MUDr. Dana Maňasková


Vyhledávání na medicinman.cz
 

Sekundární lymfedém obecně

Secondary lymphedema

  • Often develops years after an invasive treatment.
  • Lymphangiogenesis
    • Control of a tightly orchestrated equilibrium between numerous players.
  • Nevznikne u každého člověka, a pokud vznikne, liší se
    • V době, za jak dlouho se objevil
    • Tíží, průběhem
    • I šancí na vyléčení či stabilizaci aj.
  • Je jasné, že o tom rozhoduje celá mozaika faktorů
    • Počínaje geny, konče životním stylem a důsledností péče o končetinu

Nejčastější příčiny

  • Cancer
  • Surgery
    • Po Ca prsu
    • Po Ca prostaty
    • Po Ca dělohy
    • Po Ca močového měchýře
    • Po Sarkomu stehenní kosti,...
    • Po Ca měkkých tkání krku,...
  • Radiation therapy
  • Trauma
  • Infection
    • Filariasis
    • Erysypel, erysypeloid,...

LMs - Local Mutations, post-zygotic mosaic mutations, Somatic mutations

  • Localized lesions
    • Dilated lymphatic channels filled with lymph
    • Disconnected from the normal lymphatic system
  • LMs are congenital
  • Enlarge when infected
  • Sporadic
  • Usually unifocal
  • Etiopathogenesis is unknown
  • The lack of familial forms
  • Unifocality of the lesions
  • Cause could be a somatic mutation, restricted to cells of the lesion
    • Might be too damaging (i.e., lethal) if occurring germline
  • True for sporadic venous malformations
    • Result from strongly activating somatic mutations in TIE2 [55]
  • Can be part of a syndrome, such as
    • Turner syndrome (due to monosomy X),
    • Overgrowth syndromes, such as
      • Proteus syndrome (OMIM 176920)
      • Klippel-Trenaunay-Weber syndrome (capillary-lymphatico-venous malformation; OMIM 149000)
      • CLOVES syndrome (OMIM 612918) due to mutations in the PI3K/AKT pathway [55]
  • PTEN mutations are found in a subset of patients with PTEN hamartoma tumor syndrome; some are inherited [55]
  • Recurrent somatic activating mutation in AKT1 in patients with proteus syndrome
    • Akt1–/– mice have reduced lymphatic capillaries and valve numbers, underscoring an important function in lymphangiogenesis [55]
  • Somatic mutations that activate PIK3CA were identified in patients with
    • CLOVES
    • Some with Klippel-Trenaunay-Weber syndrome
    • Other overgrowth syndromes as well as sporadic LMs may be caused by somatic changes in the components of this pathway. [55]
  • VEGF-C/VEGFR-3 axis are a central mechanism in the etiopathogenesis of inherited lymphedema
    • proteins of the RAS/ MAPK pathway that regulate VEGFR-3 expression
    • Some lymphatic dysplasia are caused by somatic mutations that tend to cluster around PI3K/AKT signaling [55]
      • There are connections between the two pathways
      • Etiopathogenic causes of primary (and secondary) lymphedema may cluster around these intracellular functions [55]

Combination of mildly mutated germline alleles

  • More difficult to identify

Mutations in regulatory regions


Epigenetic changes



Výčet náhodně nalezených informací přímo v souvislosti se sekundárním lymfedémem

Cx47 mutations

  • Identified in individuals having secondary lymphedema following breast cancer treatment

GJC2 gene mutations

  • Increase risk for developing secondary lymphedema following breast cancer treatment

Vyšší intersticiální onkotický tlak



Lymph node infarction

  • Rare phenomenon reported in literature in association with various conditions

Non-neoplastic conditions

  • Polyarteritis nodosa
  • Viral infections
    • Parvovirus B19
    • Infectious mononucleosis
    • Dengue fever [50]
      • Infarction of the lymph node occurred before the DIC became clinically evident [50]
  • cholesterol atheromatous embolism
  • Thrombosis
  • Gold injections
  • Intestinal volvulus
  • Postmediastinoscopy
  • Mononeuritis multiplex
  • Fine needle aspiration cytology
  • Idiopathic [50]
  • Hemorrhagic infarction of hilar lymph nodes has also been reported recently in association with heart lung transplantation [50]

Neoplastic lesions

  • Malignant lymphoma
    • Most common cause [50]
  • Metastatic melanoma [50]

Dif. dg.

  • Necrotizing lymphadenitis (Kikuchi's lymphadenitis)
  • Mucocutaneous lymph node syndrome
  • Necrotizing granulomatous inflammation [50]

MET gene


Těsné prádlo


Lymfatická obstrukce


Lymphatic filariasis

  • Three different species of worms known as
    • Brugia malayi
    • Brugia timori
    • Wuchereria bancrofti
  • Cause damage and inflammation to the lymphatic system
  • Introduced into the human body through the bite of infected mosquitoes
  • Certain drugs are used to kill filarial larvae and worms [3]

Sekund. lymfedem

  • Manifestace od překážky distálně


Traumatické iatrogenní

  • CVO, varixy, OP. štěpy, TEP, popáleniny, frct., OP kloubů, obezity, polymorbidita
O úroveň výše

Poslední aktualizace: 6. 10. 2024 23:26:09