Kaori Kanda

Clinical improvement of diffuse lymphangiomatosis with pegylated interferon alfa-2b therapy: case report and review of the literature.

Diffuse lymphangiomatosis is a very rare congenital disease, characterized by diffuse or multifocal lymphangioma in the skeletal tissue, spleen, liver, mediastinum, and/or lung. The prognosis is usually poor, especially for children with thoracic lesion, and treatments for the disease are controversial. The authors report a 9-year-old boy with diffuse lymphangiomatosis involving the thorax with pleural effusions, the spleen, and systemic bone. The patient was treated with pegylated interferon alfa-2b, and achieved good clinical and radiological improvement.

Propranolol for infantile hemangioma: Effect on plasma vascular endothelial growth factor

Propranolol has recently been shown to be highly effective for infantile hemangioma (IH), but the mechanism of action of propranolol and the usefulness of measurement of vascular endothelial growth factor (VEGF) remain poorly understood. The aim of this study was therefore to determine the efficacy of propranolol treatment and to evaluate changes in plasma VEGF in IH patients who underwent propranolol treatment.

Successful Everolimus Treatment of Kaposiform Hemangioendothelioma With Kasabach-Merritt Phenomenon: Clinical Efficacy and Adverse Effects of mTOR Inhibitor Therapy.

Kasabach-Merritt phenomenon (KMP) is a life-threatening consumptive coagulopathy associated with underlying kaposiform hemangioendothelioma (KHE) in infancy. We describe the case of a 3-month-old girl with KHE complicated by KMP who responded dramatically to treatment with everolimus, a mechanistic target of rapamycin (mTOR) inhibitor. Immunohistochemical expression of mTOR was found in the KHE biopsy specimens, which may explain the improvement of KMP and reduction in KHE tumor size with mTOR inhibitor treatment. This effective use of everolimus may shed light on the emerging role of mTOR signaling in the development and pathogenesis of KHE and KMP.

Everolimus for Treatment of Pseudomyogenic Hemangioendothelioma

Pseudomyogenic hemangioendothelioma (PMH) is a recently described vascular neoplasm that occurs most commonly in the soft tissue of the distal extremities of young adults. Metastatic PMH can be fatal and there are no effective medications. We describe a case of a 15-year-old boy with metastatic PMH, who responded to treatment with everolimus, a mammalian target of rapamycin inhibitor. Immunohistochemistry showed that mammalian target of rapamycin was expressed in PMH biopsy specimens, which may explain the reduction in PMH tumor size following treatment.

Everolimus for Primary Intestinal Lymphangiectasia With Protein-Losing Enteropathy.

Primary intestinal lymphangiectasia (PIL), also known as Waldmann’s disease, is an exudative enteropathy resulting from morphologic abnormalities in the intestinal lymphatics. In this article, we describe a 12-year-old boy with PIL that led to protein-losing enteropathy characterized by diarrhea, hypoalbuminemia associated with edema (serum albumin level: 1.0 g/dL), and hypogammaglobulinemia (serum IgG level: 144 mg/dL). Severe hypoalbuminemia, electrolyte abnormalities, and tetany persisted despite a low-fat diet and propranolol. Everolimus (1.6 mg/m2/day) was added to his treatment as an antiangiogenic agent. With everolimus treatment, the patient’s diarrhea resolved and replacement therapy for hypoproteinemia was less frequent. Hematologic and scintigraphy findings also improved (serum albumin level: 2.5 g/dL). There were no adverse reactions during the 12-month follow-up. To the best of our knowledge, this is the first report of everolimus use in a patient with PIL.

Facial palsy as an unusual presenting symptom associated with acute myeloid leukemia

Extramedullary infiltration is common in acute myeloid leukemia (AML) patients. Although AML can cause neurological symptoms, especially when associated with extramedullary infiltration, a presenting manifestation of facial palsy is rare. We report on a 1‐year‐old boy who developed right facial palsy. Detailed examination led to a diagnosis of AML (French–American–British classification M1). Magnetic resonance imaging enhanced with gadolinium‐diethylenetriamine penta‐acetic acid showed abnormal enhancement of the right facial nerve, which disappeared after chemotherapy. AML should be considered as a differential diagnosis of facial palsy. Enhanced magnetic resonance imaging may be useful for diagnosing facial palsy associated with AML and for evaluating treatment outcome.

Complete bone regeneration in hemophilic pseudotumor of the mandible.

Hemophilic pseudotumor (HP) is rare, seen in 1–2% of patients with hemophilia, and is extremely uncommon in the mandible. A 6‐year‐old boy with moderate hemophilia A presented to our hospital with left mandibular swelling. Based on clinical and radiological findings, a tentative diagnosis of HP was made. After factor VIII administration, the lesion was curetted and HP was confirmed on histopathology. The patient was treated with twice‐weekly factor VIII until the lesion had completely resolved and bone had regenerated at 1 year. The best treatment for HP is not established; however, appropriate initial treatment and postoperative prophylaxis are effective.

Pediatric acute lymphoblastic leukemia mimicking Henoch–Schönlein purpura

Effectiveness of imiquimod 5% cream for treatment of extensive anogenital warts in a seven-year-old child. Pediatr. Inf. Dis. J. 2007; 26: 265–6. 5 Silverberg NB. Human papillomavirus infections in children. Curr. Opin. Pediatr. 2004; 16: 402–9. 6 Gruber PC, Wilkinson J. Successful treatment of perianal warts in a child with 5% imiquimod cream. J. Dermatolog. Treat. 2001; 12: 215–17. 7 Schaen L, Mercurio MG. Treatment of human papilloma virus in a 6-month-old infant with imiquimod 5% cream. Pediatr. Dermatol. 2001; 18: 450–2. 8 Majewski S, Pniewski T, Malejczyk M, Jablonska S. Imiquimod is highly effective for extensive, hyperproliferative condyloma in children. Pediatr. Dermatol. 2003; 20: 440–2. 9 Grussendorf-Conen EI, Jacobs S. Efficacy of imiquimod 5% cream in the treatment of recalcitrant warts in children. Pediatr. Dermatol. 2002; 19: 263–6. 10 Barba AR, Kapoor S, Berman B. An open label safety study of topical imiquimod 5% cream in the treatment of Molluscum contagiosum in children. Dermatol. Online J. 2001; 7: 20.

Anaphylactoid transfusion reactions associated with a positively charged white-cell reduction filter: A case report

The effectiveness of a white-cell reduction filter to deplete contaminated leucocytes in preventing the harmful effects of transfusion is evident. However, several complications associated with a white-cell reduction filter have been identified. We report the first case of anaphylactoid reactions caused by a white-cell reduction filter with a positively charged surface.

P1-238Clinical efficacy of mTOR inhibitor for Kaposiform hemangioendotheliomas with Kasabach-Merritt phenomenon

Case presentation: A 56-year-old female was received the resection of retroperitoneal leiomyosarcoma in December 2011. In July 2015, CT showed multiple liver metastases, and she received the treatment with ADR as first-line chemotherapy. After two cycles of ADR, her performance status was deteriorative, so she was treated with pazopanib as second-line chemotherapy. The PFS of pazopanib was 5 months and eribulin was 2.5 months. She was then treated with trabectedin as 4th-line chemotherapy from July 2016. Liver metastases was reduced and the disease has been controlled for 7 months from administration of trabectedin. We have still treated with trabectedin.