Case report
Primary cutaneous CD30-positive anaplastic large cell lymphoma in an 80-year-old man: a case report from Nepal Sudip Parajuli1, MBBS, MD, Dipendra Gurung2, MBBS, MD, Upama Paudel1, MBBS, MD, Dinesh Binod Pokhrel1, MBBS, MD, and Werner Kempf3, MD
1 Department of Dermatology, Tribhuvan University Teaching Hospital, Kathmandu, Nepal, 2DI Skin Hospital and Research Centre, Kathmandu, Nepal, 3Kempf and Pfaltz Histological Diagnostics, Zu¨rich, Switzerland, and Department of Dermatology, University Hospital Zu¨rich, Zu¨rich, Switzerland
Correspondence Sudip Parajuli, MBBS, MD Department of Dermatology Tribhuvan University Teaching Hospital Maharajgunj Medical Campus Kathmandu Nepal E-mail: [email protected] Conflicts of interest: None.
1498
Introduction
Case history
Primary cutaneous CD30-positive lymphoproliferative disorders (LPDs) represent about 25% of all cases of cutaneous T-cell lymphomas.1 LPDs are thought to represent a spectrum of diseases, with lymphomatoid papulosis at one end and primary cutaneous anaplastic large cell lymphoma (PCALCL) at the other.2,3 CD30-positive primary cutaneous large cell lymphomas (PCLCL) are typically associated with poor prognosis when they are systemic, though they have a favorable prognosis when confined to the skin. CD30 expression is a much more important prognostic parameter than is the extent of skin disease at presentation. Beljaards et al.4 described that patients with CD30-negative PCLCL died of progressive disease compared to patients with CD30-positive PCLCL. Lymphoma is a rare diagnosis in Nepal where such noduloulcerative growths are mainly due to cutaneous tuberculosis and deep fungal infections such as sporotrichosis. However, in a tertiary center such as the Tribhuvan University Teaching Hospital (Kathmandu, Nepal), cutaneous lymphomas should also be considered in the differential diagnosis of such presentations, and relevant investigations should be done to confirm them.
An 80-year-old man presented to us with a single noduloulcerative growth on the flexor aspect of the left forearm of three months’ duration. It started as a small 0.5 9 0.5 cm asymptomatic papule that was slowly increasing in size. He pricked the lesion, which then turned into a noduloulcerative lesion. The histopathological examination showed predominant mononuclear cells, mostly lymphocytes, plasma cells, macrophages, and ill-defined granulomas, in the dermis. With this finding, two possibilities were considered: lupus vulgaris and localized cutaneous leishmaniasis. The patient was started on empirical antitubercular therapy (ATT). However, no response was seen after three months of treatment; instead the number of lesions increased around the previous lesion (Fig. 1). Repeat histopathological examination showed atrophic epidermis. The dermis was heavily infiltrated by monomorphous cells having large vesicular nuclei and one or more prominent nucleoli, accompanied by numerous mitoses. These features were suggestive of cutaneous lymphoma. Immunohistochemistry was not done because it was unavailable in Nepal.
International Journal of Dermatology 2014, 53, 1498–1500
ª 2014 The International Society of Dermatology
Parajuli et al.
A case report of cutaneous lymphoma from Nepal
Case report
with the clinical presentation, the negative results of staging examinations, and lack of expression of ALK-1 and EMA, the proliferation of pleomorphic and anaplastic CD30-positive large lymphoid tumor cells was assigned to PCALCL (according to WHO classification 2008). The patient was started on low-dose oral methotrexate (MTX) 7.5 mg/week. There was complete resolution of the lesion within 1½ months. No relapse was observed during the follow-up period of one year. Discussion
Figure 1 Multiple satellite lesions of the left forearm
Complete systemic evaluation and investigations for lymphoma were done and were normal. He was referred to an oncology physician where it was decided to put him on chemotherapy; however, this was declined by the patient. He presented again after six months with history of a nodular lesion on the left cubital region approximately 4 9 4 cm in size. In addition, there were multiple nodules of varying size on the left forearm. A nodule on the left cubital fossa was excised in toto. Histopathology showed ulceration and acanthotic epidermis. In all dermal layers and extending into the subcutaneous fat tissue, there was a nodular proliferation of cohesive sheets of medium-sized to large pleomorphic and anaplastic lymphoid cells with moderately dense chromatin nuclei with atypical mitotic figures and numerous apoptotic tumor cells. CD2 was reactive in almost all tumor cells. CD3 was only focally expressed by tumor cells (
Primary cutaneous CD30-positive anaplastic large cell lymphoma in an 80-year-old man: a case report from Nepal Sudip Parajuli1, MBBS, MD, Dipendra Gurung2, MBBS, MD, Upama Paudel1, MBBS, MD, Dinesh Binod Pokhrel1, MBBS, MD, and Werner Kempf3, MD
1 Department of Dermatology, Tribhuvan University Teaching Hospital, Kathmandu, Nepal, 2DI Skin Hospital and Research Centre, Kathmandu, Nepal, 3Kempf and Pfaltz Histological Diagnostics, Zu¨rich, Switzerland, and Department of Dermatology, University Hospital Zu¨rich, Zu¨rich, Switzerland
Correspondence Sudip Parajuli, MBBS, MD Department of Dermatology Tribhuvan University Teaching Hospital Maharajgunj Medical Campus Kathmandu Nepal E-mail: [email protected] Conflicts of interest: None.
1498
Introduction
Case history
Primary cutaneous CD30-positive lymphoproliferative disorders (LPDs) represent about 25% of all cases of cutaneous T-cell lymphomas.1 LPDs are thought to represent a spectrum of diseases, with lymphomatoid papulosis at one end and primary cutaneous anaplastic large cell lymphoma (PCALCL) at the other.2,3 CD30-positive primary cutaneous large cell lymphomas (PCLCL) are typically associated with poor prognosis when they are systemic, though they have a favorable prognosis when confined to the skin. CD30 expression is a much more important prognostic parameter than is the extent of skin disease at presentation. Beljaards et al.4 described that patients with CD30-negative PCLCL died of progressive disease compared to patients with CD30-positive PCLCL. Lymphoma is a rare diagnosis in Nepal where such noduloulcerative growths are mainly due to cutaneous tuberculosis and deep fungal infections such as sporotrichosis. However, in a tertiary center such as the Tribhuvan University Teaching Hospital (Kathmandu, Nepal), cutaneous lymphomas should also be considered in the differential diagnosis of such presentations, and relevant investigations should be done to confirm them.
An 80-year-old man presented to us with a single noduloulcerative growth on the flexor aspect of the left forearm of three months’ duration. It started as a small 0.5 9 0.5 cm asymptomatic papule that was slowly increasing in size. He pricked the lesion, which then turned into a noduloulcerative lesion. The histopathological examination showed predominant mononuclear cells, mostly lymphocytes, plasma cells, macrophages, and ill-defined granulomas, in the dermis. With this finding, two possibilities were considered: lupus vulgaris and localized cutaneous leishmaniasis. The patient was started on empirical antitubercular therapy (ATT). However, no response was seen after three months of treatment; instead the number of lesions increased around the previous lesion (Fig. 1). Repeat histopathological examination showed atrophic epidermis. The dermis was heavily infiltrated by monomorphous cells having large vesicular nuclei and one or more prominent nucleoli, accompanied by numerous mitoses. These features were suggestive of cutaneous lymphoma. Immunohistochemistry was not done because it was unavailable in Nepal.
International Journal of Dermatology 2014, 53, 1498–1500
ª 2014 The International Society of Dermatology
Parajuli et al.
A case report of cutaneous lymphoma from Nepal
Case report
with the clinical presentation, the negative results of staging examinations, and lack of expression of ALK-1 and EMA, the proliferation of pleomorphic and anaplastic CD30-positive large lymphoid tumor cells was assigned to PCALCL (according to WHO classification 2008). The patient was started on low-dose oral methotrexate (MTX) 7.5 mg/week. There was complete resolution of the lesion within 1½ months. No relapse was observed during the follow-up period of one year. Discussion
Figure 1 Multiple satellite lesions of the left forearm
Complete systemic evaluation and investigations for lymphoma were done and were normal. He was referred to an oncology physician where it was decided to put him on chemotherapy; however, this was declined by the patient. He presented again after six months with history of a nodular lesion on the left cubital region approximately 4 9 4 cm in size. In addition, there were multiple nodules of varying size on the left forearm. A nodule on the left cubital fossa was excised in toto. Histopathology showed ulceration and acanthotic epidermis. In all dermal layers and extending into the subcutaneous fat tissue, there was a nodular proliferation of cohesive sheets of medium-sized to large pleomorphic and anaplastic lymphoid cells with moderately dense chromatin nuclei with atypical mitotic figures and numerous apoptotic tumor cells. CD2 was reactive in almost all tumor cells. CD3 was only focally expressed by tumor cells (
