American Journal of Hematology 24:111-112 (1987)

Letter to the Editor: Thalassemia Major and M al ig nanci es

Stricker et al [l] have reported four cases of hematologic malignancy in sickle disease and have raised a concerned suspicion that a genetic link may exist between the two diseases affecting the hematopoietic system. Therefore, it is recommendable that cases of association between hereditary hematologic diseases and various malig- nancies are reported in order to establish if the incidence is higher than expected.

In 1984, 909 patients with P-thalassemia major were living in Sicily (prevalence 38.4/100,000 between 0 and 30 years of age) [2]. From 1974 to 1985 242 children and adolescents with P-thalassemia major have been followed at the Thalassemia Centre of our Pediatric Department. In our experience we have observed one case of non-Hodgkin lymphoma with bone marrow involvement and one case of stage IV neuroblastoma among these thalassemic children. The 4-year-old boy with lym- phoma, reported in detail elsewhere [3], had a rapidly progressive disease. The other patient, a 30-month-old boy, had adrenal neuroblastoma with diffuse skeletal metas- tasis and survived 13 months after diagnosis. It is paradoxical that, in spite of the high degree of medical attention owing to thalassemia, in both cases the possibility of malignancy was only late considered in the differential diagnosis of their presenting symptoms. In thalassemic patients anemia is obviously masked by regular transfu- sions, and splenomegaly may be overlooked. Another reason for late diagnosis may be the scepticism about the coexistence of two potentially fatal diseases in the same patient. However, our experience indicates that malignancies in thalassemia occur with an incidence not lower than that expected in normal children.

Antonio Russo Gino Schiliro Department of Pediatrics Chair of Pediatric Hematology University of Catania 95125 Catania, ltaly

REFERENCES

1 . Stricker RE, Linker CA, Crowley TJ, Embury SH: Hematologic malignancy in sickle cell disease:

2. Giambelluca SE: Registro Siciliano delle Talassemi; Distribuzione della talassemia major in Sicilia.

3. Schilirb G, Russo A, Marino S, Musumeci S, Russo G: Occurrence of lymphoma with bone marrow

Report of four cases and review of the literature. Am J Hematol21:223, 1986.

Rapport0 No. 2, Novembre 1985.

involvement in a boy with p+ thalassemia major. Clin Lab Haematol 1:325, 1979.

0 1987 Alan R. Liss, Inc.