PROCLIPI: registry & establishment of a prognostic index for MF and SS
Establishing a registry and prognostic index for mycosis fungoides, the most common type of cutaneous T-cell lymphoma (CTCL), and Szary syndrome, a rare type of CTCL, is important to improve the mana... Author: VJHemOnc Added: 10/02/2018
ConclusionThe reason for the association between the high serum copper levels and adverse prognosis is unknown. We hypothesized that interleukin-6 is secreted primarily by non-neoplastic cells at MF skin sites, leading to release of copper by the liver. Local production of interleukin-6 at the lesion sites might conceivably also promote neoplastic cell progression by stimulation of the STAT3 pathway. Further studies on the relationship between activated tumor-associated macrophages, serum copper levels, interleukin-6, or C-reactive protein and prognosis might be informative.
A 34-year-old Japanese woman presented with widespread scaly erythema that had enlarged over 2 years. A skin biopsy revealed the diagnosis of mycosis fungoides (patch stage, T1b N0 M0 B0), a most frequent cutaneous T-cell lymphoma. 18F-FDG PET/CT scan unexpectedly showed intense uptake on the left sole, which suggested a tumorous mycosis fungoides lesion (SUVmax = 6.2). Careful examination revealed the mass to be a typical plantar wart of 2 cm in diameter that the patient had not recognized. With repeated cryotherapy, the wart disappeared in 6 months, and follow-up 18F-FDG PET/CT showed no abnormal uptake on the left sole.
Follicular mucinosis in association with systemic hematologic disorders other than mycosis fungoides is poorly characterized. Clinicopathological features of follicular mucinosis in the setting of hematopoietic stem cell transplantation and diverse systemic hematologic malignancies differ from mycosis fungoides-associated cases and follicular mucinosis is not necessarily a precursor to cutaneous T-cell lymphoma.
Mycosis Fungoides (MF) and S ézary Syndrome (SS) are the most common types of primary cutaneous T cell lymphomas. The clinical presentation of MF is generally indolent whereas SS represents a more aggressive variant. Stage at diagnosis is the most important determinant of long-term survival outcome. Although most patients pres ent with early stage, patients who develop progressive disease or have advance stage represent a therapeutic challenge owing to the lack of effective therapies. Allogeneic HCT has been used as a potentially curative treatment modality because autologous HCT, conventional chemotherapies and new...
We report a case of an 83-year-old woman with folliculotropic MF characterized by erythematous, scaly plaques on the forehead along with poliosis and alopecia of the right medial eyebrow.
The objective of this research was to develop and validate the first QoL instrument for MF/SS-CTCL using a patient-centered approach. Methods: A conceptual framework for the MF/SS-CTCL QoL was developed through a literature review and interviews with key opinion leaders. Concept elicitation with patients was utilized to refine the conceptual model and generate preliminary items. The items were then revised based on qualitative and quantitative feedback obtained through cognitive debriefing surveys and interviews with patients. Next, participants (N=126) completed the preliminary MF/SS-CTCL QoL and a comparator measure of h...
ConclusionThe reason for the association between high serum copper and adverse prognosis is unknown. We hypothesize that IL-6 is secreted primarily by non-neoplastic cells at MF skin sites, leading to release of copper by the liver. Local production of IL-6 at lesion sites conceivably might also promote neoplastic cell progression via stimulation of the STAT3 pathway. Further studies on the relationship between activated tumor-associated macrophages, serum copper, IL-6 or C-reactive protein and prognosis might be informative.
This article is protected by copyright. All rights reserved. PMID: 30604871 [PubMed - as supplied by publisher]
Greater than normal serum copper levels in a subset of patients with early mycosis fungoides lymphoma correlated with a worse prognosis compared with that of patients with normal copper levels. The reason for this association is unknown. We hypothesized that inflammatory cytokines such as interleukin-6 secreted by inflammatory cells in skin lesions might play a role in disease progression.
An above normal serum copper level in a subset of patients with early mycosis fungoides lymphoma correlated with a worse prognosis compared to patients with normal copper levels. The reason for this association is unknown. We hypothesize that inflammatory cytokines such as interleukin-6 secreted by inflammatory cells in skin lesions may play a role in disease progression.