OBJECTIVE: To report the clinical characteristics, diagnosis, treatment and prognosis of one patient with primary cutaneous CD8⁺ aggressive epidermotropic cytotoxic T-cell lymphoma (CD8⁺ PCAECTL), and to strengthen the understanding of this extremely rare type of lymphoma. METHODS: The clinical manifestations, diagnosis, treatment course, and prognosis of one patient with CD8⁺ PCAECTL admitted to our hospital were retrospectively analyzed. RESULTS: The patient is a 42-year-old female, with infiltrative skin rash on naso-facial and back as the main clinical manifestations. After pathological examination of the affected skin tissue, immunohistochemistry, molecular biology, and imaging, the diagnosis was confirmed as CD8⁺ PCAECTL, T3aN₀M₀ stage. Alternating chemotherapy with CHOP/HD-MTX (methotrexate, 6 g/m²) regimen was administered, and achieved complete remission (CR) after 4 cycles. After undergoing chemotherapy with DHAP regimen (cisplatin 100 mg/m², d 1 + cytarabine 2 g/m², q 12h, d 2 + dexamethasone 40 mg/d, d 1-4), the patient was mobilized for peripheral blood stem cells using recombinant human granulocyte colony-stimulating factor (G-CSF), and a sufficient number of CD34⁺ cells were successfully collected. Preconditioning was conducted with the BEAM regimen, followed by consolidation therapy with autologous hematopoietic stem cell transplantation (AHSCT). The patient remained in a disease-free survival state after 20 months of follow-up post-AHSCT. CONCLUSION CD8⁺ PCAECTL is extremely rare in clinical practice, with insidious onset and difficult early diagnosis. It is mainly characterized by the proliferation of epidermotropic CD8⁺ cytotoxic T cells and aggressive clinical course. At present, there is still no unified standard for the optimal treatment regimen, and the prognosis is very poor. Consolidation therapy with AHSCT after achieving remission through induction chemotherapy can improve the survival and prognosis of the CD8⁺ PCAECTL patients.
Humans ; Female ; Adult ; Antineoplastic Combined Chemotherapy Protocols/therapeutic use* ; Lymphoma, T-Cell, Cutaneous/diagnosis* ; Retrospective Studies ; Prognosis ; CD8-Positive T-Lymphocytes ; Skin Neoplasms/diagnosis* ; T-Lymphocytes, Cytotoxic

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

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

PURPOSE: We aimed to evaluate clinical outcomes including progression-free survival (PFS), overall survival (OS), partial response, and complete response in patients who underwent radiation therapy (RT) for mycosis fungoides (MF). Also, we sought to find prognostic factors for clinical outcomes. MATERIALS AND METHODS: Total 19 patients confirmed with MF between 1999–2015 were retrospectively reviewed. Clinical and treatment characteristics, clinical outcomes, and and toxicities were analyzed. RESULTS: Eleven patients were treated with total skin electron beam radiotherapy (TSEBT) and 8 patients with involved field radiation therapy (IFRT) with median dose of 30 Gy, respectively. The median time interval from diagnosis to RT was 2.6 months (range, 0.4 to 87.3 months). The overall response rate was 100%; 11 patients (57.9%) had a complete response and 8 patients (42.1%) a partial response. The presence of positive lymph node at the time of consultation of RT was associated with lower OS (p = 0.043). In multivariate analysis, PFS was significantly lower for patients with increased previous therapies experienced following RT (p = 0.019) and for patients showing PR during RT (p = 0.044). There were no reported grade 3 or more skin toxicities related with RT. CONCLUSION: Both IFRT and TSEBT are effective treatment for MF patients. Patients with short disease course before RT or complete response during RT are expected to have longer PFS. Positive lymph node status at the initiation of RT was associated woth poor OS, suggesting other treatment modalities such as low-dose RT for patients with low life-expectancy.
Diagnosis ; Disease-Free Survival ; Humans ; Lymph Nodes ; Lymphoma, T-Cell, Cutaneous ; Multivariate Analysis ; Mycosis Fungoides ; Radiotherapy ; Retrospective Studies ; Skin

Mycosis fungoides is the most common form of cutaneous T-cell lymphoma, and it rarely exhibits predilection for hair follicle and eccrine gland infiltration. Here, we present 2 similar cases that display folliculotropism with varying amounts of follicular mucinosis, with and without syringotropism. The features observed in both cases were cystic, comedo-like, acneiform lesions; generalized involvement with loss of body hair; pruritus; and hidradenitis suppurativa-like lesions. Hypohidrosis as well as nail and palmoplantar involvement with lichen planopilaris-like clinical features were unique characteristics of the first case. Despite the well-known aggressive behavior of follicular mycosis fungoides, the presented cases had a subtle, slowly progressive, but persistent, clinical course. Folliculotropic and syringotropic mycosis fungoides are variants of cutaneous T-cell lymphoma. Clinical presentations might be challenging, and multiple, deep biopsy specimens containing adnexal structures are required for this critical diagnosis. Aggressive treatment may not be necessary in cases having an indolent course, especially in those with syringotropism.
Biopsy ; Diagnosis ; Eccrine Glands ; Hair ; Hair Follicle ; Hidradenitis ; Hypohidrosis ; Lichens ; Lymphoma ; Lymphoma, T-Cell, Cutaneous ; Mucinosis, Follicular ; Mycosis Fungoides* ; Pruritus

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

Adolescent ; Adult ; Aged ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Cisplatin ; administration & dosage ; Dexamethasone ; administration & dosage ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Humans ; Immunohistochemistry ; Lymphoma, Extranodal NK-T-Cell ; pathology ; Lymphoma, Primary Cutaneous Anaplastic Large Cell ; pathology ; Lymphoma, T-Cell, Cutaneous ; drug therapy ; pathology ; Lymphomatoid Granulomatosis ; pathology ; Male ; Middle Aged ; Natural Killer T-Cells ; pathology ; Neoplasm Recurrence, Local ; Skin Neoplasms ; drug therapy ; pathology ; Young Adult

CD4 Antigens ; metabolism ; CD56 Antigen ; metabolism ; Child ; Humans ; Lymphoma, T-Cell, Cutaneous ; diagnosis ; metabolism ; Male ; Skin Neoplasms ; diagnosis ; metabolism

Humans ; Lymphoma, T-Cell, Cutaneous ; diagnosis ; Male ; Middle Aged ; Sezary Syndrome ; diagnosis

CD3 Complex ; metabolism ; CD5 Antigens ; metabolism ; Diagnosis, Differential ; Female ; Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor ; Humans ; Leukocyte Common Antigens ; metabolism ; Leukosialin ; metabolism ; Lymphoma, T-Cell, Cutaneous ; metabolism ; pathology ; Middle Aged ; Skin Diseases ; pathology