Kasabach-Merritt Syndrome with Profound Platelet Support

Abstract

The Kasabach-Merritt Syndrome describes thrombocytopenia occurring in patients with giant hemangiomata. The resultant thrombocytopenia may be profound and occasionally even life-threatening. An 11-month-old infant with prolonged thrombocytopenia whose course was complicated by recurrent hemorrhaging requiring intense platelet transfusions is reported. During her 19-month hospitalization she received 6,622 platelet concentrates. This represents the most extensive platelet support ever given to an infant with this syndrome.

Features of the community–acquired pneumonia in a child with Kasabach-Merritt syndrome: case-report and mini-review

“…As many researchers consider, the transfusion of plasma and platelets is the basis of the Kasabach-Merritt syndrome treatment 25 . Blood transfusion should be retained if there is no evidence of bleeding, since cytokines from the transfusive blood can aggravate the angiogenic process and lead to increase in hemangioma's size 21 .…”

Kasabach–Merritt phenomenon in an adult man with a tufted angioma and cirrhosis responding to radiation, bevacizumab, and prednisone

“…One hypothesis is that unique architectural abnormalities, turbulent flow, and shear stress in proliferating endothelium of TAs and KHEs favors adhesion of platelets, with their subsequent aggregation and activation . Once platelets are activated, secondary consumption of fibrinogen and coagulation factors occurs, followed by fibrinolysis . KMP usually occurs while the vascular tumor is actively proliferating.…”

“…Because severe hemorrhage is infrequent despite severe thrombocytopenia, it is recommended to refrain from giving platelets unless the patient is actively bleeding or needs surgery . Additional rationale for withholding platelet transfusion in KMP patients is two‐fold: (i) transfused platelets tend to be rapidly consumed by the vascular tumor (half‐life 1–24 h) and do not seem to ever correct the consumptive coagulopathy, and (ii) platelet transfusions are often associated with coincident rapid expansion of the vascular tumor, suggesting infused platelets trapped within the tumor release pro‐angiogenic molecules that stimulate tumoral proliferation and further exacerbate KMP …”

Kasabach–Merritt Syndrome: Identification of Platelet Trapping in a Tufted Angioma by Immunohistochemistry Technique Using Monoclonal Antibody to CD61

“…Many authors have reported various treatment modalities, such as corticosteroid (11), platelet replacement (12), irradiation (13), and interferon‐α(4,14), but the results have been inconsistent. Thus the choice of treatment modality should be individualized on the basis of the size and location of the lesion and the severity of the coagulopathy.…”