Adults with indolent systemic mastocytosis (SM) now have a medicine to treat their disease after the groundbreaking approval of Ayvakit® (avapritinib) by the US Food and Drug Administration (FDA). “Today’s approval of Ayvakit reflects more than a decade of collaboration and leadership in the field of SM, during which our team at Blueprint Medicines has…
An open-label, 2-part phase 2 study investigating CGT9486 (bezuclastinib) for the treatment of patients with advanced systemic mastocytosis (SM), including patients with aggressive SM, SM with associated hematologic neoplasm, and mast cell leukemia, is now recruiting. The study will enroll 120 eligible adult participants who will take bezuclastinib oral tablets for continuous 28-day cycles. The…
Researchers presented a case of alopecia in a patient with cutaneous manifestations of systemic mastocytosis in Annals of Clinical Case Reports. A 12-year-old boy had a history of alopecia on his scalp and body for the previous 9 years. The lesions were described as being reddish-brown in color and itchy. Upon physical examination, the patient…
Patients with systemic mastocytosis are at significant risk of developing anaphylaxis during surgical procedures.
The diagnostic workup in patients with a history of anaphylactic reaction to an insect sting involves multiple levels of exploration, including the presence of systemic mastocytosis (SM), according to findings from a review published in The Journal of Allergy and Clinical Immunology: In Practice. A patient’s evaluation typically involves a number of steps, known as…
Although increased levels of basal serum tryptase (BST) may indicate a diagnosis of systemic mastocytosis (SM), high elevations can still be tolerated in people with hereditary α-tryptasemia, according to an article published in Blood Advances. To examine the overall utility of tryptase genotyping, the researchers cloned, long-read sequenced, and assembled the human tryptase locus containing…
In a letter to the editor of Annals of Hematology, researchers described the case study of a patient diagnosed with well-differentiated systemic mastocytosis. A woman in her 20s presented with a large hematoma following a hip injury. Magnetic resonance imaging revealed splenomegaly and abnormal bone marrow signaling throughout her pelvis and her spine. Her past…
Researchers reported the case study of a woman with breast carcinoma who had radiotherapy-associated cutaneous mastocytosis in Extraordinary Case Report. A 64-year-old woman presented with multiple, red-brown macules located on her left breast and her adjacent central chest. These macules were smooth in texture and measured approximately 1 to 3 mm in diameter. The right…
A study conducted on the effects of omalizumab in treatment-resistant systemic and cutaneous mastocytosis yielded a mixed picture of the benefits of this treatment in this group of patients, according to a paper published in The Journal of Allergy and Clinical Immunology: In Practice. Omalizumab is a novel drug that binds to free immunoglobulin E,…
In spite of advances, response assessment in advanced systemic mastocytosis remains complex and difficult. Refined response criteria were recently proposed.
