title: Hairy cell leukemia in a patient with situs inversus totalis: an extremely rare combination.
abstract: Hairy cell leukemia is a rare cancer of the blood. The occurrence of hairy cell leukemia with another very rare genetic disorder makes us question whether it is just a coincidence. This article reports the first case of hairy cell leukemia in a patient with situs inversus totalis in western literature. There have been studies into the pathogenesis of situs inversus totalis that suggest it is caused by the failure of embryonic cells to properly rotate during embryogenesis. On the molecular level, the nodal cilia, which are responsible for embryonic rotation, are built by transport through the KIF3 complex - a kinesin superfamily of molecular motors. The KIF3 complex is also responsible for N-cadherin movement in cells. Furthermore, it is well known that these cell adhesion molecules play an important role in carcinogenesis and its progression. This report attempts to link the rare conditions and propose a possible genetic relationship between the two.
authors: Department of Internal Medicine/Hematology & Oncology, Nassau University Medical Center, NY, USA. pathak_prajwol@yahoo.com
authors: Prajwol
authors: P
authors: Pathak
authors: Valeria
authors: V
authors: Zilberman
authors: Boris
authors: B
authors: Avezbakiyev
authors: Vladimir
authors: V
authors: Gotlieb
mesh_terms: D000368:Aged; D015820:Cadherins / Q000378:metabolism; D015815:Cell Adhesion Molecules / Q000235:genetics / Q000378:metabolism; D002471:Cell Transformation, Neoplastic / Q000235:genetics*; D002923:Cilia / Q000378:metabolism; D006801:Humans; D016547:Kinesins / Q000378:metabolism; D007943:Leukemia, Hairy Cell / Q000150:complications / Q000000981:diagnostic imaging / Q000473:pathology*; D008297:Male; D011859:Radiography; D012857:Situs Inversus / Q000150:complications / Q000000981:diagnostic imaging / Q000196:embryology / Q000473:pathology*
chemical_list: D015820:Cadherins; D015815:Cell Adhesion Molecules; C087415:KIF3A protein, human; D016547:Kinesins