SARS-CoV-2 precipitated Kasabach-Merritt syndrome in a child with Kaposiform Hemangiendothelioma and acute lymphoblastic leukemia
To the editor: The association between giant hemangioma and thrombocytopenia was described by Haig Kasabach and Katharine Merritt in 1940 [1]. Kasabach Merritt syndrome is usually a rapidly growing vascular anomaly causing consuming coagulopathy, thrombocytopenia, hypofibrinogenemia, D-dimer, microangiopathic hemolytic anemia, prolonged thromboplastin and thromboplastin time; this syndrome is 90% associated with Kaposi’s hemangioendothelioma [2,3]. HEK has an unpredictable course and with visceral infiltration are considered extensive and unresectable lesions, so they have a poor prognosis [4]. We describe an 8-month-old boy with Kasabach Merritt syndrome due to Kaposi’s hemangioendothelioma precipitated by COVID-19 infection. According to the mother, 5 days ago he presented fever, rhinorrhea and describes a violaceous lesion in the right dorsal region. RNA testing for SARS coronavirus 2 was performed on a respiratory specimen by RT-PCR and was positive. A 6 x 7 cm diameter, indurated, purplish-red lesion with superficial telangiectasias was observed [Figure 1]. Thoracic angiography showed vascular pedicle in the dorsal trajectory related to vascular structures branches of the intercostal 11 and 12 without organ involvement [Figure 2]. Blood tests on the fifth day show severe thrombocytopenia 7000/mm3, leukopenia 1740/mm3, and severe neutropenia 130/mm3, positive C-reactive protein 24.9 mg/dl and hypoalbuminemia 2.89 g/dl, elevated prothrombin time 103.48 sec, INR 11. 45, d-dimer 15.4 ug/ml, partial thromboplastin time and uncoagulable fibrinogen, ferritin 561.3 ng/ml, receiving platelet transfusions, cryoprecipitate, propanolol and methylprednisolone. After 2 weeks he presented vomiting, abdominal distension, painful abdomen on palpation, with suspicion of intestinal obstruction and underwent surgery, showing abdominal perforation and ending with functional ileostomy. The biopsy of the intestinal segment describes hemorrhagic infarction lesions with foci of ischemic necrosis, without evidence of thrombi.
The mechanisms of vascular injury in COVID-19 appear to be related to inflammation, endothelial injury and thrombosis [5]. Above all, increased immune responses and cytokine storms cause thrombotic disorders in patients with severe COVID-19, with high levels of d-dimer being found in most patients with COVID-19 [6 - 7]. Acute lymphoblastic leukemia and SARS-CoV-2 infection may be a precipitating factor for vascular complications (Kasabach-Merritt phenomenon) and coagulopathies at the intestinal level.
It should be reported that the observed vascular cutaneous manifestations, should also pay attention in predicting the prognosis of patients with COVID - 19 [8]. Treatment of KM is supportive and prevention of life-threatening complications while addressing the underlying tumor [9]. This condition caused by COVID-19 focuses on gastrointestinal decompression, fluid resuscitation, hemodynamic support, surgical resection of necrotic bowel, and restoration of blood flow to the ischemic bowel [10].
SARS-CoV-2 in children with cancer can precipitate the formation of giant vascular lesions with Kasabach-Merritt phenomenon and the coagulopathy caused requires evaluation of all organs at risk for thrombosis.
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