A case report: CD8 + aggressive cutaneous epidermotropic T cell lymphoma (ACLAE), a rare and ill-defined subtype

doi:10.21203/rs.3.rs-1249327/v1

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A case report: CD8 + aggressive cutaneous epidermotropic T cell lymphoma (ACLAE), a rare and ill-defined subtype

https://doi.org/10.21203/rs.3.rs-1249327/v1

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Background: Aggressive" CD8+ cutaneous epidermotropic T-lymphoma is a rare entity belonging to the group of cutaneous lymphomas with a cytotoxic T/NK phenotype and which is characterized by: rapidly evolving tumor lesions at the onset, keratinocyte necrosis, a cytotoxic T immunophenotype and an unfavorable prognosis

Case presentation: In this study, we report the case of a A 36-year-old patient with no notable pathological history, presenting a rash made up of plaques evolving for 6 months, associated with scalp lesion and alopecia.

Conclusion: LCEA CD8 + is a lymphoma with a poor prognosis, despite the use of aggressive chemotherapy drugs. The use of gemcitabine, pralatrexate, brentuximab, as well as allogeneic stem cell transplantation seems to open up new therapeutic avenues.

Aggressive cutaneous epidermotropic T cell lymphoma

Pathology

Cytometry

CD8 ⁺ aggressive cutaneous epidermotropic T lymphoma is a rare entity that represents less than 1% of all T lymphomas, which explains the lack of well-defined diagnostic criteria. It is characterized by its aggressiveness and its often fatal course even under treatment (1, 2).

The aim of this study is to focus on the diagnosis difficulty of epidermotropic cytotoxic cutaneous T lymphoma, to insist on the contribution of biopsies from the various affected sites and to demonstrate the advantage of the correlation between clinical, flow cytometry and histology in earlier ACLAE diagnosis to initiate appropriate chemotherapy.

A 36-year-old patient with no notable pathological history, presenting a rash made up of plaques evolving for 6 months (Figure 1), associated with scalp lesion and alopecia evolving for 3 months (Figure 2).

The clinical examination aimed a quasi-erythroderma, dry, site of infiltrated hyperkeratotic plaques, a palmoplantar keratoderma, with an ulcerative, bleeding scalp tumor of 4cm, with alopecia.

Examination of the lymph node areas found multiple lymphadenopathy in the cervical, axillary and inguinal areas.

The quantification of the blood lymphocyte subpopulations by flow cytometry has revealed a majority of CD45⁺/CD3⁺ T-type lymphoid population with an expression of CD8⁺ (cytotoxic T lymphocyte) higher than CD4⁺ (T helpers). (Figure 3)

Histological examination of the skin biopsy taken from the scalp was in favor of a dense lymphoid cell infiltrate of perivascular arrangement with epidermotropism ulcerating the epidermis almost entirely (figure 4) and intense CD8 +, CD3 + expression (figures, 5 and 6), moderate expression of granzyme B and TIA-1, minimal expression of CD4, profile compatible with a CD8 + LCAE. The other biopsies taken from the forearms and hands were in favor of palmoplantar keratoderma.

Thoraco-abdominal-pelvic CT revealed multiple axillary lymphadenopathy as well as subpleural micronodules.

The patient was put on CHOP protocol multidrug therapy (cyclophosphamide, doxorubicin, vincristine, prednisolone), and has already received 2 cures. She is still being treated.

CD8⁺ aggressive cutaneous epidermotropic T lymphoma (LCAE) is a rare and poorly characterized variant, still considered to be a temporary entity according to the WHO (World Health Organization) classification of 2017. About twenty cases have been reported in the literature (2, 3.4).

Clinically, it presents as ulcerated, necrotic, sometimes hemorrhagic plaques, nodules or tumors, which develop rapidly and aggressively, giving rise to extra-cutaneous damage. Our observation illustrates a clinical presentation also associating a quasi-erythroderma, and a palmoplantar keratoderma (4.9).

Histologically, the infiltrate is composed of lymphocytes of varying sizes. Epidermotropism is constant. Skin immunostaining shows tumor CD8⁺T lymphocytes expressing cytotoxic proteins, which probably explains the local and general aggressiveness of the disease, the angiodestructive nature of the infiltrate and the necrotic lesions (2, 3, 9).

The differential diagnosis arises with other indolent cutaneous T lymphomas, mainly pagetoid reticulosis and CD8⁺ mycosis fungoides (8).

There is no standardized treatment to date. The first-line treatment generally involves the CHOP (cyclophosphamide, doxorubicin, vincristine, prednisolone) or EPOCH (Etoposide, Prednisone, Oncovin, Cyclophosphamide hydrochloride dexorubicin) protocols, but generally results in treatment failure. Recent studies suggest switching to second-line therapies such as gemcitabine, pralatrexate, brentuximab, as well as allogeneic stem cell transplantation, which may have allowed complete remission in a few rare cases (5, 6, 7). Recurrence is the rule with a median survival not exceeding 34 months (4, 8, 9)

LCEA CD8 + is a lymphoma with a poor prognosis, despite the use of aggressive chemotherapy drugs. The use of gemcitabine, pralatrexate, brentuximab, as well as allogeneic stem cell transplantation seems to open up new therapeutic avenues.

Ethics approval and consent to participate

Yes
Consent for publication

Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal."

Availability of data and material

Data Availabiles

Competing interests

The authors declare no conflict of interest
Funding

None
Authors' contributions

Imane.Boujguenna and Mohamed Amine Haouane: drafting of the manuscript

Anass Belbachir and Hanane Rais: correction of the manuscript

Fatimazahra Marhoume and Anass Belbachir: analysis of cytometry data

Imane Bahbouhi, Kenza Kandri and Ouafa hocar: clinical management of the patient

All authors contributed to the conduct of this work

Acknowledgements Consent for publication :

To anyone who has participated in the care of this patient directly or indirectly

Wang Y, Li T, Tu P, Wu LS, Zhu XJ. Primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma clinically simulating pyoderma gangrenosum. Clin Exp Dermatol. 2009 Apr 27.
Introcaso CE, Kim EJ, Gardner J, et al. CD8+ epidermotropic cytotoxic T-cell lymphoma with peripheral blood and central nervous system involvement. Arch Dermatol. 2008 Aug; 144(8):1027–9.
Csomor J, Bognár A, Benedek S, et al. Rare provisional entity: primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma in a young woman. J Clin Pathol. 2008 Jun; 61(6):770–2.
Fika Z, Karkos PD, Badran K, Williams RE. Primary cutaneous aggressive epidermotropic CD8 positive cytotoxic T-cell lymphoma of the ear. J Laryngol Otol. 2007 May;121(5):503–5.
Cyrenne -BM, Gibson JF, Subtil A, Girardi M, Isufi I, Seropian S. F. Foss- Transplantation in the Treatment of Primary Cutaneous Aggressive Epidermotropic Cytotoxic CD8-Positive T-Cell Lymphoma- Clinical Lymphoma, M Clinical Lymphoma, Myeloma & Leukemia, 2017 Elsevier.
Nofal -A, Abdel-Mawla MY, Assaf M, E. Salah- Primary cutaneous aggressive epidermotropic CD8 T-cell lymphoma: Proposed diagnostic criteria and therapeutic evaluation, J AM ACAD DERMATOLOGY Volume 67, Number 4 2011.
DU-THANH -A. Lymphome T cutané épidermotrope cytotoxique CD8+ « agressif »: difficultés de prise en charge chez un malade atteint d'une dystrophie myotonique de Steinert. ANNALES DE DERMATOLOGIE ET DE VÉNÉRÉOLOGIE; 2006.
-Saggini AMD. A Variant of Lymphomatoid Papulosis Simulating Primary Cutaneous Aggressive Epidermotropic CD8+ Cytotoxic T-cell Lymphoma. Description of 9 Cases. The American Journal of Surgical Pathology: August 2010 - Volume 34 - Issue 8 - p 1168–1175.
-EmilioBerti*.Primary Cutaneous CD8-Positive Epidermotropic Cytotoxic T Cell Lymphomas: A Distinct Clinicopathological Entity with an Aggressive Clinical Behavior. The American Journal of Surgical Pathology: August 2009.

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A case report: CD8 + aggressive cutaneous epidermotropic T cell lymphoma (ACLAE), a rare and ill-defined subtype

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