What to Know About Advanced Systemic Mastocytosis

Watch this video short from the podcast: Marina Kremyanskaya, MD, discusses the progression of systemic mastocytosis and available treatment options to improve quality of life and extend lifespan.

The final episode of our mast cell disease series explores advanced systemic mastocytosis. We delve into the patient journey from indolent to advanced forms of mastocytosis and examine the progression of the disease, the monitoring techniques doctors use, and various treatment options available.

You can listen to or download the podcast on ITCHPODCAST.COM for listening anytime, anywhere. The podcast can also be downloaded at:

The podcast series is sponsored by Blueprint Medicines.

Understanding the Role of Bone Marrow in Advanced Systemic Mastocytosis

Joining podcast hosts Kortney Kwong Hing and Payel Gupta, MD, is Marina Kremyanskaya, MD, PhD, a hematologist and Associate Professor of Medicine, Hematology, and Medical Oncology, at Mount Sinai, Icahn School of Medicine.

Dr. Kremyanskaya brings her expertise in myeloid malignancies to the podcast, offering invaluable insights into advanced systemic mastocytosis.

Dr. Kremyanskaya explains how bone marrow plays a crucial role in mastocytosis progression and discusses its broader impact on the body’s organs. She addresses some common patient concerns and clarifies the course of advancing mastocytosis.

Treatment Options for Advanced Systemic Mastocytosis

When systemic mastocytosis progresses, treatment plans must adapt. Dr. Kremyanskaya emphasizes the use of kinase inhibitors, including avapritinib, as a treatment option.

Dr. Kremyanskaya also considers stem cell transplants. She delves into the complexities of stem cell transplantation, highlighting its effectiveness in curing mastocytosis while acknowledging its challenging nature and possible side effects like graft-versus-host disease (GvHD).

 

Podcast Timestamps

  • 01:07 Introduction to Dr. Kremyanska
  • 01:42 Understanding indolent systemic mastocytosis
  • 04:45 Recognizing signs of advancing mastocytosis
  • 08:28 Changes in treatment and what to expect with aggressive mastocytosis
  • 12:32 Explaining advanced systemic mastocytosis, with or without blood cancer
  • 14:21 Learning about myeloid malignancies
  • 16:38 Why bone marrow is important
  • 16:49 Discovering which organs are most affected by advanced mastocytosis
  • 17:39 The benefits of starting treatment early to slow down advanced mastocytosis
  • 19:22 Considering stem cell transplant as a treatment option
  • 21:32 Understanding how chemotherapy fits into stem cell transplant
  • 23:16 Exploring the risks of graft-versus-host disease
  • 24:42 Investigating if stem cell transplant can cure mast cell disease

Additional Resources