| Abstract | Kronični oblik reakcije presatka protiv primatelja (eng. graft versus host disease,
GVHD) je kasna komplikacija koja se javlja nakon transplantacije alogeničnih krvotvornih
matičnih stanica (eng.hematopoetic stem-cell transplantation, HSCT) uz zahvaćanje više
tkiva i organa s varirajućom težinom i tijekom kliničke slike. Neurološke promjene kroničnog
GVHD-a su rijetke i mogu zahvatiti centralni i periferni živčani sustav te se obično javljaju
nekoliko mjeseci do godina nakon HSCT. Kod ovih bolesnika je često prisutna kronična bol,
nociceptivnog,visceralnog ili neuropatskog karaktera i svojom pojavnošću može značajno
utjecati na kvalitetu života i funkcioniranje bolesnika.
Kliničko prospektivno istraživanje provedeno je na 15 bolesnika (raspon godina 20-73) s dijagnozom kroničnog GVHD-a u sklopu dijagnostičke obrade i sumnje na neuropatiju
tankih vlakana (NTV). Svakom bolesniku je učinjen kompletan neurološki pregled,
elektroneurografija (ENG), kvantitativno senzorno testiranje (KST), ispunjen PainDETECT
upitnik i ocijenjen totalni neuropatski skor (TNS).
Prema nalazu KST-a je 11 bolesnika imalo oštećenu funkciju Aδ vlakana, a 3
bolesnika oštećenu funkciju i Aδ i C vlakana. Za 7 bolesnika se pokazalo da imaju izoliranu
neuropatiju tankih vlakana, 7 je imalo neuropatiju i tankih i debelih vlakana, a samo 1
bolesnik izoliranu neuropatiju debelih vlakana. Prema PainDETECT upitniku moguća
prisutnost neuropatske boli klasificirana je kao pozitivna kod 4 bolesnika, za 3 bolesnika je
klasificirana kao nejasna, a 8 bolesnika je imalo negativan rezultat. Najčešća hematološka
dijagnoza je bila akutna mijeloična leukemija (AML), kod ukupno 6 bolesnika. Prema nalazu
neurološkog pregleda, mišićna atrofija je bila prisutna kod 4 bolesnika, mišićni grčevi su bili
prisutni u anamnezi kod 5 bolesnika, parestezije kod 9 bolesnika a oštećen osjet vibracije
(≤4/8) je imalo 3 bolesnika. Srednja ocjena TNS-a kod bolesnika je bila 6,60 bodova.
Neurološke manifestacije kroničnog GVHD-a su rijetke ali mogu imati velik utjecaj na
tijek bolesti, kvalitetu života i postotak preživljenja kod ovih bolesnika. Upravo je rano
prepoznavanje neuroloških komplikacija korištenjem ENG-a, KST-a, PainDETECT upitnika
ključno za početak adekvatnog liječenja. |
| Abstract (english) | Chronic graft-versus-host disease (cGVHD) is a multisystemic disorder with variable
severity and clinical course, occuring as a late complication after allogeneic hematopoetic
stem cell transplantation. Neurological manifestations are rare and can affect central or
peripheral nervous system. Patients with cGVHD often experience chronic pain, which can
have nociceptive, visceral or neuropathic character and have significant negative impact on
quality of life.
This clinical prospective study was conducted on 15 patients (year range 20-73) with
cGVHD who complained of symptoms suggestive of peripheral nervous system
complications and small fibre affection. Each patient underwent neurological examination,
nerve conduction studies (NCS), quantitative sensory testing (QST), filled painDETECT
questionnaire and had total neuropathy score (TNS) graded.
According to QST results, 11 patients had Aδ fibers affected and 3 patients had both
Aδ and C fibers affected. For 7 patients neruophisiology revealed isolated small fibre
polineuropathy, another 7 patients had mixed fibre neuropathy and only 1 patient had
predominantly large fibre polyneuropathy. Based on painDETECT questionnaire, neuropathic
pain was determined as possible in 4 patients, indistinct in 3 patients and negative in 8
patients. The most common underlying hematological malignancie was acute myeloid
leukaemia (AML) in 6 patients. Muscle atrophy was found in 4 patients, muscle cramps in 5
patients, paresthesia in 9 patients and vibration sense was impaired (≤4/8) in 3 patients.
Averege total neuropathy score was 6,60 points.
Neurological manifestations of cGVHD are rare, but often disabling and can have a
great impact on disesase course and quality of life. Early diagnosis of potential neurologic
complications using NCS, QST and different screening tools is essential for establishing
appropriate and effective therapy. |
