累及中枢神经系统的皮肤CD30~+间变性大细胞淋巴瘤1例

患者男,75岁,反复皮肤肿块11年。1个月前第3次复发伴语言、记忆和生活自理能力减退。头颅MRI示:左额叶,右顶叶多发占位,颅内淋巴瘤浸润。左大腿肿块组织病理示:真皮内大量异型细胞,体积较大,形态不规则,核扭曲,核分裂相可见;免疫组化:CD3(+),UCHL-1(+),CD30(+),ALK(-),MIB-1>90%。诊断:皮肤CD30+间变性大细胞淋巴瘤。中枢神经系统(central nerve system,CNS)累及可以是皮肤间变性大细胞淋巴瘤发生皮外累及的唯一部位,患者的意识改变是进行CNS筛查的重要提示。     

原发性皮肤CD30~+间变性大细胞淋巴瘤1例

目的患者男,70岁,右下肢肿块伴疼痛2月。皮损组织病理示异型淋巴细胞浸润于真皮,部分异型细胞有亲表皮现象。免疫组化证实为CD30+T细胞性淋巴瘤。     

原发性皮肤CD30阳性大细胞淋巴瘤1例

报告1例原发性皮肤CD30阳性大细胞淋巴瘤。患者女,65岁。左上臂出现结节并逐渐扩大6年,组织病理检查发现真皮弥漫性致密淋巴样细胞浸润,多数细胞核大、深染,可见病理分裂相。免疫组织化学染色结果示:CD30、LCA、CD4、CD45RO阳性,CD20、CD68、CD79a、TIA阴性。诊断为原发性皮肤CD30阳性大细胞淋巴瘤,行局部放疗后治愈,随访3年未复发。     

原发性皮肤CD30~+间变性大T细胞淋巴瘤1例

患者男,68岁。左臀部巨大溃疡伴离心性扩大10余年。皮肤组织病理检查:真皮全层可见弥漫性肿瘤细胞浸润。肿瘤细胞体积大,细胞异型性明显。细胞核染色质深,可见明显核分裂相。多核瘤巨细胞及R-S样细胞未见。免疫组化示肿瘤细胞约90%以上细胞CD30+染色阳性、CD3、CD4和LCA染色(+),而CD8、CD20、CD79α、TIA-1和ALK-1均为阴性。诊断为原发性皮肤CD30+间变性大细胞淋巴瘤。     

Primary Cutaneous Anaplastic Large Cell Lymphoma of the Nasal Tip in a Child

Abstract: A 4‐year‐old boy with primary cutaneous anaplastic large cell lymphoma located on the nasal tip is presented. The lesion healed spontaneously in 4 months without any evidence of residual disease. Cutaneous anaplastic large cell lymphoma, though rare in children, may occur, and should be considered when a rapidly growing ulcerating skin lesion appears.     

种痘样水疱病样T细胞淋巴瘤伴发原发性皮肤CD30阳性大细胞淋巴瘤1例

患者女,17岁。全身反复起丘疹、水疱、坏死、凹陷状瘢痕伴瘙痒、发热15年,四肢起肿块2年。血清抗EBV-IgM(-),抗EBV-IgG(+)。肿块处皮损组织病理示真皮中下层和皮下组织见弥漫性致密的瘤细胞浸润,细胞核呈间变性;免疫组化示CD3(+),浸润的大细胞CD30(+),CD43(+),80%浸润细胞Ki-67(+)。水疱处皮损组织病理示表皮网状变性及多个水疱,真皮和皮下组织可见血管和附属器周围以淋巴细胞为主的、伴少量嗜酸粒细胞浸润,部分浸润细胞呈明显异形性;免疫组化示CD3(+),CD30(-),CD43(+),Ki-67(+)。诊断:种痘样水疱病样T细胞淋巴瘤伴发原发性皮肤CD30阳性大细胞淋巴瘤。确诊后建议患者转肿瘤科化疗,随访中。     

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.     

原发性皮肤CD_(30)~+间变性大细胞淋巴瘤1例

患者男,71岁。右上肢反复出现结节2年。右肘部外侧密集数十个蚕豆至核桃大结节,部分表面破溃。右上胸部有片状浸润性斑块、结节。右肘部结节组织病理示:真皮全层及皮下脂肪均可见肿瘤细胞呈弥漫、致密浸润性生长,无亲表皮现象,肿瘤细胞为淋巴样细胞,体积较大,形态多样,明显异型性。免疫组化示:肿瘤细胞CD30强阳性,Ki67细胞增殖指数约80%,CD43阳性,LCA,CD45RO,CD3部分阳性,ALK,EMA,CD15,CD10,CD20,CK-P,S-100,HMB45,Vimentin,TDT及CD68均阴性。诊断:原发性皮肤CD3+0间变性大细胞淋巴瘤(PC-ALCL)。采用COP方案化疗一个周期,2个月后皮损全部复发,死亡。     

A Case of Primary Cutaneous CD4 Positive Small/medium T Cell Lymphoma

Primary cutaneous CD4 positive small/medium T cell lymphoma (PCSM-TCL) is a provisional entity in the new WHO-EORTC classification for cutaneous lymphoma, and it is a rare disease with a favorable clinical course. PCSM-TCL may present with different clinical and pathologic features associated with the biologic behavior of the disease. Herein we report on a 63-year-old woman with multiple, multifocal, erythematous to violaceous papules and plaques that progressed despite local radiotherapy.     

Anaplastic Large Cell Lymphoma Masquerading as an Infectious Syndrome

BACKGROUND: Nodular vasculitis is a disorder that can be associated with several disease states, including infection, medications, malignancy, and collagen vascular disorders. The pathogenesis is unknown, but may be related to immune complex deposition, leading to subsequent vascular damage. METHODS: We report a case of nodular vasculitis occurring in a 40‐year‐old African‐American woman with end‐stage liver disease and a 3 month history of ulcerating lesions on her lower extremities. Although these lesions were both clinically and histologically consistent with erythema indratum, all studies directed at determining a mycobacterial etiology were negative. RESULTS: Biopsy of the skin lesions revealed a neutrophil‐predominant vasculitis in the setting of a lobular panniculitis. The histopathology of the liver biopsies, and the patient's clinical course were consistent with auto‐immune cholangitis. A course of oral prednisone was initiated, which resulted in improvement of the lower extremity lesions. CONCLUSION: In this case report we document a case of nodular vasculitis occurring in the setting of auto‐immune cholangitis. The pathogenesis of this condition remains obscure, however possible causes include immune complex deposition or a delayed‐type hypersensitivity reaction.     

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 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.     

Subcutaneous Tissue Involvement of CD30-Positive Large Cell Lymphoma

There are several types of T-cell lymphoma presenting with subcutaneous tissue involvement showing different clinical features and prognoses. The entity of subcutaneous involvement of T-cell lymphoma should be differentiated. We report a case of CD30-positive large cell lymphoma which initially presented with subcutaneous tissue involvement only, but progressed to systemic dissemination.     

CD30‐Positive Peripheral T‐Cell Lymphoma

The hematoxylin and eosin (H&E) stain is the most common method used worldwide for routine pathologic evaluation of tissue specimens. Evaluation of cutaneous specimens frequently requires differentiation of elastic fibers, melanin, hemosiderin, fungal elements, mast cells and depositions. For this reason, we used acid‐orcein and Giemsa (AOG) stain to evaluate various skin disorders to determine the extent of utility of this infrequently reported stain. As expected, AOG stain functioned well as an elastic fiber stain, highlighting loss of elastic fibers in areas of scar and in disorders such as anetoderma. In pigmentary disorders, AOG stains melanin a dark green to greenish‐black pigment while hemosiderin remained yellow‐brown to light green color. Fungal hyphae in superficial dermatophytosis and leishmania organisms stained a deep blue. Amyloid stained a "sky" blue color in macular, lichenoid and nodular amyloid cases. The granules of mast cells stain purple in cases of urticaria pigmentosa and mastocytomas. Dermal mucin stained metachromatic lavender. Due to the multifunctional nature of the AOG stain, this simple technique can improve dermatopathologic diagnosis while decreasing the number of special stains necessary to have available in the laboratory setting.     

Primary Cutaneous Natural Killer Cell Blastic Lymphoma

An 81 yo male presented with several asymptomatic firm 1–5 cm red purple plaques on the trunk and lower extremities associated with a mild pancytopenia. Histological examination revealed a diffuse, monotonous dermal infiltrate of atypical medium sized cells with fine chromatin and scanty cytoplasm. Immunoperoxidase staining demonstrated positivity for CD 45, CD43, CD4, Bcl‐2, and TdT; but was negative for cytokeratin, melan‐A, CD30 and hematopoietic lineage specific markers. A subsequent bone marrow aspirate demonstrated a dense population of cells that were morphologically consistent with blasts. Immunophenotyping by flow cytometry revealed lesional cells that expressed CD56, CD4, CD7, CD5, HLA‐DR and TdT. However, lineage specific markers for B‐cells (CD19, CD20, cCD79a, and CD10), T‐cells (sCD3 and cCD3), and myeloid cells (CD13, CD33, CD117, cCD13, CD14, CD41, CD61, myeloperoxidase, and alpha napthyl butyrate esterase) were not expressed. Molecular studies by PCR exhibited no evidence of T‐cell receptor or heavy chain gene rearrangements. Collectively, these findings are consistent with a primary cutaneous blastic natural killer cell lymphoma. Blastic natural killer cell lymphomas are characterized by a high incidence of cutaneous involvement and an aggressive clinical course. Our patient responded dramatically to one cycle of CHOP chemotherapy with resolution of his cutaneous tumors.     

CD10 and CD20 Expression in a Primary Cutaneous Lymphoma of Mature T Cell CD8 Phenotype.

An 85 year‐old woman presented with two pearly papules on her left cheek. The clinical impression was BCC. Biopsy revealed a proliferation of atypical keratinocytes emanating from the epidermis and extending beyond the papillary dermis. A diagnosis of invasive squamous cell carcinoma was rendered. Excison with frozen section margin control was performed. Permanent sections revealed melanoma, at least 2.06 mm in depth, with melanoma in situ extending to all peripheral margins. Upon re‐examination of the initial biopsies, pseudoepitheliomatous epidermal hyperplasia (PEH) with marked squamous atypia was noted. Intimately admixed with the squamous proliferation was a growth of large malignant epithelioid cells. The epitheliod cells lacked pigment production and extended to the shave biopsy margins. This second population of cells was strongly S‐100 positive, diagnostic for amelanotic verrucous melanoma. A revised diagnosis of verrucous malignant melanoma, Clark level III, 0.82 mm in depth was rendered. The patient's course was complicated by multiple positive surgical margins and she ultimately received XRT. This case spotlights the rare occurrence of PEH in conjunction with melanoma. PEH can mask the underlying melanoma and lead to a diagnosis of squamous cell carcinoma. As demonstrated, immunostaining is essential in diagnosis of this difficult tumor.     

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.     

Lymphomatoid Papulosis Followed by Ki-1 Positive Anaplastic Large Cell Lymphoma: Proliferation of a Common T-Cell Clone

We report a case of lymphomatoid papulosis (LyP) followed by Ki-1 positive anaplastic large cell lymphoma (LCL). A 33-year-old man developed subcutaneous nodules in the left inguinal region and the left thigh after a seven-year-history of self-healing papulonecrotic lesions of LyP. Histological and immunohistochemical examination of the subcutaneous nodules revealed LCL. DNA was isolated from a nodule of the initial stage of LyP in 1988, a subcutaneous nodule of LCL in 1993, and a papule of LyP in 1993 which appeared after chemotherapy for LCL. T cell receptor gene rearrangement analysis demonstrated an identical rearranged pattern in all the three specimens, indicating that a common T cell clone proliferated throughout the course in both the LyP and LCL lesions.