Sex/age | Phenotype/CRIM status | Symptoms | Immune reaction | Premedication | Desensitization: initial concentration | Desensitization: total dose | Desensitization: adverse reactions |
---|---|---|---|---|---|---|---|
M, 11 months [79] | IOPD CP | Urticarial rash | Elevated serum tryptase, activated complement, IgE and eosinophil count normal | Tranexamic acid, deflazacort, cetirizine, ranitidine | 0.5 μg/ml | 10 mg/kg (only for the first infusion) | minimal skin reactions |
M, 4 months [80] | IOPD CP | Anaphylaxis | IgE and eosinophil count normal | Tranexamic acid, deflazacort, cetirizine, ranitidine | 15 μg/ml | Full dose 20 mg/kg EOW | NO |
F, 4 months [82] | IOPD, CRIM ND | Urticarial rash | intradermal test positive, SPT negative | Diphenhydramine, methylprednisolone | 0.1 μg/ml | 20 mg/kg EOW | NO |
M, 3 years 8 months [69] | Juvenile onset PD CP | Anaphylaxis | IgG 1:51200, negative IgE titers, complement activation, normal serum tryptase | Diphenhydramine, acetaminophen | 0.07 μg/ml | 10 mg/kg/week | severe reactions (requiring epinephrine) |
M, 10 months [69] | IOPD CP | Urticarial rash | Elevated IgE and serum tryptase, negative IgG and no complement activation | No | 0.05 μg/ml | 10 mg/kg/weekly | minor skin reaction |
F, 6 years 9 months [74] | IOPD CP | Urticarial rash | SPT negative, IgG negative | Prednisolone | 0.01 μg/ml | 10 mg/kg/weekly | Urticarial rash |
F, 15 months [75] | IOPD CP | Anaphylaxis | IgG 1:6400, normal IgE, tryptase and complement | No | 1:100 | 0.2 mg/kg. The dose was doubled every week | No |
F 7 months [82] | IOPD CP | Urticarial rash, intractable cough and stridor | Intradermal test positive, SPT negative | No | 0.05 μg/ml | 10 mg/kg/week | Urticarial rash |
F 28 y [56] | LOPD | Anaphylaxis | IgE positive, IgG 1600, SPT negative, intradermal testing positive, complement testing negative, elevated tryptase levels | Diphenhydramine and prednisone | 0.1 μg/ml | 10 mg/kg/week | severe reactions (requiring epinephrine) |
F, 46 years [56] | LOPD | Anaphylaxis | SPT and intradermal tests negative, normal IgE, eosinophil counts, complement, tryptase | Antihistamines H1 (cetirizine), antileukotriene (montelukast) | Drop by drop of ERT solution 2 mg/ml with increasing flow + saline solution with continuous flow by another perfusion pocket | Full dose | Minor reaction: anxiety, crying, abdominal pain |