Clinical and Pathological Features of Hydroa Vacciniforme-Like Lymphoproliferative Disorder Along with Risk Factors Indicating Poor Prognosis

Abstract

Purpose

To examine the clinical and pathological features, laboratory markers, therapeutic options and risk factors indicating poor prognosis of hydroa vacciniforme-like lymphoproliferative disorder (HVLPD).

Patients and Methods

Seven patients with HVLPD had their clinical and pathological data collected. Immunohistochemical staining, Epstein-Barr virus-encoded RNA (EBER) in situ hybridization experiments, T-cell receptor (TCR) gene rearrangement, RT-PCR tests and the Elisa assay were carried out.

Results

The main clinical manifestations were papulovesicular lesions and ulcers on the face, neck, or trunk. Five cases had systemic symptoms. Three of the deceased patients had significant facial edema, deep body necrosis, and ulceration. The pathological results demonstrated that lymphocytes infiltrated blood vessels and sweat glands in addition to the dermis and subcutaneous tissues. All patients tested positive for CD3 and EBER. Six cases tested positive for TCRβF1, but none tested positive for TCRδ. TCRγ monoclonal rearrangement, strongly positive expression of TIA-1 and a Ki67 proliferation index of 40% occurred in 3 fatal cases. When compared to the survival group, the plasma EBV DNA in the deceased group was considerably higher (P<0.05). IFN-γ and TNF-α cytokine levels in patients were higher than in the control group, particularly in the deceased group (P<0.05). The skin lesions on all patients recovered quickly underwent conservative care. Nonetheless, 3 patients passed away as the disease progressed in its latter stages.

Conclusion

In our cases, the main infiltrating cells were T cells and the dominant lymphocyte subclass was αβT cells. A significant increase in lgE level, plasma EBV DNA, IFN-γ, and TNF-α cytokine levels, decreased hemoglobin level, strongly positive expression of TIA-1, high Ki67 proliferation index, and positive TCR gene rearrangement are all indicators of a poor prognosis.

Keywords:

• hydroa vacciniforme-like lymphoproliferative disorder
• hydroa vacciniforme
• Epstein-Barr virus
• Epstein-Barr virus-encoded RNA
• risk factors

Abbreviations

HVLPD, hydroa vacciniforme-like lymphoproliferative disorder; TCR gene rearrangement, T-cell receptor gene rearrangement; EBV, Epstein-Barr virus; NK, natural killer; T cells, T lymphocytes; HV, hydroa vacciniforme; HVLL, hydroa vacciniforme-like lymphoma; Th1 lymphocytes, T helper type 1 lymphocytes; Th2 lymphocytes, T helper type 2 lymphocytes; EBER, Epstein-Barr virus-encoded RNA; PBMC, Peripheral Blood Mononuclear Cell; HB, hemoglobin; PLT, platelets; AST, aspartate transaminase; ALT, alanine transaminase; IgE, Immunoglobulin E.

Data Sharing Statement

The datasets used and/or analysed during the current study are available from the corresponding author.

Ethics Approval and Informed Consent

This study involved anonymous use of patients’ clinical data, pathological data, tissue wax blocks and blood samples, and patients were not identified during data collection and experiment. The study does not affect patient health or privacy. The study was conducted in accordance with the ethical standards of the Declaration of Helsinki and has been approved by the Ethics Committee of the First Affiliated Hospital of Guangxi Medical University, numbered as follows:NO.2022-S007-01.

Consent for Publication

The study has confirmed that details of all images, clinical data and experimental data are publishable, and the purpose and content of the study have been detailed to those who have provided consent. If requested, corresponding author may provide copies of signed consent forms to the journal editorial office.

Acknowledgments

The authors wish to thank the patients and their families for making this study possible. We would like to express our gratitude to all participants involved in this study. In addition, we would also like to thank our families and the people who helped us for their support.

Author Contributions

All authors made a significant contribution to the work reported, whether that is in the conception, study design, execution, acquisition of data, analysis and interpretation, or in all these areas; took part in drafting, revising or critically reviewing the article; gave final approval of the version to be published; have agreed on the journal to which the article has been submitted; and agree to be accountable for all aspects of the work.

Disclosure

The authors report no conflicts of interest in this work.

Additional information

Funding

There is no funding to report.