PMC5428202_01
Classic clinical case of Kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon (KMP) diagnosed in a 5-week-old patient. Diagnosis, response to blood product transfusion, and resolution of KMP with sirolimus + steroids therapy. (A) Platelet counts (normal: 150-450 x 103/microL), (B) hemoglobin (normal: 9.5-13.5 g/dL), (C) fibrinogen (normal: 220-440 mg/dL), and (D) d-dimer (normal: <0.4 microg/mL).
10.1177_1179545X17699849-fig1.jpg
PMC5428202_01_10.1177_1179545X17699849-fig1.jpg
fig1-1179545X17699849
To illustrate the presentation and management of KMP, we report 1 patient diagnosed at 5 weeks of age with KMP associated with KHE (Figures 1 and 2). Within a few days after initial presentation, KMP was evident (Figure 1). The platelet count improved immediately after transfusion (Figure 1) and the patient tolerated the procedure well with no complications. Hemoglobin improved from 6.8 to 10 g/dL; however, within 4 days, it was again low at 7.4 g/dL (Figure 1). The KMP stabilized within a couple of days, and prior to completion of 5 days of therapy, thrombocytopenia, hemoglobin, and fibrinogen significantly improved (Figure 1). Figure 1 illustrates the first month of therapy to demonstrate the classic effect of therapy on KHE and KMP.
Classic clinical case of Kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon (KMP) diagnosed in a 5-week-old patient. Extension of cutaneous involvement. Photos presented with mother's permission. (A) At the peak of activity of KMP (day 4):patient with purpura, edema, irregular margins, and extensive size. (B) After 3 weeks of treatment, the cutaneous component improved significantly. (C) After 2 months of therapy with sirolimus and steroids, complete resolution of the cutaneous component was achieved.
10.1177_1179545X17699849-fig2.jpg
PMC5428202_01_10.1177_1179545X17699849-fig2.jpg
fig2-1179545X17699849
To illustrate the presentation and management of KMP, we report 1 patient diagnosed at 5 weeks of age with KMP associated with KHE (Figures 1 and 2). The periorbital and periauricular edema on the affected side was prominent (Figure 2A), and the patient had significant pain. After 3 weeks of treatment, the cutaneous component improved significantly (Figure 2A) and a steroid wean was initiated. The cutaneous component of the patient's KHE has now resolved as well (Figure 2C).