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Signs and Symptoms of Mast Cell Proliferation, Accumulation and Infiltration (Mastocytosis)

 

Advanced disease symptoms may include the following signs of mast cell proliferation, accumulation and infiltration: anemia, thrombocytopenia, ascites, bone fractures, gastrointestinal abnormalities, and enlargement of the liver, spleen, and lymph nodes.1, 2 Mast cell proliferation, accumulation and infiltration can occur in systemic mastocytosis (SM), smoldering SM (SSM), aggressive SM (ASM), SM with an associated hematologic neoplasm (SM-AHN) [previously called “SM with associated clonal hematologic non mast cell lineage disease” (SM-AHNMD)],3 or mast cell leukemia (MCL). B and C findings, in addition to meeting the criteria for SM, clearly define these signs and assist physicians with the diagnosis.

Symptoms and Signs

People with mastocytosis may experience the following symptoms or signs. Sometimes, people with mastocytosis do not show any of these symptoms. Or, these symptoms may be caused by another medical condition. If you are concerned about a symptom or sign on this list, please talk with your doctor or a dermatologist.

General symptoms:

  • Hives
  • Red, itchy rash
  • Diarrhea
  • Abdominal pain
  • Fainting
  • Shortness of breath
  • Wheezing (trouble breathing)
  • Psychological changes (for example, irritability and inability to concentrate)

Urticaria pigmentosa:

  • Tan or red-brown spots on the skin
  • Abdominal pain
  • Nausea
  • Vomiting
  • Diarrhea
  • Headache
  • Increased heart rate
  • Facial flushing (reddening of the face)
  • Psychological changes (for example, irritability and inability to concentrate)

Solitary mastocytoma:

  • Raised or flat reddish-brown spot on the skin
  • Hives
  • Itching

 Diffuse erythrodermic mastocytosis:

  • Thickening of the skin
  • Blisters

Telangiectasia macularis eruptiva perstans:

  • Small lesions that do not itch

Systemic mastocytosis:

  • Skin lesions
  • Urticaria pigmentosa
  • Facial flushing
  • Itching
  • Nausea
  • Vomiting
  • Diarrhea
  • Abdominal pain
  • Ulcers in the stomach and duodenum (small intestine)
  • Headache
  • Light-headedness
  • Heart palpitations (an irregular or unusually rapid beating of the heart)
  • Bone pain
  • Anemia (low red blood cell count, which can cause fatigue)
  • Psychological changes (for example, irritability and inability to concentrate)

Symptoms of systemic mastocytosis can sometimes occur as “attacks or events,” where multiple symptoms appear all at once. Following an attack, the person may feel fatigued (tired) and lethargic (drowsy, lacking energy).

Your doctor will ask you questions about the symptoms you are experiencing to help find out the cause of the problem, called a diagnosis. This may include how long you’ve been experiencing the symptom(s) and how often.

If mastocytosis is diagnosed, relieving symptoms and side effects remains an important part of your care and treatment. This may also be called symptom management, palliative care, or supportive care. Be sure to talk with your health care team about symptoms you experience, including any new symptoms or a change in symptoms.

References

  1. Gotlib J, Pardanani A, Akin C, Reiter A, George T, Hermine O, et al. International Working Group-Myeloproliferative Neoplasms Research and Treatment (IWG-MRT) & European Competence Network on Mastocytosis (ECNM) consensus response criteria in advanced systemic mastocytosis. Blood. 2013 Mar 28;121(13):2393-401. http://www.ncbi.nlm.nih.gov/pubmed/23325841
  2. Lim KH, Tefferi A, Lasho TL, Finke C, Patnaik M, Butterfield JH, et al. Systemic mastocytosis in 342 consecutive adults: survival studies and prognostic factors. Blood. 2009 Jun 4;113(23):5727-36. http://www.ncbi.nlm.nih.gov/pubmed/19363219
  3. Arber DA, Orazi A, Hasserjian R, Thiele J, Borowitz MJ, Le Beau MM, et al. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood. 2016 May 19;127(20):2391-405. http://www.ncbi.nlm.nih.gov/pubmed/27069254

Sources

Paragraph 1 and Resources: TAMS wish to acknowledge The Mast Cell Diseases Society (TMS) as the original authors of the above information. Reproduced with permission. View original source on the TMS website.

List of symptoms: Cancer.Net Guide & Mastocytosis American Society of Clinical Oncology (ASCO) | 28 February 2012

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