Outcome in children with purpura fulminans: Report on 16 patients

Abstract

Purpura fulminans (PF) is a severe disorder of acute onset with high morbidity and mortality. In children, this rapidly progressive illness is usually associated with severe bacterial or viral infections. However, some other conditions may participate in the development of PF. Our objective was to investigate the underlying and associated disorders and the outcomes of the disease in 16 children, 7 males and 9 females ranging in age from 3.5 months to 12 years (median age, 2 years). Thirteen of the 16 children (81%) were 4 years of age or younger. The remaining 3 patients were 9, 10, and 12 years of age. Among these 13 infants and small children, 7 (43%) had infection, 2 infants had congenital cardiac disorders necessitating minor or major surgical intervention, and 1 infant and 3 children had different miscellaneous disorders. The factor V G1691A mutation was present in six of the 13 small children (46%). None of the 3 older children carried the mutation. Six (37.5%) of the 16 patients had protein C deficiencies, and 9 (56%) had protein S deficiencies. These deficiencies, except one for protein S, were acquired. Ten patients except two who were diagnosed at this center were treated with fresh frozen plasma. They were also given heparin. Nine (69%) of the 13 children 4 years of age or younger and one of the older children (33%) required amputation. Five of the six patients (83%) who had factor V G1691A mutation, and who also exhibited severe infection, required amputation. This study suggests that an age of 4 years or less is a risk factor for the development of PF during severe infections, especially in the presence of factor V G1691A mutation and congenital heart disease, necessitating major or minor surgical interventions. This study also shows that the amputation rate in 10 patients, after excluding the patients who had been referred to our center after development of sequelae, was 60%. The survival rate among these 10 patients may indicate that, with the treatment protocol, PF need not be regarded as a lethal disease any more. It is also suggested that effective immunization programs and better health care have probably resulted in some changes in the etiological profile of PF. Am. J. Hematol. 80:20–25, 2005. © 2005 Wiley‐Liss, Inc.

Novel management of post varicella purpura fulminans owing to severe acquired protein S deficiency

“…In many cases a combination of all of the above are used. These patients are severely prothrombotic and so the mainstay of treatment is prompt anticoagulation with therapeutic doses of heparin (either unfractionated or low molecular weight) as soon as the consumptive coagulopathy has been corrected, in order to reduce the risk of limb and/or life-threatening thrombosis [12]. In our patient, the deficiency of protein S was counteracted by aiming for treatment levels of i.v.…”

Human protein C concentrate in the treatment of purpura fulminans: a retrospective analysis of safety and outcome in 94 pediatric patients

“…Although improvement in health care delivery for these very sick patients has dramatically improved survival in recent years [11,12], permanent disability as a result of amputation of limbs or digits, extensive scarring, or neurological injury remains problematic. Gurgey and colleagues reported a series of 16 children; nine (69%) of the 13 children aged 4 years or younger and one of the older children (age range 9 - 12 years) (33%) required amputation [13]. A case series by Wheeler and colleagues on 21 patients reported amputations in nine (43%) patients [14].…”

Purpura fulminans in a newborn infant with galactosemia

“…Dysfunction of the protein C activation pathway appears to be critical to the development of thrombosis in PF [5,6,11]. The other prothrombotic risk factors including factor V Leiden, prothrombin G20210A mutation, MTFHR C677T mutation, homocysteine, and antiphospholipid antibody may also play a role in development of PF [7]. Although bleeding diathesis is a well-known presenting sign of galactosemia PF in which thrombosis is predominant rather than bleeding, as seen in our patient, has to our knowledge not been described.…”