the treatment course.1,2 These data were from the pivotal, multicenter, randomized, double-blind, placebo-controlled phase 2 SUMMIT trial and were presented at the American...
Long-Term PIONEER Data Reinforce Avapritinib’s Benefit-Risk Profile in ISM, with Cem Akin, MD
Avapritinib continues to deliver durable symptom control and quality-of-life improvements nearly 4 years into treatment for patients with indolent systemic mastocytosis (ISM), reinforcing its role as a long-term targeted therapy for this chronic disease.1 Avapritinib was approved for treating ISM by the United States (US) Food and Drug Administration (FDA) in May 2023.2 Updated data from the Phase 2/3 PIONEER trial (NCT03731260) were presented at the American Academy of Allergy, Asthma &...
KIT Targeting: A New Frontier in ISM Management, With Cem Akin, MD
Published on: Akin discussed updated data on elenestinib from the HARBOR trial in indolent systemic mastocytosis. Indolent systemic mastocytosis (ISM) is a rare, chronic condition characterized by the accumulation of abnormal mast cells that drive a range of often debilitating symptoms, including flushing, gastrointestinal distress, fatigue, musculoskeletal pain, and in some cases, life-threatening anaphylaxis.
Pipeline Therapies for Indolent Systemic Mastocytosis: Growing Confidence in the Treatment Landscape of Indolent Systemic Mastocytosis
Advancing Treatment in Indolent Systemic Mastocytosis: Updates at EAACI 2025 - Episode 10 Published on: A panelist discusses how they have growing confidence in pipeline therapies for indolent systemic mastocytosis based on their experience with the PIONEER trials, particularly noting improvements in patient symptoms and quality of life, while expressing hope for additional data on the drug's effects on bone health and anaphylactic events, and describing other investigational treatments...
Multiorgan Relief with KIT Inhibition: Broad Symptom Relief for Patients
Advancing Treatment in Indolent Systemic Mastocytosis: Updates at EAACI 2025 - Episode 9 Published on: A panelist discusses how avapritinib provides broad, multi-organ symptom relief in indolent systemic mastocytosis—improving skin, gastrointestinal, and neurocognitive symptoms—leading to significant quality-of-life gains, though its effects on osteoporosis and anaphylaxis remain under investigation.
Identifying Ideal Candidates for Avapritinib Up-Titration
Advancing Treatment in Indolent Systemic Mastocytosis: Updates at EAACI 2025 - Episode 8 Published on: A panelist discusses how patients with indolent systemic mastocytosis who have high mast cell burdens, inadequate symptom control, and stable tolerance at 25 mg may be ideal candidates for avapritinib dose escalation to 50 mg.
The Clinical Significance of a Durable Treatment for Indolent Systemic Mastocytosis
Advancing Treatment in Indolent Systemic Mastocytosis: Updates at EAACI 2025 - Episode 7 Published on: A panelist discusses how the durability and safety of avapritinib over several years support its long-term use in indolent systemic mastocytosis, potentially increasing confidence among community providers to prescribe it beyond specialized centers.
Candidates for Consideration: Who Would Benefit from Avapritinib?
Advancing Treatment in Indolent Systemic Mastocytosis: Updates at EAACI 2025 - Episode 6 Published on: A panelist discusses how appropriate candidates for avapritinib include patients with moderate to severe indolent systemic mastocytosis who remain symptomatic despite standard therapies, while those with low platelet counts, pregnancy, bleeding risks, or certain comorbidities should be excluded through shared decision-making.
Inside PIONEER: 3-Year Long-Term Safety and Efficacy Study of Avapritinib
Advancing Treatment in Indolent Systemic Mastocytosis: Updates at EAACI 2025 - Episode 5 Published on: A panelist discusses how the three part PIONEER study shows that avapritinib 25 mg (with optional escalation to 50 mg) sustains meaningful symptom and quality of life improvements with a favorable safety profile for up to five years, supporting its FDA approval for indolent systemic mastocytosis.
Current Standards of Care to Treat Patients With Indolent Systemic Mastocytosis
Advancing Treatment in Indolent Systemic Mastocytosis: Updates at EAACI 2025 - Episode 3 Published on: A panelist discusses how the current standard of care for indolent systemic mastocytosis involves stepwise symptom management with antihistamines and other antimediator therapies, though treatment challenges include limited efficacy, adverse effects, and the burden of polypharmacy.
The Heterogeneous Clinical Presentations of Indolent Systemic Mastocytosis
Advancing Treatment in Indolent Systemic Mastocytosis: Updates at EAACI 2025 - Episode 2 Published on: A panelist discusses how indolent systemic mastocytosis is driven by a KIT D816V mutation that causes constant mast cell activation, leading to heterogeneous clinical presentations and highlighting the importance of targeted therapies like selective KIT inhibitors.
Understanding Indolent Systemic Mastocytosis (ISM): Symptoms and Impact
Advancing Treatment in Indolent Systemic Mastocytosis: Updates at EAACI 2025 - Episode 1 Published on: A panelist discusses how indolent systemic mastocytosis presents with chronic, mast cell–mediated symptoms that significantly reduce quality of life, often persist despite treatment, and may lead to serious complications like anaphylaxis and osteoporosis.
Mastocytosis - Nature Reviews Disease Primers
Abstract Mastocytosis is a spectrum of clonal myeloid disorders defined by abnormal growth and accumulation of mast cells in various organ systems. The disease is divided into cutaneous mastocytosis, systemic mastocytosis (SM) and mast cell sarcoma. SM is further categorized into several non-advanced and advanced forms. The prognosis of cutaneous mastocytosis and non-advanced SM is mostly favourable, whereas prognosis and survival in advanced SM and mast cell sarcoma are poor.
Neutrophil extracellular traps activate Notch-γ-secretase signaling in hidradenitis suppurativa
Supported by the , National Institute of Arthritis and Musculoskeletal and Skin Diseases / (ZIA AR041199 to M.J.K.), Danby grant (C.C.-R.), and the Skin of Color Society CDA (C.C.-R.). Disclosure of potential conflict of interest: A. S. Byrd has been a consultant for Senté and Sonoma Biotherapeutics. G. A. Okoye serves on the advisory boards for Pfizer, Unilever, Janssen, UCB, Novartis, Sanofi-Genzyme,
Ara h 2-expressing cucumber mosaic virus-like particle (VLP Peanut) induces in vitro tolerogenic cellular responses in peanut-allergic individuals
Section snippets Subjects Whole blood samples were collected from 6 untreated PA children (aged 6 to 18 years) who were recruited under the BOPI-2 study protocol that consented to optional blood sampling (Imperial College London, United Kingdom; NCT03937726) (Table I, and see Table E1 in this article’s Online Repository at www.jacionline.org). In addition, a first-in-human study with VLP Peanut (PROTECT, NCT05476497) comprising 3 phases has commenced (see Fig E1 in the Online Repository).
Antibody-Based and Cell Therapies for Advanced Mastocytosis: Established and Novel Concepts
This is an early access version, the complete PDF, HTML, and XML versions will be available soon.
Evaluating the Relationship of Skin Involvement, Gender and Anaphylaxis in Mastocytosis
Mastocytosis is associated with an increased risk of anaphylaxis. However, there is limited understanding of the clinical factors that predispose patients to anaphylaxis. We analyzed mastocytosis patients with and without skin lesions and observed gender differences to determine predictors of anaphylaxis.
Mastocytosis and Anaphylaxis to Subcutaneous Inhalant Allergen Immunotherapy
Subcutaneous allergen immunotherapy (SCIT) may induce anaphylaxis on rare occasions through a variety of patient and environmental related factors. Systemic mastocytosis is known to be a risk factor for anaphylaxis as well as systemic reactions to venom immunotherapy, however the prevalence of undiagnosed mastocytosis in patients experiencing anaphylaxis to subcutaneous inhalant immunotherapy is not known.
Qualitative Research to Understand the Patient Experience in Non-Advanced Systemic Mastocytosis
Patients with Non-Advanced Systemic Mastocytosis (NonAdvSM), which includes the subvariants indolent systemic mastocytosis and smoldering systemic mastocytosis, experience a variety of disabling, unpredictable and potentially life-threatening symptoms, including anaphylaxis in over 25% of patients and decrements to their health-related quality of life (HRQoL).
Avapritinib Improved Symptoms and Quality of Life in Patients With Indolent Systemic Mastocytosis in the PIONEER Study
Indolent systemic mastocytosis (ISM), is a disorder that can be characterized by debilitating cutaneous, gastrointestinal, neurological, and musculoskeletal manifestations, high symptom burden, anaphylaxis, and poor quality of life (QoL). Avapritinib, an investigational selective KIT D816V inhibitor targets the primary driver of ISM.
Tyrosine kinase inhibitors for the treatment of indolent systemic mastocytosis: are we there yet?
This paper is only available as a PDF. To read, Please Download here. Abstract Indolent systemic mastocytosis (ISM) is the most prevalent form of systemic mastocytosis. Many patients with ISM suffer from mast cell (MC) mediator-related symptoms In a small number of patients, hematologic progression is seen in the follow up.
This paper is only available as a PDF. To read, Please Download here. Abstract Mast cell (MC) neoplasms are an emerging challenge in the fields of internal medicine, allergy, immunology, dermatology, laboratory medicine and pathology. In this review, we discuss the current standards for the diagnosis and prognostication of MC neoplasms with special reference to clinically relevant germline and somatic gene variants.
Drug-Induced Mast Cell Eradication: A Novel Approach to Treat Mast Cell Activation Disorders?
This paper is only available as a PDF. To read, Please Download here. Abstract Mast cell activation is a key event in allergic reactions, other inflammatory states, and mast cell activation syndromes. Mast cell-stabilizing agents, mediator-targeting drugs and drugs interfering with mediator effects are often prescribed in these patients. However, the clinical efficacy of these drugs varies, depending on the numbers of involved mast cells and the underlying pathology.
Utility of a Validated Disease-Specific Measure to Assess Symptomology in Patients with Indolent Systemic Mastocytosis (ISM)
A Phase 2/3 Study of BLU-263 in Patients with Indolent Systemic Mastocytosis or Monoclonal Mast Cell Activation Syndrome
Allergic Diseases and Anaphylaxis in Mastocytosis
Mastocytosis is characterized by abnormal clonal mast cell expansion and activation in one or more organ systems. When mediator release is excessive and involves multiple systems, anaphylaxis may occur. Patients with mastocytosis present with symptoms similar to an allergic reaction and are at increased risk of anaphylaxis.
Reply
To the Editor: 1 Lyons J. On the complexities of tryptase genetics and impact on clinical phenotypes. J Allergy Clin Immunol. J Allergy Clin Immunol 2021;148:xxx-xxx.
Developing a standardized approach for assessing mast cells and eosinophils on tissue biopsies: A Work Group Report of the AAAAI Allergic Skin Diseases Committee - Journal of Allergy and Clinical Immunology
Mast cells and eosinophils are commonly found, expectedly or unexpectedly, in human tissue biopsies. Although the clinical significance of their presence, absence, quantity, and quality continues to be investigated in homeostasis and disease, there are currently gaps in knowledge related to what constitutes quantitatively relevant increases in mast cell and eosinophil number in tissue specimens for several clinical conditions.
Hereditary alpha tryptasemia is not associated with specific clinical phenotypes
This paper is only available as a PDF. To read, Please Download here. ABSTRACT Background Hereditary alpha tryptasemia (HαT) is found in approximately 7% of the population. Associations with a variety of clinical symptoms including gastric reflux, joint hypermobility, dysautonomia, flushing and pruritus, and Hymenoptera allergy have variably been described in prior reports.
A novel case of beta-tryptase/TPSB2 allele duplication
Serum tryptase is the product of two genes – TPSAB1 and TPSB2. While TPSB2 reliably contains the β2 and β3 alleles, TPSAB1 contains either the α or β1 allele to produce three possible genotypes - αα:ββ, αβ:ββ, and ββ:ββ. Hereditary alpha tryptasemia is a genetic trait defined by one or more extra copies of the α-tryptase allele. This is the first report of an extra copy of the β-tryptase allele.
Patients with mast cell activation symptoms and elevated baseline serum tryptase have unique bone marrow morphology
This paper is only available as a PDF. To read, Please Download here. Abstract Background Patients with mast cell activation symptoms and elevated baseline serum tryptase (MCAS-T) may not necessarily have a clonal mast cell disorder. Many are diagnosed with hereditary alpha tryptasemia (HαT), a genetic trait characterized by autosomal dominant inheritance of multiple copies of TPSAB1 encoding alpha-tryptase and increased risk for severe anaphylaxis.
Risk and management of patients with mastocytosis and MCAS in the SARS-CoV-2 (COVID-19) pandemic: Expert opinions
The coronavirus disease 2019 (COVID-19) (caused by severe acute respiratory syndrome coronavirus 2) pandemic has massively distorted our health care systems and caused catastrophic consequences in our affected communities. The number of victims continues to increase, and patients at risk can only be protected to a degree, because the virulent state may be asymptomatic.
Midostaurin improves quality of life and mediator-related symptoms in advanced systemic mastocytosis
Background Advanced systemic mastocytosis (advSM) is characterized by presence of the KIT D816V mutation and pathologic accumulation of neoplastic mast cells (MCs) in various tissues, leading to severe symptoms and organ damage (eg, cytopenias, liver dysfunction, portal hypertension, malabsorption, and weight loss).
Clinical programs | Blueprint Medicines
Purposefully designed medicines and clinical candidates Header Graphic Avapritinib Avapritinib is a potent and selective inhibitor of activated KIT and PDGFRA mutant kinases. In certain diseases, mutations in KIT and PDGFRA force protein kinases into an increasingly active state. Avapritinib is uniquely designed to bind and inhibit the active conformation of these proteins.
Idiopathic anaphylaxis yardstick: Practical recommendations for clinical practice
To view the full text, please login as a subscribed user or purchase a subscription. Click here to view the full text on ScienceDirect. Figure 2 Diagnostic algorithm for idiopathic anaphylaxis. ASA, acetylsalicylic acid; HATS, hereditary alpha tryptasemia; HIAA, hydroxyindolacetic acid; NET, neuroendocrine tumor; PGF2α, prostaglandin F2α. *Diagnosis according to accepted criteria (e.g., Sampson et al, 20067).
AAAAI Mast Cell Disorders Committee Work Group Report: Mast cell activation syndrome (MCAS) diagnosis and management
AAAAI Position Statements,Work Group Reports, and Systematic Reviews are not to be considered to reflect current AAAAI standards or policy after five years from the date of publication. The statement below is not to be construed as dictating an exclusive course of action nor is it intended to replace the medical judgment of healthcare professionals. The unique circumstances of individual patients and environments are to be taken into account in any diagnosis and treatment plan.
A Simple Guide to Fresh Fruit at Home
There’s no end to the benefits of planting fruit around your house: you have unlimited access to a fresh supply of produce; a bountiful landscape in your own yard and neighborhood; and an ecosystem ready for pollinating bees and delightful songbirds to visit. The Home Orchard Handbook (Quarry Books, 2011) by Cem Akin and Leah Rottke can be your guide to creating this lovely backdrop.
Venom‐immunotherapy in patients with clonal mast cell disorders: IgG4 correlates with protection - Jarkvist - - Allergy - Wiley Online Library
The full text of this article hosted at iucr.org is unavailable due to technical difficulties.
How to Prune and Weed Your Home Orchard
There’s no end to the benefits of planting fruit around your home: you have unlimited access to a fresh supply of produce; a bountiful landscape in your own yard and neighborhood; and an ecosystem ready for pollinating bees and delightful songbirds to visit. That’s not to say that a home orchard cannot come with a new set of challenges, however.
Mast Cell Activation Syndrome: Importance of Consensus Criteria and Call for Research
Publication HistoryPublished online: June 18, 2018Accepted: June 7, 2018; Received in revised form: May 25, 2018; Received: April 22, 2018;
Proposed Terminology and Classification of Pre-Malignant Neoplastic Conditions: A Consensus Proposal
Cancer evolution is a step-wise process that involves multiple molecular defects, and is characterized by clonal diversification, sub-clone selection, and clonal expansion (Cahill et al., 1999, Greaves and Maley, 2012, Nowell, 1976, Vogelstein and Kinzler, 1993, Yates and Campbell, 2012).
Journal of Allergy and Clinical Immunology
To view the full text, please login as a subscribed user or purchase a subscription. Click here to view the full text on ScienceDirect. FiguresFig 1Relationship between clonal mast cell disorders, mast cell activation syndrome (MCAS), and idiopathic anaphylaxis (IA). Circle sizes do not represent prevalence.Mast cell activation is common and possibly necessary for maintenance of survival.
Advances in the Classification and Treatment of Mastocytosis: Current Status and Outlook toward the Future
Reviews Advances in the Classification and Treatment of Mastocytosis: Current Status and Outlook toward the Future Peter Valent, Cem Akin, Karin Hartmann, Gunnar Nilsson, Andreas Reiter, Olivier Hermine, Karl Sotlar, Wolfgang R. Sperr, Luis Escribano, Tracy I. George, Hanneke C. Kluin-Nelemans, Celalettin Ustun, Massimo Triggiani, Knut Brockow, Jason Gotlib, Alberto Orfao, Lawrence B. Schwartz, Sigurd Broesby-Olsen, Carsten Bindslev-Jensen, Petri T. Kovanen, Stephen J. Galli, K. Frank Austen, Daniel A.
How to Prune and Weed Your Home Orchard
There’s no end to the benefits of planting fruit around your home: you have unlimited access to a fresh supply of produce; a bountiful landscape in your own yard and neighborhood; and an ecosystem ready for pollinating bees and delightful songbirds to visit. That’s not to say that a home orchard cannot come with a new set of challenges, however.
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