eMedicine Specialties > Dermatology > Lymphoma and Related Processes

Cutaneous CD30+ (Ki-1) Anaplastic Large-Cell Lymphoma: Follow-up

Author: Chung-Che Chang, MD, PhD, Medical Director and Program Director of Hematopathology, Associate Professor of Pathology, Department of Hematopathology, Methodist Hospital, Weil Medical College, Cornell University, Houston
Coauthor(s): Scott M Acker, MD, Associate Professor, Director of Dermatopathology, Departments of Dermatology and Pathology, University of Alabama at Birmingham
Contributor Information and Disclosures

Updated: Nov 30, 2009

Follow-up

Further Outpatient Care

  • In cutaneous CD30+ (Ki-1) anaplastic large-cell lymphoma (cutaneous CD30+ ALCL), arrange for follow-up care to look for any new skin lesions or lymphadenopathy.
  • Obtain a CBC count if the patient is undergoing chemotherapy.

Complications

  • Chemotherapy drugs are myelosuppressive.

Prognosis

  • Generally, cutaneous CD30+ (Ki-1) anaplastic large-cell lymphoma (cutaneous CD30+ ALCL) has a favorable clinical course (5-y survival rate of 90%) for the primary cutaneous form with occasional spontaneous regression (up to 25% of cases) of the skin lesions. The systemic form has a worse prognosis than the primary cutaneous form. The primary cutaneous CD30- large-cell lymphoma also has a much worse prognosis (5-y survival rate of 15%) than that of the primary cutaneous form of CD30+ ALCL.
  • Primary cutaneous disease, spontaneous regression, absence of extracutaneous involvement, and younger age at onset (£60 y) have been suggested to be associated with a better prognosis.
  • The expression of either NPM-ALK transcripts or ALK protein is not correlated with prognosis or age in the primary cutaneous form of CD30+ cutaneous lymphoproliferations; however, expression of either NPM-ALK transcripts or ALK protein indicates a better prognosis in the systemic form of ALCL.
  • Prior studies have suggested that the stage of disease may be more important than the cytologic subtype.
  • CD30 ligand expression is detected in regressing lesions only and indicates a better prognosis. Cutaneous CD30+ ALCL developing from preexistent mycosis fungoides are often associated with a poor prognosis (5-y survival rate of 10-30%).
  • Regional lymph node involvement is not necessarily associated with an unfavorable prognosis.
  • P53 expression by immunohistochemistry is not associated with spontaneous regression, extracutaneous spreading, or survival.

Patient Education

  • Regular follow-up care is indicated for possible relapse or recurrent disease.

Miscellaneous

Medicolegal Pitfalls

  • Failure to properly treat this condition is a pitfall. Overtreatment of this indolent lymphoma may expose patients to unnecessary complications.

Special Concerns

  • Local excision may be an alternative to radiation therapy for solitary lesions in either pregnant patients or pediatric patients.
  • Rare serious complications such as hemophagocytic syndrome have been reported.8
 
Acknowledgments




More on Cutaneous CD30+ (Ki-1) Anaplastic Large-Cell Lymphoma

Overview: Cutaneous CD30+ (Ki-1) Anaplastic Large-Cell Lymphoma
Differential Diagnoses & Workup: Cutaneous CD30+ (Ki-1) Anaplastic Large-Cell Lymphoma
Treatment & Medication: Cutaneous CD30+ (Ki-1) Anaplastic Large-Cell Lymphoma
Follow-up: Cutaneous CD30+ (Ki-1) Anaplastic Large-Cell Lymphoma
References
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References

  1. Kinney MC, Kadin ME. The pathologic and clinical spectrum of anaplastic large cell lymphoma and correlation with ALK gene dysregulation. Am J Clin Pathol. Jan 1999;111(1 Suppl 1):S56-67. [Medline].

  2. Bonzheim I, Geissinger E, Roth S, Zettl A, Marx A, Rosenwald A, et al. Anaplastic large cell lymphomas lack the expression of T-cell receptor molecules or molecules of proximal T-cell receptor signaling. Blood. Nov 15 2004;104(10):3358-60. [Medline].

  3. Kummer JA, Vermeer MH, Dukers D, Meijer CJ, Willemze R. Most primary cutaneous CD30-positive lymphoproliferative disorders have a CD4-positive cytotoxic T-cell phenotype. J Invest Dermatol. Nov 1997;109(5):636-40. [Medline].

  4. Yamaguchi T, Ohshima K, Karube K, Kawano R, Nakayama J, Suzumiya J, et al. Expression of chemokines and chemokine receptors in cutaneous CD30+ lymphoproliferative disorders. Br J Dermatol. May 2006;154(5):904-9. [Medline].

  5. Vonderheid EC, Sajjadian A, Kadin ME. Methotrexate is effective therapy for lymphomatoid papulosis and other primary cutaneous CD30-positive lymphoproliferative disorders. J Am Acad Dermatol. Mar 1996;34(3):470-81. [Medline].

  6. Fanin R, Sperotto A, Silvestri F, Cerno M, Geromin A, Stocchi R, et al. The therapy of primary adult systemic CD30-positive anaplastic large cell lymphoma: results of 40 cases treated in a single center. Leuk Lymphoma. Sep 1999;35(1-2):159-69. [Medline].

  7. Bekkenk MW, Geelen FA, van Voorst Vader PC, Heule F, Geerts ML, van Vloten WA, et al. Primary and secondary cutaneous CD30(+) lymphoproliferative disorders: a report from the Dutch Cutaneous Lymphoma Group on the long-term follow-up data of 219 patients and guidelines for diagnosis and treatment. Blood. Jun 15 2000;95(12):3653-61. [Medline].

  8. Muhlhoff C, Rubben A, Gabler N, Megahed M. [Primary cutaneous CD30(+) ALK(-) anaplastic large cell T-cell lymphoma.]. Hautarzt. Nov 18 2009;[Medline].

  9. de Bruin PC, Beljaards RC, van Heerde P, Van Der Valk P, Noorduyn LA, Van Krieken JH, et al. Differences in clinical behaviour and immunophenotype between primary cutaneous and primary nodal anaplastic large cell lymphoma of T-cell or null cell phenotype. Histopathology. Aug 1993;23(2):127-35. [Medline].

  10. Duncan LM. Cutaneous lymphoma. Understanding the new classification schemes. Dermatol Clin. Jul 1999;17(3):569-92. [Medline].

  11. Kempf W, Dummer R, Burg G. Approach to lymphoproliferative infiltrates of the skin. The difficult lesions. Am J Clin Pathol. Jan 1999;111(1 Suppl 1):S84-93. [Medline].

  12. LeBoit PE. Lymphomatoid papulosis and cutaneous CD30+ lymphoma. Am J Dermatopathol. Jun 1996;18(3):221-35. [Medline].

  13. Mori M, Manuelli C, Pimpinelli N, Mavilia C, Maggi E, Santucci M, et al. CD30-CD30 ligand interaction in primary cutaneous CD30(+) T-cell lymphomas: A clue to the pathophysiology of clinical regression. Blood. Nov 1 1999;94(9):3077-83. [Medline].

  14. Paulli M, Berti E, Boveri E, Kindl S, Bonoldi E, Gambini C, et al. Cutaneous CD30+ lymphoproliferative disorders: expression of bcl-2 and proteins of the tumor necrosis factor receptor superfamily. Hum Pathol. Nov 1998;29(11):1223-30. [Medline].

  15. Paulli M, Berti E, Rosso R, Boveri E, Kindl S, Klersy C, et al. CD30/Ki-1-positive lymphoproliferative disorders of the skin--clinicopathologic correlation and statistical analysis of 86 cases: a multicentric study from the European Organization for Research and Treatment of Cancer Cutaneous Lymphoma Project Group. J Clin Oncol. Jun 1995;13(6):1343-54. [Medline].

  16. Shehan JM, Kalaaji AN, Markovic SN, Ahmed I. Management of multifocal primary cutaneous CD30 anaplastic large cell lymphoma. J Am Acad Dermatol. Jul 2004;51(1):103-10. [Medline].

  17. Tomaszewski MM, Lupton GP, Krishnan J, May DL. A comparison of clinical, morphological and immunohistochemical features of lymphomatoid papulosis and primary cutaneous CD30(Ki-1)-positive anaplastic large cell lymphoma. J Cutan Pathol. Aug 1995;22(4):310-8. [Medline].

  18. Vergier B, Beylot-Barry M, Pulford K, Michel P, Bosq J, de Muret A, et al. Statistical evaluation of diagnostic and prognostic features of CD30+ cutaneous lymphoproliferative disorders: a clinicopathologic study of 65 cases. Am J Surg Pathol. Oct 1998;22(10):1192-202. [Medline].

  19. Willemze R, Beljaards RC. Spectrum of primary cutaneous CD30 (Ki-1)-positive lymphoproliferative disorders. A proposal for classification and guidelines for management and treatment. J Am Acad Dermatol. Jun 1993;28(6):973-80. [Medline].

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Further Reading

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Keywords

cutaneous CD30+ (Ki-1) anaplastic large-cell lymphoma, cutaneous CD30+ ALCL, cutaneous ALCL, CD30+ ALCL, regressing atypical histiocytosis, RAH, CD30+ cutaneous large T-cell lymphoma, pseudo-Hodgkin disease, pseudo-Hodgkin's disease, non-Hodgkin lymphoma, NHL, pseudo-Hodgkin lymphoma

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Contributor Information and Disclosures

Author

Chung-Che Chang, MD, PhD, Medical Director and Program Director of Hematopathology, Associate Professor of Pathology, Department of Hematopathology, Methodist Hospital, Weil Medical College, Cornell University, Houston
Chung-Che Chang, MD, PhD is a member of the following medical societies: American Medical Association, American Society for Clinical Pathology, College of American Pathologists, and International Academy of Pathology
Disclosure: Nothing to disclose.

Coauthor(s)

Scott M Acker, MD, Associate Professor, Director of Dermatopathology, Departments of Dermatology and Pathology, University of Alabama at Birmingham
Scott M Acker, MD is a member of the following medical societies: Alpha Omega Alpha, American Medical Association, American Society for Clinical Pathology, and Southern Medical Association
Disclosure: Nothing to disclose.

Medical Editor

Günter Burg, MD, Professor and Chairman Emeritus, Department of Dermatology, University of Zürich School of Medicine; Delegate of The Foundation for Modern Teaching and Learning in Medicine Faculty of Medicine, University of Zürich, Switzerland
Günter Burg, MD is a member of the following medical societies: American Academy of Dermatology, American Dermatological Association, International Society for Dermatologic Surgery, North American Clinical Dermatologic Society, and Pacific Dermatologic Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA
Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Daniel S Loo, MD, Associate Professor of Dermatology, Residency Program Director, Department of Dermatology, Tufts Medical Center
Daniel S Loo, MD is a member of the following medical societies: American Academy of Dermatology and Association of Professors of Dermatology
Disclosure: Nothing to disclose.

CME Editor

Joel M Gelfand, MD, MSCE, Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania
Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology
Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds Investigator; Genentech Grant/research funds investigator; Centocor Consulting fee Consulting; Abbott Grant/research funds investigator; Abbott Consulting fee Consulting; Novartis  investigator; Pfizer Grant/research funds investigator; Celgene Consulting fee DMC Chair; NIAMS and NHLBI Grant/research funds investigator

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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