Cutaneous CD30+ (Ki-1) Anaplastic Large-Cell Lymphoma Follow-up
- Author: Cary Chisholm, MD; Chief Editor: Dirk M Elston, MD more...
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 nucleophosmin-anaplastic lymphoma kinase (NPM-ALK) transcripts or anaplastic lymphoma kinase (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+ ALCLs 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.
Diamantidis MD, Papadopoulos A, Kaiafa G, et al. Differential diagnosis and treatment of primary, cutaneous, anaplastic large cell lymphoma: not always an easy task. Int J Hematol. Sep 2009;90(2):226-9. [Medline].
Savage KJ, Lee-Harris N, Vose JM, et al. ALK-negative anaplastic large-cell lymphoma (ALCL) is clinically and immunophenotypically different from both ALK-positive ALCL and peripheral T-cell lymphoma, not otherwise specified: report from the International Peripheral T-Cell Lymphoma Project. Blood. 2008;111:5496-5504.
[Guideline] Willemze R, Jaffe ES, Burg G, Cerroni L, Berti E, Swerdlow SH. WHO-EORTC classification for cutaneous lymphomas. Blood. May 15 2005;105(10):3768-85. [Medline].
Kong YY, Dai B, Kong JC, Lu HF, Shi DR. Neutrophil/eosinophil-rich type of primary cutaneous anaplastic large cell lymphoma: a clinicopathological, immunophenotypic and molecular study of nine cases. Histopathology. Aug 2009;55(2):189-96. [Medline].
Massone C, El-Shabrawi-Caelen L, Kerl H, Cerroni L. The morphologic spectrum of primary cutaneous anaplastic large T-cell lymphoma: a histopathologic study on 66 biopsy specimens from 47 patients with report of rare variants. J Cutan Pathol. Jan 2008;35(1):46-53. [Medline].
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].
Mao X, Orchard G, Lillington DM, Russell-Jones R, Young BD, Whittaker S. Genetic alterations in primary cutaneous CD30+ anaplastic large cell lymphoma. Genes Chromosomes Cancer. Jun 2003;37(2):176-85. [Medline].
Prochazkova M, Chevret E, Beylot-Barry M, Sobotka J, Vergier B, Delaunay M. Chromosomal imbalances: a hallmark of tumour relapse in primary cutaneous CD30+ T-cell lymphoma. J Pathol. Nov 2003;201(3):421-9. [Medline].
van Kester MS, Tensen CP, Vermeer MH, et al. Cutaneous anaplastic large cell lymphoma and peripheral T-cell lymphoma NOS show distinct chromosomal alterations and differential expression of chemokine receptors and apoptosis regulators. J Invest Dermatol. Feb 2010;130(2):563-75. [Medline].
Feldman AL, Law M, Remstein ED, Macon WR, Erickson LA, Grogg KL. Recurrent translocations involving the IRF4 oncogene locus in peripheral T-cell lymphomas. Leukemia. Mar 2009;23(3):574-80. [Medline].
Bonzheim I, Geissinger E, Roth S, 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].
Braun FK, Hirsch B, Al-Yacoub N, et al. Resistance of cutaneous anaplastic large-cell lymphoma cells to apoptosis by death ligands is enhanced by CD30-mediated overexpression of c-FLIP. J Invest Dermatol. Mar 2010;130(3):826-40. [Medline].
Kim YC, Yang WI, Lee MG, et al. Epstein-Barr virus in CD30 anaplastic large cell lymphoma involving the skin and lymphomatoid papulosis in South Korea. Int J Dermatol. Nov 2006;45(11):1312-6. [Medline].
Woo DK, Jones CR, Vanoli-Storz MN, Kohler S, Reddy S, Advani R. Prognostic factors in primary cutaneous anaplastic large cell lymphoma: characterization of clinical subset with worse outcome. Arch Dermatol. Jun 2009;145(6):667-74. [Medline].
Skiljevic D, Nikolic MM, Milinkovic M, Bonaci-Nikolic B. Regressing anaplastic CD30-positive large-cell lymphoma of the skin. Acta Dermatovenerol Alp Panonica Adriat. Sep 2006;15(3):131-4. [Medline].
Le Delay MC, Reiter A, Williams D, et al. Prognostic factors in childhood anaplastic large cell lymphoma: results of a large European intergroup study. Blood. 2008;111(3):1560-1566.
Lowe EJ, Sposto R, Perkins SL, et al. Intensive chemotherapy for systemic anaplastic large cell lymphoma in children and adolescents: final results of Children's Cancer Group Study 5941. Pediatr Blood Cancer. Mar 2009;52(3):335-9. [Medline].
Lamant L, Pileri S, Sabattini E, Brugieres L, Jaffe ES, Delsol G. Cutaneous presentation of ALK-positive anaplastic large cell lymphoma following insect bites: evidence for an association in 5 cases. Haematologica. Nov 30 2009;[Medline].
Madray MM, Greene JF Jr, Butler DF. Glatiramer acetate-associated, CD30+, primary, cutaneous, anaplastic large-cell lymphoma. Arch Neurol. Oct 2008;65(10):1378-9. [Medline].
Morizane S, Setsu N, Yamamoto T, Hamada T, Nakanishi G, Asagoe K. Ichthyosiform eruptions in association with primary cutaneous T-cell lymphomas. Br J Dermatol. Jul 2009;161(1):115-20. [Medline].
Benner MF, Willemze R. Bone marrow examination has limited value in the staging of patients with an anaplastic large cell lymphoma first presenting in the skin. Retrospective analysis of 107 patients. Br J Dermatol. Nov 2008;159(5):1148-51. [Medline].
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].
Werner B, Massone C, Kerl H, et al. Large CD30-positive cells in benign, atypical lymphoid infiltrates of the skin. J Cutan Pathol. 2009;35:1100-1107.
Medeiros LJ, Elenitoba-Johnson KS. Anaplastic Large Cell Lymphoma. Am J Clin Pathol. May 2007;127(5):707-22. [Medline].
Goteri G, Simonetti O, Rupoli S, et al. Differences in survivin location and Bcl-2 expression in CD30+ lymphoproliferative disorders of the skin compared with systemic anaplastic large cell lymphomas: an immunohistochemical study. Br J Dermatol. 2009;157:41-48.
Yamaguchi T, Ohshima K, Karube K, et al. Expression of chemokines and chemokine receptors in cutaneous CD30+ lymphoproliferative disorders. Br J Dermatol. May 2006;154(5):904-9. [Medline].
Kleinhans M, Tun-Kyi A, Gilliet M, Kadin ME, Dummer R, Burg G. Functional expression of the eotaxin receptor CCR3 in CD30+ cutaneous T-cell lymphoma. Blood. Feb 15 2003;101(4):1487-93. [Medline].
Kempf W, Kutzner H, Cozzio A, Sander CA, Pfaltz MC, Müller B. MUM1 expression in cutaneous CD30+ lymphoproliferative disorders: a valuable tool for the distinction between lymphomatoid papulosis and primary cutaneous anaplastic large-cell lymphoma. Br J Dermatol. Jun 2008;158(6):1280-7. [Medline].
Benner MF, Jansen PM, Meijer CJ, Willemze R. Diagnostic and prognostic evaluation of phenotypic markers TRAF1, MUM1, BCL2 and CD15 in cutaneous CD30-positive lymphoproliferative disorders. Br J Dermatol. Jul 2009;161(1):121-7. [Medline].
Hernandez-Machin B, de Misa RF, Montenegro T, et al. MUM1 expression does not differentiate primary cutaneous anaplastic large-cell lymphoma and lymphomatoid papulosis. Br J Dermatol. 2009;160:713.
Assaf C, Hirsch B, Wagner F, Lucka L, Grünbaum M, Gellrich S. Differential expression of TRAF1 aids in the distinction of cutaneous CD30-positive lymphoproliferations. J Invest Dermatol. Aug 2007;127(8):1898-904. [Medline].
Querfeld C, Kuzel TM, Guitart J, Rosen ST. Primary cutaneous CD30+ lymphoproliferative disorders: new insights into biology and therapy. Oncology (Williston Park). May 2007;21(6):689-96; discussion 699-700,. [Medline].
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].
Fujita H, Nagatani T, Miyazawa M, Wada H, Koiwa K, Komatsu H. Primary cutaneous anaplastic large cell lymphoma successfully treated with low-dose oral methotrexate. Eur J Dermatol. May-Jun 2008;18(3):360-1. [Medline].
Fanin R, Sperotto A, Silvestri F, 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].
Bekkenk MW, Geelen FA, van Voorst Vader PC, 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].
Rosen ST, Querfeld C. Primary cutaneous T-cell lymphomas. Hematology Am Soc Hematol Educ Program. 2006;323-30, 513. [Medline].
Shehan JM, Kalaaji AN, Markovic SN, et al. Management of multi-focal primary cutaneous CD30+ anaplastic large cell lymphoma. J Am Acad Dermatol. 2004;51:103-110.
Seifert G, Tautz C, Seeger K, Henze G, Laengler A. Therapeutic use of mistletoe for CD30+ cutaneous lymphoproliferative disorder/lymphomatoid papulosis. J Eur Acad Dermatol Venereol. Apr 2007;21(4):558-60. [Medline].
Muhlhoff C, Rubben A, Gassler N, Megahed M. [Primary cutaneous CD30+ ALK(-) anaplastic large cell T-cell lymphoma]. Hautarzt. Dec 2009;60(12):954-6. [Medline].
de Bruin PC, Beljaards RC, van Heerde P, 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].
Duncan LM. Cutaneous lymphoma. Understanding the new classification schemes. Dermatol Clin. Jul 1999;17(3):569-92. [Medline].
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].
LeBoit PE. Lymphomatoid papulosis and cutaneous CD30+ lymphoma. Am J Dermatopathol. Jun 1996;18(3):221-35. [Medline].
Lee JH, Cheng AL, Lin CW, Kuo SH. Multifocal primary cutaneous CD30+ anaplastic large cell lymphoma responsive to thalidomide: the molecular mechanism and the clinical application. Br J Dermatol. Apr 2009;160(4):887-9. [Medline].
Mori M, Manuelli C, Pimpinelli N, 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].
Olsen SH, Ma L, Schnitzer B, Fullen DR. Clusterin expression in cutaneous CD30-positive lymphoproliferative disorders and their histologic simulants. J Cutan Pathol. Mar 2009;36(3):302-7. [Medline].
Paulli M, Berti E, Boveri E, 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].
Paulli M, Berti E, Rosso R, 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].
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].
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].
Vergier B, Beylot-Barry M, Pulford K, 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].
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].
Willemze R, Kerl H, Sterry W, et al. EORTC classification for primary cutaneous lymphomas: a proposal from the Cutaneous Lymphoma Study Group of the European Organization for Research and Treatment of Cancer. Blood. Jul 1 1997;90(1):354-71. [Medline].

