AI Article Synopsis
- The study focused on hydroa vacciniforme-like lymphoproliferative disorder (HVLPD), examining clinical features, laboratory markers, treatment options, and factors linked to poor prognosis.
- Seven patients were analyzed, with findings showing papulovesicular lesions, systemic symptoms in some, and significant pathological indicators, including lymphocyte infiltration and elevated plasma EBV DNA.
- Key markers such as high levels of IgE, IFN-γ, TNF-α, and TCR gene rearrangements were associated with a poor prognosis, with three patients ultimately passing away despite quick recovery of skin lesions from treatment.
Article 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 (<0.05). IFN-γ and TNF-α cytokine levels in patients were higher than in the control group, particularly in the deceased group (<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.
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| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC10027612 | PMC |
| http://dx.doi.org/10.2147/IDR.S402040 | DOI Listing |
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