Kaposi's sarcoma (KS) appears to develop in association with kidney transplantation, but unlikely with dialysis. We report two cases of classic KS that occurred in patients receiving short-term (less than 3 yr) dialysis. They have been suffering from chronic renal failure due to tuberculosis and diabetes mellitus, respectively. Several to multiple, reddened-violaceous patches, plaques and nodules were found on the hand and the lower extremities. Laboratory studies showed no evidence suggesting immunosuppressed state and there was no history of taking immunosuppressive agents. The biopsies of the two cases revealed proliferation of spindle-shaped cells focally arranged in bundles and multiple dilated vascular spaces outlined by an attenuated endothelium with intravascular and extravasated erythrocytes. The specimens expressed positivity with CD34 antigen. Human herpesvirus 8 (Kaposi's sarcoma-associated herpesvirus) was detected in one case by polymerase chain reaction method.
Aged ; Case Report ; Herpesvirus, Kaposi Sarcoma-Associated/isolation & purification ; Human ; Kidney Transplantation/adverse effects ; Male ; Middle Age ; Renal Dialysis/adverse effects* ; Sarcoma, Kaposi/virology ; Sarcoma, Kaposi/therapy ; Sarcoma, Kaposi/etiology*

OBJECTIVETo study the pathogenesis role of immune system activation in AIDS related Kaposi's sarcoma(AIDS-KS).

METHODSThe serum levels of sFas, beta 2-microglobin, IL-10, IL-16, IL-18, IL-6 and sIL-4R were detected by ELISA in 8 AIDS-KS patients, 28 patients with HIV infection but without Kaposi's sarcoma(HIV-NKS) and 16 normal controls. The lymphocyte and their subsets, CD38(+) CD8, HLA-DR(+)CD8 in the peripheral blood mononuclear cell (PBMCs) in 12 AIDS-KS and 32 HIV-NKS were detected by flow cytometer.

RESULTSBeta 2-MG and sIL-4R in HIV-NKS were significantly higher than those in normal controls(P<0.05), IL-16 in HIV-NKS was significantly lower than that in controls(P<0.05). IL-18 was higher in both AIDS-KS and HIV-NKS compared with normal controls. In AIDS-KS, CD3, CD4, CD8, NK and HLA-DR(+)CD8 were lower than those in HIV-NKS whereas CD19 and CD38(+)CD8 were higher than those in HIV-NKS. But the difference was not statistically(P<0.05).

CONCLUSIONAlthough both AIDS-KS and HIV-NKS demonstrate some activation of immune system, there appears to be no significant difference between immune responses in KS and NKS patients. These data suggest that the activation of the immune system is unlikely to contribute significantly to the pathogenesis of AIDS-KS.

Acquired Immunodeficiency Syndrome ; complications ; immunology ; Cytokines ; blood ; HLA-DR Antigens ; blood ; Humans ; Interleukin-16 ; blood ; Sarcoma, Kaposi ; etiology ; immunology

OBJECTIVETo explore the clinicopathologic features of kaposiform hemangioendothelioma (KHE).

METHODSThe clinicopathologic data were studied in three cases of KHE and review the literatures.

RESULTSTwo cases were female and one was male. All cases occurred in infancy. Two tumor located in axillary chest wall and one in lumbar region. All of the three patients had Kasabach-Merritt syndrome. Histologically, the tumor was composed of spindle-shaped cells. in all cases nodular growth pattern was seen. Immunohistochemically, Neoplastic spindled cells expressed CD34 and CD31. Associated lymphangiomatosis was present in two cases. Two tumors were resected completely, one was resected partly. the follow-up period ranged from 6 months to 3 years, and all were alive.

CONCLUSIONSKaposiform hemangioendothelioma is a rare locally aggressive vascular tumor that mainly occurred in early infancy. It is frequently complicated by Kasabach-Merritt syndrome, and it has features common to both capillary hemangioma and Kaposi sarcoma. The prognosis of KHE is determined by the size, location and the hemorrhage degree of vascular tumor. Better outcome might be achieved in patients with KHE of the skin and in the soft tissues under the skin. It appears that the main treated measure should be wide local excision.

Diagnosis, Differential ; Female ; Hemangioendothelioma ; complications ; pathology ; Humans ; Infant ; Male ; Purpura, Thrombocytopenic ; etiology ; Sarcoma, Kaposi ; complications ; pathology ; Skin Neoplasms ; complications ; pathology

Increased incidences of Kaposi's sarcoma and lymphoid malignancies have been observed in patients with pemphigus, and the human herpesvirus 8 (HHV-8) is very strongly associated with these tumors. Because the virus may be one of the triggering factors of pemphigus, we undertook this study to screen for the presence of HHV-8 in chronic blistering skin diseases including pemphigus. A total of 45 paraffin-embedded specimens were studied using nested polymerase chain reaction (PCR) with primers to amplify a 160-base pair HHV-8 fragment. HHV-8 DNA could be detected in 7 of 9 patients with pemphigus vulagris, and 1 of 2 with pemphigus foliaceus. All specimens of other blistering skin diseases were negative for HHV-8. On sequencing PCR products, the sequences were almost identical with the prototypic sequence for HHV-8, and a few base- pair substitutions at 1086C-T and 1139A-C were detected. The results of our study suggests that HHV-8 might have trophism for pemphigus lesions. Further studies including comparison of HHV-8 DNA load in both lesional and normal skin in the same patient, serological and animal studies would be helpful to study the relationship between HHV-8 and pemphigus.
Adult ; Comparative Study ; DNA Mutational Analysis ; DNA, Viral/genetics ; DNA, Viral/analysis+ACo- ; Female ; Herpesviridae Infections/virology+ACo- ; Herpesviridae Infections/epidemiology ; Herpesvirus, Kaposi Sarcoma-Associated/pathogenicity ; Herpesvirus, Kaposi Sarcoma-Associated/isolation +ACY- purification+ACo- ; Herpesvirus, Kaposi Sarcoma-Associated/genetics ; Human ; Korea/epidemiology ; Male ; Middle Age ; Paraffin Embedding ; Pemphigus/virology+ACo- ; Pemphigus/etiology ; Polymerase Chain Reaction ; Prevalence ; Retrospective Studies ; Skin Diseases, Vesiculobullous/virology+ACo- ; Skin Diseases, Viral/virology+ACo- ; Skin Diseases, Viral/epidemiology ; Tissue Fixation

Increased incidences of Kaposi's sarcoma and lymphoid malignancies have been observed in patients with pemphigus, and the human herpesvirus 8 (HHV-8) is very strongly associated with these tumors. Because the virus may be one of the triggering factors of pemphigus, we undertook this study to screen for the presence of HHV-8 in chronic blistering skin diseases including pemphigus. A total of 45 paraffin-embedded specimens were studied using nested polymerase chain reaction (PCR) with primers to amplify a 160-base pair HHV-8 fragment. HHV-8 DNA could be detected in 7 of 9 patients with pemphigus vulagris, and 1 of 2 with pemphigus foliaceus. All specimens of other blistering skin diseases were negative for HHV-8. On sequencing PCR products, the sequences were almost identical with the prototypic sequence for HHV-8, and a few base- pair substitutions at 1086C-T and 1139A-C were detected. The results of our study suggests that HHV-8 might have trophism for pemphigus lesions. Further studies including comparison of HHV-8 DNA load in both lesional and normal skin in the same patient, serological and animal studies would be helpful to study the relationship between HHV-8 and pemphigus.
Adult ; Comparative Study ; DNA Mutational Analysis ; DNA, Viral/genetics ; DNA, Viral/analysis+ACo- ; Female ; Herpesviridae Infections/virology+ACo- ; Herpesviridae Infections/epidemiology ; Herpesvirus, Kaposi Sarcoma-Associated/pathogenicity ; Herpesvirus, Kaposi Sarcoma-Associated/isolation +ACY- purification+ACo- ; Herpesvirus, Kaposi Sarcoma-Associated/genetics ; Human ; Korea/epidemiology ; Male ; Middle Age ; Paraffin Embedding ; Pemphigus/virology+ACo- ; Pemphigus/etiology ; Polymerase Chain Reaction ; Prevalence ; Retrospective Studies ; Skin Diseases, Vesiculobullous/virology+ACo- ; Skin Diseases, Viral/virology+ACo- ; Skin Diseases, Viral/epidemiology ; Tissue Fixation

Kaposi sarcoma (KS) is a vascular neoplasm, which is fairly prevalent in acquired immunodeficiency syndrome (AIDS) patients. Mucocutaneous and lymph node involvements are characteristic features of KS in AIDS patients. The involvement of gastrointestinal tract occurs in 40% of KS patients and leads to significant morbidity and mortality. In the highly active antiretroviral therapy (HAART) era, the rate of AIDS related KS has fallen with control of human immunodeficiency virus (HIV) viremia. However, it is still recognized as the primary AIDS-defining illness, and the proportion of AIDS diagnoses made due to KS ranged from 4.1% to 7.5%. In Korea, AIDS-related KS has been report in low rate incidence. Its gastrointestinal involvements are rarely reported. To date, five cases have been recorded in Korea. Herein, we present an additional case of gastrointestinal KS as the AIDS-defining illness and review of the Korean medical literature.
Acquired Immunodeficiency Syndrome/complications/*diagnosis ; Anti-HIV Agents/therapeutic use ; Endoscopy, Digestive System ; HIV Infections/complications/diagnosis/drug therapy ; Humans ; Male ; Middle Aged ; Republic of Korea ; Sarcoma, Kaposi/*diagnosis/etiology/pathology ; Tomography, X-Ray Computed

Kaposi sarcoma herpes virus (KSHV), also known as human herpesvirus-8, plays an important role in the pathogenesis of Kaposi sarcoma (KS), multicentric Castleman disease (MCD) of the plasma cell type, and primary effusion lymphoma. KSHV is rarely associated with the hemophagocytic syndrome (HPS), but when it does occur, it most occurs in immunocompromised patients. We report herein an unusual case of KSHV-associated HPS in an immunocompetent patient. A previously healthy 62-yr-old male was referred for evaluation of leukocytopenia and multiple lymphadenopathies. After a lymph node biopsy, he was diagnosed with MCD of the plasma cell type. KSHV DNA was detected in the lymph node tissue by polymerase chain reaction. Following a short-term response of the leukocytopenia to prednisolone, mental change, left side weakness, fever, thrombocytopenia, hemolytic anemia, and renal failure developed. Despite intravenous immunoglobulin therapy and plasmapheresis, he expired. The lymph nodes were infiltrated by hemophagocytic histiocytes in the sinuses. Pulmonary nodules and gastric erosions were shown to be KS. KSHV DNA was detected in the stomach, lung, and liver. This is the first case of multiple KSHV associated diseases including MCD and KS with KSHV-associated hemophagocytic syndrome in an HIV-negative, non-transplant, immunocompetent patient.
Autopsy ; Giant Lymph Node Hyperplasia/complications/*diagnosis/pathology ; HIV Seronegativity ; Herpesviridae Infections/*diagnosis ; Herpesvirus 8, Human/*isolation & purification ; Humans ; Immunocompetence ; Lymphohistiocytosis, Hemophagocytic/*diagnosis/etiology ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Polymerase Chain Reaction ; Sarcoma, Kaposi/complications/*diagnosis

Abdominal Cavity ; Adult ; Anemia, Aplastic ; therapy ; Antigens, CD34 ; metabolism ; Carcinoma ; metabolism ; pathology ; Dendritic Cell Sarcoma, Follicular ; metabolism ; pathology ; Diagnosis, Differential ; Hematopoietic Stem Cell Transplantation ; adverse effects ; Humans ; Ki-67 Antigen ; metabolism ; Lymph Nodes ; pathology ; Male ; Neoplasms, Muscle Tissue ; metabolism ; pathology ; Platelet Endothelial Cell Adhesion Molecule-1 ; metabolism ; Sarcoma, Kaposi ; etiology ; metabolism ; pathology ; Viral Proteins ; metabolism