Intravascular hemolysis and transjugular intrahepatic portosystemic stent shunt.
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Biomedical subjects
Publications and source records attributed to G Girelli.
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In HCL patients some cases of familial occurrence of the disease were reported, without an association with a specific haplotype or HLA antigen. In a series of a non familial HCL patients an increase in DR11 frequency was reported. We performed HLA class I and class II antigens in a series of 38 consecutive HCL patients. An increased frequency of B17 (10/38-27%) and DR11 (22/38-57%) antigens compared with the normal Caucasian population was recorded. As clinical characteristics, no significant differences were recognized between DR11 positive and DR11 negative patients. In response to IFN treatment, a complete response (CR) was achieved in 20% of DR11 patients and in 46% of DR11 negative patients. In this study an increase frequency of B17 and DR11 antigens was found; furthermore DR11 positive patients seem to be less responsive to IFN treatment but a larger series of patients will have to be studied in the future in order to establish this observation with more certainty.
A 73-year-old man with hydatid disease developed autoimmune hemolytic anemia due to IgM cold agglutinin with anti-I specificity. Hemolysis decreased after initiating mebendazole therapy. The cysts notably diminished in size and the red cell autoantibodies disappeared at the end of this treatment. On the basis of these observations, 44 patients with hydatid disease were investigated. One patient showed IgM cold autoantibody with no signs of anemia. In addition, cleavage fragments of C3 were detected on the erythrocyte membranes of 6 patients, following chronic activation of the complement system. We suggest that parasitic antigens may evoke antibodies cross-reacting with the red blood cells of the host.
Thyroid function was evaluated in patients affected by Fisher-Evans syndrome (FES) and compared to that of patients affected only by autoimmune hemolytic anemia (AIHA) and to that of patients affected only by idiopathic thrombocytopenic purpura (ITP). The study population consisted of 20 patients with FES, 44 with AIHA and 20 with ITP. All patients were examined for thyroid function abnormalities and thyroid autoantibodies. Abnormal thyroid function test results were observed in 40, 25 and 10% of the patients, respectively. The prevalence of antithyroid antibodies (ATA) in FES was 25%; this is higher than the sum of the prevalences of ATA in patients affected only by AIHA (11.4%) or only by ITP (none). Subclinical primary hypothyroidism and hyperthyroxinemia with or without hypertriiodothyroninemia, with TSH serum levels below normal, were present in 20% and 10% of patients affected by FES, respectively. Of the former, 75% were positive for ATA. These results: i) confirm the high prevalence of abnormal thyroid test results in patients affected by AIHA, ITP and FES; ii) demonstrate the higher prevalence of autoimmune hypothyroidism in FES; iii) lead to the possibility of including FES as one of the multiple autoimmune syndromes.
The increasingly common use of seat belts in the standard features of cars has markedly reduced the seriousness of lesions due to motor vehicle accidents but, at the same time, has caused the appearance of a specific pattern of injuries strictly related to the type of restraint. Thus, the seat belt syndrome consists of soft-tissues, skeletal and visceral injuries associated with the use of the passenger restraint system. After describing the site of injuries, we report the most common features of traumatic lesions, also comparing the consequences of the three possible occurrences: unrestrained, with two- and three- point restraints. With lap seat belts, which are the less complex, injuries most frequently involve the abdomen and vertebrae while with the three-point variety of seat belts the chest and shoulder girdle are involved. No matter what the type of restraint, skin abrasions are nearly always observed, and associated with internal injuries in 30% of cases. From the analysis of distribution, type and seriousness of injuries we can summarize that: a) the use of passenger restraint systems has surely reduced mortality and morbidity associated with motor vehicle accidents; b) the seat belt will never be able to avoid traumatic events because strong forces act and strike in a very short time on small areas of textile fabric; c) seat belts can be still considered the easiest, most reliable and cost-effective preventive method.
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Five patients who received cyanidanol for 4-36 months are presented. Three developed both hemolytic anemia and thrombocytopenia, while 2 had only thrombocytopenia. After suspending the drug the hematological values returned to normal in all of the patients. Drug-dependent platelet antibodies were detected in 4 of the 5 patients and cyanidanol-dependent red blood cell antibodies were present in 3. There are various mechanisms involved in the cyanidanol-induced immune cytopenias and, as in the present study, were sometimes simultaneously observed in the same patient.
The authors describe the case of a 75-year-old female who was hospitalized for anemia of unknown origin. Physical examination revealed a swelling in the right mammary region, where a mastectomy scar was present from surgery for a breast carcinoma. On admission, laboratory tests disclosed anemia (Hb, 8.5 g/dl), with a reticulocyte count of 65,000/mm3 and slightly increased bilirubin. Immunohematologic study revealed the presence of a red cell autoantibody with anti-D specificity in the serum and in the eluate from the patient's erythrocytes. A biopsy of the swelling was performed and histologic examination showed the presence of metastatic cells of breast carcinoma. The patient was given chemotherapy and radiotherapy. At this writing the anemia was absent, the immunohematologic study was negative, the swelling was greatly reduced, and no other metastatic lesions of breast carcinoma were present.
Antiglobulin test (AT) and Dixon tests were performed in 100 patients with CLL. Thirty-five of them had Rai stages 0 or 1, 19 stage 2, 13 stage 3, and 33 stage 4. Twelve patients showed red blood cells autoantibodies (RBCAb) positivity; positivity at Dixon test (direct, indirect, or both) was observed in 74%. The presence of autoantibodies against erythrocytes and platelets did not influence survival curves, but anemia and thrombocytopenia are considered risk factors, independently of the presence of an autoimmune disorder. Nine RBCAb positive patients with positive Dixon test had the worst survival curves, 5 of these were anemic and 1 thrombocytopenic and anemic.
Four cases of spontaneous regression of herniated nucleus polposus are described, which were followed after conservative medical treatment. Three female and one male patients who suffered from severe lumbago were evaluated; their age ranged 24-59 years. From the first CT scan on which herniation was diagnosed to follow-up control after complete regression of symptoms an average period of 14 months elapsed. Comparing ours with literature data, a substantial agreement was observed of both timing of spontaneous regression and location (most often involved was the last lumbar intervertebral space). The age of one of our patients (59 years) is to be stressed, which is well above the mean age reported in literature (35 years). The pathogenesis of regression is still unknown. The most reliable hypothesis seems to be that of dehydration and progressive atrophy of the herniated nucleus polposus. The appearance, in some cases, of the vacuum disk phenomenon seems to support such a thesis. Therefore, the diagnosis of a herniated nucleus polposus of relatively small size, still contained in an intact posterior longitudinal ligament, widely justifies the choice of waiting tactics.
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Antiglobulin Test (AT), and Dixon Tests (DT) have been studied in 100 patients with CLL. Thirty-five patients were Rai stage 0 and I, 19 patients stage 2, 13 patients stage 3 and 33 patients stage 4. Twelve patients showed a Red Blood Cells Autoantibodies (RBCAb) positivity; a positivity (direct, indirect, or both) of DT was present in 74% of patients. The presence of autoantibodies against erythrocytes and platelets did not influence survival curves, but anemia and thrombocytopenia are risk factors for survival, independently of the presence of an autoimmune disorder. Nine patients RBCAb positive and DT positive showed the worst survival curve, five out of these were anemic and one thrombocytopenic and anemic.
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The paper deals with sixteen cases of acetabular fractures studied with CT. After a short description of the normal CT findings, the different kind of fractures are reported. The usefulness of CT examination in evaluating acetabular fractures and their complications is confirmed both in conservative treatment and surgical approach.
We describe the first case of paroxysmal nocturnal hemoglobinuria with Evans' syndrome. The immunohematological studies of this patient, a 27-year-old man, revealed the presence of red cell and platelet autoantibodies, related to an episode of anemia and thrombocytopenia.
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