2025 – PAGE 293 – HEMATOLOGY & ONCOLOGY

(DOUBLE TAKE) KASABACH-MERRITT SYNDROME

In Kasabach-Merritt syndrome, there are large, congenital vascular tumors. They are not true hemangiomas but can cause a severe CONSUMPTIVE COAGULOPATHY (in the form of thrombocytopenia and the consumption of coagulation factors) and death. It is most common in infants.

IMAGE: www.pbrlinks.com/KASABACH1
IMAGE: www.pbrlinks.com/KASABACH2

PEARL: Look at the above images closely. Make sure you look closely at images so that you do not get this vascular tumor confused with hemihypertrophy.

MNEMONIC:

• >—< is used by many of us when recording CBC results.

The name alert is for: KLIPPEL-TRENAUNAY SYNDROME, with nevus flammeus, vascular malformations, and overgrowth of the affected limb.

GLANZMANN THROMBASTHENIA

For Glanzmann thrombasthenia, look for unexplained bleeding in a patient with a normal platelet count, normal PTT, and normal INR. The bleeding is from dysfunctional platelets.

BERNARD-SOULIER SYNDROME

In Bernard-Soulier syndrome, there is mild thrombocytopenia + LARGE platelets + Prolonged Bleeding Time.

MNEMONIC: Platelets are LARGE, like a Saint Bernard.