Kasabach-Merritt syndrome in two successive pregnancies

A 20-year-old Indian Hindu woman, a primigravida at 8 months of pregnancy, presented with symptoms of progressively worsening dyspnea and anasarca of 1 month duration. Physical examination showed signs of severe congestive cardiac failure. She had multiple hemangiomas, 5 cm in size, located over the right temporal region, right side of the chin, right side of the nose, left upper part of the abdominal wall, and anterior aspect of the left leg ( Fig. 1). These hemangiomas had been present since birth and had enlarged considerably during pregnancy. The clinical diagnosis was pregnancy—34 weeks—with multiple hemangiomas and congestive cardiac failure. Figure 1. Hemangiomas Laboratory investigations revealed hemoglobin (Hb) 11 g/dL, total leucocyte count 7000/mm3, differential count P 60 L 40, random blood sugar 110 mg/dL, blood urea (BU) 34 mg/dL, creatinine 0.9 mg/dL, and serum electrolytes: Na, 130 mEq/L; K, 4 mEq/L; Cl, 110 mEq/L. Electrocardiogram (ECG) was normal. Chest X-ray showed cardiomegaly with congestion. Echocardiogram (ECHO) showed enlarged right atrium and right ventricle. The patient was refractory to antifailure therapy and went into labor at the end of 35 weeks of pregnancy and delivered a 1.3 kg male baby. The patient developed post partum hemorrhage and was treated with blood transfusion. During labor, the patient developed purpuric eruption and epistaxis. At that time, the bleeding time was more than 15 min and the platelet count was 46,000. At this juncture, a diagnosis of Kasabach–Merritt syndrome was considered. Skin biopsy revealed hemangiomatous changes. After delivery, the patient’s edema and dyspnea decreased considerably and the eruption disappeared. The hemangiomas regressed in size. A platelet count performed 3 days after delivery was 120,000/mm3. The bleeding time was 7 min, and a chest X-ray showed the reduction of heart size to normal. ECHO repeated 1 week after delivery showed normal-sized chambers. Follow-up as an outpatient showed the patient returning to normalcy. She had a similar presentation during her second pregnancy, 1 year later.