Exploratory comparative transcriptomic analysis of CD8+ mycosis fungoides and type D lymphomatoid papulosis

Abstract Introduction: Lymphomatoid papulosis (LyP) is a CD30+ lymphoproliferative disorder, with a self-regressing clinical course and malignant histopathology. Objective: The aim of the study was to evaluate characteristics, risk factors, associated malignancies, long-term outcome and treatment of LyP in a large cohort representing the experience of the MD Anderson Cancer Center. Methods: This was a retrospective study of patients with LyP seen between 1999 and 2015. Patient charts, clinical and histopathological data were evaluated, searching the MD Anderson Cancer Center cutaneous T-cell lymphoma database. A total of 237 patients with the diagnosis of LyP were identified, with 180 patients meeting the inclusion criteria of both clinical diagnosis of LyP and confirmatory biopsy. Results: A total of56.6% of patients were male and 43.3% were female. The majority of patients were Caucasians (77.7%, n=139). The mean age at diagnosis was 52.7±17.2 years. The mean time from symptom onset to a biopsy proven diagnosis of LyP was 45.0±75.4 months. The majority of patients (51.6%, n=83) had less than twelve lesions at the worst time point. Histologic subtype A was found in 47.2%, type B in 17.2%, type C in 22.8%, type D in 7.8%, type E in 0.6% and mixed subtype in 4.4% of the patients. Associated lymphomas (n=114) were observed in 93 patients with mycosis fungoides (61.4%, n=70) and anaplastic large cell lymphoma (26.3%, n=30) being the most common forms (Table). Men were 2.85 times (95%CI: 1.55-5.24) more likely to develop a secondary lymphoma than women. Other risk factors for development of lymphoma included histologic subtypes B and C and older age. Number of lesions, symptom severity and ethnicity were not associated with lymphoma development. Patients with type D (CD8+) were less likely to develop lymphomas. Despite the high number of detected secondary malignancies, the prognosis of LyP is excellent. Only eight out of 180 evaluated patients died of their disease. A broad variety of treatment options are available for symptomatic relief only, since no treatment modality was found to prevent progression to lymphoma. Conclusion: We could demonstrate that LyP is associated with other cutaneous and extra-cutaneous malignancies, the most common being MF and ALCL. To date the risk of developing associated lymphomas cannot be altered by medication. Long term follow-up exams and proper patient counseling are required to ensure early detection of evolving secondary malignancies. Table 1. A total of 114 hematologic malignancies were observed in 93 patients with lymphomatoid papulosis diagnosed before, concomitant and after diagnosis of lymphomatoid papulosis Hematologic malignancy Before (n=39) Concomitant (n=28) After (n=47) All (n=114) %(n) %(n) %(n) %(n) MF (all stages) 17.5% (20) 17.5%(20) 26.3%(30) 61.4% (70) MFIA 9.6% (11) 7.0% (8) 20.2%(23) 36.8%(42) MFIB 4.4% (5) 4.4% (5) 2.6%(3) 11.4%(13) MFIIA 1.0% (1) - - 1.0%(1) MFIIB 1.0% (1) 1.0% (1) 1.0%(1) 2.6%(3) MFIVA - 1.0% (1) - 1.0% (1) FMFIA - 1.8% (2) 1.0%(1) 2.6%(3) FMFIB 1.8%(2) 1.0% (1) - 2.6%(3) FMFIIB - 1.0% (1) - 1.0% (1) FMFIVA - - 1.0%(1) 1.0% (1) Hypopigmented MF - - 1.0%(1) 1.0% (1) Granulomatous MF - 1.0% (1) - 1.0% (1) ALCL 12.3%(14) 7.0% (8) 7,0%(8) 26.3% (30) HD 1.0%(1) - 2.6%(3) 3.5%(4) CLL 2.6%(3) - 1.0%(1) 3.5%(4) AML - - 1.0%(1) 1.0% (1) B-cell lymphoma - - 1.8%(2) 1.8% (2) LGL 1.0%(1) - 1.0%(1) 1.8% (2) Multiple Myeloma - - 1.0%(1) 1.0% (1) Abbreviations: ALCL, anaplastic large cell lymphoma; AML, acute myeloid leukemia; CLL, chronic lymphatic leukemia; FMF, folliculotropic mycosis fungoides; HD, Hodgkin disease; LGL, T-cell large granular lymphocytic leukemia; MF, mycosis fungoides; n, number of lymphoma cases. Disclosures Duvic: Therakos: Research Funding.

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Case 20. Lymphomatoid papulosis type D in a child with CD8+ hypopigmented mycosis fungoides

Cutaneous Lymphomas ◽

10.1007/978-3-030-59129-8_20 ◽

2021 ◽

pp. 44-45

Author(s):

I. E. Belousova ◽

W. Kempf

Keyword(s):

Mycosis Fungoides ◽

Lymphomatoid Papulosis ◽

Type D

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CD8+ mycosis fungoides: A wolf in sheep’s clothing?

Our Dermatology Online ◽

10.7241/ourd.20221.34 ◽

2022 ◽

Vol 13 (1) ◽

pp. 118-119

Author(s):

Asmae Abdelmouttalib ◽

Sanae Sialiti ◽

Soumaya Hamich ◽

Kawtar Znati ◽

Mariame Meziane ◽

...

Keyword(s):

Mycosis Fungoides ◽

Large Cell ◽

Good Prognosis ◽

Axillary Lymph Nodes ◽

Axillary Lymph ◽

Upper Respiratory Tract ◽

Ki 67 ◽

Lymphomatoid Papulosis ◽

Type D ◽

Cutaneous Lymphomas

Sir, CD8+ mycosis fungoides (MF) is a rare form of MF with an indolent course. Herein, we report a rare case of CD8+ fungoid mycosis preceded by lymphomatoid papulosis type D with an aggressive course. A 36-year-old female presented with several papular lesions on the trunk and extremities with a relapsing–remitting course. Histopathology and an immunohistochemical study confirmed CD8-positive lymphomatoid papulosis type D and methotrexate at 25 mg/week was initiated. After a temporary clinical improvement, the lesions worsened, became infiltrated, and grouped as vaguely annular and angular patches with serpiginous borders (Fig. 1). A second scalp biopsy was performed and a diagnosis of CD8+ MF was established. An extension workup was normal, and MF was classified as stage IB. PUVA therapy was started with acitretin at 25 mg/day. After four weeks from the beginning of treatment, the patches completely disappeared but with the concomitant appearance of four subcutaneous tumors. The evolution was spectacular in fifteen days, with the tumors rapidly increasing in size, becoming ulcerative and necrotic, and one being localized in the left cervical area compressing the upper respiratory tract (Fig. 2). A subsequent biopsy revealed massive epidermal and dermal large cell infiltration (Fig. 3a); the tumor cells were positive for CD3, CD8, and CD7 (Fig. 3b) and negative for CD4, CD5, CD3, CD2, and CD30. Antigen Ki-67 was expressed by more than 80% of T-cells (Fig. 3c). A cerebral and thoraco-abdominal CT scan revealed multiple axillary lymph nodes with hypermetabolism on a PET scan. An osteomedullar biopsy was normal, and lactate dehydrogenase (LDH) was increased to 358 U/L. Chemotherapy was performed, but the patient died after two cycles of CHOEP. In contrast to classical CD4+ mycosis fungoides, CD8+ MF is a rare cytotoxic phenotype constituting about 5% of all cases of MF [1]. It belongs to the first group of primary cytotoxic cutaneous lymphomas (PCCL) with a good prognosis, an indolent course, and a slow progression of the lesions over several years [2]. However, rare cases with a more aggressive course have been reported in the literature [3]. The main differential diagnosis of aggressive CD8+ MF is an aggressive epidermotropic cutaneous CD8+ lymphoma that is a rare cutaneous lymphoma with a poor prognosis due to rapid extracutaneous dissemination [4]. The prognosis of the CD8+ subtype of mycosis fungoides (MF) is controversial. More studies are necessary to clarify this subject.

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