Kaposi sarcoma (KS) is an angioproliferative tumor affecting the blood and lymphatic vessels. The human herpesvirus 8 (HHV8) is directly implicated in the development of the disease. Non-AIDS kaposi sarcoma is a rare clinical type of KS and must be distinguished from AIDS-associated KS as it differs in clinical presentation, course, prognosis, and management.

A 73-year old male, Filipino, rice farmer, from Quezon Province, Philippines, presented with a progressive 10-year history of violaceous to hyperpigmented plaques and nodules on all extremities, with associated pruritus and difficulty in performing fine motor tasks. Histopathology showed proliferation of vascular channels and immunohistochemical stains were positive for CD31, CD34, and human herpesvirus 8 (HHV8), consistent with KS. Work-up revealed non-reactive HIV serology with an adequate CD4 count and computed tomography (CT) scan of the chest and abdomen were unremarkable. The case was classified as non-AIDS KS. He was then referred to oncology for chemotherapy with paclitaxel.

This case highlights that KS can occur in non-endemic areas and in immunocompetent individuals. Non-AIDS KS must be differentiated from other types of KS since non-AIDS KS is less aggressive, limited to the skin, and is highly responsive to therapy. It is therefore crucial to correlate clinical and histopathologic findings, utilizing immunohistochemical stains, as histology of KS can mimic benign vascular tumors, and it is crucial to achieve an early and accurate diagnosis. There are currently no treatment guidelines for KS and management aims to decrease morbidity and improve a patient’s quality of life.

OBJECTIVE: To investigate the cytokine/chemokine profile in patients with Epstein-Barr virus (EBV)-related hemophagocytic lymphohistiocytosis (HLH), and assess the prognostic value of survival. METHODS: Serum levels of thirty-eight cytokines/chemokines were measured by multiple cytokine assay kit in EBV-related HLH patients, EBV-infected patients, and controls. The expression profile of cytokines/chemokines was compared among groups. The changes of cytokine/chemokine expression in active and remission stage of EBV-related HLH patients were also compared, and the prognostic values for survival were evaluated. RESULTS: Serum levels of interferon-α2 (IFN-α2), interleukin (IL)-6, and IL-7 in EBV-related HLH patients were 33.67(23.23-68.78) pg/ml, (74.95±25.53) pg/ml, and 35.35(19.50-63.55) pg/ml, respectively, which were significantly higher than those in EBV-infected patients［IFN-α2: 16.07(9.87-29.63); IL-6: 55.91±20.29; IL-7: 20.40(13.35-31.40)］ and controls ［IFN-α2: 11.02(4.67-21.25); IL-6:42.64±13.41; IL-7: 16.95(14.95-33.78)］(all P<0.05). Serum levels of IL-8, IL-9, and marcophage-derived chemokine (MDC) in EBV-related HLH patients were 11.00(7.50-15.27) pg/ml, 81.30(40.79-111.0) pg/ml, and (512.6±128.7) pg/ml, respectively, which were significantly higher than those in controls ［IL-8: 6.80(5.56-8.38); IL-9: 41.30(29.82-67.91); MDC: 384.1±156.6］(all P<0.05), but there was no remarkable differences compared with EBV-infected patients (P>0.05). Serum IFN-α2, IL-6, IL-7, IL-8, IL-9, and MDC in survival and death groups of EBV-related HLH patients were analyzed by receiver operating characteristic curve with area under curve of 0.781, 0.778, 0.633, 0.805, 0.562, and 0.657, respectively (P=0.019, 0.021, 0.269, 0.015, 0.607, and 0.190). IFN-α2, IL-6, and IL-8 had good predictive effect on survival. Serum level of IFN-α2, IL-6, and MDC of EBV-related HLH patients in remission stage were significantly lower than those in active stage (P<0.05), while IL-7, IL-8, and IL-9 were not different (P>0.05). CONCLUSION IFN-α2, IL-6, IL-7, IL-8, IL-9, and MDC may take part in the pathogenesis of EBV-related HLH.
Humans ; Lymphohistiocytosis, Hemophagocytic/complications* ; Herpesvirus 4, Human ; Cytokines/metabolism* ; Epstein-Barr Virus Infections/complications* ; Interleukin-6 ; Clinical Relevance ; Interleukin-7 ; Interleukin-8 ; Interleukin-9 ; Chemokines ; Interferons

OBJECTIVES: Varicella-zoster virus (VZV) is one of the most common etiologies of viral meningitis/encephalitis. The early clinical manifestations and cerebrospinal fluid (CSF) changes of VZV meningitis/encephalitis lack specificity, and it is easy to be misdiagnosed as other viral encephalitides or tuberculous meningitis. This study aims to investigate whether the clinical characteristics, CSF analysis findings, and CSF cytokine levels could distinguish VZV meningitis/encephalitis from central nervous system (CNS) herpes simplex virus (HSV) and Mycobacterium tuberculosis (MTB) infections. METHODS: The medical records from 157 CNS infections, including 49 HSV (45 HSV-1, 4 HSV-2), 55 VZV, and 53 MTB infections between January 2018 and June 2021 in the Cytology Laboratory, Department of Neurology, Third Xiangya Hospital of Central South University were retrospectively reviewed. The data of 3 groups included demographic characteristics, laboratory results, radiographic findings, and outcomes. The levels of 12 cytokines (IL-1β, IL-2, IL-4, IL-5, IL-6, IL-8, IL-10, IL-12p70, IL-17, IFN-γ, IFN-α, and TNF-α) in the CSF of 68 patients (13 HSV, 22 VZV, and 33 MTB infection cases) were quantified. Clinical and laboratory data were compared among the 3 groups. RESULTS: The most common clinical manifestations in the 3 groups were fever, headache, vomiting, and neck stiffness. The clinical manifestations of HSV and VZV CNS disease were similar, although fever and altered consciousness were less common in the VZV group than those in the HSV and MTB groups (63.6% vs 87.8% vs 96.2%, P<0.001, and 14.5% vs 26.5% vs 47.2%, P=0.004, respectively). Seven patients (7/55, 12.7%) presented cutaneous zoster in the VZV group. CSF leukocyte count was significantly higher in the VZV group (230×10⁶ cells/mL) and MTB groups (276×10⁶ cells/mL) than that in the HSV group (87×10⁶ cells/mL, P=0.002). CSF protein level was significantly higher in the VZV than that in the HSV group (1 034 mg/L vs 694 mg/L, P=0.011) but lower than that in the MTB group (1 744 mg/L, P<0.001). IL-6 (VZV vs HSV vs MTB: 2 855.93 pg/mL vs 2 128.26 pg/mL vs 354.77 pg/mL, P=0.029) and IL-8 (VZV vs HSV vs MTB: 4 001.46 pg/mL vs 1 578.11 pg/mL vs 1 023.25 pg/mL, P=0.046) levels were significantly different among the 3 groups and were elevated in the VZV group.Post hoc analysis revealed that IL-6 and IL-8 were significantly higher in the VZV group than those in the MTB group (P=0.002 and P=0.035, respectively), but not in the HSV group (P>0.05). CONCLUSIONS VZV meningitis/encephalitis presents with CSF hypercellularity and proteinemia, challenging the classical view of CSF profiles in viral encephalitis. CSF IL-6 and IL-8 levels are elevated in patients with VZV meningitis/encephalitis, indicating a more intense inflammatory response in these patients.
Humans ; Central Nervous System Infections ; Encephalitis ; Encephalitis, Varicella Zoster/diagnosis* ; Encephalitis, Viral/diagnosis* ; Herpesvirus 3, Human ; Interleukin-6 ; Interleukin-8 ; Meningitis ; Retrospective Studies

Kaposi's sarcoma (KS) is an unusual vascular tumor associated with human herpesvirus-8 (HHV-8) infection, which is common in immunosuppressors. Although extremely rare, iatrogenic (drug-related) KS can occur in human immunodeficiency virus (HIV)-negative patients under immunosuppressive therapy. We report a 64-year-old male diagnosed with ulcerative colitis for 1 year. He was treated with methylprednisolone because of an acute severe disease flare. He presented with several popular violet lesions on the body 4 months after steroid therapy. Histological examination of skin biopsies showed Kaposi's sarcoma associated with HHV-8. The skin lesions regressed after steroid withdrawal and chemotherapy. Two key words "Kaposi's sarcoma" and "inflammatory bowel disease" were searched in Wanfang data and CNKI, but no relevant articles were found. Thirty-eight articles in English were retrieved on PubMed with the key words of ("ulcerative colitis" OR "Crohn's disease" OR "inflammatory bowel disease") AND (Kaposi sarcoma). Twenty-five cases of Kaposi's sarcoma related to inflammatory bowel disease (IBD) were reported. Including this case, the majority of 26 Kaposi's sarcoma related IBD patients were male (80.8%, 21/26). The average age was (51.1 ± 16.4) years. Twenty cases were ulcerative colitis and 6 were Crohn's disease. All the patients received immunomodulatory therapy, including glucocorticoid, azathioprine/mercaptopurine, methotrexate, cyclosporin and anti tumor necrosis factor α antibody. Thirteen cases were positive for HHV-8. There were 18 cases involving the distal ileum and colorectum only, 3 cases involving skin only, and 5 cases involving both skin and colorectum at the same time. Overall, the prognosis was good. Three patients only stopped immunosuppressive therapy, 1 received radiotherapy, 1 received chemotherapy, and 20 received surgery. Kaposi's sarcoma could be seen in IBD patients with immunomodulatory therapy. It is very important to distinguish from the skin lesions related to IBD or drug treatment. The adverse reactions of immunomodulatory therapy should not be ignored. In addition, attention should be paid to the cooperation of multi-disciplinary team, which can diagnose and treat rare cases earlier and more accurately.
Adult ; Aged ; Colitis, Ulcerative ; Crohn Disease ; Herpesvirus 8, Human ; Humans ; Immunosuppression Therapy ; Male ; Middle Aged ; Sarcoma, Kaposi

PURPOSE: Primary effusion lymphoma (PEL) is a type of body cavity–based lymphoma (BCBL). Most patients with PEL are severely immunocompromised and seropositive for human immunodeficiency virus (HIV). We investigated the distinctive clinicopathologic characteristics of BCBL in a country with low HIV burden. MATERIALS AND METHODS: We retrospectively collected data on the clinicopathologic characteristics, treatments, and outcomes of 17 consecutive patients with BCBL at nine institutions in Korea. RESULTS: Latency-associated nuclear antigen 1 (LANA1) immunostaining indicated that six patients had PEL, six patients had human herpesvirus 8 (HHV8)-unrelated BCBL, and five patients had HHV8-unknown BCBL. The patients with PEL exhibited no evidence of immunodeficiency except for one who was HIV positive. One (20%) and four (80%) patients with PEL and six (100%) and zero (0%) patients with HHV8-unrelated BCBL were positive for CD20 and CD30 expression, respectively. The two patients with PEL (one HIV-positive and one HIV-negative patient) with the lowest proliferation activity as assessed by the Ki-67 labeling index survived for > 1 and > 4 years without chemotherapy, respectively, in contrast to the PEL cases in the literature, which mostly showed a high proliferation index and poor survival. CONCLUSION: PEL mostly occurred in ostensibly immunocompetent individuals and had a favorable outcome in Korea. A watchful waiting approach may be applicable for managing HIV-seronegative patients with PEL with a low Ki-67 labeling index. A possible trend was detected among LANA1, CD20, and CD30 expression in BCBL.
Drug Therapy ; Herpesvirus 8, Human ; HIV ; Humans ; Korea ; Lymphoma ; Lymphoma, Primary Effusion ; Prevalence ; Retrospective Studies ; Watchful Waiting

OBJECTIVETo investigate the infection of HHV-8 virus in free blood donors in Wuhan.

METHODSWhole blood samples were collected from 450 free blood donors in Wuhan, and the genomic DNA extraction and serum collection were performed respectively. And then, the positive rate of HHV-8 was detected by PCR and ELISA, the positive detection rates were compared between them. Finally, HHV-8 ORFK1 gene was cloned by PCR in the positive samples, and the HHV-8 ORFK1 gene th was genetyped through sequencing analysis, homology comparison and phylogenetic tree construction.

RESULTS25 and 23 cases of positive samples were detected by PCR and ELISA, their positive rate were 5.56% and 5.11% respectively, and without statistically significant difference using χ test analysis (P > 0.05). Based on the results of ORFK1 gene cloning and sequence analysis in 23 positive samples, 15 samples C subtype of had HHV-8 ORFK1 gene, and 8 cases had A subtype had HHV-8 ORFK1 gene.

CONCLUSIONThere is a certain percentage of HHV-8 infection in the free blood donors in Wuhan. It is suggested to increase the HHV-8 virus screening for free blood donors, so as to ensure the quality of blood.

No abstract available.
Biopsy* ; Herpesvirus 8, Human ; Sarcoma, Kaposi*

No abstract available.
Herpesvirus 8, Human* ; Humans* ; Kidney Transplantation ; Lymphohistiocytosis, Hemophagocytic* ; Sarcoma, Kaposi* ; Transplant Recipients*

Primary effusion lymphoma (PEL) is a rare type of non-Hodgkin’s lymphoma arising from a B-cell lineage characterized by the formation of malignant effusion in body cavities without evidence of a detectable tumor. The effusion contains tumor cells universally infected with human herpesvirus 8 (HHV8), which is the critical factor differentiating PEL from HHV8-unrelated PEL-like lymphoma (PEL-LL). This report describes a 77-year-old male patient with pleural effusion and ascites, containing lymphoma cells expressing a B-cell phenotype, but without markers of HHV8 in immunocytochemical analysis. The patient was diagnosed with PEL-LL and treated with six cycles of rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisolone (R-CHOP), which resulted in a complete remission. The patient is currently disease-free 15 months post-treatment. To the best of our knowledge, this is the first report on administration of R-CHOP in a PEL-LL patient in South Korea.
Aged* ; Ascites ; B-Lymphocytes ; Cyclophosphamide* ; Doxorubicin* ; Herpesvirus 8, Human ; Humans* ; Korea ; Lymphoma* ; Lymphoma, Primary Effusion ; Male ; Phenotype ; Pleural Effusion ; Prednisolone* ; Rituximab* ; Vincristine*

Kaposi's sarcoma is an angioproliferative neoplasm that is derived from endothelial cells of blood and lymphatic vessels. Although the etiology is not yet clearly revealed, human herpes virus (HHV)-8 is believed to play an important role in the occurrence of Kaposi's sarcoma. A 57-year-old man presented with hyperpigmented patches on the right lower leg for 2 months. The patient received a kidney transplantation 19 years ago and has taken immunosuppressants since then. He had undergone a percutaneous coronary intervention twice, and his right lower leg was swollen for a year. We performed a skin biopsy on the right lower leg. Histopathological examination showed neovascularization and vascular dilatation in the dermis with perivascular proliferation of spindle cells. Immunohistochemistry revealed positive findings for CD31 and CD34. An HHV-8 test using polymerase chain reaction (PCR) showed positive findings. We report an interesting case of Kaposi's sarcoma presenting as hyperpigmented patches with percutaneous coronary intervention-induced lymphedema in an immunosuppressed patient.
Biopsy ; Dermis ; Dilatation ; Endothelial Cells ; Herpesvirus 8, Human ; Humans ; Immunohistochemistry ; Immunosuppressive Agents ; Kidney Transplantation ; Leg ; Lymphatic Vessels ; Lymphedema* ; Middle Aged ; Percutaneous Coronary Intervention ; Polymerase Chain Reaction ; Sarcoma, Kaposi* ; Skin