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Reversibility of Cirrhosis in Patients Cured of Thalassemia by Bone Marrow Transplantation

Pietro Muretto, MD; Emanuele Angelucci, MD; and Guido Lucarelli, MD
[+] Article and Author Information

From Unità Operativa di Anatomia Patologica and Unità Operativa di Ematologia e Centro Trapianto Midollo Osseo, Azienda Ospedaliera San Salvatore di Pesaro, Italy.


Acknowledgment: The authors thank Professor Stanley L. Schrier for suggestions and for editing of the manuscript.

Requests for Single Reprints: Pietro Muretto, MD, Servizio di Anatomia Patologica, Azienda Ospedaliera San Salvatore di Pesaro, 61100 Pesaro, Italy; e-mail, p.muretto@ospedalesansalvatore.it.

Current Author Addresses: Dr. Muretto: Servizio di Anatomia Patologica, Azienda Ospedale San Salvatore di Pesaro, 6110 Pesaro, Italy.

Dr. Angelucci: Unità Operativa di Ematologia e Centro Trapianti Midollo Osseo, Ospedale A Businco, via Edward Jenner, 09121 Cagliari, Italy.

Dr. Lucarelli: Unità Operativa di Ematologia, Azienda Ospedale San Salvatore di Pesaro, 61100 Pesaro, Italy.

Author Contributions: Conception and design: P. Muretto, E. Angelucci.

Analysis and interpretation of the data: P. Muretto, E. Angelucci, G. Lucarelli.

Drafting of the article: P. Muretto, E. Angelucci.

Critical revision of the article for important intellectual content: P. Muretto, E. Angelucci, G. Lucarelli.

Final approval of the article: P. Muretto, E. Angelucci, G. Lucarelli.

Provision of study materials or patients: P. Muretto, E. Angelucci.

Statistical expertise: E. Angelucci.

Obtaining of funding: G. Lucarelli.

Collection and assembly of data: E. Angelucci.


Ann Intern Med. 2002;136(9):667-672. doi:10.7326/0003-4819-136-9-200205070-00009
Text Size: A A A

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.

Design: Retrospective study.

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

Patients: 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.

Measurements: 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.

Conclusion: In some patients in whom bone marrow transplantation has cured thalassemia, cirrhosis may be reversible after iron removal treatment.

Figures

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Figure.
Percutaneous liver biopsy specimens. A.B.C.D.E.F.G.H.

Patient 1. Specimen obtained at diagnosis of cirrhosis (4 years after marrow transplantation) shows cirrhosis with active hepatitis. (Masson trichrome stain; original magnification, ×60.) Patient 1. Specimen obtained at diagnosis shows severe iron overload (hepatic iron concentration, 22.94 mg/g of liver, dry weight). (Perls Prussian blue reaction; original magnification, ×60.) Patient 1. Specimen obtained 10 years after transplantation shows regression of cirrhosis and marked improvement of hepatitis. (Masson trichrome stain; original magnification, ×60.) Patient 1. Specimen obtained 10 years after transplantation shows absence of iron overload (hepatic iron concentration, 0.94 mg/g of liver, dry weight). (Perls Prussian blue reaction; original magnification, ×60.) Patient 2. Specimen obtained at diagnosis (4 years after transplantation) shows cirrhosis. (Masson trichrome stain; original magnification, ×25.) Patient 2. Specimen obtained 10 years after transplantation shows regression of cirrhosis. (Masson trichrome stain; original magnification, ×25.) Patient 6. Specimen obtained at diagnosis (before transplantation) shows definite cirrhosis. (Masson trichrome stain; original magnification, ×60.) Patient 6. Specimen obtained 5 years and 4 months after transplantation shows stage 2 fibrosis. (Masson trichrome stain; original magnification, ×60.).

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Summary for Patients

Reversibility of Liver Scarring in Patients Who Are Cured of Thalassemia

The summary below is from the full report titled “Reversibility of Cirrhosis in Patients Cured of Thalassemia by Bone Marrow Transplantation.” It is in the 7 May 2002 issue of Annals of Internal Medicine (volume 136, pages 667-672). The authors are P Muretto, E Angelucci, and G Lucarelli.

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