CD8+ Epidermotropic Cytotoxic T-Cell Lymphoma With Peripheral Blood and Central Nervous System Involvement

Constitutive JAK3 activation induces lymphoproliferative syndromes in murine bone marrow transplantation models

“…Together, these results indicate that constitutive JAK3 activation in this model mimics some important phenotypic features of a rare subset of human CD8 ϩ primary cutaneous peripheral T-cell lymphomas. [45][46][47] Finally, the results obtained in this murine model informed a screen that identified JAK3A572V as an infrequent allele in human CD4 ϩ CTCL. A larger cohort of patients will be required to determine its precise incidence in the different CTCL subsets, and whether other activating alleles in the JAK-STAT pathway contribute to human CTCL.…”

Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma

“…[1][2][3][4][5][7][8][9][10][11][12][13][14][15][16][17] Clinically, the disease manifests as localized or disseminated papules, papules, nodules, and tumors, often with ulceration, hemorrhage, and necrosis, or with superficial hyperkeratotic patches and plaques. [1][2][3][4][5][7][8][9][10][11][12][13][14][15][16][17] Unlike classic MF, patients do not generally have long-standing precursor lesions and do not follow the typical progression through patch-, plaque-, and finally tumor-stage disease, but rather present from the onset with widespread plaque-and tumor-stage disease. 3,9,12 Involvement of mucosal surfaces, and acrally accentuated lesions of the palms and soles, is commonly seen.…”

Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma: Proposed diagnostic criteria and therapeutic evaluation

“…1,3,6,26 Multiagent chemotherapy, particularly doxorubicinbased regimens such as cyclophosphamide, hydroxydoxorubicin, Oncovin, and prednisone (CHOP) and the hyper-cyclophosphamide, vincristine, Adriamycin (doxorubicin), and dexamethasone regimen (hyper-CVAD) that consists of alternating A cycles (hyperfractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone) and B cycles (high-dose methotrexate and cytarabine), in 6 to 8 courses, is the most commonly used option for patients with primary cutaneous aggressive CD8 1 epidermotropic T-cell lymphoma. [6][7][8][9][10][11][13][14][15][19][20][21][22][23]25,26 Intensified regimens such as methotrexate, Adriamycin, cyclophosphamide, Oncovin, prednisone, and bleomycin (MACOP-B) have not shown a survival advantage over CHOP. 8,35 Generally, the results of multiagent chemotherapy are unsatisfactory and no available therapy seems to be curative because the response is usually partial, and if complete is associated with rapid relapse, in addition to the high incidence of toxic effects, particularly myelosuppression and opportunistic infections.…”

“…6,11,19,26 Bexarotene is a new synthetic rexinoid that selectively binds to the retinoid x receptor subfamily and is formulated as either as capsule or a topically applied gel. 36 Oral bexarotene has been used as a single agent, 21 in combination with other modalities such as total skin electron beam therapy, or for postchemoradiotherapy refractory patients. 26,29 Bexarotene is usually associated with partial remission of the tumor; however, one patient was well controlled on oral bexarotene with no new emerging skin lesions, but a long-term cure was not established.…”