OBJECTIVE: To study the clinical features, treatment, and prognosis of neonates with Kasabach-Merritt syndrome (KMS), and to provide a reference for optimizing the diagnosis and treatment of this disease. METHODS: A retrospective analysis was performed for the clinical and follow-up data of 16 neonates with KMS who were admitted to the Anhui Children's Hospital, Anhui Medical University, from January 2016 to December 2020. RESULTS: Of the 16 neonates, there were 13 boys (81%) and 3 girls (19%), with an age of 1 hour to 10 days on admission. Among these neonates, 13 (81%) had cutaneous hemangioma (2 in the head and face, 5 in the trunk, and 6 in the extremities) and 3 (19%) had liver hemangioma. The main clinical manifestations of bleeding tendency and scattered petechiae and ecchymosis were observed in 10 neonates (62%). All the 16 neonates had varying degrees of thrombocytopenia and coagulation disorders. They all received glucocorticoid treatment after admission and 7 (44%) of them had response, among whom 4 experienced recurrence. Among the neonates with no response to glucocorticoid treatment, 3 received sirolimus treatment, among whom 1 had the tumor volume reduced by 58.8% after 4 weeks of treatment, with platelet count and coagulation function returning to normal, while 2 had no significant reduction in tumor volume or significant increase in platelet count and achieved a tumor volume reduced by (43.7±0.4)% after 4 weeks of combined treatment with bleomycin arterial embolization, with platelet count and coagulation function returning to normal. After 4 weeks of bleomycin arterial embolization alone for 4 neonates, tumor volume was reduced by (52.0±3.4)%, and platelet count and coagulation function returned to normal. Blunt and sharp dissection was performed for 2 neonates. The tumor was removed completely during surgery in the 2 neonates, with no infection or recurrence after surgery, and platelet count and coagulation function returned to normal. The postoperative pathological examination showed Kaposiform hemangioendothelioma in 1 out of the 2 neonates. CONCLUSIONS KMS has characteristic clinical manifestations, histopathological features, and laboratory examination results. The KMS neonates who are not sensitive to glucocorticoids can achieve a good curative effect through arterial embolization and sirolimus treatment.
Child ; Female ; Hemangioendothelioma ; Humans ; Infant, Newborn ; Kasabach-Merritt Syndrome/therapy* ; Male ; Neoplasm Recurrence, Local ; Retrospective Studies ; Sarcoma, Kaposi

Introduction: Acquired immunodeficiency syndrome-Kaposi sarcoma (AIDS-KS) has unique clinical characteristics, often dis- seminated on presentation, a rapidly progressive course, and often fatal outcome. Describing the epidemiology and clinical characteristics of AIDS-KS in the Philippines may lead to early recognition, diagnosis, and management of this condition, which are the keys to preventing significant complications. Case Series: AIDS-KS in 11 Filipino MSM patients with a mean age of 36.55 years (SD 11.54) was described. Violaceous plaques and nodules were present for an average of 5.1 months prior to diagnosis confirmed by biopsy. Histopathologic findings from all pa- tients were consistent with KS. The median CD4+ count of patients was 44 cells/microliter (range, 4 to 181). Six patients presented with opportunistic infections (OI)/AIDS-related conditions (ARC). The most common OIs observed were pulmonary tuberculosis, oropharyngeal candidiasis, and Pneumocystis jiroveci pneumonia. Nine patients improved with highly active antiretroviral therapy (HAART). One patient required modification on his HAART regimen, which was shifted to 2 NRTI and ritonavir-boosted protease inhibitor, and one patient died due to AIDS-related complications. Conclusion This series of 11 cases of AIDS-KS showed similar demographic, clinical and histopathologic characteristics to pre- viously published studies. Findings suggest the need for earlier recognition and diagnosis. While HAART afforded clinical improve- ment in a majority of patients, other treatment options such as chemotherapy should be considered for appropriate patients.
Sarcoma, Kaposi ; Antiretroviral Therapy, Highly Active

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

Since the introduction of highly active antiretroviral therapy, the life span of people with human immunodeficiency virus (HIV) or AIDS (PWHA) has been extended significantly. Therefore, the importance of non-AIDS-defining cancers (NADCs), as well as AIDS-defining cancers (ADCs) has increased. There is little information concerning the epidemiology of malignancies in PWHA in Korea. A descriptive epidemiologic study was conducted at a tertiary care hospital in Korea. PWHA who visited Pusan National University Hospital from January 2000 to October 2014 were included. Demographics and clinical data were obtained from the medical records and analyzed. A total of 950 PWHA were observed for 4,439.71 person-years. Forty-eight episodes (5.05%) of cancers were diagnosed in 47 patients. Mean age of the enrolled patients was 40.66 ± 12.15 years and 88% were male. Among the 48 cancer episodes, 20 (42%) were ADCs and 28 were NADCs. The most common ADCs was non-Hodgkin's lymphoma (53.6%), followed by Kaposi's sarcoma (17.9%). The most common NADCs were lung cancer (25%) and hepatocellular carcinoma (25%). The overall incidence of total cancers, ADCs, and NADCs was 10.8 (95% confidence interval [CI], 8.0–14.3), 4.5 (95% CI, 2.8–7.0), and 6.3 (95% CI, 4.2–9.1)/1,000 person-years, respectively. NADCs accounted for 12/15 (80%) of cancers among PWHA with good adherence to care. The 5-year survival rate of PWHA and NADC was 26.3%. NADCs have become the main type of malignancy among Korean PWHA with good adherence to care. Effective strategies to improve screening of NADCs among PWHA are required in Korea.
Antiretroviral Therapy, Highly Active* ; Busan ; Carcinoma, Hepatocellular ; Demography ; Epidemiologic Studies ; Epidemiology ; HIV* ; Humans ; Humans* ; Incidence ; Korea ; Lung Neoplasms ; Lymphoma, Non-Hodgkin ; Male ; Mass Screening ; Medical Records ; Sarcoma, Kaposi ; Survival Rate ; Tertiary Healthcare

No abstract available.
Aged, 80 and over ; Female ; Humans ; Injections, Intralesional ; Sarcoma, Kaposi/*drug therapy ; Skin Neoplasms/*drug therapy ; Sodium Tetradecyl Sulfate/*administration & dosage/*therapeutic use

OBJECTIVETo summarize clinical features of eye Kaposis' sarcoma ( KS ) in leukemia child after peripheral blood stem cell transplantation (PBSCT).

METHODSOne 13 years-old child with acute lymphoblastic leukemia (ALL) and negative HIV test who developed KS restricted in right conjunctiva, cornea and sclera after successful allogeneic PBSCT was reviewed retrospectively.

RESULTSThe child suffered from T cell type ALL. He received immunosuppressive treatment after PBSCT, and had once extensive herpes zoster restricted in skin. Seven months after PBSCT, he had blurred vision with right eye and slowly neoplasm formed in cornea and conjunctiva. Pathological examination confirmed KS with changes like capillary hemangioma, atypical fusiform cell, typical immunochemistry and positive immunofluorescent result of HHV8. He received excision of lump of cornea, conjunctiva, sclera and transplantation of cornea and sclera. Antiviral therapy was given together with anti-infection, prevention of cornea rejection and biotherapy. He kept right eye and hand-move eyesight, survived without GVHD or recurrence of ALL and KS.

CONCLUSIONThis was the first ocular KS case in ALL child after PBSCT, without correlation with HIV infection. Complete excision combined with biotherapy was safe and effective for single ocular lesions.

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

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

Angiokeratomas are cutaneous vascular lesions that are characterized by dilated thin-walled blood vessels that lay in the upper part of the dermis, and this condition is mostly associated with epidermal reactions such as acanthosis, hyperkeratosis and elongation of rete ridges. Four variants have been described (angiokeratoma of Fordyce, angiokeratoma of Mibelli, angiokeratoma corporis diffusum, and solitary or multiple angiokeratomas). Among these variants, angiokeratoma of Fordyce develops mainly in elderly Caucasian men and it is common on the genitalia. Angiokeratomas are blue to purple, smooth, 2- to 5-mm papules on the scrotum, penile shaft or glans. They generally appear to multiply during life, but they occasionally present as single lesions. They may bleed after trauma and they may be mistaken for a nevus, melanoma or Kaposi sarcoma. Electrocautery or laser ablation can be offered for treatment. Various studies have recently reported successful treatment with argon laser, copper vapor laser, variable pulse width 532 nm neodymium:yttrium-aluminium-garnet (Nd:YAG) laser, 532 nm KTP (potassium-titanyl-phosphate) laser and flashlamp-pumped pulsed dye laser (PDL). We report here on a case of angiokeratoma that was treated with a 532nm KTP laser.
Aged ; Angiokeratoma ; Argon ; Blood Vessels ; Dermis ; Electrocoagulation ; Fabry Disease ; Genitalia ; Humans ; Laser Therapy ; Lasers, Dye ; Lasers, Gas ; Lasers, Solid-State ; Male ; Melanoma ; Nevus ; Sarcoma, Kaposi ; Scrotum

Kaposi sarcoma in acquired immunodeficiency syndrome (AIDS) is rarely seen in Korea and the incidence has declined in the era of highly active antiretroviral therapy (HAART). Visceral involvement in patients having AIDS with Kaposi sarcoma is more common than in patients infected with non-human immunodeficiency virus. Visceral involvement may be asymptomatic and usuallydoes not require specific therapy. Chemotherapy is usually used in symptomatic or rapidly progressive disease, and interferon- alpha can be applied as an alternative treatment option. Compared to cutaneous Kaposi sarcoma in AIDS, only three cases of gastrointestinal Kaposi sarcoma in patients with AIDS have been reported in Korea, and no experience with interferon therapy for AIDS-associated visceral Kaposi sarcoma has been reported. We report a case of gastrointestinal Kaposi sarcoma in a patient with AIDS who had combined treatment with interferon-alpha and HAART.
Acquired Immunodeficiency Syndrome ; Antiretroviral Therapy, Highly Active ; Ciprofloxacin ; Drug Resistance ; Humans ; Incidence ; Interferon-alpha ; Interferons ; Korea ; Nalidixic Acid ; Sarcoma, Kaposi ; Typhoid Fever ; Viruses