1. 評估治療 卡莎巴梅凝血病變的新指標
李建智 陳明庭
國泰綜合醫院
台大醫院

2. 卡莎巴梅凝血病變＝血管瘤＋血小板損耗 Kasabach-merritt syndrome

3. 8 M/O，female baby
Platelet 4000

4. 5 M/O, female baby
PLT 6800

5. 2 M/O, male baby
PLT 4000

6. Case 1：Pathology and IHC stain （H&E, CD31, D2-40）

7. Case 2：Pathology and IHC stain （ H&E, CD31, D2-40）

8. Case 3：Pathology and IHC stain （ H&E, CD31, D2-40）

9. Kasabach & Merritt first reported an infant with giant capillary hemangioma with extensive purpura in 1940
Mortality rate of 30%, higher in non-responders
Two types of vascular tumors
Kaposiform hemangioendotheliomas (KHE)
Tufted angiomas (TA)

10. KHE TA
Rare
a blue-red mass located in the superficial or deep soft tissue plane
Pathology: Irregular nodules growing in an infiltrative fashion
Also rare
pink reddish macules or plaques on the skin
Pathology: Tufts of hypertrophied endothelial cells scattered throughout the whole dermis in a cannonball-like distribution

12. Our results

14. FIGURE 3 . In correspondence to Figure 1A, (A) illustrates the cannonball-like aggregates of vessels within the dermis typical of tufted angioma histopathologically; (B) demonstrates fat cell deposition between endothelial cells during tumor involution, clinically corresponding to child in Figure 1D.

15. Systemic corticosteroids
Treatment
Systemic corticosteroids
Vincristine: neurotoxin & peripheral neuropathy
Interferon alpha: thrombocytopenia, spastic diplegia
Excision of the vascular tumor: bleeding and complex anatomical locations
Embolization
Radiation therapy
Topical steroid injection
Sirolimus
Kim T, Roh MR, Cho S et al. Kasabach-Merritt syndrome arising from tufted angioma successfully treated with systemic corticosteroid. Ann Dermatol 2012;22:
Enjolras O, Riche MC, Merland JJ, et al. Management of alarming hemangiomas in infancy: a review of 25 cases. Pediatrics 1990; 85: 491-8

16. Study design and sample
Ante grade data collection
Pathology proved hemangioma with Kasabach-Merritt syndrome
Five cases
Follow-up for 30 months
Take photo, and check CBC + D/C, PT, APTT, fibrinogen, FDP, D-Dimer, every 2 months

17. Photo measurement
Digital photo were analyzed with image analysis software
color change, L*a*b* system
L* : lightness
a* : values from green to red
b*: values from blue to yellow
√(∆L*)2+(∆a*)2+(∆b*)2
N Engl J Med Apr 9;338(15): Effect of the timing of treatment of port-wine stains with the flash-lamp-pumped pulsed-dye laser.

18. L*a*b* data

19. Results

20. Platelet count and color change
Spearman Correlation= 0.550
P= 0.036

21. Fibrinogen and color change
Spearman Correlation= 0.175
P= 0.576

22. Spearman Correlation= -0.7
FDP and color change
Spearman Correlation= -0.7
P= 0.005

23. D-Dimer and color change
Spearman Correlation=
P= 0.013

24. Fibrinogen, FDP, D-Dimer
Liver
Wikipedia

25. Conclusion
FDP and D-Dimer are better indicators than platelet and fibrinogen
Treatment should be maintained until coagulopathy resolved.

26. 謝謝各位的聆聽！