Hydroa vacciniforme-like lymphoma of an adult: a case report with review of the literature
- Mi Wang†1,
- Sheng Wang†1,
- Qun-Pei Yang†2,
- Yan-Mei Liu2,
- Li-Min Gao2,
- Hong Sun2 and
- Wei-Ping Liu2Email author
Hydroa vacciniforme-like lymphoma (HVL) is a rare type of Epstein-Barr virus (EBV)-positive lymphoma of cytotoxic T-cell or natural killer cell origin that mainly affect children, characterized by a vesicopapular skin eruption that clinically resemble hydroa vacciniforme (HV). In current study, we report an adult patient with the tumor. The patient presented similar morphologic, immunophenotypic and genotypic changes of the disease with that occurred in children, whereas clinically, he showed a prolonged clinical course without hepatosplenomegaly or generalized lymphadenopathy. Whether there are some differences in biologic behavior between children and adults still remains unknown and it is necessary to collect more data to observe and to investigate in the future.
KeywordsHydroa vacciniforme-like lymphoma Epstein-Barr virus Adult
Hydroa vacciniforme-like lymphoma (HVLL) is a cutaneous cytotoxic T-cell or natural killer (NK) cell originated lymphoma occurring in children and adolescents, but not in adults, mainly reported from Asia, Central and South America. In the updated WHO Classification of tumor of Haematopoietic and lymphoid tissues (2008), hydroa vacciniforme-like lymphoma has been recognized as one of the Epstein-Barr virus (EBV)-positive lymphoproliferative disorders of childhood . In current study, an adult patient with hydroa vacciniforme-like lymphoma was reported with review of the literature.
Histopathology, immunophenotype, genotype, and EBV status
Treatment and follow-up
The patient was treated with Haitangheji, a kind of Chinese homeopathic medicine which is used for immuno-suppression and anti-inflammation, ketotifen which is most commonly used to treat IgE-mediated allergic diseases, and other supportive treatments. α-interferon was used to control the fever. Levofloxacin and sirolimus were used to control fever, but the temperature still kept above 38°C; then prednisone was added. At last, the patient’s temperature fell down to normal level and skin lesions were also gradually disappeared and he was discharged from the hospital. Three month later, the patient died, and no more detail information about the disease was available.
We reported an exceptional case of EBV-associated T/NK-cell lymphoproliferative disorder in an adult who shares similar characteristics of hydroa vacciniforme-like lymphoma in the current WHO category.
EBV-positive T/NK-cell lymphomas recognized by the WHO classification (2008) include extranodal NK/T-cell lymphoma nasal type, aggressive NK-cell leukemia, systemic EBV-positive T-cell lymphoproliferative disorder of childhood, and hydroa vacciniforme-like T-cell lymphoma. All occur more frequently in Asians, and in the Native Americans from Central and South America and Mexico . Except for hydroa vacciniforme-like lymphoma, all are aggressive diseases, with a final fatal outcome in most cases.
Hydroa vacciniforme-like lymphoma (HVLL) has also been called angiocentric cutaneous T-cell lymphoma of childhood , and hydroa-like cutaneous T-cell lymphoma [4–6]. Most reports come from Asian area, including Japan, China and Korea . Up to now, limited researches about Hydroa vacciniforme-like lymphoma in adult were reported in the literature due to its rarity.
Hydroa vacciniforme-like lymphoma should be distinguished from a group of T-cell and NK cell lymphoproliferative disorders presenting in the skin. The first is Hydroa vacciniforme (HV). Typical HV is a self-limited skin disease characterized by vesiculopapular eruptions on the sun-exposed regions with vacciniforme scarring. Systemic involvement is absent. Patients with HV-like lymphoproliferative disorders may show some clinical features mimicking typical HV at the onset of the disease . In addition, some cases of severe HV reported in the literature before are actually included in Hydroa vacciniforme-like lymphoma now . The second differential diagnosis is extranodal NK/T-cell lymphoma, nasal type (ENKTCL-N). Unlike HVLL, cutaneous ENKTCL-N usually presents multiple, generalized and severe skin lesions, involving extremities and trunks simultaneously, in sun-exposed and also nonexposed area of the skin. The neoplastic cells infiltrated diffusely in both dermis and subcutis, resulting in a column-like low-power appearance and featured positive for CD56 and usually lack of T-cell receptor gene rearrangement . Furthermore, subcutaneous panniculitis-like T-cell lymphoma (SPTCL) presents with deep subcutaneous nodules rather than vesiculopapalar skin eruptions and is invariably negative for EBV. In addition, primary cutaneous gamma-delta T-cell lymphoma, which is now included in primary cutaneous peripheral T-cell lymphoma, not otherwise specified is extremely rare and is also negative for EBV.
With regard to the treatment of the tumor, Interferon-α (IFN-α) is the most commonly selected whose major side effects are fever and cytopenia. Corticosteroid is usually used to control the symptoms, but it is not suitable to be used persistently. Chemotherapy is uncommonly used, because only 30% of patients may have partial response. Xu reported six Chinese cases of childhood, in which, four patients received IFN-α, and two of them also had chemotherapy (the regimen was unknown) . In current report, the patient was treated by IFN-α and corticosteroid. Although the effect is not so ideal, the progression is relatively slow. The prognosis-related factor is unclear right now. According to the limited reports in the literature, the clinical course may be indolent in patients with the NK-cell phenotype; whereas cases with T-cell phenotype are characterized by intermittent fever and hepatosplenomegaly. High titers of EBV-related antibodies in the serum often predict a progressive clinical course and poor prognosis.
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