Defining early mycosis fungoides: validation of a diagnostic algorithm proposed by the International Society for Cutaneous Lymphomas

Vandergriff, Travis; Nezafati, Kaveh A.; Susa, Joseph; Karai, Laszlo J.; Sanguinetti, Amy; Hynan, Linda S.; Ambruzs, Josephine M.; Oliver, Dwight; Pandya, Amit G.

doi:10.1111/cup.12470

Cited by 35 publications

(36 citation statements)

References 17 publications

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“…This may lead to vague, non‐specific and inconclusive pathology interpretations of early stage lesions of MFwhich ultimately contribute to diagnostic and treatment delays. Diagnostic algorithms for early MF, note the presence of epidermotropism in the absence of spongiosis as a criterion with low specificity (60%) . This is supported by multiple reports that have described spongiosis as a relatively common feature in early MF.…”

Section: Discussionmentioning

confidence: 70%

Mycosis fungoides with spongiosis: A potential diagnostic pitfall

et al. 2019

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Background Mycosis fungoides (MF) is characterized by epidermotropic atypical lymphocytes in the absence of spongiosis. However, we describe an unusual presentation of MF with spongiosis, mimicking benign inflammatory dermatoses and highlight the importance of pathologic interpretation for diagnostic confirmation. Methods A cross‐sectional study of consecutive patients diagnosed with MF with spongiosis was conducted. The clinical, histopathologic, immunophenotypic, and molecular genetic features of cases were reviewed. Results We identified nine cases of MF (age range 34‐82 years; mean 75 years), with an initial diagnosis of dermatitis (6/9), psoriasis (4/9), or other inflammatory dermatoses (2/9). Pruritus, pain, and blisters were common clinical manifestations. The most common areas of involvement were the extremities (8/9). Epidermotropism with spongiosis was a central histopathological feature in all cases. Conclusion These cases highlight prominent spongiosis in MF and overlap with common benign inflammatory dermatoses. We present these cases to show the diagnostic pitfalls associated with spongiotic presentations of MF. Dermatitis, psoriasis, and other inflammatory skin conditions not responsive to standard therapy warrant further work‐up including biopsy to rule out MF. Multiple skin biopsies and review by a dermatopathologist with expertise in the diagnosis of cutaneous lymphoma is highly recommended.

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Section: Discussionmentioning

confidence: 70%

Mycosis fungoides with spongiosis: A potential diagnostic pitfall

et al. 2019

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“…ISCL diagnostic algorithm Confirming MF cases (score ≥ 4): patients with typical MF features in clinical features; (2) suspecting MF cases (4 points > score >2 points): It means that clinical features and pathological examination are not enough to confirm MF, but all cases are highly suggestive of MF; (3) not completely excluding MF cases (score ≤ 2 points): cases with insufficient pathological evidence but highly consistent clinical biological behavior with MF; and (4) eliminating MF cases (score = 0): diagnosed as parapsoriasis, psoriasis, pityriasis rosea, lichen planus, dermatitis, and other diseases. 5% and the specificity was 60%, and the low specificity of this algorithm may limit its clinical application 11. In this study, MF was highly suspected in one patient in clinical lesions and course of disease, while his pathological result score was only 1 point.…”

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confidence: 71%

Reflectance confocal microscopy may be included as part of the diagnostic algorithm of early‐stage mycosis fungoides

Zhu

Wang

et al. 2020

Skin Research and Technology

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Mycosis fungoides (MF), a kind of primary cutaneous lymphoma with relatively indolent biological behavior, represents the most common of cutaneous T-cell lymphomas. According to its biological processes, the characteristics of typical MF lesions can be divided into three stages: patch, plaque, and tumor. 1 Most patients are in the early stage (patch and plaque) for decades due to its unique indolent biological behavior, but once entering the tumor stage, the disease develops rapidly and eventually affects lymph nodes or internal organs. 2 Therefore, early diagnosis and implementing dynamic monitoring of MF are of great significance for its prognosis. Multiple, persistent patch or plaque lesions that occur at unexposed sites may be clinically suspected cases of MF,

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“…20 Furthermore, the diagnostic criteria for MF were formally fulfilled and the clinical course of the disease, including the clinical manifestation of the relapse lesions after autologous SCT, strongly favoured the diagnosis of MF. [22][23][24] In patients with MF, cerebral involvement is rare and presents most frequently as cranial nerve dysfunction or fluctuation of higher cognitive functions. 25 In line with this, loss of eagerness to engage in conversation dominated the clinical appearance of the patient presented here.…”

Section: Discussionmentioning

confidence: 99%

Interferon alfa‐2a maintenance after salvage autologous stem cell transplantation in atypical mycosis fungoides with central nervous system involvement

Doerschner

Pekar-Lukacs²,

Messerli-Odermatt

et al. 2019

Br J Dermatol

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Summary Mycosis fungoides (MF) is a primary cutaneous T‐cell lymphoma with unfavourable prognosis for patients with advanced stages of the disease. Refractory disease and advanced‐stage disease require systemic therapy. We report on a rare case of an atypical predominantly CD8+ folliculotropic MF, a subtype of MF with poorer prognosis, in a 59‐year‐old woman. She was initially diagnosed with MF restricted to the skin, of T3N0M0B0/stage IIB according to the current World Health Organization–European Organisation for Research and Treatment of Cancer classification. First‐line treatment with local percutaneous radiotherapy in combination with systemic interferon alfa‐2a resulted in complete remission. However, 21 months later the disease progressed to T3N0M1B0/stage IVB with development of cerebral manifestation and thus very poor prognosis. Allogeneic stem cell transplantation (SCT) was not a therapeutic option due to the lack of a suitable donor. We initiated methotrexate and cytarabine chemotherapy, followed by high‐dose chemotherapy with thiotepa and carmustine with autologous SCT. Despite rapid response and complete remission of the cerebral lesions, disease recurrence of the skin occurred soon after. Interestingly, readministration of interferon alfa‐2a as a maintenance treatment after the salvage autologous SCT resulted in a durable complete remission during the follow‐up period of currently 17 months after autologous SCT. What's already known about this topic? Mycosis fungoides is a primary cutaneous T‐cell lymphoma with unfavourable prognosis for the advanced stages of the disease. A refractory course of disease requires systemic therapy. What does this study add? We report on an unusual case of a patient with mycosis fungoides with cerebral involvement, in which a durable complete remission was achieved upon autologous stem cell therapy and interferon alfa‐2a maintenance therapy.

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Defining early mycosis fungoides: validation of a diagnostic algorithm proposed by the International Society for Cutaneous Lymphomas

Cited by 35 publications

References 17 publications

Mycosis fungoides with spongiosis: A potential diagnostic pitfall

Mycosis fungoides with spongiosis: A potential diagnostic pitfall

Reflectance confocal microscopy may be included as part of the diagnostic algorithm of early‐stage mycosis fungoides

Interferon alfa‐2a maintenance after salvage autologous stem cell transplantation in atypical mycosis fungoides with central nervous system involvement

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