Oral manifestations of aplastic anemia in children

Resident: Roberts

Date: 3/8/2011

Article title: Oral manifestations of aplastic anemia in children

Authors: Supulveda Ester

Journal: Jada

Volume 137 pages: 474 - 478

Year: 2006

Aplastic Anemia is a rare blood dyscrasia in which peripheral blood pancytopenia( defects in host defense) results from reduced or absent blood cell production in the bone marrow, and normal hematopoietic replaced by fatty marrow. The estimated incidence of AA is two new cases per 1 million person per year. The disease is rare in children and the peak age of occurrence is between and 5 years. The disorder can be inherited, idiopathic or acquired, the causes include radiation therapy, intake of drugs and chemicals, viral infections, thymoma, pregnancy and paroxysmal nocturnal hemoglobinuria. Severe AA is defined by two of three criteria: 1. low neutrophil count (500 cells/cubic mm) 2. low platelet count (20,000 cells/cubic mm) 3. low reticulocyte count (200 cells/ cubic mm). Therapy includes Bone marrow transplantation and immunosuppresive therapy in circumstances where identical siblings is present and immunosuppressive therapy for all other children.

Purpose: to describe oral lesions in children with AA.

Methods: Retrospective review of patients seen at a hospital in Concepcion, Chile between the years March 1996 and May 2001.

Results: Twelve children were diagnosed with AA. Their age range was 3 to 12 years (median age range was 7). Nine subjects were receiving immunosuppresive therapy, and three only supportive care. The most common oral manifestation of the disease was hemorrhage, which developed most often in patients with low platelet counts. This was followed by candidiasis and viral infection.

Conclusion: Neutropenia, caused by the disorder itself and its treatment, leads to an increased susceptibility to infection and thrombocytopenia which leads to bruising and mucosal bleeding: both of theses complications correspond to sepsis (including oral manifestations - often the first sign) and hemorrhage, the main causes of death in these patients.

Assessment: This study was obviously limited by the number of sample size. Further investigation is needed.