Untreated priapism can lead to ischemic damage of the penis and impotence. This case report describes a 14-year-old boy who presented with a history of priapism for 2 months, which was undiagnosed, ridiculed, and ignored even by medical practitioners. The underlying etiology was later identified to be chronic myeloid leukemia. Despite the usage of multimodal treatment, it took 7 days for control of priapism. The young boy is now left with an erectile dysfunction. The case highlights that priapism in children is a medical emergency needing aggressive evaluation and treatment.
Objective
To analyze clinical and laboratory parameters, and treatment outcomes of children with autoimmune hemolytic anemia (AIHA).
Methods
Retrospective analysis of 50 children aged 0–18 years. Monospecific direct antiglobulin test (DAT) and investigations for secondary causes were performed. Disease status was categorized based on Cerevance criteria.
Results
Median (range) age at diagnosis was 36 (1.5–204) months. AIHA was categorized as cold (IgM+,C3+/cold agglutinin+) (35%), warm (IgG+ with/without C3+) (28%), mixed (IgG+, IgM+, C3+) (15%) and paroxysmal cold hemoglobinuria (4%). Primary AIHA accounted for 64% cases. Treatment modalities included steroid (66%), intravenous immunoglobulin (IVIg) (4%), steroid+IVIg (4%), and steroid+rituximab (4%). Treatment duration was longer for secondary AIHA than primary (11 vs 6.6 months,
P
<0.02) and in patients needing polytherapy than steroids only (13.3 vs 7.5 months,
P
<0.006). During median (range) follow-up period of 73 (1–150) months, 29 (58%) remained in continuous complete remission, 16 (32%) remained in complete remission.
Conclusion
Infants with AIHA have a more severe presentation. Monospecific DAT and a thorough search for an underlying cause help optimize therapy in most patients of AIHA.
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