Polyneuropathy and myopathy in beta-thalassemia major patients.

The thalassemias are the most common single gene disorder in the world. Nowadays, the average life expectancy of patients in developed countries has increased significantly, while, there was an increase of complications. We aimed to investigate peripheral neuropathy and myopathy in this patient group using a neurophysiological study. We performed nerve conduction studies and electromyography of upper and lower extremities on 36 beta-thalassemia major (ß-thal) patients. The electrophysiological findings were correlated with demographic data and laboratory parameters of the disease. Patients with ß-thal present polyneuropathy or myopathy at (50%). Polyneuropathy was detected in (38.9%) and myopathy in (27.8%), while polyneuropathy and myopathy were present at (16.7%) with an overlap of the diseases in 1/3 of the patients. There was not a statistically significant correlation of polyneuropathy and myopathy with age, sex, splenectomy, nor with respect to laboratory parameters, hemoglobin, and ferritin. However, there was a statistically significant correlation of polyneuropathy and myopathy with iron overload, as recorded by the magnetic resonance imaging (MRI) of the heart and the liver. Our findings suggest that iron overload plays a key role in the pathogenesis of polyneuropathy and myopathy in ß-thal patients, and performing heart and liver MRI for the prediction of such lesions in an annual basis is warranted.

Non-Hodgkin's lymphomas in Greece according to the WHO classification of lymphoid neoplasms. A retrospective analysis of 810 cases.

The purpose of this retrospective study, the largest unselected series in our country, was to illustrate the clinicopathological features of non-Hodgkin's lymphoma (NHL) classified according to the World Health Organization (WHO) classification of lymphoid neoplasms. A retrospective analysis was conducted and clinical features of histological subtypes were established in 810 patients (age > or = 15 years) with NHL who were treated at 8 major centers representative of Greece. There were 435 males and 375 females 95% of them aged >30 years. B symptoms were present in 34% of the patients, while 45.3% had stages I-II and 54.6% had stages III-IV. LDH was increased in 37% of the patients. B cell lymphomas formed 88% of the cases whereas T cell lymphomas formed 12% of the total. Indolent lymphomas accounted for 31.1%, aggressive ones for 66.7% and very aggressive ones for 2.4% of all NHLs. Among indolent lymphomas extranodal ones (MALT B cell lymphoma) were the most common subset while follicular lymphoma grade I and II and small lymphocytic ones presented with equal frequency. Among the aggressive lymphomas diffuse large cell lymphoma (DLCL) was the most common subtype; this entity along with large-cell immunoblastic lymphomas accounted for 45.2% of all B cell lymphomas. Among the T cell lymphomas, peripheral T cell lymphomas and anaplastic large cell lymphomas of the T/null-cell type were the most common subtypes. The most common extranodal presentation was the gastrointestinal tract (GI). Next in frequency were primary extranodal NHL of the head and neck region. MALT B cell lymphomas were found in almost half of the patients with GI tract NHL, whereas in all other extranodal places DLCL was the predominant histological subtype. The median survival for indolent and aggressive NHL was 123.5 and 55.5 months, respectively. This is the first report of a large series of malignant lymphomas in Greece using the WHO classification. It appears that there are no significant differences between NHL in Greece and other large series as far as clinical and extranodal presentation is concerned. The frequency of follicular lymphoma in the current study is comparable to that reported from Asian countries and mainland Europe, but lower than that of US and Northern European series. There were no important differences in the incidence of the remaining histological subtypes between Greece and other European countries.

Responsiveness to recombinant human erythropoietin (rh-Epo) of marrow erythroid progenitors (CFU-E and BFU-E) from B-chronic lymphocytic leukemia (B-CLL).

The responsiveness of bone marrow erythroid progenitors (CFU-E and BFU-E) to various concentrations of recombinant human erythropoietin (rh-Epo) (2,5,20,40,100,200 and 500 U/ml) was investigated in vitro in 18 patients with B-chronic lymphocytic leukemia to assess the clinical usefulness of rh-Epo in this disease. Bone marrow mononuclear cells were cultured by methylcellulose methods for CFU-E and BFU-E assays. The B-chronic lymphocytic leukemia patients were divided into two groups according to the percentage of lymphocytes in the bone marrow (under 70% and over 70%). Among the patients with few lymphocytes, more than one third demonstrated some degree of response to rh-Epo. Among the patients with a high percentage of lymphocytes in the bone marrow, some revealed no response to rh-Epo, but there were patients who showed a good response to rh-Epo. Because erythroid progenitors from B-chronic lymphocytic leukemia appeared sensitive to rh-Epo in vitro, we propose that high doses of this drug may be clinically effective in some patients with this disease, regardless of the degree of lymphocytic inflitration of the bone marrow.

Increased peripheral blood normal myeloid progenitor cells (CFU-GM) in chronic lymphocytic leukemia: a perspective for autologous peripheral blood stem cell transplantation.

We assayed granulocyte-macrophage committed progenitor cells (CFU-GM) in the peripheral blood of 34 patients with chronic lymphocytic leukemia (CLL) and 12 normal individuals. The patients were divided into separate groups on the basis of previous therapy (i.e. analysis performed at diagnosis, during and after chemotherapy) and clinical features of the disease (i.e. disease stage, pattern of bone marrow infiltration, peripheral blood lymphocytosis). The mean CFU-GM colony count of the patients was 30 times higher than that of the controls (206.4 +/- 197.8 (SD) CFU-GM per 5 x 10(5) cells plated versus 6.5 +/- 3.6). There was no statistical difference in the numbers of circulating CFU-GM between the patients studied at diagnosis (257 +/- 215.4 CFU-GM/5 x 10(5) cells) and those studied during (117.6 +/- 169.2 CFU-GM/5 x 10(5) cells) or after chemotherapy (207.5 +/- 105.9 CFU-GM/5 x 10(5) cells), although a trend towards a higher recovery of myeloid progenitors was observed as a function of time elapsing from the last treatment. In addition, we found no significant difference in the in vitro CFU-GM growth of patients grouped according to their disease stage, pattern of bone marrow infiltration and degree of peripheral blood lymphocytosis. In conclusion, our data indicate that intensification with peripheral blood stem cell support may be feasible in CLL, since progenitor cells of myeloid-monocytic series are markedly increased in the peripheral blood of these patients. Moreover, it is possible to extend this kind of therapy to patients who have undergone previous extensive chemotherapy and who might have persisting bone marrow infiltration.