Literature Review




(1)
Department of Health Management, New York Medical College, Valhalla, New York, USA

(2)
University of British Columbia, Vancouver, British Columbia, Canada

(3)
Misdiagnosis Association, Seattle, Washington, USA

 



Keywords
Psoralen and ultraviolet A (PUVA)InterferonIFNMycosis fungoidesSézary syndromeNarrow band ultraviolet B (NB-UVB)


Treatment outcomes in 113 patients with mycosis fungoides (MF) and Sézary syndrome (SS) were studied by Anadolu et al. [1]. In their study of 113 patients, 110 were diagnosed with MF while 3 were diagnosed with SS, 101 patients (89.4 %) were diagnosed at early phases of the disease (IA, IB, IIA), and 12 (10.6 %) were diagnosed at advanced phases (IIB, III, IVA, IVB). The age of diagnosis was between 12 and 81 years (mean 45.6 ± 15.8 years), and 55 patients (48.7 %) were male and 58 patients (51.3 %) were female. Skin lesion duration varied between 1.5 months and 32 years (average 6.1 years). Psoralen and ultraviolet A (PUVA) was the primary treatment used (91 %) at early phases of the disease with a complete remission (CR) rate of 80.4 %. Treatment with PUVA + interferon-alpha resulted in 57 % complete remission in early phases and 33.3 % in advanced phases. Of 113 patients with MF, 8 patients (7 % of all patients and 57.1 % of advanced phase) passed away, 21.4 % of advanced phase patients showed relative recovery, and 14.2 % had complete recovery. None of the patients in the early phase died; however, 2 patients (1.9 %) progressed to an advanced phase of the disease.

Photochemotherapy in the form of PUVA is the first-line, effective, and tolerable treatment for the early phases of MF. In patients with thin patches and plaques, narrow band ultraviolet B (NB-UVB) is preferred. PUVA is used in patients with thick plaques and those who relapsed after initial treatment with NB-UVB. To induce recovery, three sessions of PUVA treatment per week, or three sessions of NB-UVB per week, were recommended until full recovery of the patient was achieved. In recurrent cases, PUVA monotherapy and/or a combination of PUVA with adjuvants such as methotrexate and interferon were used. Patients in early MF phases showed a good clinical response to combination therapy, such as PUVA with methotrexate, bexarotene, or interferon-α(alpha)-2b. In advanced phases of MF, this combination therapy may be used as first-line therapy. Currently, however, there is no consensus on maintenance therapy using phototherapy in attaining CR [2].

Interferon-alpha-2a was studied as a treatment for T-cell lymphoma in a study conducted by Olsen et al. [3]. In their study, 22 patients with T-cell lymphoma at IA to IVA phases entered into an interferon-alpha-2a controlled study (Roferon-A). Patients received 3 million IU interferon-alpha-2a at first, or received 36 million IU doses as intramuscular injection daily for a period of 10-week induction. At the end of induction, 14 out of 22 patients (64 %) had objective response against tumor: 3 patients had full response, 10 patients had relative response (≥50 % clinical improvement), and 1 patient showed slight response. Responders include individuals who were at IA to IVA phases of T-cell lymphoma and there was at least 4–27.5 months until recovery. Through continued treatment, 3 progressed from partial response to complete response and overall complete response was 27 %. Side effects included acute influenza-like symptoms, which were generally mild and transient. Weakness/fatigue, depression, anorexia, and weight loss were common dose-dependent side effects and were also the most common reasons to decrease the dose. Dose-dependent leukopenia was the most common laboratory side effect seen.

One study performed a systematic review of combination therapy in MF [4]. The results of this study showed that a combination of PUVA with interferon-alpha and/or retinoids does not lead to increased general response. Adding methotrexate but not retinoids to interferon-alpha may increase general response. In MF, no combination therapy is superior to monotherapy [4]. In some cases, patients may benefit from a combination of PUVA with interferon-alpha and/or one retinoid and/or a combination of two latter therapies. In addition, patients at advanced phases of the disease may benefit from a combination of methotrexate and interferon-alpha and/or bexarotene. The combination of bexarotene with vorinostat or gemcitabine does not increase overall response and can in fact lead to more severe complications; thus, it is not a recommended regimen.

Only gold members can continue reading. Log In or Register to continue

Stay updated, free articles. Join our Telegram channel

Dec 3, 2017 | Posted by in Dermatology | Comments Off on Literature Review

Full access? Get Clinical Tree

Get Clinical Tree app for offline access