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Table 3 Treatment modalities for idiopathic purpura fulminans from recently reported casesa

From: Empiric treatment of protracted idiopathic purpura fulminans in an infant: a case report and review of the literature

Case report

Patient and history

PS

Trigger

Treatment (target levels)b

Outcome and treatmentc

Boccara et al. [15]

Two-year-old girl

Varicella three months prior

Low

HHV-6

IVIg 2 g/kg, LMWH, plasmapheresis q12 h, FFP (repeat IVIg if PS < 50%)

Pre-treatment: right leg amputation

Post-treatment: resolution, IVIg 2 ×/day

Özbek et al. [9]

Eight-year-old boy

FVL+/-

G20210A+/-

Low

Not known

Prior treatment: SQ UH, IV ceftriaxone, metronidazole

(One) Bolus 75U UH, 15 days continuous 20 U/kg/hour, twice daily FFP × 5 daysb

(Two) 80 to 100 U/kg SQ heparin for 1 month

(Three) Warfarin (INR Two or Three)

Pre-treatment: necrosis in lower half of body

Post-treatment: resolution

al-Ismail et al. [10]

4.8-year-old boy

G20120A+/-

ANA+

ASO+

Viral titer-

Low

Streptococcus?

(One) Antibiotics, FFP, vitamin K

(Two) IV UH

(aPTT 1.5-2.5 × normal)b

(Three) Enoxaparin 20 mg/q12 hours (anti-Xa level 0.6 U/L)

Pre-treatment: left leg swelling, edema, left femoral vein thrombosis

Post-treatment: resolution

Levin et al.d [7]

6.4-year-old boy

Varicella

One year prior

Low

Fever, vesicular rash

(One) IV penicillin, cefotaxime, 4U FFP, IVIg 1 g/kg, exchange transfusion, methylprednisone, IV heparin 15 U/kg/hour, tPa for PE, prostacyclin

(Two) IV heparin increased to 60 U/kg/hour, FFP 40 mL/kg/day (aPTT 1.5 to 2.5 × normal)b

(Three) Warfarin

Pre-treatment: lesions stopped, right leg amputation, PE1

Post-treatment: then respiratory improvement, resolution

 

5.9-year-old girl

Low

Varicella

(One) IV cefotaxime, acyclovir and heparin bolus 100 U/kg/hour, then 25 U/kg/hour; two volume exchange transfusion, then daily FFP

Pre-treatment: right atrial thrombosis, PEe

Post-treatment: skin grafting

  1. aPS, protein S; INR, international normalized ratio; HHV-6, human herpesvirus 6; IVIg, intravenous immunoglobulin; LMWH, low-molecular-weight heparin; FFP, fresh frozen plasma; PS, protein S; FVL, factor V Leiden; G20210A, prothrombin G20210A mutation; SQ, subcutaneous; UH, unfractionated heparin; IV, intravenous; ANA, anti-nuclear antibody; ASO, anti-streptolysin O antibody; aPTT, activated partial thromboplastin time; tPa, tissue plasminogen activator; PE, pulmonary embolism; +/-, heterozygote; +, antibodies present; bmost effective treatment modality in each case when more than one treatment was applied; cpre- and post-treatment are with respect to most effective treatment in "Treatment (target levels)" column; dLevin et al. [7] case series comprised five cases, but only two had sufficient clinical information both of which are shown here; eboth PEs occurred after initiation of effective treatment but before resolution, which occurred after indicated treatments.