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Klin Padiatr ; 222(6): 374-7, 2010 Nov.
Article in English | MEDLINE | ID: mdl-21058224

ABSTRACT

BACKGROUND: Childhood immune thrombocytopenia (ITP) is a bleeding disorder characterized by decreased platelet counts. Assessment of the individual bleeding risk during the course of the disease would allow more accurately guiding treatment-related decisions in these patients. PATIENTS AND METHODS: We conducted a pilot study and prospectively evaluated platelet counts and bleeding signs using an established bleeding (Buchanan) score in 30 patients with newly diagnosed ITP at 3 different time points (at diagnosis [TP1], on day 2-3 [TP2], and on day 5-8 [TP3]) during the first week after diagnosis. 15 patients received immune modulatory therapy. RESULTS: Median platelet counts at the 3 different time points were 13, 19, 32×10 (9)/L (untreated patients) and 2, 7, 37×10 (9)/L (treated patients). Corresponding median cumulative bleeding scores were 5, 2, 0 (untreated patients) and 7, 6, 2 (treated patients). Cumulative median bleeding scores and platelet counts were inversely correlated in treated and untreated patients at all 3 time points. Cumulative median bleeding scores significantly decreased in both groups. CONCLUSIONS: Bleeding signs in children with newly diagnosed ITP rapidly improve within one week after diagnosis. Serial grading of bleeding severity seems to be useful to comprehensively assess and monitor the individual bleeding risk in these patients, but has to be evaluated and validated in a larger cohort.


Subject(s)
Hemorrhage/diagnosis , Hemorrhage/immunology , Purpura, Thrombocytopenic, Idiopathic/diagnosis , Purpura, Thrombocytopenic, Idiopathic/immunology , Adrenal Cortex Hormones/therapeutic use , Child , Child, Preschool , Combined Modality Therapy , Female , Hemorrhage/therapy , Humans , Immunization, Passive , Male , Pilot Projects , Platelet Count , Prospective Studies , Purpura, Thrombocytopenic, Idiopathic/therapy
3.
Klin Padiatr ; 222(3): 184-6, 2010 May.
Article in English | MEDLINE | ID: mdl-20514624

ABSTRACT

A 14-year-old girl was diagnosed with alveolar soft part sarcoma (ASPS) of the thigh and lung metastases. She underwent tumor resection and pulmonary metastasectomy followed by hyperfractionated local radiotherapy (44.8 Gy). A mesh graft transplant was used to cover the postoperative skin defect on the thigh. Since ASPS do not respond to conventional chemotherapy antiangiogenic treatment with peginterferon alfa-2b and thalidomide was started. Immunohistochemical analysis of tumor tissue showed expression of vascular endothelial growth factor receptors (VEGFR) 1, 2, 3, and platelet derived growth factor receptor (PDGFR)-alpha and -beta. Hence, additional treatment with multitargeted receptor tyrosine kinase inhibitor sunitinib (Sutent) was started on a compassionate use basis. 2 weeks later the patient presented with necrosis of the skin transplant requiring necrectomy and skin grafting. This case illustrates that drugs inhibiting vascular endothelial growth factor receptors have to be used very cautiously in cancer patients with severe pre-existing skin damage.


Subject(s)
Angiogenesis Inhibitors/adverse effects , Antineoplastic Agents/adverse effects , Indoles/adverse effects , Lung Neoplasms/drug therapy , Lung Neoplasms/secondary , Pyrroles/adverse effects , Sarcoma, Alveolar Soft Part/drug therapy , Sarcoma, Alveolar Soft Part/secondary , Soft Tissue Neoplasms/drug therapy , Surgical Flaps/blood supply , Surgical Flaps/pathology , Thigh , Adolescent , Angiogenesis Inhibitors/therapeutic use , Antineoplastic Agents/therapeutic use , Chemotherapy, Adjuvant , Combined Modality Therapy , Compassionate Use Trials , Female , Follow-Up Studies , Humans , Indoles/therapeutic use , Lung Neoplasms/pathology , Lung Neoplasms/surgery , Necrosis , Postoperative Complications/chemically induced , Postoperative Complications/pathology , Postoperative Complications/surgery , Pyrroles/therapeutic use , Radiotherapy, Adjuvant , Reoperation , Sarcoma, Alveolar Soft Part/surgery , Skin Transplantation , Soft Tissue Neoplasms/pathology , Soft Tissue Neoplasms/surgery , Sunitinib , Thigh/surgery
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