Erythrocyte content of free protoporphyrin in thalassaemic syndromes.
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Biomedical subjects
Publications and source records attributed to N Stathakis.
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BACKGROUND: The development of acute non-lymphoblastic leukaemia (ANLL) or myelodysplastic syndromes (MDS) secondary to treatment of multiple myeloma (MM) is well known. In some cases the simultaneous appearance of MM and ANLL has been described. METHODS: In this series the simultaneous appearance of MM and various myelopathies in 91 untreated patients with MM, and the development of myelopathies during the course of the disease in 72 treated patients were studied. RESULTS: Simultaneous appearance of MM (IgA/lambda) and refractory anaemia with ring sideroblasts (RAS) was observed in one case (1.1%). Development of myelopathies in treated patients with MM was found in 4 out of 72 cases (cumulative risk at 8 years 28.3%). In one case (IgG/lambda MM) a myeloproliferative disorder (MPD) developed 6 years after the initial diagnosis. Cytogenetic analysis was normal. In the second patient (IgG/k MM) a similar MPD was observed 5 years after the initial diagnosis. The karyotype was 46, XX, -5 + t (20;?). The third patient with lambda light chain disease developed RAS 11 months after the initial diagnosis. The karyotype was 46, XY/hypodiploidy + M. Finally, the fourth case (IgG/k MM) developed ANLL (M4) 28 months after the initial diagnosis and the karyotype was 45, XX, -7, t(1;3). CONCLUSIONS: The simultaneous appearance of MM and various myelopathies is unusual and probably represents a neoplastic transformation of a single progenitor in both lymphoid and myeloid malignancies. On the contrary, the development of myelopathies during the course of treated patients is a common phenomenon. The time of development and the cytogenetic findings strongly suggest that they are related to treatment with cytostatics.
The risk and the type of second malignancies (SM) developing in 217 treated Hodgkin's disease (HD) patients were studied. The median age of the patients was 35 years (range 14-83) and the M/F ratio 1.8. Treatment consisted of radiotherapy alone (24 patients, 11%), chemotherapy alone (96 patients, 44.3%), or a combination of both modalities (43 patients, 19.8%), while 54 patients (24.9%) received salvage treatment. The median follow-up time was 67 months (range 12-224). Ten patients developed a SM with a 5-year and 10-year actuarial risk of 3.3% and 5.4%, respectively. There were 3 cases of ANLL and MDS (actuarial risk of 2.4% at 6 years), 1 case of non-Hodgkin's lymphoma and 6 cases of solid tumors (actuarial risk of 2.4% at 6 years). The risk of developing SM was higher in males and older patients (> 40 years). SM represent a serious late side effect of successful treatment for HD. The possibility of developing a SM must be taken into consideration in the initial treatment of the disease.
Primary extranodal NHL of the head and neck (HN-NHL) accounts for 10-20% of all cases of NHL. Despite their frequency, the natural history and biological behaviour of these lymphomas is poorly understood. In this study we analysed the data 116 cases of HN-NHL. There were 65 males and 51 females with a median age of 56 years. The distribution among different anatomical sites was: tonsils 56 cases (48.3%), nasopharynx 15 (12.9%), mandible/gingiva 9 (7.8%), hard palate 7 (6%), parotis 6 (5.2%), nasal cavity 6 (5.2%), hypopharynx/larynx 6 (5.2%), thyroid 5 (4.3%), ocular adnexa 4 (3.5%), paranasal sinuses 2 (1.7%). The patients were treated with radiotherapy alone (14 cases), combined chemotherapy (52 cases) and combined modality (50 cases). According to the WF histological classification 73 cases (62.9%) had intermediate, 32 (27.6%) high and 11 (9.5%) low grade. Patients were separated in two groups: Tonsillar NHL (56 cases) and NHL of all other sites (non-tonsillar group-60 cases). A comparison between the two groups showed that there was no statistically significant difference with respect to age, sex, and histological subtypes. Also treatment response was similar (82.1% for the tonsillar vs 83.3% for the non-tonsillar). The two groups differed in stage distribution, survival and pattern of relapse. Stage I was more frequent in the non-tonsillar NHL (60%) in contrast to tonsillar NHL where stage II was more prominent (51.8%). Median survival was 86 months for the tonsillar while it has not been reached yet for the non-tonsillar patients. Patients in stage I and stage II of the non-tonsillar group had better survival compared to stages I and II of the tonsillar patients. Finally GI tract was a common site of relapse in the tonsillar group while a considerable number in CNS relapses were observed in the non-tonsillar group. We concluded that HN-NHL constitutes a heterogeneous group of patients. Tonsillar lymphomas represent a distinct group with some special clinicopathological findings.