Reversibility of cirrhosis in patients cured of thalassemia by bone marrow transplantation.
BACKGROUND
Cirrhosis is a well-known complication of thalassemia major. In this context, it is a consequence of iron overload and hepatitis C virus infection and generally seems to be irreversible.


OBJECTIVE
To determine whether cirrhosis in thalassemia major can be reversible.


METHODS
Retrospective study.


METHODS
Bone Marrow Transplantation Unit and Pathology Service, Pesaro Hospital, Pesaro, Italy.


METHODS
6 patients who developed liver cirrhosis before or after their thalassemia was cured by bone marrow transplantation (age at transplantation, 11 to 25 years). After diagnosis of cirrhosis, the patients received iron depletion and antiviral therapies.


METHODS
Each liver biopsy specimen was coded. A liver pathologist and a member of the transplantation center with expertise in hepatopathology graded the specimens by using the Ishak staging and grading systems. Neither knew the patient's identity or the sequence of biopsy with regard to the time of treatment.


RESULTS
After the patients received iron depletion and antiviral therapies, liver biopsy specimens showed impressive reduction in liver iron stores. In 4 patients, iron removal was complete. Serum aminotransferase levels decreased in all patients and normalized in 5; histologic inflammatory activity decreased in all patients and disappeared in 2. Follow-up biopsies showed regression of incomplete or definite cirrhosis in all patients; 3 patients had presented with portal fibrosis without bridging, and the others had portal fibrosis and portal-to-portal bridging. Several biopsies and the presence of many portal spaces confirmed the diagnosis of cirrhosis; follow-up biopsies confirmed regression of cirrhosis.


CONCLUSIONS
In some patients in whom bone marrow transplantation has cured thalassemia, cirrhosis may be reversible after iron removal treatment.
Muretto
Pietro
P
Unità Operativa di Anatomia Patologica, Azienda Ospedaliera San Salvatore di Pesaro, 6110 Pesaro, Italy. p.muretto@ospedalesansalvatore.it
Angelucci
Emanuele
E
Lucarelli
Guido
G
D000293:Adolescent; D000328:Adult; D000998:Antiviral Agents / Q000627:therapeutic use; D001706:Biopsy; D016026:Bone Marrow Transplantation; D002648:Child; D003131:Combined Modality Therapy; D003676:Deferoxamine / Q000627:therapeutic use; D006801:Humans; D007501:Iron / Q000378:metabolism; D007502:Iron Chelating Agents / Q000627:therapeutic use; D008103:Liver Cirrhosis / Q000209:etiology* / Q000378:metabolism / Q000628:therapy*; D018962:Phlebotomy; D012074:Remission Induction; D012189:Retrospective Studies; D013789:Thalassemia / Q000150:complications* / Q000628:therapy*; D000637:Transaminases / Q000097:blood
D000998:Antiviral Agents; D007502:Iron Chelating Agents; D007501:Iron; D000637:Transaminases; D003676:Deferoxamine