第四讲 皮肤淋巴瘤皮肤T细胞淋巴瘤向大细胞淋巴瘤转化

阐述皮肤T细胞淋巴瘤向大细胞淋巴瘤转化的临床表现、组织病理、免疫组织化学染色、分子生物学特征、诊断和鉴别诊断与治疗。     

皮肤B细胞淋巴瘤

报告２例皮肤Ｂ细胞淋巴瘤。例１．５６岁，男性，躯干、四肢多数皮肤结节，伴有淋巴结肿大５个月。例２，５１岁，男性胸部皮肤斑块３个月入院。２例皮损活检，组织病 理均示为淋巴瘤性浸润。侵犯皮肤附属器及血管周围，乳头层浸润稀疏，有无浸润滞。免疫组化均证实为Ｂ细胞性淋巴瘤。便１免疫球蛋白标记双重链（ＩｇＧ，ＩｇＭ）和双轻链（κ，γ）均呈阳性。     

Intravascular large cell lymphoma

A 19‐year‐old female presented at our hospital with a 1‐year history of a 5‐cm indurated nontender lesion on the left shoulder. Two months later, a similar lesion appeared in the left mammary region, accompanied by necrosis and fever. At admission to our department, she presented disseminated lesions in the left submandibular region, the sternal area and the posterior part of the upper and lower extremities. The lesions were indurated erythematous‐violaceous plaques affecting the adipose tissue, some with ulcerations and necrotic crusting ( Fig. 1 ). The first histopathological report was thrombosis of the capillary vessels and focal vasculitis ( Fig. 2 ).

Figure 1 Open in figure viewer PowerPoint Ulceration and necrotic crusting lesion in the left mammary region     

Primary cutaneous B cell lymphoma

A 54-year-old man, a road layer by occupation, presented with a 'leonine facies' and multiple tumors that were more commonly present over the exposed parts of the body. On investigation, he turned out to be a case of primary cutaneous B cell lymphoma with a distinctive histopathology. Chemotherapy was given with a good therapeutic response.     

Primary cutaneous anaplastic large cell lymphoma

Primary cutaneous anaplastic large cell lymphoma (PC‐ALCL) is a CD30+ lymphoproliferative disorder (LPD) of the skin with a relatively good prognosis in the absence of high‐stage disease. CD30+ LPDs comprise approximately 25%‐30% of primary cutaneous lymphomas and as a group represent the second most common clonal T‐cell neoplasm of the skin behind mycosis fungoides. Diagnosis of PC‐ALCL relies strongly on clinicopathologic correlation given the potential morphologic, clinical and molecular overlap with the other cutaneous CD30+ LPD, lymphomatoid papulosis, and more aggressive hematolymphoid neoplasms.     

种痘水疱病样皮肤T细胞淋巴瘤

种痘水疱病样淋巴瘤是近年来认识到的一种皮肤T细胞淋巴瘤,儿童多见,主要累及面部和四肢,表现为水肿性红斑、水疱、结痂和坏死,可伴有高热、肝脾大等全身症状。与EB(Epstein-Barr)病毒感染有关。组织病理显示肿瘤细胞侵犯血管和脂膜。该文报告1例该病患者。     

Cutaneous pleomorphic large cell lymphoma.

The case of a primary cutaneous pleomorphic large cell lymphoma occurring in a twenty-one-year-old woman who presented with a blue-reddish nodule on her left cheek of three months' duration is presented. The tumor consisted of pleomorphic blast cells showing high mitotic activity. On immunohistochemical examination, the majority of the tumor cells expressed CD 3 (Leu-4), CD 4 (Leu-3), HLA-DR CD 30 (Ki-1/Ber-H 2), and CD 25 (IL2 receptor). Twenty-two months after excision of the tumor there is no detectable systemic spread of the lymphoma. This case provides further evidence for recent observations that primary cutaneous Ki-1-positive large cell lymphomas without lymph node involvement may have a favorable prognosis after local treatment despite showing histologic pattern of malignancy.     

种痘水疱病样皮肤T细胞淋巴瘤

报告1例长期复发的种痘水疱病样皮肤T细胞淋巴瘤.患者女,47岁.从2岁开始反复发作水肿性红斑、水疱、坏死及痘疮样萎缩性瘢痕形成,冬轻夏重,主要累及面部和四肢.组织病理示真皮层内可见较多淋巴样细胞灶状和散在浸润,可见核分裂象.免疫组化检测显示淋巴样细胞CD2、CD3、CD4、CD5阳性,CD7、CD8少数细胞阳性,CD2...     

Treatment of cutaneous T cell lymphoma

Primary cutaneous T cell lymphomas encompass a wide variety of lymphomas that are characterized by a distinct clinical presentation. Advanced biological techniques have allowed a more precise classification in recent years. Stage-adapted therapy is at present the best approach to treat cutaneous T cell lymphoma.     

Multinodular keratoacanthoma and T cell lymphoma

This is a report of a case of multinodular keratoacanthoma (MNKA), a rare variety of solitary keratoacanthoma (KA) characterized by a collection of multiple KA nodules on the margins of a necrotic ulcerative lesion spreading centrifugally for 3 weeks in a patient with malignant non-Hodgkin's T-cell lymphoma. The very infiltrative nature of the main lesion made differential diagnosis difficult in the presence of extremely differentiated squamous cell carcinoma (SCC). Later, a KA lesion was to appear opposite a cutaneous lymphoma. The authors discuss the close links between the MNKA and the KA lesions and the immunosuppressed state of the patient.     

Cutaneous intravascular anaplastic large cell lymphoma

Intravascular lymphoma (IL) is a rare variant of non‐Hodgkin lymphoma with a predilection for skin. Most reported cases are large B cell lymphomas. Intravascular anaplastic large cell lymphoma (IALCL) is extremely rare. Retrospective analysis of a case of cutaneous IALCL was performed. Hematoxylin and eosin stained sections and immunohistochemical staining results were analyzed. The patient was a 47‐year‐old woman who had developed multiple erythematous patches and plaques on her back. The lesions responded well to CHOP (cyclophosphamide, hydroxydoxorubicin, oncovin, prednisone) chemotherapy, but relapsed shortly after therapy. The patient was surviving with the disease for eight years but was ultimately lost to follow up. Histopathologically, the neoplasm evolved from IL to extravascular lymphoma. This was showed in biopsies obtained at different stages of the disease. The lymphoma cells stained positively for CD30, CD45, CD3, CD4, CD5 and Ki67, and lacked expression of anaplastic lymphoma kinase (ALK), CD8, CD45RA, CD45RO, CD20, CD79, CD56, perforin and granzyme B. Our results suggest that IALCL represents a distinct subtype of IL and is histopathologically and biologically different from IL with B, NK or T cell phenotype. Wang L, Li C, Gao T. Cutaneous intravascular anaplastic large cell lymphoma.     

Skin manifestation of mantle cell lymphoma

Mantle cell lymphoma (MCL) is a distinct type of non-Hodgkin's lymphoma that commonly affects extranodal sites. The most commonly affected sites are bone marrow, gastrointestinal tract and Waldeyer's ring, however, skin is rarely involved. We reported a 62-year-old Japanese patient with MCL, exhibiting multiple small dome-shaped red nodules and skin ulcers. Histopathological examination demonstrated numerous atypical lymphoid cells in the dermis and subcutis. Immunohistochemically, tumor cells were positive for CD20 (L26), CD5, CD43 and cyclin D1, but negative for CD45RO (UCHL-1), CD3, CD10 and CD23. Our patient showed a significant improvement of skin lesions and lymphadenopathy with a combination chemotherapy. Awareness of skin manifestations of MCL is essential for dermatologists to establish an early diagnosis and perform appropriate treatment.     

Lymphoepithelioid cell lymphoma (Lennert's lymphoma) presenting as atypical granuloma annulare

We describe a case presenting as atypical granuloma annulare where the underlying diagnosis, confirmed by lymph node biopsy, was lymphoepithelioid cell lymphoma (Lennert's lymphoma). Lennert's lymphoma is a peripheral T-cell lymphoma which may follow a variable course and transform into an aggressive phase. Cutaneous manifestations of this condition have only rarely been reported in the literature. The presence of granulomas in the skin may have either obscured the lymphoma infiltrate or may have reflected a more generalized immune response to the underlying malignancy.