Question 1 · Hard
A 4-month-old girl has a firm, purple mass on her shoulder and chest wall that has grown quickly over 2 weeks and is now tense and bruised. She has no fever, and blood cultures are negative. Results: hemoglobin 9.0 g/dL (90 g/L), platelets 12 × 10^3/µL (12 × 10^9/L), fibrinogen 68 mg/dL (0.68 g/L; reference 200-400), PT 15.9 s (reference 11.0-13.5 s), APTT 37 s (reference 25-35 s), D-dimer 9.2 µg/mL FEU (9.2 mg/L FEU; reference below 0.5). The film shows a few schistocytes. Urea and creatinine are normal. What best explains these results?
- ASepsis-induced disseminated intravascular coagulation
- BImmune thrombocytopenia of infancy
- CLocalized consumption in a vascular tumor
- DTypical hemolytic uremic syndrome
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Answer: C · Localized consumption in a vascular tumor
Severe thrombocytopenia, low fibrinogen and a high D-dimer show consumption, and the rapidly growing vascular mass is where it happens: this is the Kasabach-Merritt phenomenon. Platelets are trapped and activated on the abnormal endothelium of a kaposiform hemangioendothelioma or tufted angioma, setting off local clotting and fibrinolysis; common infantile hemangiomas do not do this. Sepsis is unlikely without fever and with negative cultures. ITP and typical HUS do not lower fibrinogen or raise D-dimer this much, and HUS would damage the kidneys. The consumption settles only when the tumor responds to treatment, and platelet transfusions can make the lesion swell.
- A: There is no fever, cultures are negative, and the clotting is centered on the enlarging mass.
- B: ITP lowers platelets only; fibrinogen and D-dimer stay normal.
- D: HUS damages the kidneys, and urea and creatinine are normal; fibrinogen is usually not consumed.