Tooth developmental anomalies in severe combined immunodeficiency disease and juvenile myelomonocytic leukemia: common clinical features and treatment outcomes

Authors:

ABSTRACT

Aim

Human Severe Combined Immunodeficiency (SCID) is a prenatal disorder of T lymphocyte development that depends
on the expression of numerous genes. Juvenile myelomonocytic leukemia (JMML), previously known as juvenile chronic myeloid
leukemia (JCML), is a rare, myelodysplastic/myeloproliferative disease typically presenting in early childhood. CASE REPORTS:
Two cases are described of immunodeficiency disorders, both treated with chemotherapeutic drugs (Busulfan plus cyclophosphamide)
before bone marrow transplantation. After treatment, these two different cases showed several similar oral lesions: microdontia, root
alterations, numerous tooth ageneses, incomplete calcification, enamel hypoplasia, premature apexification and hypodontia. Both
subjects underwent dental and orthodontic treatment. The first phase comprised orthopaedic treatment using a removable appliance
(Interim-G) followed by rapid palatal expansion; in the second phase patients underwent tooth extraction and were treated using fixed
appliances for 19 and 26 months, respectively (mean 2 years) to obtain final alignment and maximum intercuspation. In the third and
final phase, reconstruction of malformed teeth was completed, and implant-supported protheses were applied.

Conclusion

The
difficulties of managing and treating these diseases are discussed, with particular focus on tooth anomalies and malocclusion disorders.
Collaboration between dentist and paediatrician in dealing with patients with a variety of oral lesions and tooth anomalies is important in
order to prevent any other possible tooth lesions and ensure correct jaw development.

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