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CASE REPORT
Year : 2016  |  Volume : 5  |  Issue : 3  |  Page : 230-233

Multifocal primary cutaneous anaplastic lymphoma


Department of Dermatology, Venereology and Leprology, Kurnool Medical College, Kurnool, Andhra Pradesh, India

Date of Web Publication10-Oct-2016

Correspondence Address:
Panthalla Vijaya Lakshmi
Department of Dermatology, Venereology and Leprology, Kurnool Medical College, Flat No. 309, MS9 Priya Towers, Deva Nagar, Kurnool, Andhra Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2277-8632.191853

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  Abstract 

Primary cutaneous anaplastic large cell lymphoma (CALCL) is a rare lymphoproliferative disorder characterized by proliferation of CD4 T helper cells that express CD30+ marker with anaplastic cytology. These lymphomas are seen in adults and present as solitary or multiple and often ulcerated nodules most often on the trunk. Multifocal lesions are seen in only 20% of patients. Careful staging with computed tomography scan is required to exclude systemic CD30+ anaplastic large cell lymphoma. Multifocal primary CALCL, not extensively reported is more likely to progress to extracutaneous involvement than localized disease. Herein, we report a case of an 83-year-old male with a 6 months history of minimally pruritic generalized ulcerated nodules.

Keywords: Anaplastic large cell lymphoma, CD30+, multifocal lesions


How to cite this article:
Lakshmi PV, Reddy IC, Ala M. Multifocal primary cutaneous anaplastic lymphoma. J NTR Univ Health Sci 2016;5:230-3

How to cite this URL:
Lakshmi PV, Reddy IC, Ala M. Multifocal primary cutaneous anaplastic lymphoma. J NTR Univ Health Sci [serial online] 2016 [cited 2019 Jul 22];5:230-3. Available from: http://www.jdrntruhs.org/text.asp?2016/5/3/230/191853


  Introduction Top


As per the WHO-EORTC classification,[1] primary cutaneous lymphoproliferative disorders consist of a spectrum of conditions including lymphomatoid papulosis and CD30+ anaplastic large cell lymphoma (ALCL), which are defined on the basis of clinical and pathological features. Primary cutaneous ALCL (CALCL) is a rare disorder with an incidence of only 1.2-1.9 cases in 1,000,000. Multifocal lesions are seen in only 20% of these cases. Primary ALCL must be differentiated from systemic ALCL which requires aggressive therapy. We report a case of primary CALCL with multifocal lesions.


  Case Report Top


An 83-year-old man presented with multiple ulcerated plaques over face, trunk, both upper and lower extremities for 6 months. The lesions began as erythematous papules over upper extremities, which subsequently enlarged to form nodules, which eventually ulcerated. The lesions were mildly itchy and there was a history of serosanguinous discharge from the lesions. There were no constitutional symptoms.

He was initially treated with oral antibiotics and topical steroids by a local physician as the condition was presumed to be of infectious origin, but there was no improvement.

Clinically the plaques were indurated and tender with dusky erythematous rolled edges. There was a large ulcer of size 10 cm × 15 cm size over right forearm. Similar lesions were present over trunk and extremities. Multiple hyper-pigmented patches were present over the back, and a granulomatous plaque was present over the nose [Figure 1], [Figure 2], [Figure 3].
Figure 1: Granulomatous plaque over nose and a ulcerated plaque over chin, front of chest

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Figure 2: Indurated plaque over back with dusky erythematous rolled out edges

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Figure 3: Ulcerated lesion over right forearm

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There was no cervical, axillary or inguinal lymphadenopathy.

Routine blood investigations were normal.

Skin biopsy revealed a nodular interstitial infiltrate of predominantly large lymphocytes and plasmacytoid cells, involving the whole of reticular dermis and extending to the sub-cutis. The cells had pleomorphic nuclei and nucleoli, frequent mitosis was evident. The histopathological features were consistent with ALCL [Figure 4] and [Figure 5].
Figure 4: Nodular interstitial infiltrate of predominantly large lymphocytes and plasmacytoid cells involving whole of reticular dermis suggestive of anaplastic large cell lymphoma

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Figure 5: Large anaplastic lymphocytic infiltrate in dermis

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In order to rule out systemic involvement ultrasonogram of abdomen, computed tomography scan with contrast of thorax and abdomen, bone marrow aspiration studies were done, which revealed no abnormality.

Immunohistochemistry showed that the cell stained positive to CD30, CD4, CD8, and anaplastic lymphoma kinase (ALK) 1 was faintly positive [Figure 6], [Figure 7], [Figure 8].
Figure 6: Immunohistochemistry showing CD30+ anaplastic lymphocytes

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Figure 7: Immunohistochemistry showing CD4 and CD8 positive lymphocytes

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Figure 8: Anaplastic lymphocytes showing faintly positive anaplastic lymphoma kinase on immunohistochemistry

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This patient was referred to the medical oncologist who started him on multiagent chemotherapy consisting of cyclophosphamide, adriamycin, vincristine, and prednisolone.

The lesions regressed in size, and there was decreased discharge from the lesions after starting chemotherapy. However, the patient developed severe cytopenia, fever, and altered sensorium 1 month after completion of the first cycle of chemotherapy and he died a week later due to side effects of chemotherapy.


  Discussion Top


Primary CALCL is a rare lymphoproliferative disorder that mainly affects adults with a male to female ratio of 1.47:1 and with a mean age of onset of 52 years.

The clinical presentations range from solitary or localized lesions to multifocal nodules or tumors, some of which can be ulcerated and some regress spontaneously.[2] Multifocal lesions are seen only in 20% of cases.

Primary CALCL must be distinguished from anaplastic transformation of other cutaneous lymphomas, especially mycosis fungoides and cutaneous infiltration of systemic ALCL.[3],[4]

Transformed mycosis fungoides can be distinguished by the presence of patches or plaques for years and the histological presence of epidermotropism.

Systemic ALCL should be suspected if any extracutaneous site other than regional lymph nodes is affected.

Anaplastic lymphoma kinase + primary systemic ALCL frequently affects younger patients and is more responsive to chemotherapy, whereas ALK -ve primary systemic ALCL carries a poorer prognosis.

Histological examination of a lesion of CALCL typically shows a diffuse infiltrate composed of large cells with an anaplastic, pleomorphic or immunoblastic cytomorphology and expression of CD30 antigen by more than 75% of the tumor cells. This was demonstrated in our patient. The tumor cells were CD30+ and ALK1 was faintly positive. The neoplastic cells showed an activated CD4+ T cell phenotype. Unlike systemic CD30+ lymphomas, most CALCL express the cutaneous lymphocyte antigen, but do not express epithelial membrane antigen and ALK which indicate the 2,5 chromosome translocation or its variants.

The choice of treatment in CALCL is based on the size, the extent and the clinical behavior of the skin lesion. Solitary or localized nodules or plaques can be treated with radiotherapy or surgical excision.[3],[5] Patients with multifocal skin lesions or regional lymph node involvement may respond to systemic therapies such as methotrexate, systemic retinoids, or interferon-alpha. Single or multi-agent chemotherapy may be required in resistant cases.[6]

The standard chemotherapy for this disease consisted of CHOP regimen.

Combination chemotherapy was thought to be the most appropriate first line treatment of multifocal primary cutaneous disease with or without evidence of extracutaneous disease.[7] However, a study from Dutch lymphoma group found that patients treated with CHOP chemotherapy regimen universally experienced one or more relapses in the skin.[8] On the basis of it, the authors recommended that traditional combination chemotherapy should not be used for multifocal ALCL involving only the skin. This is particularly applicable to elderly patients as in our case in whom chemotherapy induced side effects are fatal.

The prognosis being excellent for CD30+ primary CALCL, with a 5 years survival rate of 90%[9] it may be better to go for radiotherapy or low dose methotrexate as the initial modality of treatment in multifocal skin restricted disease.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.


  Acknowledgment Top


Our sincere thanks to Dr. Uday Khopkar, Dermatopathologist, Mumbai, India.

 
  References Top

1.
Willemze R, Jaffe ES, Burg G, Cerroni L, Berti E, Swerdlow SH, et al. WHO-EORTC classification for cutaneous lymphomas. Blood 2005;105:3768-85.  Back to cited text no. 1
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2.
Stein H, Foss HD, Dürkop H, Marafioti T, Delsol G, Pulford K, et al. CD30(+) anaplastic large cell lymphoma: A review of its histopathologic, genetic, and clinical features. Blood 2000;96:3681-95.  Back to cited text no. 2
    
3.
Beljaards RC, Kaudewitz P, Berti E, Gianotti R, Neumann C, Rosso R, et al. Primary cutaneous CD30-positive large cell lymphoma: Definition of a new type of cutaneous lymphoma with a favorable prognosis. A European Multicenter Study of 47 patients. Cancer 1993;71:2097-104.  Back to cited text no. 3
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4.
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 1998;22:1192-202.  Back to cited text no. 4
    
5.
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 2000;95:3653-61.  Back to cited text no. 5
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6.
Rijlaarsdam JU, Huijgens PC, Beljaards RC, Bakels V, Willemze R. Oral etoposide in the treatment of cutaneous large-cell lymphomas. A preliminary report of four cases. Br J Dermatol 1992;127:524-8.  Back to cited text no. 6
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7.
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 1993;28:973-80.  Back to cited text no. 7
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8.
Shehan JM, Kalaaji AN, Markovic SN, Ahmed I. Management of multifocal primary cutaneous CD30 anaplastic large cell lymphoma. J Am Acad Dermatol 2004;51:103-10.  Back to cited text no. 8
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9.
Fiorani C, Vinci G, Sacchi S, Bonaccorsi G, Artusi T. Primary systemic anaplastic large-cell lymphoma (CD30+): Advances in biology and current therapeutic approaches. Clin Lymphoma 2001;2:29-37.  Back to cited text no. 9
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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]



 

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Acknowledgment
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