Lymphomatoid Papulosis Followed by Anaplastic Large Cell Lymphoma in a Pediatric Patient

Lymphomatoid papulosis (LyP) is a benign, self-healing, papular eruption that can wax and wane over time. Transformation to T-cell lymphoma has been well documented in 10% to 20% of adults with LyP. However, this transformation rarely occurs in patients younger than 20 years of age. Here, we present the first known pediatric patient in Korea, a 12-year-old boy who developed a subcutaneous nodule on the scrotum 13 months after papulonecrotic lesions of LyP were identified on both lower extremities and face. Histological and immunohistochemical examination of the subcutaneous nodule revealed anaplastic large cell lymphoma (ALCL). A T-cell receptor gene rearrangement analysis demonstrated an identical rearranged pattern in the two specimens, indicating that a common T-cell clone had proliferated over time in both the LyP and ALCL lesions.     

Primary Cutaneous Ki-1+ Anaplastic Large Cell Lymphoma: A Morphologic,Immunohistochemical and Genetic Study of an Indolent Case

A 59-year-old woman with a large nodular ulcerative lesion on her neck was presented. She had a 3 year history of recurrent cutaneous nodules which spontaneously regressed before regional lymphadenopathies appeared. She has followed an indolent clinical course for seven years after the first overt lymphadenopathies appeared. Histological findings were compatible with anaplastic large cell lymphoma (ALCL). The tumor cells strongly expressed Ki-1 (CD30), HLA-DR, IL-2 receptor (CD25) and leukocyte common antigen. These findings led to the diagnosis of primary cutaneous Ki-1⁺ ALCL. Although the majority of the tumor cells did not express T-cell related antigens, the detection of monoclonal TCR gene rearrangement clearly established the T-cell lineage nature.     

A Case of Cutaneous Ki-1 Positive Anaplastic Large Cell Lymphoma in a Hemodialysed Patient

A 66-year-old woman who suffered from chronic glomerulonephritis had been undergoing hemodialysis for about 10 years. A reddish papule on her waist developed gradually into a nodule (1.9 × 1.4 cm). Histopathological findings showed that the tumor cells had oval to reniform nuclei; multinucleated neoplastic cells and erythrophagocytosis were also present. Immunohistochemical analyses revealed that the membranes of the tumor cells stained for Ber-H2 (Ki-1) and epithelial membrane antigen (EMA), Vimentin was partially positive, but keratin, S-100, chromogranin, leukocyte common antigen (LCA), UCHL-1, MT-1, L-26, MB-1 and C3D-1 were all negative. Anti-human T-cell leukemia virus-1 (HTLV-1) was also negative. No gene rearrangement of the T-cell receptor β-, γ- and δ-chain could be detected. From these results, we diagnosed cutaneous Ki-1 anaplastic large cell lymphoma (ALCL), but the origin could not be determined. The relationship between lymphoma and chronic renal failure and/or hemodialysis was far from clear.     

A Case of Ki-1 Positive Anaplastic Large Cell Lymphoma Transformed from Mycosis Fungoides

A case of cutaneous Ki-1 positive anaplastic large cell lymphoma which developed in the plaque stage of mycosis fungoides was described. A 73-year-old woman who had suffered from pruritic scaly eruptions over her entire body for more than two decades was admitted because of an ulcerated tumor measuring 45 times 55 times 15 mm and several satellite tumors on the buttock. All tumorous lesions were resected without recurrence to date. Histochemical study revealed that the tumor consisted of large anaplastic cells which were Ki-1 (CD30)-positive and LCA-negative. Some of the erythematous plaques contained LCA-positive, small-sized atypical lymphocytes. In other plaques which developed two years later, there were large Ki-1-positive atypical cells. In the specimens obtained from the tumor and the plaque, the same pattern of T-cell receptor gene rearrangements was detected. These findings indicate that both Ki-1 positive anaplastic cells in the tumor and atypical lymphoid cells in the plaques were derived from the same T cell clone.     

Anaplastic large cell lymphoma, so-called Ki-1 lymphoma: a report of a case of long course.

A 55-year-old female with a complaint of tumors on the right lower extremity was reported. The condition was diagnosed as anaplastic large cell lymphoma, so-called Ki-1 lymphoma, by its histological and immunohistochemical features. The clonal proliferation of the infiltrating cells of the skin lesions was confirmed by the analysis of T cell receptor gene rearrangement. The lesions have repeatedly occurred on the right lower extremity for more than ten years. In this report, we also discuss the prognosis of anaplastic large cell lymphoma with or without skin lesions.     

淋巴瘤样丘疹病继发蕈样肉芽肿:共同T细胞克隆增生一例报告

目的 报道一例淋巴瘤样丘疹病(LyP)继发蕈样肉芽肿(MF)，随访27年，并检测其克隆性是否与疾病相关。方法 比较不同时期的皮肤组织病理和免疫表型特点，同时应用聚合酶链反应(PCR)和Southern印迹杂交法，检测皮损组织和外周血标本的T细胞受体基因重排。结果 此例在不同时期各具临床病理学特征，增生的淋巴细胞为成熟的Th细胞，且示共同T细胞克隆。结论 LyP继发MF示相同T细胞克隆，克隆性T细胞与疾病的生物行为无关。     

Lymphomatoid papulosis in children: a study of 10 children registered by the Dutch Cutaneous Lymphoma Working Group

Lymphomatoid papulosis (LyP) is a chronic recurrent self-healing condition, with histological features suggestive of a malignant lymphoma. Only a few cases have been described in children. We report 10 children with this skin disease and compare them with the adult type of LyP and childhood cases described in the literature. Although LyP has the same clinical picture and histology in both age groups, in contrast with the adult type no transformation into malignancy has been described in childhood.     

Lymphomatoid papulosis: clinicopathological comparative study with pityriasis lichenoides et varioliformis acuta.

We have compared the clinical and histopathological features of 6 patients with lymphomatoid papulosis (LP) and 14 patients with pityriasis lichenoides et varioliformis acuta (PLEVA). There were some differences between the clinical features in the two diseases, including the size and appearance of skin lesions and the duration of the course of disease. Ki-1 Ag positive, large, atypical, lymphoid cells were always seen in lymphomatoid papulosis; none of lymphoid cells of pityriasis lichenoides et varioliformis acuta demonstrated this antigen. We conclude that lymphomatoid papulosis and PLEVA, although sharing some common features, should be considered to be different clinical and immunopathological entities.     

Lymphomatoid papulosis: a study of 18 cases*

Lymphomatoid papulosis (LyP) is a cutaneous eruption that is clinically benign but histologically malignant. To date, more than 300 cases have been published. About 10–20% of the patients develop a lymphoma. The purpose of this study was to make a clinicopathological study of 18 patients diagnosed with LyP in our hospital from 1973 to 1990, to characterize cellular infiltrates in the lesions, to find clonal populations of T-cells and to look for predictive factors of malignant lymphoma in LyP patients. Mean age was 48.7 years. The most frequent clinical lesions were papules (88.8%) followed by plaques (38.8%). The localizations were on extremities (100%), trunk (88%), face (22%), palms or soles (11%), perigenital (11%) and scalp (5%). Two patients have been free of disease for more than 5 years. IgA levels are increased in LyP patients. Neither HTLV I nor III can be considered as a cause of the LyP in any of our patients. Associated diseases were found in 6 cases (1 mycosis fungoides, 1 Hodgkin's disease, 2 anaplastic large-cell lymphoma and 2 large plaque parapsoriasis). Some types of parapsoriasis should be included in the ‘spectrum of Ki-1 lymphomas’. 52 skin biopsies were studied. 17% were type A of Willemze, 67% were type B and 15% were transitional. In 12 of the samples follicular or perifollicular infiltration was found. Follicular LyP should not be considered as a distinct type of LyP. Vasculitis is an uncommon finding in LyP. In all the cases studied, large atypical cells were CD30 +; 5/7 cases had lost CD5 and 4/5 cases had lost CD7. In one case, all T-cell antigens were negative. Cerebriform mononuclear cells were always recognized by T-cell antibodies and they were CD30 positive in only two cases. In one case there were more CD8 + than CD4 + cells. In 5 patients skin and blood samples for genetic rearrangement (beta-T) were taken. Only germinal line was found. We did not find any significant difference between those cases in which malignant lymphoma developed and those in which it did not.     

CD20 Positive T Cell Lymphoma Involvement of Skin

CD20 positive T cell lymphoma is a rare condition that is associated with the coexpressions of CD20 and T cell markers, such as, CD3, CD5, or UCHL-1. Positivity for CD20 in this tumor represents an aberrant immunophenotype, but the presence of monoclonal T cell receptor (TCR) gene rearrangements and negativity for immunoglobulin heavy chain gene rearrangement indicate that this tumor is a T cell lymphoma. The majority of cases of CD20 positive T cell lymphoma have been reported as immature peripheral T cell lymphoma not otherwise specified. However, we believe that this disease is likely to be re-listed as a new disease entity after its pathogenesis has been elucidated and more cases have been evaluated. Here, we present a case of peripheral T cell lymphoma coexpressing CD20 and T cell markers with a demonstrable TCR gene rearrangement, in a patient who had been misdiagnosed as having B cell type lymphoma 4 years previously. We hypothesize that in this case initially circulating normal CD20+ T cell subsets underwent neoplastic transformation and CD20 positive T cell lymphoma subsequently developed in the lymph node, and then recurred in the skin due to systemic disease or metastasized from the nodal disease.     

Manual microdissection technique in a case of subcutaneous panniculitis-like T-cell lymphoma: a case report and review

Demonstration of T-cell receptor gene monoclonality often plays an important role in the diagnosis of T-cell lymphoma. When a test to detect monoclonality is performed on whole tissue sections, the presence of a reactive lymphocyte population may reduce sensitivity. This may be especially true for early or borderline cases of lymphoma. Microdissection techniques may be utilized to more readily identify a clonal population of lymphocytes. Subcutaneous panniculitis-like T-cell lymphoma represents a cutaneous lymphoid neoplasm whose clinical course may vary from an indolent, waxing and waning course to an aggressive course resulting in death. We report the first case of a microdissection technique used to facilitate diagnosing a case of subcutaneous panniculitis-like T-cell lympoma.     

Immunophenotypic shift of CD4 and CD8 antigen expression in primary cutaneous T‐cell lymphomas: a clinicopathologic study of three cases

Primary cutaneous T‐cell lymphomas (CTCL) comprise a heterogeneous group of neoplasms with diverse clinical behavior. Mycosis fungoides (MF) is the most common type of CTCL. Immunophenotypical shift during progression of the disease is a rare event and its significance is unknown. We present three primary CTCL cases that showed an immunophenotypical shift and poor prognosis. Conventional hematoxylin/eosin and immunohistochemical‐stained sections were examined in all the cases. Molecular analysis for rearrangement of the T‐cell receptor (TCR) gene was performed in two cases. One case was classified as MF, while the other two lacked epidermotropism, and were considered primary cutaneous peripheral T‐cell lymphoma (PTCL), NOS. Two cases were CD3+/CD4+ and one case was CD3+/CD8+ at diagnosis. The first two patients suffered many relapses and eventually, new CTCL lesions with a CD3+/CD8+ phenotype were observed. Both cases revealed identical clonal TCR rearrangements on the initial and late lesions, supporting the interpretation of a single clonal proliferation with different phenotypes. The third case progressed with skin recurrences and pulmonary lesions with a predominant CD3+/CD4+/CD8? phenotype. All cases manifested poor prognosis and two patients died of lymphoma. Immunophenotypical shift between CD4 and CD8 in CTCL seems to be a rare phenomenon that may be associated with disease progression.