eMedicine Specialties > Hematology > Stem Cells and Disorders

Mycosis Fungoides: Differential Diagnoses & Workup

Author: Lauren C Pinter-Brown, MD, Director, Lymphoma Program, Clinical Professor of Medicine, Department of Internal Medicine, Division of Hematology and Oncology, UCLA Medical Center
Contributor Information and Disclosures

Updated: Oct 4, 2009

Differential Diagnoses

Lymphoma, Diffuse Large Cell
Lymphoma, Non-Hodgkin

Other Problems to Be Considered

Eczema
Neurodermatitis
Pseudolymphoma syndrome

Workup

Laboratory Studies

  • Consider human immunodeficiency virus (HIV) and human T-cell lymphotrophic virus type I (HTLV-I) testing.
  • Conduct a complete blood cell (CBC) count with differential and review the buffy coat smear for Sézary cells.
  • Liver-associated enzyme abnormalities and lactate dehydrogenase (LDH)
    • LDH is a marker of bulky or biologically aggressive disease.
    • Abnormal transaminase values may indicate hepatic involvement.
  • Conduct flow cytometric study of the blood (include available T cell–related antibodies) to detect a circulating malignant clone and to assess immunocompetence by quantifying the level of CD8-expressing lymphocytes.
  • Perform a uric acid study in cases involving a bulky disease and/or biologically aggressive disease.
  • Polymerase chain reactions (PCRs), Southern blot testing of the blood, or both: Consider these studies if detecting a circulating clone of malignant cells in the blood will change medical management. Ideally, the abnormal clonal T-cell gene rearrangement detected in the blood should match that found in the skin. However, T-cell gene rearrangement by itself will not allow the physician to make a diagnosis of mycosis fungoides or Sézary syndrome. Disorders that are considered benign (eg, lymphomatoid papulosis) can have T-cell gene rearrangement.

Imaging Studies

  • Chest radiography
  • Computed tomography (CT) scanning of the abdomen and pelvis in patients with advanced mycosis fungoides (stage IIB to stage IVB) or in patients with clinically suspected visceral disease.
  • Positron emission tomography (PET) scanning can be considered in individual cases.

Procedures

  • Skin biopsy: Perform a punch biopsy, which is submitted on sodium chloride–soaked gauze to allow for both fixation and snap freezing.
  • Bone marrow examination: Perform this procedure only if the patient has proven blood or nodal involvement.
  • Lymph node biopsy: Conduct this procedure if the nodes are palpable.
    • In a study by Pai et al, the investigators evaluated the utility of fine-needle aspiration (FNA) biopsy in patients with cutaneous T-cell lymphoma (CTCL).11 The authors assessed a series of 11 FNA biopsy specimens from 10 mycosis fungoides and Sezary syndrome patients. Flow cytometric immunophenotyping and T-cell receptor gamma chain PCR (TCR-gamma PCR) were performed on the FNA biopsy material and correlated with cytologic findings. Pai et al reported 7 of 10 patients had "lymph node involvement by cutaneous T-cell lymphoma (CTCL), with 3 cases exhibiting large-cell transformation and 4 cases exhibiting a small-cell pattern." Another 6 cases were identified by flow cytometric immunophenotyping as having an abnormal T-cell population. In 1 case, TCR-gamma PCR demonstrated a clonal T-cell rearrangement, whereas insufficient events were present for evaluation by flow cytometry, as well as in another case in which flow cytometry was not diagnostic of T-cell lymphoma. In addition, immunohistochemistry on cell block material revealed classic Hodgkin lymphoma in 2 cases.11 The authors concluded that FNA biopsy combined with immunophenotyping and TCR-gamma PCR "is significantly useful in the evaluation of lymphadenopathy in patients with mycosis fungoides and S é zary syndrome, especially for triaging lymph nodes that would otherwise not be sampled or for evaluating multiple lymph nodes."11

Histologic Findings

The criteria for the diagnosis of mycosis fungoides include the following12 :

  • A bandlike, upper dermal infiltrate of lymphocytes and other inflammatory cells, with no grenz zone, is present.
  • Epidermotropism of mononuclear cells occurs.
  • When a clear halo surrounds an intraepidermal mononuclear cell singly or in clumps, this is called a Pautrier microabscess. Its presence is suggestive of mycosis fungoides, but it is not necessary for the diagnosis.
  • Little spongiosis of the epidermis is found.
  • Lymphocytes have nuclei that are hyperchromatic and convoluted or cerebriform.

More on Mycosis Fungoides

Overview: Mycosis Fungoides
Differential Diagnoses & Workup: Mycosis Fungoides
Treatment & Medication: Mycosis Fungoides
Follow-up: Mycosis Fungoides
Multimedia: Mycosis Fungoides
References
Further Reading
[ CLOSE WINDOW ]

References

  1. Diamandidou E, Cohen PR, Kurzrock R. Mycosis fungoides and Sezary syndrome. Blood. Oct 1 1996;88(7):2385-409. [Medline]. [Full Text].

  2. Olsen E, Vonderheid E, Pimpinelli N, et al. Revisions to the staging and classification of mycosis fungoides and Sezary syndrome: a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the cutaneous lymphoma task force of the European Organization of Research and Treatment of Cancer (EORTC). Blood. Sep 15 2007;110(6):1713-22. [Medline]. [Full Text].

  3. Hoff NP, Groffik A, Mota R, et al. [Successful use of allogeneic stem cell transplantation for treatment-refractory mycosis fungoides] [German]. Hautarzt. Oct 2008;59(10):779-82. [Medline].

  4. Rook AH, Yoo EK, Grossman DJ, et al. Use of biological response modifiers in the treatment of cutaneous T-cell lymphoma. Curr Opin Oncol. Mar 1998;10(2):170-4. [Medline].

  5. Koh HK, Charif M, Weinstock MA. Epidemiology and clinical manifestations of cutaneous T-cell lymphoma. Hematol Oncol Clin North Am. Oct 1995;9(5):943-60. [Medline].

  6. Girardi M, Heald PW, Wilson LD. The pathogenesis of mycosis fungoides. N Engl J Med. May 6 2004;350(19):1978-88. [Medline].

  7. Weinstock MA, Gardstein B. Twenty-year trends in the reported incidence of mycosis fungoides and associated mortality. Am J Public Health. Aug 1999;89(8):1240-4. [Medline]. [Full Text].

  8. Kim YH, Hoppe RT. Mycosis fungoides and the Sézary syndrome. Semin Oncol. Jun 1999;26(3):276-89. [Medline].

  9. Fink-Puches R, Wolf P, Kerl H, Cerroni L. Lichen aureus: clinicopathologic features, natural history, and relationship to mycosis fungoides. Arch Dermatol. Sep 2008;144(9):1169-73. [Medline].

  10. Kim ST, Sim HJ, Jeon YS, Lee JW, Roh HJ, Choi SY, et al. Clinicopathological features and T-cell receptor gene rearrangement findings of mycosis fungoides in patients younger than age 20 years. J Dermatol. Jul 2009;36(7):392-402. [Medline].

  11. Pai RK, Mullins FM, Kim YH, Kong CS. Cytologic evaluation of lymphadenopathy associated with mycosis fungoides and Sezary syndrome: role of immunophenotypic and molecular ancillary studies. Cancer. Sep 17 2008;epub ahead of print. [Medline].

  12. Furmanczyk PS, Wolgamot GM, Kussick SJ, Sabath DE, Olerud JE, Argenyi ZB. Diagnosis of mycosis fungoides with different algorithmic approaches. J Cutan Pathol. Jul 7 2009;[Medline].

  13. Querfeld C, Rosen ST, Guitart J, Kuzel TM. The spectrum of cutaneous T-cell lymphomas: new insights into biology and therapy. Curr Opin Hematol. Jul 2005;12(4):273-8. [Medline].

  14. Duvic M, Cather JC. Emerging new therapies for cutaneous T-cell lymphoma. Dermatol Clin. Jan 2000;18(1):147-56. [Medline].

  15. Foss FM, Kuzel TM. Novel treatment approaches for cutaneous T-cell lymphoma. Cancer Treat Res. 1999;99:227-40. [Medline].

  16. Herrmann JJ, Roenigk HH Jr, Hönigsmann H. Ultraviolet radiation for treatment of cutaneous T-cell lymphoma. Hematol Oncol Clin North Am. Oct 1995;9(5):1077-88. [Medline].

  17. Zackheim HS. Cutaneous T cell lymphoma: update of treatment. Dermatology. 1999;199(2):102-5. [Medline].

  18. Sinha AA, Heald P. Advances in the management of cutaneous T-cell lymphoma. Dermatol Clin. Apr 1998;16(2):301-11. [Medline].

  19. Ramsay DL, Meller JA, Zackheim HS. Topical treatment of early cutaneous T-cell lymphoma. Hematol Oncol Clin North Am. Oct 1995;9(5):1031-56. [Medline].

  20. Prince HM, Whittaker S, Hoppe RT. How we treat mycosis fungoides and Sezary syndrome. Blood. Aug 20 2009;[Medline].

  21. Gardner JM, Evans KG, Musiek A, Rook AH, Kim EJ. Update on treatment of cutaneous T-cell lymphoma. Curr Opin Oncol. Mar 2009;21(2):131-7. [Medline].

  22. Wu PA, Kim YH, Lavori PW, Hoppe RT, Stockerl-Goldstein KE. A meta-analysis of patients receiving allogeneic or autologous hematopoietic stem cell transplant in mycosis fungoides and Sézary syndrome. Biol Blood Marrow Transplant. Aug 2009;15(8):982-90. [Medline].

  23. D'Acunto C, Gurioli C, Neri I. Plaque stage mycosis fungoides treated with bexarotene at low dosage and UVB-NB. J Dermatolog Treat. Jan 1 2009;1-4. [Medline].

[ CLOSE WINDOW ]

Further Reading

Related eMedicine Topics


Clinical Trials
 Clinical Guideline



[ CLOSE WINDOW ]

Keywords

mycosis fungoides, MF, cutaneous T-cell lymphoma, CTCL, malignant T-cell lymphoma of the skin, Sézary syndrome, SS, pagetoid reticulosis, erythroderma, malignant lymphoma, skin lesions, Staphylococcus aureus, , Pseudomonas aeruginosa, , non-Hodgkin lymphoma, non-Hodgkin's Lymphoma, NHL

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Lauren C Pinter-Brown, MD, Director, Lymphoma Program, Clinical Professor of Medicine, Department of Internal Medicine, Division of Hematology and Oncology, UCLA Medical Center
Lauren C Pinter-Brown, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American Society of Hematology, and Southwest Oncology Group
Disclosure: Nothing to disclose.

Medical Editor

Paul Schick, MD, Emeritus Professor, Department of Internal Medicine, Thomas Jefferson University Medical College; Research Professor, Department of Internal Medicine, Drexel University College of Medicine; Adjunct Professor of Medicine, Lankenau Hospital, Wynnewood, PA
Paul Schick, MD is a member of the following medical societies: American College of Physicians, American Heart Association, American Society of Hematology, International Society on Thrombosis and Haemostasis, and New York Academy of Sciences
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Marcel E Conrad, MD, (Retired) Distinguished Professor of Medicine, University of South Alabama
Marcel E Conrad, MD is a member of the following medical societies: Alpha Omega Alpha, American Association for the Advancement of Science, American Association of Blood Banks, American Chemical Society, American College of Physicians, American Physiological Society, American Society for Clinical Investigation, American Society of Hematology, Association of American Physicians, Association of Military Surgeons of the US, International Society of Hematology, Society for Experimental Biology and Medicine, and Southwest Oncology Group
Disclosure: No financial interests None None

CME Editor

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, 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 Hematology, and New York Academy of Sciences
Disclosure: Nothing to disclose.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
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.