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.
CONFLICT OF INTEREST
The authors of this manuscript certify that they have no affiliations
with or involvement in any organization or entity with any financial
interest (such as honoraria; educational grants; participation in
speakers’ bureaus; membership, employment, consultancies, stock
ownership, or other equity interest; and expert testimony or
patent-licensing arrangements) or nonfinancial interest (such as
personal or professional relationships, affiliations, knowledge or
beliefs) in the subject matter or materials discussed in this
manuscript.
AUTHOR CONTRIBUTIONS
All persons who meet authorship criteria are listed as authors, and all
authors certify that they have participated sufficiently in the work to
take public responsibility for the content, including participation in
the concept, design, analysis, writing, or revision of the manuscript.
CONSENT TO PARTICIPATE
The authors of this manuscript certify that informed consent was
obtained and signed from the patient’s parent/legal guardian to
participate in this case report for submission to the journal.
CONSENT TO PUBLISH
The authors of this manuscript certify that informed consent was
obtained and signed from the participant’s parent/legal guardian
regarding publishing their data and photographs.
REFERENCES
- HH K, KK M. Capillary hemangioma with extensive purpura: Report of a
case. Am J Dis Child. 1940 May 1;59(5):1063–70.
- Wang P, Zhou W, Tao L, Zhao N, Chen X-W. Clinical analysis of
kasabach-merritt syndrome in 17 neonates. BMC Pediatr. 2014 Jun
11;14:146.
- Acharya S, Pillai K, Francis A, Criton S, Parvathi VK. Kasabach
merritt syndrome: management with interferon. Indian J Dermatol.
2010;55(3):281–3.
- Drolet BA, Trenor CC, Brandão LR, Chiu YE, Chun RH, Dasgupta R, et al.
Consensus-derived practice standards plan for complicated Kaposiform
hemangioendothelioma. J Pediatr. 2013;163(1):28591.
- Roumen ‐ Klappe EM, den Heijer M, van Uum SH, et al. Respuesta
inflamatoria en la fase aguda de la trombosis venosa profunda . J Vasc
Surg . 2002; 35 ( 4 ): 701‐706.
- José RJ, Manuel A. Tormenta de citocinas COVID-19: la interacción
entre la inflamación y la coagulación. Lancet Raspir Med. 2020; 8 (6):
e46 ‐ e47.
- Zhou F, Yu T, Du R y col. Curso clínico y factores de riesgo para la
mortalidad de adultos en pacientes con COVID-19 en Wuhan, China: un
estudio de cohorte retrospectivo. Lancet. 2020; 395 (10229):
1054‐1062.
- Afsaneh Sadeghzadeh-Bazargan MD, Mahdi Rezai MD. Skin manifestations
as potential symptoms of diffuse vascular injury in critical COVID-19
patients. J Cutan Pathol. 15 de junio de 2021: 10.1111 / cup.14059.
- Priya Mahajan, Judith Margolin. Kasabach-Merritt Phenomenon: Classic
Presentation and Management Options. Clin Med Insights Blood Disord.
2017 Mar 16; doi:10.1177/1179545X17699849.
- Balraj Singh, Ashesha Mechineni. Acute Intestinal Ischemia in a
Patient with COVID-19 Infection. Korean J Gastroenterol 2020; 76(3):
164-166. https://doi.org/10.4166/ kjg.2020.76.3.164.