Systemic Mastocytosis Follow-up

  • Author: Koyamangalath Krishnan, MD, FRCP, FACP; Chief Editor: Emmanuel C Besa, MD   more...
 
Updated: Feb 15, 2012
 

Complications

See Clinical.

Next

Prognosis

The prognosis of systemic mastocytosis (systemic mast cell disease) is variable.[30] Young children and patients who present with primarily cutaneous and flushing manifestations tend to have little or no progression of the disease over a considerable length of time. However, patients with more extensive systemic disease involving organ systems other than the skin have a poorer prognosis. Although their median duration of survival is not known, it appears to be a few years. Other poor prognostic factors include older age at diagnosis, anemia, leukopenia and/or thrombocytopenia, elevated lactate dehydrogenase levels, and a myeloproliferativelike disorder.[31]

Previous
Next

Patient Education

Patients should carry epinephrine-filled syringes at all times and should be taught to administer epinephrine in cases of emergency.

Previous
 
Contributor Information and Disclosures
Author

Koyamangalath Krishnan, MD, FRCP, FACP  Paul Dishner Endowed Chair of Excellence in Medicine, Professor of Medicine and Chief of Hematology-Oncology, James H Quillen College of Medicine at East Tennessee State University

Koyamangalath Krishnan, MD, FRCP, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, American Society of Hematology, and Royal College of Physicians

Disclosure: Nothing to disclose.

Coauthor(s)

Devapiran Jaishankar, MBBS  Associate Professor, Division of Oncology, East Tennessee State University, James H Quillen College of Medicine

Devapiran Jaishankar, MBBS is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American Society of Clinical Oncology, and American Society of Hematology

Disclosure: Nothing to disclose.

Specialty Editor Board

Thomas H Davis, MD, FACP  Associate Professor, Fellowship Program Director, Department of Internal Medicine, Section of Hematology/Oncology, Dartmouth Medical School

Thomas H Davis, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American Association for Cancer Education, American College of Physicians, New Hampshire Medical Society, Phi Beta Kappa, and Society of University Urologists

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Ronald A Sacher, MB, BCh, MD, FRCPC  Professor, Internal Medicine and Pathology, Director, Hoxworth Blood Center, University of Cincinnati Academic Health Center

Ronald A Sacher, MB, BCh, MD, FRCPC is a member of the following medical societies: American Association for the Advancement of Science, American Association of Blood Banks, American Clinical and Climatological Association, American Society for Clinical Pathology, American Society of Hematology, College of American Pathologists, International Society of Blood Transfusion, International Society on Thrombosis and Haemostasis, and Royal College of Physicians and Surgeons of Canada

Disclosure: Glaxo Smith Kline Honoraria Speaking and teaching; Talecris Honoraria Board membership

Rajalaxmi McKenna, MD, FACP  Southwest Medical Consultants, SC, Department of Medicine, Good Samaritan Hospital, Advocate Health Systems

Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis

Disclosure: Nothing to disclose.

Chief Editor

Emmanuel C Besa, MD  Professor, Department of Medicine, Division of Hematologic Malignancies, Kimmel Cancer Center, Jefferson Medical College of Thomas Jefferson University

Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Clinical Oncology, American Society of Hematology, and New York Academy of Sciences

Disclosure: Nothing to disclose.

Additional Contributors

The authors and editors of eMedicine gratefully acknowledge the contributions of previous authors Stephen J Smith, MD, Harsha G Vardhana, MD, and Guha Krishnaswamy, MD, to the development and writing of this article.

References

  1. [Guideline] Vardiman JW, Thiele J, Arber DA, Brunning RD, Borowitz MJ, Porwit A. The 2008 revision of the World Health Organization (WHO) classification of myeloid neoplasms and acute leukemia: rationale and important changes. Blood. Jul 30 2009;114(5):937-51. [Medline].

  2. Akin C, Metcalfe DD. Systemic mastocytosis. Annu Rev Med. 2004;55:419-32. [Medline].

  3. Bain BJ. Systemic mastocytosis and other mast cell neoplasms. Br J Haematol. Jul 1999;106(1):9-17. [Medline].

  4. Karnam U, Rogers A. Systemic mastocytosis. Dig Dis. 1999;17(5-6):299-307. [Medline].

  5. Bunimovich O, Grassi M, Baer MR. Systemic mastocytosis: classification, pathogenesis, diagnosis, and treatment. Cutis. Jan 2009;83(1):29-36. [Medline].

  6. Pettigrew HD, Teuber SS, Kong JS, Gershwin ME. Contemporary challenges in mastocytosis. Clin Rev Allergy Immunol. Apr 2010;38(2-3):125-34. [Medline].

  7. Ehrlich P. Beitrage zur Kenntnis der Anilinfarbungen und ihrer Verwendung in der mikroskopischen Technik. Arch mikr Anat. 1877;13:263-7.

  8. Parker RI. Hematologic aspects of systemic mastocytosis. Hematol Oncol Clin North Am. Jun 2000;14(3):557-68. [Medline].

  9. Kirshenbaum AS, Goff JP, Semere T, Foster B, Scott LM, Metcalfe DD. Demonstration that human mast cells arise from a progenitor cell population that is CD34(+), c-kit(+), and expresses aminopeptidase N (CD13). Blood. Oct 1 1999;94(7):2333-42. [Medline].

  10. Ho CL, Kito H, Squillace DL, Lasho TL, Tefferi A. Clinical correlates of serum pro-major basic protein in a spectrum of eosinophilic disorders and myelofibrosis. Acta Haematol. 2008;120(3):158-64. [Medline].

  11. Jensen RT. Gastrointestinal abnormalities and involvement in systemic mastocytosis. Hematol Oncol Clin North Am. Jun 2000;14(3):579-623. [Medline].

  12. Kirsch R, Geboes K, Shepherd NA, et al. Systemic mastocytosis involving the gastrointestinal tract: clinicopathologic and molecular study of five cases. Mod Pathol. Dec 2008;21(12):1508-16. [Medline].

  13. Lim KH, Tefferi A, Lasho TL, et al. Systemic mastocytosis in 342 consecutive adults: survival studies and prognostic factors. Blood. Jun 4 2009;113(23):5727-36. [Medline].

  14. Tefferi A, Levine RL, Lim KH, Abdel-Wahab O, Lasho TL, Patel J, et al. Frequent TET2 mutations in systemic mastocytosis: clinical, KITD816V and FIP1L1-PDGFRA correlates. Leukemia. May 2009;23(5):900-4. [Medline].

  15. Bonadonna P, Zanotti R, Pagani M, Caruso B, Perbellini O, Colarossi S. How much specific is the association between hymenoptera venom allergy and mastocytosis?. Allergy. Sep 2009;64(9):1379-82. [Medline].

  16. Weingarten TN, Volcheck GW, Sprung J. Anaphylactoid reaction to intravenous contrast in patient with systemic mastocytosis. Anaesth Intensive Care. Jul 2009;37(4):646-9. [Medline].

  17. Butterfield JH. Survey of aspirin administration in systemic mastocytosis. Prostaglandins Other Lipid Mediat. Apr 2009;88(3-4):122-4. [Medline].

  18. Ma Y, Zeng S, Metcalfe DD, et al. The c-KIT mutation causing human mastocytosis is resistant to STI571 and other KIT kinase inhibitors; kinases with enzymatic site mutations show different inhibitor sensitivity profiles than wild-type kinases and those with regulatory-type mutations. Blood. Mar 1 2002;99(5):1741-4. [Medline]. [Full Text].

  19. Pardanani A, Elliott M, Reeder T, et al. Imatinib for systemic mast-cell disease. Lancet. Aug 16 2003;362(9383):535-6. [Medline].

  20. Garcia-Montero AC, Jara-Acevedo M, Teodosio C, Sanchez ML, Nunez R, Prados A, et al. KIT mutation in mast cells and other bone marrow hematopoietic cell lineages in systemic mast cell disorders: a prospective study of the Spanish Network on Mastocytosis (REMA) in a series of 113 patients. Blood. Oct 1 2006;108(7):2366-72. [Medline].

  21. Schwartz LB, Irani AM. Serum tryptase and the laboratory diagnosis of systemic mastocytosis. Hematol Oncol Clin North Am. Jun 2000;14(3):641-57. [Medline].

  22. Horny HP. Mastocytosis: an unusual clonal disorder of bone marrow-derived hematopoietic progenitor cells. Am J Clin Pathol. Sep 2009;132(3):438-47. [Medline].

  23. Metcalfe DD. Classification and diagnosis of mastocytosis: current status. J Invest Dermatol. Mar 1991;96(3 suppl):2S-4S; discussion 4S, 60S-65S. [Medline]. [Full Text].

  24. Worobec AS. Treatment of systemic mast cell disorders. Hematol Oncol Clin North Am. Jun 2000;14(3):659-87, vii. [Medline].

  25. van der Veer E, van der Goot W, de Monchy JG, Kluin-Nelemans HC, van Doormaal JJ. High prevalence of fractures and osteoporosis in patients with indolent systemic mastocytosis. Allergy. Mar 2012;67(3):431-8. [Medline].

  26. Ustun C, Corless CL, Savage N, et al. Chemotherapy and dasatinib induce long-term hematologic and molecular remission in systemic mastocytosis with acute myeloid leukemia with KIT D816V. Leuk Res. May 2009;33(5):735-41. [Medline].

  27. Aichberger KJ, Sperr WR, Gleixner KV, Kretschmer A, Valent P. Treatment responses to cladribine and dasatinib in rapidly progressing aggressive mastocytosis. Eur J Clin Invest. Nov 2008;38(11):869-73. [Medline].

  28. Pardanani A, Ketterling RP, Brockman SR, et al. CHIC2 deletion, a surrogate for FIP1L1-PDGFRA fusion, occurs in systemic mastocytosis associated with eosinophilia and predicts response to imatinib mesylate therapy. Blood. Nov 1 2003;102(9):3093-6. [Medline]. [Full Text].

  29. Chaar CI, Bell RL, Duffy TP, Duffy AJ. Guidelines for safe surgery in patients with systemic mastocytosis. Am Surg. Jan 2009;75(1):74-80. [Medline].

  30. Lim KH, Tefferi A, Lasho TL, et al. Systemic mastocytosis in 342 consecutive adults: survival studies and prognostic factors. Blood. Jun 4 2009;113(23):5727-36. [Medline].

  31. Pardanani A, Lim KH, Lasho TL, et al. Prognostically relevant breakdown of 123 patients with systemic mastocytosis associated with other myeloid malignancies. Blood. Oct 29 2009;114(18):3769-72. [Medline].

  32. Akin C, Schwartz LB, Kitoh T, et al. Soluble stem cell factor receptor (CD117) and IL-2 receptor alpha chain (CD25) levels in the plasma of patients with mastocytosis: relationships to disease severity and bone marrow pathology. Blood. Aug 15 2000;96(4):1267-73. [Medline]. [Full Text].

  33. Kumar S, Moody P. Mastocytosis. Pediatr Rev. Jan 2001;22(1):33-4. [Medline].

  34. Longley BJ, Metcalfe DD. A proposed classification of mastocytosis incorporating molecular genetics. Hematol Oncol Clin North Am. Jun 2000;14(3):697-701, viii. [Medline].

  35. Pauls JD, Brems J, Pockros PJ, et al. Mastocytosis: diverse presentations and outcomes. Arch Intern Med. Feb 22 1999;159(4):401-5. [Medline].

  36. Soter NA. Mastocytosis and the skin. Hematol Oncol Clin North Am. Jun 2000;14(3):537-55, vi. [Medline].

  37. Sperr WR, El-Samahi A, Kundi M, Girschikofsky M, Winkler S, Lutz D. Elevated tryptase levels selectively cluster in myeloid neoplasms: a novel diagnostic approach and screen marker in clinical haematology. Eur J Clin Invest. Oct 2009;39(10):914-23. [Medline].

  38. Swolin B, Rodjer S, Roupe G. Cytogenetic studies in patients with mastocytosis. Cancer Genet Cytogenet. Jul 15 2000;120(2):131-5. [Medline].

  39. Tefferi A, Li CY, Butterfield JH, Hoagland HC. Treatment of systemic mast-cell disease with cladribine. N Engl J Med. Jan 25 2001;344(4):307-9. [Medline].

  40. van Daele PL, Beukenkamp BS, Geertsma-Kleinekoort WM, et al. Immunophenotyping of mast cells: a sensitive and specific diagnostic tool for systemic mastocytosis. Neth J Med. Apr 2009;67(4):142-6. [Medline].

  41. Vega-Ruiz A, Cortes JE, Sever M, et al. Phase II study of imatinib mesylate as therapy for patients with systemic mastocytosis. Leuk Res. Nov 2009;33(11):1481-4. [Medline].

 
Previous
Next
 
Bone marrow aspirate, Romanowsky stain, high-definition magnification. Diagnosis is mastocytosis, and morphology is abnormal mast cells. This is a bone marrow smear from a patient with systemic mastocytosis. Several mast cells are present in this photograph. These mast cells are larger than normal mast cells and have more irregularly shaped nuclear outlines and less densely packed mast cell granules. Courtesy of the American Society of Hematology Slide Bank. Used with permission.
Bone marrow aspirate, toluidine stain, low magnification. Diagnosis is mastocytosis, and morphology is abnormal mast cells. This is a toluidine blue stain of a bone marrow smear from a patient with marrow involvement by systemic mastocytosis. Five mast cells are present in this field. The mast cell granules are metachromatic with the toluidine blue reaction. Courtesy of the American Society of Hematology Slide Bank. Used with permission.
Bone marrow biopsy, toluidine stain, low magnification. Diagnosis is mastocytosis, and morphology is abnormal mast cell infiltrate. This is a toluidine blue stain of a bone marrow biopsy from a patient with systemic mastocytosis. The mast cells are metachromatic with toluidine blue and contain numerous purple granules. Courtesy of the American Society of Hematology Slide Bank. Used with permission.
Lymph node biopsy. Diagnosis is mastocytosis, morphology is mast cell infiltrate, and the organ is the lymph nodes. This is a lymph node biopsy from a person with systemic mastocytosis. The mast cells have a characteristic perifollicular distribution. Courtesy of the American Society of Hematology Slide Bank. Used with permission.
Lymph node biopsy, chloroacetate esterase stain. Diagnosis is mastocytosis, and morphology is mast cell infiltrate. This is a portion of a lymph node biopsy from a patient with systemic mastocytosis. The mast cells are chloroacetate esterase positive, which is characterized by an orange granular appearance. Courtesy of the American Society of Hematology Slide Bank. Used with permission.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.