2017, Number 5
<< Back Next >>
Rev Fac Med UNAM 2017; 60 (5)
Treatment for neonatal fulminans purpura. Case report and literature review
Cruz HV, Escárraga VYS, León TS, Cruz ADA, Majluf CAS
Language: Spanish
References: 19
Page: 17-21
PDF size: 580.50 Kb.
ABSTRACT
Neonatal Purpura Fulminans (PF) is an infrequent hypercoagulable
state but very severe. Its clinical manifestation is
sudden with purpuric-necrotic injuries. It can leave permanent
sequels or even have a fatal evolution. It is characterized
by thrombosis in the skin’s microcirculation, accompanied
with perivascular hemorrhage. The most affected areas are
the pelvic and thoracic limbs, and pressure zones. The most
common molecular alteration is the protein C defect, which
physiologically regulates coagulation; the defect can be of
a primary or secondary cause.
Case report: A male newborn with 8 days of extrauterine life
suddenly presents oral rejection, irritability and a 39
º C fever.
Two days later, he was hospitalized for dehydration and oral
rejection. He didn’t show signs of fever or infection at the
time of his admission. Twenty-four hours after his entry, he
presented purpuric-necrotic injuries in the right foot, hence,
he was diagnosed with purpura fulminans. D-dimer studies
and doppler ultrasound were taken. They confirmed venous
and arterial thrombosis. The treatment was initiated with
fresh frozen plasma, an anticoagulant and an antiplatelet,
with a good response.
Conclusion: PF is a serious hypercoagulable state that requires
an early diagnosis and therapy to improve the outcome.
REFERENCES
Spicer Te, Rau Jm. Purpura fulminans. Am J Med. 1976; 61:566-71.
Darmstadt GL. Acute infectious purpura fulminans: Pathogenesis and medical management. Pediatr Dermatol. 1998; 15:169-83.
Dahlback B, Carisson M, Svensson PJ. Familial thrombophilia due to a previously unrecognized mechanism characterized by poor anticoagulant response to activated protein C: prediction of a cofactor to activated protein C. Proc Natl Acad Sci USA. 1993;90:1004-8.
Ruiz-Arguelles GJ, González-Estrada S, Garces-Eisele J. Primary trombophilia in Mexico: a prospective study. Am J Hematol. 1999;60:1-5.
Baur A, Pouyau R, Meunier S, Nougier C, Teyssedre S, Javouhey E, et al. Varicella-associated purpura fulminans and deep vein thrombosis: a pediatric case report. Arch Pediatr. 2011 Jul;18(7):783-6.
John H Griffin, Berislav V Zlokovic, and Laurent O MosnierInt J. Protein C anticoagulant and cytoprotective pathways Hematol. Apr 2012;95(4):333-45.
Andrew M. Developmental haemostasis: relevance to thrombo- embolic complications in paediatric patients. Thromb Haemostas. 1995;74:415-25.
Lau KK, Stoffman JM, Williams S, McCusker P, Brandao L, Patel S, et al; Canadian Pediatric Thrombosis and Hemostasis Net- work. Neonatal renal vein thrombosis: review of the English-language literature bet- ween 1992 and 2006. Pediatrics. 2007;120(5):1278-84.
Schmidt B, Ansdrew M. Neonatal thrombosis: report of a prospective Canadian and International registry. Pediatrics. 1995;96:939-43.
Hagstrom JN, Walter J, Bluebond-Langner R, Amatniek JC, Manno CS, High KA. Prevalence of the factor V Leiden mutation in children and neonates with thromboembolic disease. J Paediatr.1998:133:777-81.
Pereira JG. Trombofilia y trombosis arterial. Rev Chil Cardiol 2007;26:97-103.
Reitsma PH, Bernardi F, Doig RG, Gandrille S, Greengard JS, Ireland H, et al. Protein C deficiency: a database of mutations, 1995 update. Thromb Haemost. 1995;73:876.
Elf JL, Strandberg K, Nilsson C, Svensson PJ. Clinical probability assessment and D-dimer determination in patients with suspected deep vein thrombosis, a prospective multicenter management study. Thromb Res. 2009;123(4):612- 616.
Cogo A, Lensing AW, Koopman MM, Piovella F, Siragusa S, Wells PS, et al. Compression ultrasonography for diagnostic management of patients with clinically suspected deep vein thrombosis: prospective cohort study. BMJ. 1998;316(7124):17-20.
Price VE, Ledingham DL, Krümpel A, Chan AK. Diagnosis and management of neonatal purpura fulminans. Semin Fetal Neonatal Med. 2011 Dec;16(6):318-22.
De Kort EH, Vrancken SL, van Heijst AF, Binkhorst M, Cuppen MP, Brons PP. Long-term Subcutaneous Protein C Replacement in Neonatal Severe Protein C Deficiency. Pediatrics. 2011;127; 1338.
Li JS, Yow E, Berezny KY, Bokesch PM, Taka-hashi M, Graham TP Jr, et al; PICOLO Investigators. Dosing of clopidogrel for platelet inhibition in infants and young children: primary results of the Platelet Inhibition in Children On clopidogrel (PICOLO) trial. Circulation. 2008;117(4):553-9. Epub 2008 Jan 14.
Fruchtman Y, Strauss T, Rubinstein M, Ben Harush M, Revel-Vilk S, Kapelushmik J, et al. Skin Necrosis and Purpura Fulminans in Children With and Without Thrombophilia- A Tertiary Center’s Experience. Pediatr Hematol Oncol. 2015;32(7):505-10.
Bacciedoni V, Attie M, Donato H; (Comité Nacional de Hematología, Oncología y Medicina Transfusional). Thrombosis in newborn infants. Arch Argent Pediatr. 2016 Apr;114(2).
Full text