Lymphomatoid papulosis (LyP) and primary cutaneous anaplastic large cell lymphoma (ALCL) are grouped under the category of cutaneous T-cell lymphomas as CD30+ lymphoproliferative disorders. Though LyP is clinically characterized by chronic recurrent papulonodular cutaneous lesions, it shows malignant features on the histologic findings. LyP is associated with lymphomas, including primary cutaneous ALCL, mycosis fungoides and other lymphoproliferative disorders. A few cases of LyP related to ALCL have been reported in Korea. We report here on an interesting case of primary cutaneous ALCL that developed in succession after LyP in a 38-year-old female.
Adult ; Female ; Humans ; Korea ; Lymphoma ; Lymphoma, Large-Cell, Anaplastic ; Lymphoma, Primary Cutaneous Anaplastic Large Cell ; Lymphoma, T-Cell, Cutaneous ; Lymphomatoid Papulosis ; Lymphoproliferative Disorders ; Mycosis Fungoides

Mycosis fungoides (MF) is the most frequent cutaneous T cell lymphoma (CTCL). Since the major tumor cell of MF is the helper T cell, positive markers are usually CD3, CD4 and CD45RO. Some MFs show CD30 positivity and the major differential diagnosis for MF with CD30 positivity includes transformed MF and MF concurrent with primary cutaneous anaplastic large cell lymphoma and lymphomatoid papulosis. As each disease shows a different prognosis, an exact diagnosis is crucial for proper treatment. We now report a case of 44-year-old male patient with mycosis fungoides which developed several papules on preexisting MF patches. On biopsy of the newly formed papules, CD 30 positive cells were observed and the histologic features were consistent with lymphomatoid papulosis. Both the lesions of lymphomatoid papulosis and MF responded well to narrow band ultraviolet B phototherapy.
Adult ; Biopsy ; Diagnosis, Differential ; Humans ; Lymphoma, Primary Cutaneous Anaplastic Large Cell ; Lymphoma, T-Cell, Cutaneous ; Lymphomatoid Papulosis ; Male ; Mycosis Fungoides ; Phototherapy ; Prognosis

OBJECTIVETo investigate the frequency of different types of mature T- and NK-cell lymphomas diagnosed in a 4-year period at Sun Yat-sen University Cancer Center, and to study baseline CD30 for potential anti-CD30 targeted therapy in mature T- and NK-cell lymphoma.

METHODSAll cases of mature T- and NK-cell lymphoma diagnosed at Sun Yat-sen University Cancer Center from September 1, 2009 to August 31, 2013, were reviewed. Paraffin-blocks of available 164 consecutive cases were stained for CD30 immunohistochemistry using EnVision protocol.

RESULTSA total of 625 cases of mature T- and NK-cell lymphomas were diagnosed and the most common type was extranodal NK/T cell lymphoma (ENKTL), nasal type 319 (51.0%) cases, followed by angioimmunoblastic T-cell lymphoma (AITL) (119 cases, 19.0%), peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS) (81 cases, 13.0%), and anaplastic large-cell lymphoma (ALCL), including 48 cases (7.7%) of systematic ALCL and 11 cases (1.8%) of primary cutaneous ALCL. Besides ALCL, ENKTL had the highest expression rate of CD30 among the 164 cases, with positivity observed in 41 cases (62.1%, 41/66). Only 1 case of PTCL-NOS was CD30 positive. CD30 was not expressed in all 28 cases of AITL and other rare types of mature T- and NK-cell lymphoma.

CONCLUSIONSThe frequency of different types of mature T- and NK-cell lymphoma encountered at Sun Yat-sen University Cancer Center was similar to that seen in other areas of China and other Asia countries. CD30 expression is different among several types of mature T- and NK-cell lymphoma. In addition to ALCL, ENKTL has the highest expression rate of CD30, which may be a candidate disease for anti-CD30 targeted therapy.

China ; epidemiology ; Humans ; Immunohistochemistry ; Killer Cells, Natural ; Lymphoma, Extranodal NK-T-Cell ; epidemiology ; pathology ; Lymphoma, Large-Cell, Anaplastic ; epidemiology ; pathology ; Lymphoma, Primary Cutaneous Anaplastic Large Cell ; epidemiology ; pathology ; Lymphoma, T-Cell, Peripheral ; epidemiology ; pathology ; T-Lymphocytes

Since the discovery of the CD30 molecule, its expression has been considered a reliable marker for CD30+ lymphomas, including lymphomatoid papulosis, primary cutaneous anaplastic large cell lymphoma and borderline cases. However, CD30+ cells can be observed in Hodgkin's lymphoma, as well as in T-cell and B-cell lymphomas or NK cell lymphomas. Furthermore, it can also be found in reactive inflammatory disorders or in non-lymphoid neoplasms. It is very important to differentiate between lymphomas and reactive inflammatory disorders using a combination of clinical, histological, phenotypic and molecular analyses. We report a case of atypical CD30+ lymphocytic hyperplasia in a 57-year-old man following tick bite.
Bites and Stings ; Hodgkin Disease ; Humans ; Hyperplasia ; Killer Cells, Natural ; Lymphoma ; Lymphoma, B-Cell ; Lymphoma, Primary Cutaneous Anaplastic Large Cell ; Lymphomatoid Papulosis ; Middle Aged ; T-Lymphocytes ; Ticks

CD30+ lymphoproliferative disorders (LPD) represent a spectrum of T-cell lymphoma including lymphomatoid papulosis and anaplastic large cell lymphoma (ALCL). Epidermis overlying cutaneous CD30+ LPD often shows epidermal hyperplasia, hyperkeratosis, crusting, and ulceration and it is difficult to distinguish from carcinoma such as keratoacanthoma (KA) or squamous cell carcinoma (SCC). Several cases of pseudocarcinomatous hyperplasia mimicking KA or SCC in CD30+ LPD have been reported. The relationship between CD30+ LPD and epithelial proliferations has not yet well understood. It was reported that a variety of mediators, including epidermal growth factor (EGF), transforming growth factor-α and EGFR from CD30+ LPD could attribute to epidermal hyperplasia. However, separate and distinct SCC occurring in CD30+ LPD has rarely been reported. Herein, we present a rare case of coexistence of SCC and cutaneous ALCL located on the same region.
Carcinoma, Squamous Cell* ; Epidermal Growth Factor ; Epidermis ; Epithelial Cells* ; Hyperplasia ; Keratoacanthoma ; Lymphoma ; Lymphoma, Large-Cell, Anaplastic ; Lymphoma, Primary Cutaneous Anaplastic Large Cell* ; Lymphoma, T-Cell ; Lymphomatoid Papulosis ; Lymphoproliferative Disorders ; Ulcer

The diagnosis of primary cutaneous lymphoma is based on a combination of clinical, histological, immunophenotypic and genetic criteria. Nineteen cases of primary cutaneous lymphomas were studied for clinicopathologic, immunophenotypic, and genetic features. Seventeen (89%) cases were T cell origin and two cases (11%) were B cell origin. CD30-positive cutaneous lymphoproliferative disorder was the most frequent subtype, occupying 42% (8 cases) of the cases. CD8 was positive in 5 cases consisting of 3 cutaneous T cell lymphomas and 2 anaplastic large cell lymphomas. CD4 was positive in 2 cases of mycosis fungoides and 3 cases of lymphomatoid papulosis. Six (67%) of 9 cases of cutaneous T cell lymphoma were positive for TIA-1. Ten (83%) out of 12 cases showed clonal rearrangements of TCR gamma genes, however, one T/NK cell lymphoma and one anaplastic large cell lymphoma did not. EBV association was detected only in T/NK cell lymphomas among 10 cases examined. In conclusion, our study showed higher proportion of CD30-positive lymphoproliferative disorders and less frequent mycosis fungoides in Korea compared to the incidences in Western countries. Our immunostaining results suggested that mycosis fungoides and lymphomatoid papulosis are CD4-positive T cell origin, however, the remaining primary cutaneous T cell lymphoma is predominantly CD8-positive cytotoxic T cell origin.
Diagnosis ; Genes, T-Cell Receptor gamma ; Herpesvirus 4, Human ; Incidence ; Korea ; Lymphoma* ; Lymphoma, Large-Cell, Anaplastic ; Lymphoma, T-Cell, Cutaneous ; Lymphomatoid Papulosis ; Lymphoproliferative Disorders ; Mycosis Fungoides

Primary cutaneous anaplastic large cell lymphoma (C-ALCL) is a rare subtype of primary cutaneous lymphoma with a favorable prognosis. Primary cutaneous CD30+ lymphoproliferative disorders, which include C-ALCL and lymphomatoid papulosis, are the second most common group of cutaneous T-cell lymphomas. C-ALCL is comprised of large cells with anaplastic, pleomorphic, or immunoblastic cytomorphology, and indeed, more than 75% of the tumor cells express the CD30 antigen. C-ALCL clinically presents with solitary or localized reddish-brown nodules or tumors, and sometimes indurated papules, and they may be with ulceration covering with dark eschar. Multifocal lesions are seen in 20% of the patients. Extracutaneous dissemination, which mainly involves the regional lymph nodes, occurs in 10% of patients. A 69-year-old man noticed a mild elevated cutaneous lesion containing central ulceration covering with brownish black necrotic tissue on the right lower lip, and the lesion was surgically removed. After the first operation, another skin lesion was developed and the histological examination confirmed the diagnosis, C-ALCL. Eight specimens were excised during the 7-month follow-up period. The patient started the treatment with low-dose oral methotrexate (15 mg/wk) and there was no recurrence for 11 months.
Aged ; Antigens, CD30 ; Diagnosis ; Follow-Up Studies ; Humans ; Lip ; Lymph Nodes ; Lymphoma ; Lymphoma, Primary Cutaneous Anaplastic Large Cell ; Lymphoma, T-Cell, Cutaneous ; Lymphomatoid Papulosis ; Lymphoproliferative Disorders ; Methotrexate ; Prognosis ; Recurrence ; Skin ; Ulcer

BACKGROUND: Recent reports suggest that Epstein-Barr virus (EBV) may play an important role in such a wide spectrum of human neoplasia. Recently, peripheral T cell lymphomas and particularly, angiocentric lymphomas (ACL), increasingly are reported to be associated with EBV.Nasal-type and nasal T/NK cell lymphoma (TNKL) have recently been reported to comprise most of ACLs. The prognosis of these tumors has been extremely poor. OBJECTIVE: The purpose of this study was to investigate EBV association in primary or secondary cutaneous T-cell lymphoproliferative disorders and to identify any prognostic association. METHODS: Thirty six patients with primary or secondary cutaneous T-cell lymphoproliferative (CTCL) disorders were examined to evaluate the presence of Epstein-Barr virus using in situ hybridization for EBV-encoded RNA (EBER). RESULTS: EBER was detected in tumor cells in one third of the total cases (13/36); 4/4 secondary skin lymphoma from nasal TNKL, 8/8 primary cutaneous nasal type TNKLs and 1/5 mycosis fungoides (MF).EBER was not detected in the following disease: 6 cases of anaplastic large cell lymphomas (ALCL) including 2 cases of probable NK-like T cell lineage, 3 lymphomatoid papulosis, 2 CD56 (-) T cell ACLs and 7 subcutaneous panniculitic T-cell lymphomas (SPTL) by Revised European-American Lymphoma (REAL) classification and recent concept of further classification into NK-cell lineage. One case of T-cell pseudolymphoma as a negative control was also negative in EBER. CONCLUSION: High incidence of EBV was observed in primary or secondary CTCLs in Koreans, with predilection for nasal and nasal type TNKL. In MFs, an erythrodermic MF with fatal outcome was associated with EBV and the EBV detection might reflect worse prognosis in MFs as seen in an aggressive course of nasal and nasal type TNKLs.
Cell Lineage ; Classification ; Fatal Outcome ; Herpesvirus 4, Human* ; Humans ; In Situ Hybridization ; Incidence ; Lymphoma ; Lymphoma, Large-Cell, Anaplastic ; Lymphoma, T-Cell ; Lymphoma, T-Cell, Peripheral ; Lymphomatoid Papulosis ; Lymphoproliferative Disorders* ; Mycosis Fungoides ; Prognosis ; Pseudolymphoma ; RNA* ; Skin ; T-Lymphocytes*

Primary cutaneous anaplastic large cell lymphoma (C-ALCL) is rare among skin malignancies. C-ALCL usually manifests as reddish or violet nodules. Surgical excision or radiation therapy is generally considered as first-line therapy, but a clinically aggressive disease may require multiagent chemotherapy. Establishing a proper diagnosis of C-ALCL is challenging but should be made to avoid inappropriate treatment and its consequences. The authors report a case of medically resolved C-ALCL in an 81-year-old man presented with well-defined nodular lesions on the forehead.
Aged, 80 and over ; Diagnosis ; Drug Therapy ; Forehead ; Humans ; Lymphoma, Large-Cell, Anaplastic ; Lymphoma, Primary Cutaneous Anaplastic Large Cell ; Lymphoma, T-Cell ; Skin ; Viola

BACKGROUND: The bcl-2 is an oncogene involved in tumorigenesis by blocking apoptosis, or programmed cell death and over-expression of bcl-2 protein has been reported in several malignant tumors such as lung cancer, basal cell carcinoma, breast cancer and malignant melanoma. However, there have been only a few studies about bcl-2 expression of cutaneous T cell lymphoma. OBJECTIVE: The purpose of this study was to examine whether there is any difference in expression of bcl-2 between mycosis fungoides(MF), angiocentric T cell lymphoma, angioimmunoblastic T cell lymphoma, subcutaneous T cell lymphoma and anaplastic large cell lymphoma. We also evaluated the statistical significance between expression of bcl-2 and the prognosis of the diseases. METHODS: Routine paraffin sections of formalin-fixed 36 tissues (14 MF, 7 angiocentric T cell lymphoma, 5 subcutaneous panniculitic T cell lymphoma, 2 anaplastic large cell lymphoma, 1 angioimmunoblastic T cell lymphoma, 1 unspecified peripheral T cell lymphoma, 2 small plaque parapsoriasis, 2 psoriasis and 2 lichen planus) were labelled with anti-bcl-2 monoclonal antibody using an avidin- biotin-peroxidase complex. Normal skin for bcl-2 served as negative controls. RESULTS: The results were as follows. l. All cases of benign inflammatory diseases, small plaque parapsoriasis and patch stages of MF showed positive staining for bcl-2. Therefore, there were no differences in expression of bcl-2 among these diseases. 2. In the plaque and tumor stages of mycosis fungoides, statistically significancant differences in bcl-2 expression were not found during disease progression. 3. bcl-2 expression in peripheral T cell lymphoma (five in seven cases of angiocentric T cell lymphoma showed positive staining but all other peripheral T cell lymphoma was negative) decreased significantly (p<0.05) than that of MF. 4. No statistical significance was found between bcl-2 expression and prognosis of cutaneous lymphoma (p>0.05). CONCLUSION: These results suggest that the loss of bcl-2 expression may play a significant role in progression of cutaneous T cell lymphoma except in MF and angiocentric T cell lymphoma.
Apoptosis ; Breast Neoplasms ; Carcinogenesis ; Carcinoma, Basal Cell ; Cell Death ; Disease Progression ; Lichens ; Lung Neoplasms ; Lymphoma ; Lymphoma, Large-Cell, Anaplastic ; Lymphoma, T-Cell ; Lymphoma, T-Cell, Cutaneous* ; Lymphoma, T-Cell, Peripheral ; Melanoma ; Mycosis Fungoides ; Oncogenes ; Paraffin ; Parapsoriasis ; Prognosis ; Psoriasis ; Skin