Severe aplastic anemia associated with hepatitis and complicated by pulmonary aspergillosis: response to immune suppression and antifungal therapy.
The true etiology of severe aplastic anemia is unknown; however, autoimmune activation of T-lymphocytes is one of the potential causes. Stem cell transplantation is regarded as a first line therapy if a fully matched sibling is available. Immunosuppressive therapy is reserved for those who have no matched sibling available for transplant, or for those individuals who fall outside the age range eligible for stem cell transplantation. This case describes a child with a hepatitis-associated severe aplastic anemia for whom a fully matched sibling was available but a transplant was postponed due to active hepatitis. While awaiting bone marrow transplantation, the child acquired a life-threatening aspergillosis infection treated with amphotericin B, granulocyte infusions, and surgical resection of the involved lung. A decision was made to proceed with immunosuppressive therapy while waiting for successful treatment of the fungal infection. Following administration of equine anti-thymocyte globulin (ATG), high dose granulocyte colony stimulating factor (G-CSF), cyclosporine, and steroids, the child had partial hematopoietic reconstitution and is now followed in our clinic. This case demonstrates the extraordinary multidisciplinary care required during the early phases of treating severe aplastic anemia. With such care, recovery is a possibility.
Alfred I. duPont Hospital for Children, Wilmington, Delaware, USA.
Robert
R
Bond
Andrew
A
Walter
Michael E
ME
Trigg
D000741:Anemia, Aplastic / Q000209:etiology / Q000628:therapy*; D001228:Aspergillosis / Q000150:complications / Q000628:therapy*; D002648:Child; D003131:Combined Modality Therapy; D006505:Hepatitis / Q000150:complications; D006801:Humans; D008172:Lung Diseases, Fungal / Q000150:complications / Q000628:therapy*; D008297:Male; D016896:Treatment Outcome