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==================== THE KASABACH–MERRITT PHENOMENON  ==================== Large congenital hemangiomas may result in shunting of blood and high-output cardiac failure or entrapment of platelets and a thrombocytopenic coagulopathy and a potentially life-threatening hemorrhage (the Kasabach–Merritt syndrome or phenomenon) (Fig). The pathogenesis of congenital hemangiomas is poorly understood. There is an association with prematurity. Kasabach-Merritt phenomenon is a rare, life-threatening condition in which either of two specific vascular tumors (tufted angioma or kaposiform hemangioendothelioma) traps and destroys platelets, which are a component of blood that helps clotting. This condition is also associated with other abnormal clotting conditions in which there is excessive consumption of clotting factors. Kasabach-Merritt phenomenon does not occur in children with infantile hemangiomas. Tumors usually occur shortly after birth and are equally common in males and females. These ...
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Nature de la cellule Sternberg-Reed
#Maladie_de_HODGKIN
#INFO_MED
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**Les cellules de Reed-Sternberg sont de grandes cellules (environ 50 μm) malignes caractéristiques des lymphomes de Hodgkin et infiltrant les ganglions. Ces cellules sont caractérisées par un noyau volumineux bi- ou polylobé, la présence d’un nucléole, souvent unique, mais volumineux, d’une basophilie soutenue. Plus la maladie est avancée et plus ces cellules sont retrouvées en nombre important.
**Elles ont été décrites pour la première fois en 1898 par Carl Sternberg (de) puis par Dorothy Reed (en) (1874-1964) en 1902, d’où leur nom de cellules de Sternberg ou de Reed-Sternberg.
**Elle n’est pas connue avec certitude et certains doutent que cette cellule soit l’expression la plus directe de la maladie.
**Son origine est équivoque : des arguments pour une origine monocytaire, lymphocytaire, de type T ou B se heurtent tous à la difficulté d’isoler les cellules pathologiques de la population ganglionnaire « réactionnelle ».
Une dernière étude de 2005 montrerait une origine lymphoïde.
**On ne décèle habituellement pas de réarrangement des gènes des immunoglobulines ni du récepteur du lymphocyte T.
**L’origine de la cellule tumorale dans la maladie de Hodgkin a été longuement débattue, mais plusieurs travaux récents indiquent qu’un certain nombre de maladies de Hodgkin
se développent à partir de lymphocytes de la lignée B. Une production locale de cytokines par la cellule de Sternberg-Reed est mise en relation avec l’infiltrat inflammatoire qui entoure ces cellules malignes.
** Le virus d’Epstein Barr (EBV) peut être détecté dans les cellules de Reed-Sternberg, dans une proportion très variable de cas et il n’y a aucune preuve de son rôle dans la survenue de la maladie.

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==================== THE KASABACH–MERRITT PHENOMENON  ==================== Large congenital hemangiomas may result in shunting of blood and high-output cardiac failure or entrapment of platelets and a thrombocytopenic coagulopathy and a potentially life-threatening hemorrhage (the Kasabach–Merritt syndrome or phenomenon) (Fig). The pathogenesis of congenital hemangiomas is poorly understood. There is an association with prematurity. Kasabach-Merritt phenomenon is a rare, life-threatening condition in which either of two specific vascular tumors (tufted angioma or kaposiform hemangioendothelioma) traps and destroys platelets, which are a component of blood that helps clotting. This condition is also associated with other abnormal clotting conditions in which there is excessive consumption of clotting factors. Kasabach-Merritt phenomenon does not occur in children with infantile hemangiomas. Tumors usually occur shortly after birth and are equally common in males and females. These ...
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=============== The bronze baby syndrome: A complication of  # phototherapy =============== **The Bronze baby syndrome is the dark grey-brown pigmentation of skin, mucous membrane and urine following phototherapy. **Hepatic dysfunction has to be there for this condition to be present. We report a neonate with dark brown pigmentation of skin. Lesions were spread over the abdomen (Fig. 1), chest, back and both upper and lower limb after receiving phototherapy for neonatal jaundice. **It is assumed that abnormal accumulation of photoisomer of bilirubin is the cause of this condition. **The second cause postulated is abnormal hepatic function leading to copper-porpyhrin complex which is photodestroyed leading to brown pigmentation. **The third explanation is accumulation of biliverdin leading to pigmentation. **It should be differentiated from grey baby syndrome exclusively seen in neonates and very young infants receiving high doses of chloramphenicol. The infant is cyanosed, acidot...
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============= Transorbital endotracheal intubation: a nonstandard approach to a difficult airway. ============= A 49-year-old gentleman with a history of adenoid cystic carcinoma of the left nare status post curative bifrontal craniotomy, left lateral rhinotomy and medial maxillectomy, adjuvant radiotherapy, and orbital exenteration for optic neuropathy, complicated by medial wall dehiscence. His course was also complicated by severe radiation trismus, for which he was schedu led to undergo bilateral mandibular coronoidectomies. Given his limited mouth opening, the surgeon requested a nasal endotracheal tube. Because of concerns of traumatizing his nare, we utilized a flexible fiberoptic bronchoscope to perform asleep transorbital intubation. Airway management in patients with severe trismus may require ingenuity. (Credit : Waldron NH, Stolp BW, Ogilvie MP, Powers DB, Shaughnessy MR.)
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