Kasabach–Merritt phenomenon: a single centre experience

Objective: Kasabach–Merritt phenomenon (KMP) can lead to life‐threatening bleeding, and its optimum treatment has not been established. We review the experience of managing KMP in a single institution. Methods: A retrospective chart review on all children with KMP treated at the Hospital for Sick Children, Toronto, over an 18 yr period was carried out. Results: All 15 patients had profound thrombocytopenia and hypofibrinogenemia at presentation, half had bleeding symptoms, and three had cardiac failure. All patients received corticosteroids. Five responded to steroids alone, given for an average of 13 wk, increasing platelets to >20 × 10⁹/L at a mean of 6.2 d and fibrinogen >1 g/dL at 25.6 d. Ten patients received at least one other therapeutic modality in addition to steroids, including vincristine, interferon, anti‐platelet agents and pentoxifylline. Five patients received vincristine, for a mean of 6 wk, with two patients responding. Eight patients received interferon, for a mean of 4 months, with two patients responding. Overall, the mean time to increasing platelets >20 × 10⁹/L was 56 d, to >150 × 10⁹/L was 88 d and fibrinogen >1 g/dL 49 d. Ten patients showed a partial response to embolisation, with a mean of 2.8 procedures performed. Thrombotic complications occurred in 7%. Twelve patients remain alive, with relapse in six patients, all treated successfully. One patient died, and two patients have been lost to follow‐up. Conclusion: KMP is a rare condition, with significant morbidity and mortality. The therapeutic approach should include a multidisciplinary team and consensus on guidelines.     

Other vascular tumors

Vascular tumors are rare in children and adults. Classification of these tumors has been difficult, especially in the pediatric population, due to the rarity of these lesions, the unusual morphologic appearance, their diverse clinical behavior, and no independent stratification for pediatric tumors. In 2013, The World Health Organization updated the classification of soft tissue vascular tumors. Pediatric tumors were not independently stratified and the terminology was mostly left unchanged, but the intermediate category of tumors was divided into locally aggressive and rarely metastasizing. These tumors are treated with multimodality therapy and therefore need the guidance of an interdisciplinary team for best care.     

Evaluation of a novel lentiviral vaccine expressing KMP11‐HASPB fusion protein against Leishmania infantum in BALB/c mice

Hydrophilic acylated surface protein B (HASPB) is an immunogenic Leishmania protein against which antibodies are produced in the sera of cutaneous and visceral Leishmaniasis (VL) patients. Kinetoplastid membrane protein 11 (KMP11) is another protein antigen of Leishmania which is reported as a promising candidate for vaccination of VL. It is a highly conserved surface protein present in all members of kinetoplastid family and is expressed in both promastigotes and amastigotes. In this study, the coding sequence of KMP11 and HASPB was cloned into a pCDH‐cGFP lentiviral vector as a fusion protein. The gene expression was confirmed using RT‐PCR and Western blot methods. After injection of the recombinant KMP11‐HASPB‐expressing lentiviruses to BALB/c mice, using ELISA technique, a significant increase in IFN‐γ and IL‐4 as well as IgG1 and IgG2a was observed compared to the control group. Furthermore, the number of parasites in the liver and spleen of vaccinated mice decreased significantly compared with the control group.     

Mapping of the antigenic determinants of the T. cruzi kinetoplastid membrane protein-11. Identification of a linear epitope specifically recognized by human Chagasic sera

The high variability among strains and isolates of Trypanosoma cruzi and the existence of shared antigenic determinants with other pathogens, particularly with members of the Leishmania genus make difficult the specific diagnosis of Chagas' disease. The data reported in this paper show that the T. cruzi KMP11 protein is an immunodominant antigen highly recognized by the sera from chagasic and leishmaniasis patients. By the use of amino- and carboxyl-terminal truncated KMP11 recombinant proteins and synthetic peptides, evidence is provided that while the sera from chagasic patients recognize linear peptides the sera from patients with visceral leishmaniasis must be predominantly directed against conformational epitopes. We found that a particular linear determinant, located in the carboxyl-terminal region of the protein, is recognized with high specificity and sensitivity only by sera from Chagas' disease patients, suggesting it could be a good candidate for differential serodiagnosis of Chagas' disease.     

小肠系膜Kaposi型血管内皮瘤致消化道出血一例及文献回顾

Kaposi型血管内皮瘤是一种定义为中间型的血管肿瘤。本文对1例发生于小肠系膜Kaposi型血管内皮瘤患者的组织病理学、免疫表型及临床资料进行回顾性分析并文献复习。该例患者以消化道出血为主要症状,影像学示肠系膜巨大软组织肿块并侵犯回肠,临床未见卡梅诺现象(Kasabach-merritt phenomenon,KMP),光镜下可见海绵状脉管样结构,部分区域间质伴淋巴细胞聚集灶,其间散在较多由短梭形细胞瘤构成的结节,结节内瘤细胞呈纵横交错排列并形成条索或裂隙样血管。免疫组化:CD31(+),CD34(+),D2-40(+),C-Kit(-),SMA(-),Ki-67(1%)。     

Opposites attract: holistic emotional regulation using dance/movement therapy and the doshas in Ayurveda

This model presents a collaborative and holistic perspective on dance/movement therapy (D/MT) and Ayurveda. This approach suggests that the dance/movement therapist takes into account all range and manner of an individual’s movement repertoire and uses these observations to construct therapeutic interventions that build upon body types and movement preferences represented in Ayurveda. Both D/MT and Ayurveda share the value of achieving a sense of regulation through the use of opposite qualities of movement, which will be explored in depth through the lenses of Laban Movement Analysis and the Kestenberg Movement Profile. Several examples of interventions the therapist might utilise are provided. Further considerations exploring the growth of this model in the future and limitations are also included in this article.