Systemic sclerosis with bilateral hilar adenopathy.
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Biomedical subjects
Publications and source records attributed to N Stathakis.
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In this prospective study, patients with "high risk' primary MDS, namely RAEB or RAEBt, were treated with combination chemotherapy (CT) supported by GM-CSF. The induction CT consisted of idarubicin 6 mg/m2 days 1-3 and cytosine-arabinoside 200 mg/m2 in 12 h infusion, days 1-5. The GM-CSF 3 micrograms/kg s.c. was given on day 6 until the neutrophil count was 1 x 10(9)/l. Postremission CT consisted of two similar courses. Patients not in remission after two courses of CT were considered as treatment failures. Twenty-two patients with a median age of 64 years, range 50-79 years (11 RAEB and 11 RAEBt) were evaluable. Twelve out of 22 patients (54.5%) achieved complete remission (CR) and four, partial remission. Six patients were resistant to treatment; there were two toxic deaths; seven patients achieved CR after the first course and five after two courses. The median time of neutrophil recovery to 1 x 10(9)/l was day 15 (range 3-22) after the first course of treatment and day 14 (range 4-21) after the second. Thirteen out of 22 patients developed febrile episodes after the first course of treatment and nine after the second. The median duration of CR was 12 months. The median survival for CR patients was 24 months, for non-CR patients, 12 months; while survival for the whole population was 18 months. In conclusion, the results of this study indicate that the administration of moderately intensive CT supported by GM-CSF in "poor risk' MDS gives promising results; the response rate is high for this disease, while the incidence of toxic death is low. GM-CSF appears to accelerate neutrophil recovery and probably reduces the incidence of infection.
Among 318 cases of non-Hodgkin's lymphoma (NHL) treated in our unit, 145 (45.6%) had primary extranodal NHL (PE-NHL). The stomach was the most common site (42.1%), followed by the PE-NHL of the head and neck region. Histologically aggressive histologies (65.5% intermediate and 20.7% high grade) predominated. 89.6% of the cases were localized (stage IE, 51% and stage II, 38.6%) but 28% had B symptoms. CR was achieved in 82.1% of the cases. 5-years disease free survival and overall survival were both 65%. Factors that influence prognosis were stage and high grade histology. Among various primary sites the Waldeyer's ring, small intestine and testes had the worse prognosis. Compared to nodal NHL, the PE-NHL were more frequently localized, belonged more often to aggressive histologies and had more often distal extranodal relapses. CR rates and disease free and overall survival were significantly better for PE-NHL. The survival rates, however, listed according to stage and histology for nodal and PE-NHL were not different. We conclude that although PE-NHL differed from nodal NHL in several respects, prognosis is mainly a factor of stage and histology rather than of the primary localization per se.
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An unusual case of a 44-year-old patient with primary left testicular granulocytic sarcoma is presented. Iliac and paraaortic lymph node involvement was also demonstrated at staging laparotomy. The patient was treated with left orchiectomy and early intensive chemotherapy, but relapsed with a right testicular mass 14 months later. No evidence of leukemic progression was found at that time, and he was treated with right orchiectomy, combined with irradiation to the scrotum, inguinal, iliac and paraaortic lymph nodes. Unfortunately, he developed myelogenous leukemia 7 months later. A complete remission of 6 months duration was achieved with combination chemotherapy. The patient then relapsed, and an effort to induce a second remission was unsuccessful. He eventually died almost 3 years after initial diagnosis. We conclude that primary granulocytic sarcoma represents a systemic disease. Despite early intensive treatment its prognosis remains poor.
In a phase II study, 21 patients with MDS (RAEB, RAEBt, CMML and RA and RAS with severe cytopenia) were randomized to be treated with 3 courses of GM-CSF (3 micrograms/kg/day s.c.) alone (11 patients) or in combination with AraC (20 mg/m2/d s.c.) (10 patients) for 14-d periods, interrupted by 14-d rest periods. Eight patients discontinued the treatment. In the GM-CSF group a marked increase in WBC and neutrophil counts during each course of treatment administration were seen in most patients. Platelet counts decreased in 14 of 24 courses of treatment in the GM-CSF plus AraC group but in none of the GM-CSF group. Although the changes in the circulating blood cells were transient and the counts tended to return to the pretreatment levels during the rest periods, some more durable effects were seen. In 3/6 patients of the GM-CSF group who completed the designed treatment, both WBC and neutrophils remained elevated above the pretreatment levels throughout the 3-month period of treatment, while in one of them thrombocytopenia improved considerably. In the GM-CSF plus AraC group, 4 out of the 7 patients who completed the treatment showed an improvement of neutropenia as well as anaemia. In these 4 patients the BM percentage of blasts was also decreased. In conclusion, the results of this study indicate that GM-CSF given intermittently improves leukopenia in some patients with MDS. In addition, the administration of GM-CSF seems to prevent granulocytopenia of concurrent AraC treatment and may be of benefit in the treatment of these diseases.
Among 387 cases of non-Hodgkin's lymphoma (NHL) treated in our units between January 1977 and December 1990, 52 (13.4%) had primary extranodal (PE) NHL of the head and neck. The median age was 55 years with a M:F ratio of 1.9:1. The most frequent primary site was the tonsil (28 cases), followed by oral cavity, parotid gland, orbit and other sites. The aggressive histological subtypes predominate. 55.2% of the patients were in stage I and 44.8% in stage II of disease. The CR rate was high (94.2%). The 5 years' overall survival rate was 65% and it was influenced mainly by stage (stage I 82.5% vs. 48.7% in stage II). Sex, age and histology did not significantly affect survival rate. Patients with primary Waldeyer's ring involvement (WR group) did not differ significantly from the other primary sites analyzed as a group (non-WR group) in respect to median age, sex distribution, histology and CR rates. They differed, however, in: (1) stage distribution with stage II disease more frequent in the WR group; (2) overall survival and disease-free survival both of which were significantly better in the non-WR group; and (3) the high incidence of GI tract involvement as initial manifestation of relapse in the WR group. It is concluded that the behaviour of the Waldeyer's ring PE-NHL is rather distinctive and should be considered separately from the other PE-NHL of the head and neck.
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The clinical and haematological findings in 131 patients with myelodysplastic syndromes (MDS), none of which had previously received chemotherapy or radiotherapy, classified according to the FAB criteria, were analysed. The distribution among the 5 subgroups was: RA 31 patients, RAS 19, RAEB 23, CMML 29 and RAEBT 29 patients. There were difficulties in the classification of 24 patients. These included, first, 8 cases with myeloid hyperplasia of the bone marrow (BM) but without monocytosis or excess of blasts of the BM. They were classified as RA. Second, 8 cases with sideroblastosis but with monocytosis or excess of blasts of the BM were classified 3 as RAEB, 2 as CMML and 3 as RAEBT. Finally, 8 cases with absolute monocytosis and BM blasts 15-30% were classified as CMML. 37 of 82 dead patients (45.1%) had transformed to acute non-lymphoblastic leukaemia (ANLL). The incidence of evolution to ANLL was low for RA and RAS (6.30% and 12.5% respectively), while it was 37.5% for RAEB, 57.1% for CMML and 77.2% for RAEBT. The median survival for each subgroup was: RA 18 months; RAS 25; RAEB 13; CMML 14 and RAEBT 10 months. It is concluded that the FAB classification with some modifications recognises group of MDS with different prognosis.
A patient with multiple myeloma who developed a myeloproliferative disorder 6 years after initial diagnosis is described. The 2 disorders coexisted for 3 years. Possible explanations for this occurrence are discussed.
We analysed 29 consecutive cases of primary gastric lymphoma (20 men and 9 women) treated in our unit between January 1977 and May 1983. Median age was 55 years. Abdominal pain and weight loss were the main presenting symptoms while there was no palpable disease in the majority of cases. Upper gastrointestinal radiology was abnormal, but not diagnostic, in all cases. Endoscopy with multiple biopsies was performed in 22 cases; carcinoma was diagnosed in 11, lymphoma in 8 while no diagnosis was made in 3 cases. Twenty six patients underwent laparotomy. Gastrectomy was performed in twenty while the tumour was unresectable in six. Histology was reported as diffuse in 28 cases (16 histiocytic, 8 lymphocytic and 4 mixed) and nodular (lymphocytic) in one. All our patients received multichemotherapy. Complete remission after 6 courses was documented in 18 patients (62%). Neither perforation nor gastrointestinal bleeding was a problem in our series. Eighty four per cent complete responders are predicted to be alive at 4 years. Advanced stage (II2B and IV) and tumour size greater than 10 cm adversely influenced survival. We suggest that in limited primary gastric lymphoma an attempt at 'curative' surgery combined with multichemotherapy currently gives very promising results.
In a retrospective study 37 patient who fitted into the clinical spectrum of myelodysplastic syndrome were reviewed. Special attention was paid to the influence of monocyte count, degree of blastic infiltration and ring sideroblasts and on the clinical presentation and the course of the disease. Monocyte count clearly distinguishes between two groups of patients with different haematological profile and clinical course. The patients with monocytosis frequently changed to acute myeloid leukaemia and had shorter survival rates compared with those without monocytosis. The degree of blastic infiltration does not affect the haematological presentation and the frequency of acute leukaemia evolution. However, increased blastic infiltration is associated with shorter survival. Patients with ringed sideroblasts presented with profound anaemia but the clinical course of the disease did not differ from the remainder.
75 cases of acute myeloid leukaemia were retrospectively classified according to the French-American-British (FAB) classification. It was found that: (1) the proposed classification was easily applicable and reproducible; (2) M2 subtype was the most frequent (48%); (3) splenomegaly was absent in M1 subtype, and (4) in M2 subtype, a considerable number of cases presented with normal neutrophil and platelet counts.
A case of the so-called 'Lennert's lymphoma' terminating as malignant histiocytosis is reported. Such a sequence of diagnoses is discussed as a possible progression of the initial 'Lennert's lymphoma' eventually under the influence of cytostatic treatment. A review of other known malignancies ending up in malignant histiocytosis is given.
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