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

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

8 M/O，female baby Platelet 4000

5 M/O, female baby PLT 6800

2 M/O, male baby PLT 4000

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

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

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

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)

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

Our results

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.

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: 426-30 Enjolras O, Riche MC, Merland JJ, et al. Management of alarming hemangiomas in infancy: a review of 25 cases. Pediatrics 1990; 85: 491-8

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

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. 1998 Apr 9;338(15):1028-33.Effect of the timing of treatment of port-wine stains with the flash-lamp-pumped pulsed-dye laser.

L*a*b* data

Results

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

Fibrinogen and color change Spearman Correlation= 0.175 P= 0.576

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

D-Dimer and color change Spearman Correlation= -0.632 P= 0.013

Fibrinogen, FDP, D-Dimer Liver Wikipedia

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

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