Sideroblastic anaemia with reactive thrombocytosis versus myelodysplastic/myeloproliferative disease.

The reported incidence of thrombocytosis among cases of refractory anaemia with ring sideroblasts (RARS) may be as high as 15%. We report four additional cases of this association, which appear to be hematologically heterogeneous. One patient clearly represents a case of RARS with reactive thrombocytosis. Two cases have features suggestive of the coincidental occurrence of essential thrombocythemia and RARS. The fourth case could be best classified as a subtype of myelodysplastic/myeloproliferative disease unclassifiable (MDS/MPD U). Only new biological or molecular markers will allow better differentiation between these disorders.

Epstein-Barr virus detection in neck metastases by polymerase chain reaction.

Cervical nodal metastasis from occult carcinomas represents a diagnostic challenge. This is a common presentation of undifferentiated nasopharyngeal carcinoma (UNPC), but metastatic carcinomas from other sites must be considered. UNPC has the distinguishing feature of a close association with Epstein-Barr virus (EBV). Since the polymerase chain reaction (PCR) can detect EBV in archival tissues, it offers significant advantages over previous methods for the detection of viral genomes. Its extreme sensitivity allows analysis of small samples from needle aspirates. Using the polymerase chain reaction to amplify EBV sequences from archival tissues, 15 of 18 NPC samples were positive for EBV. Of these 18, 14 of 14 with UNPC were positive, 1 of 2 with squamous cell carcinoma (SCC) were positive, and 10 of 2 with adenocarcinoma were positive. All 6 UNPC metastatic to lymph nodes were positive. Carcinoma metastatic to cervical nodes from 17 of 17 non-UNPC occult primaries lacked EBV. This demonstrates the utility of EBV detection by the polymerase chain reaction in the evaluation of patients with metastases to neck nodes from occult primary carcinomas in order to identify cases of UNPC.

Sclerosing hemangioma of the lung. An immunohistochemical study of intermediate filaments and endothelial markers.

Three cases of so-called pulmonary sclerosing hemangioma have been studied for endothelial markers (alkaline phosphatase, adenosine triphosphatase, factor VIII-related antigen, and Ulex europaeus I lectin), for intermediate filaments (keratin, vimentin), and for carcinoembryonic and epithelial membrane antigen. Not one of the neoplasms expressed endothelial markers, carcinoembryonic antigen, or keratin reactivity. The tumor cells showed a positive reaction for epithelial membrane antigen and vimentin. The findings exclude an endothelial origin for this group of tumors and favored an epithelial origin as the probable genesis of the neoplastic proliferation.

[Non-Mediterranean lymphoma producing heavy alpha chains]. / Linfoma no mediterráneo productor de cadenas pesadas alfa.

At the present, immunoproliferative small intestinal disease (IPSID) is considered a fairly homogeneous entity. Isolated heavy chain production is so closely related with IPSID that has been considered as a natural biologic marker for it. By contrast, we report here the case of a 63-year-old female that developed a multinodular small bowel lymphoma without clinical malabsorption symptoms. The main tumour mass was located in proximal jejunum and the neighbour intestinal mucosa did not show neoplastic cell infiltrates. Immunohistochemical methods demonstrated restrictive presence of IgA1 subclass in the neoplastic cells without presence of light chains. Cases as present are extremely infrequent and their potential relationship with IPSID has not been completely outlined, being useful in trying to understand the spectrum of gut lymphomas.

[The determination of monoclonality in B-cell lymphomas using the polymerase chain reaction technic on paraffin-embedded material]. / Determinación de monoclonalidad en linfomas B utilizando la técnica de reacción en cadena de la polimerasa semiacotada sobre material de parafina.

BACKGROUND: Clonal rearrangements of immunoglobulin are useful in the diagnosis of B-cell lymphomas. The value of the polymerase chain reaction (PCR) with consesuated primers in the variable (V) and joining (J) region is now well established. METHODS: Forty cases of B-cell lymphomas and 10 patients with reactive lymphoid proliferations have been studied. All cases were studied with two different semiacotated techniques, with similar primers in the J region (LJH and VLJH) and changing the primers of the V region (Fr2A and Fr3A). RESULTS: A 100% specificity and positive predictive value were obtained with both techniques. The sensitivity was 90% with Fr2A and 82% with Fr3A, but in 20% of the studies with Fr2A the extracted DNA was insufficient to perform the study while it could be done with Fr3A in all cases. The negative predictive value was 76% with Fr2A and 58% with Fr3A. CONCLUSIONS: Both techniques showed a good sensitivity, optimal specificity and positive predictive value, with low negative predictive value. Although the results are somewhat higher with Fr2A, this technique needs well preserved DNA and it can be an important set back when we deal with formaldehyde fixed, paraffin embedded tissues.

[Chronic autoimmune hepatitis following cholestatic hepatitis caused by droxicam]. / Hepatitis crónica autoinmune después de una hepatitis colestásica inducida por droxicam.

Droxicam in a nonsteroid antiinflammatory from the oxicam family which acts as a pro-drug, being transformed into pyroxicam after being hydrolized in the stomach and has induced several cases of cholestatic or mixed hepatitis. A clinical observation in which droxicam provoked initial cholestatic hepatitis which later developed into chronic autoimmune hepatitis is presented. It has been postulated that, after causing cholestatic hepatitis by hypersensitivity and within the context of a previous autoimmune entity such as vitiligo, this drug triggered a silent autoimmune liver disease which was demonstrated clinical, analytical and histopathological manifestations 18 months later and required permanent immunosuppressive treatment.

[Massive rectal hemorrhage caused by nonspecific colonic ulcers]. / Rectorragia masiva por úlceras cólicas inespecíficas.

Nonspecific colon ulcers are a rare disease which may produce lower gastrointestinal bleeding. A history of previous nonsteroidal anti-inflammatory drugs ingestion may be recorded in most patients. The right colon is the main location, being the cecum the most frequent site. However, they may be distributed in all the large intestine, including the rectum. We present a case of lower massive gastrointestinal hemorrhage produced by nonspecific colic ulcers requiring emergency total colectomy. Although infrequent, they have to be considered in every patient with lower gastrointestinal bleeding and previous nonsteroidal anti-inflammatory drugs ingestion.

[Surgery of benign obstruction of the bile tract. Current opinion status]. / Cirugía de la obstrucción benigna de la vía biliar. Estado actual de opinión.

Treatment of the benign obstruction of the main bile duct remains controversial. A questionnaire, containing the different aspects of the treatment, was mailed in order to learn the general opinion about its management in our country; 140 specialists were surveyed with the following results: 35% gave valid answers. Most of those surveyed (85%) use antibiotic prophylaxis, preferably (79%) pre and postoperatively. Ultrasonography is used in 100% of the cases, and the second most common examination is endoscopic retrograde colangiopan-creatography. Among the intraopreoperative examinations, colangiography is the most used (100%), followed by choledochoscopy. A majority (90.4%) of those surveyed employ the T-tube and 67.5% prefer choledochoduodenostomy if an internal bile drainage must be performed. In obstructive jaundice, only 27.5% recommend preoperative percutaneous drainage. Endoscopic sphinterotomy is the elective technique for suppurative cholangitis and for patients with choledocholitiasis and previous cholecystectomy.

[Cryoglobulinemia following liver transplantation for cirrhosis secondary to hepatitis C virus infection]. / Crioglobulinemia tras trasplante hepático por cirrosis secundaria al virus de la hepatitis C.

Hepatitis C virus (HCV) infection has been linked with some extrahepatic immunologic abnormalities. Cryoglobulinemia is one of the most frequently reported. Nevertheless, there are only a few reports of cryoglobulinemia in the setting of liver transplantation. More studies are needed to clarify the frequency of post-OLT cryoglobulinemia in patients with HCV-related cirrhosis and its impact on OLT outcome. A case of a patient who underwent liver transplantation because of HCV end-stage liver disease and in whom cryoglobulinemia appeared 3 years after transplantation is reported. Treatment with cyclophosphamide and steroids was attempted but patient died of septicemia 3 years after liver transplantation.

[Cutaneous erythematous rash as first manifestation of T-cell prolymphocytic lymphatic leukemia]. / Erupción cutánea eritematosa como primera manifestación de leucemia linfática prolinfocítica de células T.

We present a case of a man 84 years-old, whose presentation feature was a cutaneous inespecific rash, and was diagnosed of T prolymphocytic leukaemia (T-PLL). In this review we analyze actual aspects concerning biology, diagnosis, classification, prognosis and treatment of this rare mature T cell leukaemia.

[Cardiocirculatory effects of intravenous anesthetic induction in an experimental model of acute hypovolemia]. / Efectos cardiocirculatorios de la inducción anestésica intravenosa en un modelo experimental de hipovolemia aguda.

OBJECTIVE: To evaluate the cardiovascular effects of ketamine, midazolam, thiopentone and propofol in acutely hypovolemic pigs and to determine whether the association of ketamine and midazolam offers any advantage. PATIENTS AND METHODS: Twenty-two Landrace-Large-White pigs. After monitoring was begun, acute hypovolemia was induced by means of rapid exsanguination of 30% of calculated volume. Hemodynamic variables were measured: a) at baseline; b) after exsanguination; c) 2 min after anesthetic induction; d) 10 min after anesthetic induction, and e) after reinfusion of the exsanguinated volume. RESULTS: All pressures, cardiac output, cardiac index, and mixed venous oxygen saturation fell significantly with the induction of hypovolemia. Heart rate, systemic vascular resistances and arteriovenous oxygen differential increased. Ten min after anesthetic induction, heart rate in the midazolam group was significantly lower than in the ketamine-midazolam group. Arterial pressures decreased significantly after anesthetic induction with all drugs. The decrease in systolic arterial pressure was smaller in thiopenthal-anesthetized pigs than in pigs receiving either midazolam or propofol at the 10 min recording. The decrease in mean arterial and diastolic pressure after 10 min was smaller with thiopental than with any other drug. The decrease in mean arterial pressure was less in the thiopental and ketamine-midazolam groups than in the others after reinfusion. Diastolic arterial pressure at 10 min and after reinfusion had decreased less in the thiopental and ketamine-midazolam groups than in the propofol group. After anesthetic induction, the post-hypovolemic figures for cardiac output and cardiac index held steady or changes were slightly accentuated, with no statistically significant differences among the groups. CONCLUSIONS: The intravenous anesthetics evaluated were detrimental to cardiovascular function in acute hypovolemic pigs. Low-dose thiopental and ketamine plus midazolam may be the anesthetics of choice in this setting. Propofol caused the greatest degree of hemodynamic instability.