Cutaneous T‐cell lymphoma: 2014 Update on diagnosis, risk‐stratification, and management

Abstract

Disease overview: Cutaneous T‐cell lymphomas are a heterogenous group of T‐cell lymphoproliferative disorders involving the skin, the majority of which may be classified as Mycosis Fungoides (MF) or Sézary Syndrome (SS). Diagnosis: The diagnosis of MF or SS requires the integration of clinical and histopathologic data. Risk‐adapted therapy: TNMB (tumor, node, metastasis, and blood) staging remains the most important prognostic factor in MF/SS and forms the basis for a “risk‐adapted,” multidisciplinary approach to treatment. For patients with disease limited to the skin, expectant management or skin‐directed therapies is preferred, as both disease‐specific and overall survival for these patients is favorable. In contrast, patients with advanced‐stage disease with significant nodal, visceral or blood involvement are generally approached with biologic‐response modifiers or histone deacetylase inhibitors prior to escalating therapy to include systemic, single‐agent chemotherapy. Multiagent chemotherapy (e.g., CHOP) may be employed for those patients with extensive visceral involvement requiring rapid disease control. In highly selected patients, allogeneic stem‐cell transplantation may be considered. Am. J. Hematol. 89:837–851, 2014. © 2014 Wiley Periodicals, Inc.

Resimmune, an anti-CD3 recombinant immunotoxin, induces durable remissions in patients with cutaneous T-cell lymphoma

“…Currently, CTCL patients have a large number of treatment options including skin-directed therapies, systemic therapies with cytotoxic chemotherapies, histone deacetylase inhibitors and rexinoids, and allogeneic stem cell transplants. [17][18][19][20] Although objective responses to initial treatments are common, most responders, except for allogeneic stem cell transplant recipients, develop recurrent disease within several months or years. Patients with stage IB and IIB disease cycle through numerous treatments and suffer the chronic toxicities, costs, physical inconvenience of multiple physician visits, and, eventually in a significant fraction, progressive disease and death.…”

Mycosis fungoides as casus pro diagnosi

“…The peak age at presentation is in excess of 55 to 60 years [2,3]. There is a male predominance in almost all studies on CTCLs and MF, with a male:female ratio of 1.3:1 to 2:1 [4,5]. Mycosis fungoides is a disease mainly seen in older patients.…”

Primary cutaneous lymphomas – diagnostic and therapeutic guidelines of the Polish Dermatological Society

“…W przypadkach z HPS opornych na CHOP stosuje się m.in. kladrybinę, schemat DHAP (deksametazon, cytarabina, cisplatyna), alemtuzumab, przeszczepienie krwiotwórczych komórek macierzystych [5][6][7][8][15][16][17][18]. 50-99% clearance of skin lesions from baseline without new tumours (T3) in patients with T1, T2 or T4 (only skin disease)/ustąpienie zmian skórnych w 50-99% mSWAT w stosunku do stanu wyjściowego, bez nowych guzów (T3) w stadiach T1, T2 lub T4 (choroba ograniczona do skóry) stable disease (SD)/stabilizacja choroby (SD) < 50% clearance of skin lesions (mSWAT) from baseline or appearance of new skin lesions < 25% (mSWAT) in relation to baseline, without new tumours (T3) in patients with T1, T2 or T4 (only skin disease)/ustąpienie zmian skórnych < 50% mSWAT lub pojawienie się nowych zmian skórnych w liczbie nieprzekraczającej 25% mSWAT w stosunku do stanu wyjściowego, bez nowych guzów (T3) w stadiach T1, T2 lub T4 (choroba ograniczona do skóry)…”

“…In terms of prognosis, the 5-year survival rate is 91% in patients without HPS and 46% in patients with HPS. In cases of CHOP-resistant HPS, the following therapies are used: cladribine, DHAP regimen (dexamethasone, cytarabine, cisplatin), alemtuzumab and transplantation of hematopoietic stem cells [5][6][7][8][15][16][17][18].…”