Kapiller Kaçış Sendromu: Klinik Tecrübemiz ve Literatür İncelenmesi

Yıl 2017, Cilt: 14 Sayı: 1, 46 - 64, 28.04.2017

Hasan Karsen , Emine Ayça Güler Füruzan Kaya İrfan Binici Mehmet Reşat Ceylan

Öz

Amaç: Amacımız, potansiyel olarak fatal ve oldukça nadir görülen Capillaryleaksyndrome (CLS)
tanısı koyduğumuz hastaları literatür eşliğinde tartışarak klinisyenlerin dikkatine sunmaktır.
Materyal ve Metod: Çalışmaya 2010-2016 yılları arasında CLS tanısı alan6 vaka alındı. Hastaların
tanısı, hipotansiyon (sistolik kan basıncı ≤90 mmHg veya mevcut kan basıncının ≥40 mmHg düşmesi),
hemokonsantrasyon, hipoalbuminemi (albumin düzeyinin ≥0.5 gr /dL düşmesi) ve ödem (lokal veya
anazarka tarzında) klinik bulgularıyla konuldu. Hastaların bilgileri, retrospektif hasta dosyaları incelenerek
elde edildi. Bu çalışma,üniversitemiz enfeksiyon hastalıkları kliniği ve yoğun bakım ünitesinde yapıldı.
Bulgular: Hastaların üçü erkek, üçü kadın olup yaşları 22-80 arasındaydı. Hastalardan biri
bruselloz, biri tüberküloz, biri derin ventrombozu ve pulmoneremboli nedeniyle yoğun bakıma alınan, biri
hipoglisemik koma nedeniyle yoğun bakımda takip edilen, biri akut myeloid lösemi (AML) nedeniyle
kemoterapi alan diğeri büyük cerrahi girişim uygulanan hastalardan oluşuyordu. AML hastası hariç diğer
hastalarda birden fazla bakteri superpoze olarak hastaların sepsise girmelerine neden oldu. Hastaların
tümü entübe edildiğinden kan kültürü yanı sıra alınan derin trakealaspiratta da üreme oldu. Bu
enfeksiyonlar, hem CLS gelişmesine hem de mortaliteyle sonuçlanmasına neden oldu. Takip ve tedavi
sonunda bütün çabamıza rağmen hastalarımızın tamamı exitus oldu.
Sonuç: CLS oldukça nadir, mortalitesi hala yüksek, tedavisi hala belirsiz gizemli bir hastalık olarak
kalmaya devam etmektedir. Klinisyenlerin özellikle septik durumlarda hastaların CLS’ye gidişi konusunda
uyanık olmaları gerekir. Zira erken tanı ve tedavi döngüyü hastanın lehine çevirecekbilecektir

Anahtar Kelimeler

Kapiller kaçış sendromu, klinik tecrübe, tanı, tedavi, prognoz

Capillary Leak Syndrome: Our Clinical Experience and Literature Review

Öz

Background: Our objective is to discuss the cases of patients diagnosed with capillary leak
syndrome (CLS) that is a potentially fatal and extremely rare condition, in the light of literature, and present
them to clinicians’ attention.
Material and Method: Six cases diagnosed with CLS between 2010 and 2016 were included in
the study. The patients were diagnosed with clinical findings including hypotension (systolic blood pressure
≤90 mmHg, or a decline of ≥40 mmHg in immediate blood pressure), hemoconcentration,
hypoalbuminemia (a decrease of ≥0.5 gr /dL in albumin level) and edema (local or anasarca). Information
about the patients was elicited through the analysis of patient files retrospectively. The present study was
carried out at the Infectious Diseases Clinic and Intensive Care Unit of Harran University Hospital.
Results: Of the six patients, three were males and three were females, and their ages ranged
between 22-80. The patients’ diagnoses were one patient brucellosis and one patient tuberculosis, one
patient monitored in intensive care unit because of deep vein thrombosis along with pulmonary embolism
and the other monitored due to hypoglycemic coma, one patient who received chemotherapy due to acute
myeloid leukemia (AML), and the other one who have undergone a critical surgical intervention. Except
for the patient with AML, multiple bacterial species became superposed in the patients and caused them
to progress into sepsis. As all patients were intubated, culture growth was detected in deep tracheal
aspirates apart from blood culture. These infections gave rise to both the development of CLS and
increased mortality. All of the patients died, despite our all efforts of treatment and follow-up interventions
Conclusion: CLS remains to be a mysterious illness which is extremely rare. It still has high
mortality and does not have a certain treatment. Clinician needs to be alert about the patients’ progress to
CLS, partic

Anahtar Kelimeler

Capillary leak syndrome, clinical experience, diagnosis, treatment, prognosis

Kaynakça

• 1. Clarkson B, Thompson D, Horwith M andLuckey EH. Cyclicaledemaandshockduetoincrea sedcapillarypermeability.TheAmeric anJournal of Medicine. 1960; 29:193–2. 2. Guffroy A, Dervieux B, Gravier S, Martinez C, Deibener-Kaminsky J et al: Systemic capillaryleaksyndrome and autoimmunediseases: A caseseries.Semin ArthritisRheum. 2016;172:30108- 111. 3. Duron L, Delestre F, Amoura Zand Arnaud L. Idiopathicandsecondary capillarylea ksyndromes: A systematicreview of theliterature. RevMedInterne 2015; 36:386-394. [Article in French] 4. Stein DM, Scalea TM: Capillaryleaksyndrome in trauma: what is it andwhataretheconsequences? AdvSurg 2012; 46:237-53. 5. Xie Z, Chan E, Yin Y, Ghosh CC, Wisch L, Nelson C et al: InflammatoryMarkers of theSystemic CapillaryLeakSyndrom e.J Clin Cell Immunol 2014; 5:1000213. 6. McCann S, Akilov OE, Geskin L: Adverseeffects of denileukindiftitoxandtheirmanageme nt in patientswithcutaneous Tcelllymphoma. Clin J OncolNurs 2012; 16:164–172. 7. Dagdemir A, Albayrak D, Dilber C, Totan M. G-CSF relatedcapillaryleaksyndrome in a childwithleukemia. LeukLymphoma 2001; 42:1445–1447. 8. Hsiao SC, Wang MC, Chang H, Pei SN. Recurrentcapillaryleaksyndromefollo wingbortezomibtherapy in a patientwithrelapsedmyeloma. AnnPharmacother 2010; 44:587– 589. 9. Sousa A, Len O, Escolà-Vergé L, Magnifico B, Mora C, Papiol E. Influenza A virusinfection is associatedwith systemiccapillarylea ksyndrome: casereportandsystematic review of theliterature.AntivirTher 2016; 2:181-183. 10. Xie Z, Nagarajan V, Sturdevant DE, Iwaki S, Chan E, Wisch L. Genome-wide SNP analysis of thesystemiccapillaryleaksyndrome. RareDis 2013; 1: e27445. 11. Xie Z, Chan E, Yin Y, Ghosh CC, Wisch L, Nelson C. InflammatoryMarkers of theSystemicCapillaryLeakSyndrome . J Clin Cell Immunol 2014; 5:1000213. 12. Kirk MD and Philip RG. NarrativeReview: TheSystemicCapillaryLeakSyndrom e. AnnInternMed2010; 153:90-98. 13. Gousseff M, Amoura Z: [Idiopathiccapillaryleaksyndrome]. RevMedInterne 2009; 30:754-768. [Article in French] 14. Kyeong WK, Sang TH, Sang HH, et al: Systemiccapillaryleaksyndromeindu cedbyinfluenzatype A infection. ClinExpEmergMed 2014; 1:126– 129. 15. Marra AM, Gigante A, Rosato E: Intravenousimmunoglobulin in systemiccapillaryleaksyndrome: a casereportand review of literature. ExpertRevClinImmunol 2014; 10:349-352. 16. Lambert M, Launay D, Hachulla E, Morell-Dubois S, Soland V, Queyrel V. Highdoseintravenousimmunoglobulinsdr amaticallyreversesystemiccapillaryle aksyndrome. CrilCareMed 2008; 36: 2184–2187. 17. Pecker M, Adams M, Graham W: TheSystemicCapillaryLeakSyndrom e: Comment. Annals of InternalMedicine 2011; 155: 335. 18. Erkurt MA, Sari I, Gül HC, Coskun O, Eyigün CP, Beyan C. Thefirstdocumentedcase of brucellosismanifestedwithpancytope niaandcapillaryleaksyndrome. InternMed 2008; 47:863-865. 19. Dilek I, Durmuş A, Karahocagil M.K, Akdeniz H, Karsen H, Baran AI et al: ematologicalcomplications in 787 cases of acutebrucellosis in easternTurkey. Turk J MedSci 2008;38:421–424. 20. Zhang F, Yang J andLi Z: Trastuzumabinduced systemic capillary leak synd rome in a breast cancer patient. PatholOncolRes 2014; 20:435-437. 21. Pothen L, Rouvière H, Poncin R, Michaux L, Damoiseau P, Lambert M. Systemiccapillaryleaksyndromereve aling a diffuselarge B-celllymphoma. ActaClinBelg 2014; 69:305-308. 22. Durand B.M, Rouget A, Recher C, Azoulay E, Bounes V. A SystemicCapillaryLeakSyndrome i n a PatientwithChronicLymphocytic Leu kemia: A Case Report in an Out-ofHospitalSetting. Case RepEmergMed 2016; 2016:5347039. 23. Anderson BJ, Peterson LL: Systemic capillaryleaksyndrome in a patientreceivingdjuvantoxaliplatinforl ocallyadvancedcoloncancer.J OncolPharmPract 2016;22:725-8. 24. Ozawa T, Yamaguchi H, Kiyomatsu T, Saito S, Ishihara S, Sunami E et al: A casereport of idiopathic systemiccapillaryleaksynd rome thatoccurredduringthepostoper ativeperiod of abdominoperinealresectionforcolore ctalcancer. IntSurg 2015; 100:58-62. 25. Singer M, Deutschman CS, Seymour CW, Shankar-Hari M, Annane D, Bauer M et al: The Third International ConsensusDefinitionsforSepsisandS epticShock (Sepsis-3). JAMA 2016; 315:801–810.