No abstract

Waldenstrom's macroglobulinemia (WM) represents an indolent incurable human B-cell tumor. We have successfully established a permanent cell line, WSU-WM, without growth factors or viral transformation, from the pleural effusion of a 60-year-old man with IgM kappa WM. Phenotypic characterization of WSU-WM shows IgM lambda and expression of other B- cell markers. Karyotypic analysis shows a male chromosome complement with several clonal aberrations, including t(8;14)(q24;q32). Molecular characterization shows deletion of kappa and rearrangement of lambda light chain genes indicating a class switching. Both the secretory (s mu) and membrane (m mu) components of IgM are expressed. In addition, the breakpoint on 8q24 is downstream of exon 3 of the c-myc oncogene. WSU-WM grows in liquid culture and soft agar. When cells were injected subcutaneously in immune deficient mice, six of seven SCID mice developed subcutaneous tumors as opposed to three of seven in the athymic nude mice. When a WSU-WM SCID tumor was passaged in vivo in the SCID mice, the take rate was 100%. This xenograft model and a soft agar disk-diffusion assay were used to test the efficacy of standard chemotherapy agents against this tumor in vivo and in vitro, respectively. The cell line and the assays described herein can be used as a model to facilitate the discovery of new therapeutic agents or modalities for this disease.

Atlas of Genetics and Cytogenetics in Oncology and Haematology OPEN ACCESS JOURNAL AT INIST-CNRS A new case of t(4;12)(q12;p13) in a secondary acute myeloid leukemia with review of literature

Branchio-oto-renal spectrum disorders are rare genetic entities with variable penetrance and concurrently display a wide phenotypic variation. A common issue to syndromic children is a propensity for difficult bag-mask ventilation, intubation or both. Unfortunately, there is no uniformity of this challenge, assessment strategy or management plan. This case with features of branchio-otic syndrome provided the opportunity to examine several aspects of paediatric anaesthetic airway management. The child was booked for branchial cyst removal but appeared to have other features of abnormal branchial cleft development. An outline of the executed anaesthetic plan is presented and discussed. Three different techniques were sequentially tried in this patient before the airway was secured. Various case reports in the literature inconsistently describe easy to very difficult airway management in children with branchio-oto-renal spectrum disorders. Branchial arch dysgenesis is almost always associated with difficult direct laryngoscopy. There is undoubtedly no singular way to ideally manage a child with a difficult airway. Many tools for difficult airways are available. However, despite the improvement of difficult paediatric airway equipment, it would appear that for the anaesthetist the flexible bronchoscope remains an indispensable tool.

INIST-CNRS OPEN ACCESS JOURNAL Atlas of Genetics and Cytogenetics in Oncology and Haematology t(2;11)(q31;p15) NUP98/HOXD13 t(2;11)(q31;p15) NUP98/HOXD11