[Changes in incidence of malignant melanoma in the last 19 years in a tertiary hospital on the Mediterranean coast]. / Evolución del melanoma maligno cutáneo en los últimos 19 años en un hospital terciario de la cuenca mediterránea.

INTRODUCTION: The incidence of melanoma has increased more than that of any other type of malignant tumor. Our aim was to analyze the changes in incidence of cutaneous melanoma in recent years in a Mediterranean population. MATERIAL AND METHODS: Patients with melanoma diagnosed between 1988 and 2006 were included in the study. Data from the first half of this period were compared with data from the second half. RESULTS: The number of in situ melanomas increased from 36/302 cases (11.92 %) in the first half of the period to 224/724 (30.94 %) in the second half. Melanomas measuring more than 4 mm increased from 29/302 cases (9.60 %) to 62/724 (8.56 %). The mean maximum thickness for the whole study period was 1.91 mm and was similar for both halves. CONCLUSIONS: The increase in incidence of melanoma in our population was due mainly to an increase in incipient cases. The proportion of melanomas larger than 4 mm remained constant, although, in absolute terms, twice as many such melanomas were detected per year. We believe that campaigns for prevention and early detection must continue, and should focus in particular on the older population.

Cardiovascular risk factors and the long-term outcome of lupus nephritis.

We evaluated cardiovascular risk factors, morbidity and mortality in patients with lupus nephritis (LN). We prospectively studied 70 consecutive patients with LN, and 70 age- and sex-matched controls with systemic lupus erythematosus (SLE) but no evidence of nephropathy, from 1988 to 1998. Patients were evaluated at entry for hypertension, diabetes, hyperlipidaemia, smoking, menopause and antiphospholipid syndrome. The LN patients (64 women, 6 men) had a mean age of 35 years (SE 1.7, range 11-67). During the 10 years, 15 (21%) LN patients and 18 (25%) of the controls were lost to follow-up. Compared with controls, LN patients had a higher prevalence of hyperlipidaemia (44% vs. 2%, p<0.001), hypertension (44% vs. 9%, p<0.001) and antiphospholipid antibodies (45% vs. 22%, p=0.01) at study onset. At the last visit, 37 (67%) LN patients had normal plasma creatinine, 13 (24%) had renal failure and only five (9%) end-stage renal failure. Hyperlipidaemia (78% vs. 27%, p<0.001) and hypertension (67% vs. 32%, p=0.01) at study onset were associated with development of renal failure. Nine LN patients and one control died (16% vs. 2%, p=0.02). These patients showed more antiphospholipid syndrome (56% vs. 17%, p=0.03) and hyperlipidaemia (78% vs. 37%, p=0.03) at study onset. The main causes of death in LN patients were vascular complications (cardiovascular or cerebrovascular events) in five patients (four of whom had antiphospholipid antibodies) and sepsis in three.

Atypical retroperitoneal fibrosis: MRI findings.

A case of retroperitoneal fibrosis with an unusual perirenal involvement diagnosed at MR imaging is reported. Other conditions, such as metastatic disease or lymphoma, may be considered especially when the initial presentation is not typical. Imaging modalities in this condition are discussed.

High-dose busulfan and melphalan before bone marrow transplantation for acute nonlymphoblastic leukemia.

Fourteen patients with acute nonlymphoblastic leukemia (ANLL) (n = 13) or juvenile chronic myelomonocytic leukemia (n = 1) were transplanted after conditioning with high-dose busulfan (4 mg/kg daily on days -7 to -4) and melphalan (180 mg/m2 on day -2). This protocol was designed for patients considered unable to receive standard conditioning regimens with cyclophosphamide and/or TBI. Five patients (4 children and 1 adult) received a second allogeneic BMT in untreated early marrow relapse after a first BMT. There were 3 procedure-related deaths (PRD), 2 during aplasia and 1 from acute GVHD. Two patients survived the procedure; 1 relapsed at 6 months and 1 is alive at 43+ months. Nine subjects (8 children and 1 adult) received an autologous BMT, 7 in first and 2 in second complete remission (CR). Of the 7 patients grafted in first CR, there was 1 PRD, 2 relapses at 3 and 15 months, and four are alive at 38 to 82+ months. One patient grafted in second CR relapsed at 7 months and 1 is alive at 67+ months. Toxicities were mild or moderate in autologous BMT recipients, mainly affecting the gastrointestinal tract. In the allogeneic BMT group, there were more moderate to severe toxicities, including 3 cases of moderate-severe renal toxicity; no cases of such toxicity were seen in ABMT recipients. Two cases of HVOD occurred, 1 in each group. These results are encouraging, although the small patient group does not allow any firm conclusions.

Long-term evolution of patients with isolated C3 mesangial glomerulonephritis.

Isolated mesangial C3 proliferative glomerulonephritis is a nephropathy poorly defined among glomerular diseases. Clinical picture is characterized by episodes of gross hematuria and/or persistent or recurrent microhematuria and/or proteinuria. Short-term prognosis (less than 3 years) is considered to be benign, although not much information is available in reference to long-term follow-up. We reviewed all renal biopsies performed in our center between 1978 and 1992 (n = 2,200) in order to study clinical course of these patients. Isolated mesangial C3 deposition was found in 11 cases (0.5%). Isolated proteinuria (> 0.5 g/d) was present in 3 patients and nephrotic syndrome in 2. Hematuria with proteinuria was detected in 5 patients. In one patient hematuria was the only clinical finding. Arterial hypertension was observed in 4 cases. At the time of renal biopsy, renal function was accurately normal in all but one patient. In our series, renal function showed no changes during the first 3 years, accordingly to data referred to in the literature. After 7 years of follow-up, however, renal function was declining in 4 of 5 patients who developed terminal renal failure up to requiring hemodialysis. Therefore, initial benign prognosis ascribed to this glomerulonephritis would not be confirmed in a long-term follow-up.

Pulmonary hemorrhage as a clinical manifestation of hemolytic-uremic syndrome associated with mitomycin C therapy.

Mitomycin C (MMC) is an alkylating agent that has been recently associated with the hemolytic-uremic syndrome (HUS). Pulmonary impairment in the HUS is rarely reported in the literature, and no reports of pulmonary hemorrhage, as a clinical feature of the HUS, have been documented. We describe two women who developed HUS after MMC therapy and presented massive pulmonary bleeding. Pulmonary hemorrhage is an uncommon feature in the HUS, and seems to appear especially in the HUS associated with MMC therapy. It is extremely resistant to standard treatment, and confers a poor prognosis for this disease.

Coincidental amyloid nephropathy and IgA glomerulonephritis in a patient with ankylosing spondylitis.

A 43-year-old man with ankylosing spondylitis (AS) was admitted with proteinuria and renal insufficiency. Coincidental IgA nephropathy and secondary renal amyloidosis were diagnosed on renal biopsy. This is the first case reported of coincidental IgA nephropathy and renal amyloidosis as a cause of renal insufficiency in a patient with AS. Both entities should be considered in the diagnosis in a patient with AS and renal impairment. A careful study of the renal tissue including, Congo red stain and immunofluorescence studies is necessary to establish a correct diagnosis in these patients.

[Systemic lupus erythematosus: a clinical and immunological study of 300 patients]. / Lupus eritematoso sistémico: estudio clínico e inmunológico de 300 pacientes.

BACKGROUND: The aims of the present study were to analyze the clinical and immunologic characteristics of a wide group of patients with systemic lupus erythematosus (SLE) and define homogeneous subgroups with their own characteristics. METHODS: A prospective study of 300 patients diagnosed of SLE were studied. These patients were subdivided according to sex, age at the onset of the disease and immunologic profile. The statistical study was carried out by the chi (2), Fisher, Student's t and Mann-Whitney U tests. RESULTS: The series was made up of 266 (89%) females and 34 (11%) males. The mean age at onset of the disease was 31.8 +/- 14.6 years. In 48 (16%) patients the first manifestations appeared after the age of 50. Males were shown to present a lower prevalence of arthritis (59% vs 82% in woman, p < 0.005) and malar rash (29% vs 50%, p < 0.05), but had more cutaneous discoid lesions (18% vs 3% p < 0.001). In patients in whom the disease appeared after the age of 50 a lower prevalence of arthritis was presented (67% vs 82% in patients of less than 50 years of age, p < 0.005), malar rash (23% vs 53%, p < 0.001) and nephropathy (21% vs 41%, p < 0.05), but had greater myositis (17% vs 6%, p < 0.01). The absence of antinuclear antibodies (ANA) and the presence of anti-ds DNA and anti-ENA antibodies were associated with differences in the prevalence of different clinical manifestations. CONCLUSIONS: Sex, age and immunologic pattern in systemic lupus erythematosus permit the definition of homogeneous subgroups with their own characteristics: a) males present a lower prevalence of arthritis and malar rash, but a greater prevalence of cutaneous discoid lesions; b) patients over the age of 50 develop arthritis, malar rash and nephropathy with a lower prevalence but have a greater prevalence of myositis; c) patients without antinuclear antibodies and those with anti-ds DNA and anti-ENA antibodies present differences in the prevalences of different clinical manifestations.

Minimal-change glomerulopathy and carcinoma. Report of two cases and review of the literature.

We describe 2 patients with minimal-change glomerulopathy (MCG) associated with an undifferentiated carcinoma of unknown origin and urothelial carcinoma. Oliguric acute renal failure and histopathological changes consistent with acute tubular necrosis were also observed. Fourteen other cases of MCG complicating solid tumors reported in the literature are reviewed. MCG should be included in the nephropathies which cause nephrotic syndrome in adult patients with carcinoma.

Autologous hemopoietic reconstitution after fetal liver infusion in patients with bone marrow failure: consequence or coincidence?

In the past 4 years we have treated four patients with a total of 19 fetal liver infusions (FLI). Two cases of refractory anemia with excess blasts in transformation (RAEB-t) were conditioned with cyclophosphamide and total body irradiation (1400 cGy) and were treated with FLI. In spite of such intensive conditioning, one patient recovered autologous hemopoiesis 3 weeks later, remaining in remission 4 years after this procedure. The second patient died with aplastic marrow on day 154, and the third suffered from severe aplastic anemia refractory to several types of conventional treatment. After FLI and without previous conditioning therapy a partial fetal engraftment was documented. This was transient and followed by autologous hemopoietic recovery and cure of the disease. The fourth patient had bone marrow failure in the setting of a severe pneumonia following autologous bone marrow transplantation. Ten days after FLI the hematological parameters dramatically improved and the pneumonia resolved. Autologous reconstitution of hemopoiesis was demonstrated. These experiences suggest that FLI might stimulate autologous hemopoiesis. This therapeutic approach may be useful to treat bone marrow failure when there is no response to first-line therapy. In hematologic malignancies with an indication for stem cell transplantation, other sources such as allogeneic or autologous bone marrow seem preferable to fetal liver cells.

[Uremic hemolytic syndrome induced by mitomycin C. Presentation of a series of 5 cases and review of the literature]. / Síndrome hemolítico urémico inducido por mitomicina C. Presentación de una serie de cinco casos y revisión de la literatura.

Five new cases of hemolytic uremic syndrome associated mitomycin C treatment in neoplastic patients are presented and clinical, biological, histological therapeutic and the evolution data of 92 other cases from the literature are analyzed. The results point out the high incidence of GI adenocarcinomas (65%), its rare appearance with total mitomycin doses below 50 mg/m2, its frequent clinical presentation with cardiovascular manifestations, the therapeutical difficulties and the possibility of a better prognosis with an early diagnosis.

[Subacute kidney insufficiency in exclusively vascular kidney amyloidosis]. / Insuficiencia renal subaguda en la amiloidosis renal exclusivamente vascular.

Two patients are reported with secondary amyloidosis in whom renal biopsy disclosed massive amyloid deposition with exclusively extravascular localization. In one case, autopsy showed selective amyloid deposition in the vessel walls of most examined organs. In both patients, the clinical presentation consisted of subacute renal failure without proteinuria. A possible reduction of renal perfusion, secondary to a depleted blood volume in one case and to pharmacological inhibition of prostaglandin synthesis in the other, could enhance renal dysfunction in both patients, who, on the other hand, previously had mild renal failure. The clinical presentation of renal amyloidosis basically depends on the distribution and severity of amyloid deposits. Vascular localization represents an uncommon pattern of renal amyloidosis, generally associated with chronic renal failure with minimal or no proteinuria.

[Acute kidney insufficiency in ethylene glycol poisoning]. / Insuficiencia renal aguda en la intoxicación por etilenglicol.

A case of severe ethylene-glycol intoxication is presented in which there were potentially fatal plasma levels, neurological manifestations and acute renal failure and which was treated with ethanol and hemodialysis. Crystal deposits, normal glomeruli and severe tubular affectation suggestive of direct cytotoxic action, were found in renal biopsy, which was performed during the anuric phase. The evolution was favorable with no neurologic sequelae, diuresis re-onset on day 17 and return of normal renal function.

Silent renal microangiography after mitomycin C therapy.

Mitomycin C (MMC) is an alkylating agent which has been associated with microangiopathic hemolytic anemia and acute renal failure, with an overall incidence between 2 and 10%. This complication can develop several months after the initiation of chemotherapy. Isolated renal impairment without overt microangiopathic hemolytic anemia, although reported, is less frequently documented. We describe a 63-year-old man who developed progressive renal failure without any evidence of hemolysis or thrombopenia 10 months after beginning chemotherapy with MMC and Ftorafur. A renal biopsy displayed features of microangiopathy. The patient required the institution of chronic hemodialysis. In conclusion, it is important to be aware of this indolent but severe renal complication in patients treated with MMC. Urinary parameters and renal function should be monitored over a long period for an early diagnosis.

Henoch-Schönlein purpura and IgA nephropathy in father and son.

Considerable controversy exists as to whether Henoch-Schönlein purpura and Iga nephropathy are different clinical manifestations of the same disease or if, on the contrary, they are separate entities. We report on the development of Henoch-Schönlein purpura and IgA nephropathy in 2 members of the same family. Patient 1, a 63-year-old man, presented with purpura in the legs, abdominal pain, hematuria, renal failure and proteinuria. A biopsy of a purpuric skin lesion showed small-vessel vasculitis, and a renal biopsy showed diffuse proliferative glomerulonephritis with prominent IgA deposits, thus making the diagnosis of Henoch-Schönlein purpura. Serum IgA was increased. Patient 2, the 30-year-old son of patient 1, underwent renal biopsy for the investigation of microscopic hematuria and proteinuria. There was no history of skin rash, and serum creatinine was normal. A renal biopsy showed expansion of the mesangial matrix and marked IgA deposition. HLA typing confirmed that they shared a haplotype. HLA B35 or DR4 were absent. These results demonstrate that Henoch-Schönlein purpura and IgA nephropathy can possibly be genetically related and therefore support the notion that these two diseases probably share a common pathogenesis.