The JAK2 V617F mutation occurs frequently in myelodysplastic/myeloproliferative diseases, but is absent in true myelodysplastic syndromes with fibrosis.
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Diagnosis, Differential↗
Biomedical subjects
L Quintanilla-Martínez
Publications and source records attributed to L Quintanilla-Martínez.
p27(Kip1) immunostaining for the differential diagnosis of small b-cell neoplasms in trephine bone marrow biopsies.
The distinction between mantle cell lymphoma (MCL) and other small B-cell non-Hodgkin lymphomas (NHL) is important because MCL has a more aggressive clinical course. In bone marrow (BM) biopsy specimens, this distinction can be particularly difficult. Although cyclin D1 immunostaining and molecular detection of the t(11;14) translocation are highly specific markers for MCL, they fail to detect a proportion of cases. We have recently described that MCL typically lacks detectable expression of the cyclin-dependent kinase inhibitor p27(kip1) protein by immunostaining, which is expressed at high levels in most small B-cell NHL inversely correlated to the proliferation rate. We therefore examined whether p27(kip1) immunostaining could be a useful adjunct for the differential diagnosis of small B-cell NHL infiltrates in the BM. Trephine BM biopsy specimens of 96 patients, including well-characterized MCL (19 cases), B-cell chronic lymphocytic leukemia (27 cases), follicular lymphoma (18 cases), hairy cell leukemia (22 cases), and marginal zone lymphoma (10 cases) as well as 10 reactive BM, including five with benign lymphoid aggregates were investigated. In addition, the presence of a t(11;14) translocation involving the major translocation cluster was studied by PCR in all MCL. All cases of B-cell chronic lymphocytic leukemia, follicular lymphoma, and marginal zone lymphoma revealed a strong p27(kip1) nuclear staining in the majority of neoplastic cells. Fourteen (78%) cases of MCL were p27(kip1)-negative in the tumor cells, whereas four cases revealed a weak nuclear positivity. Seventeen (77%) cases of hairy cell leukemia were also either completely negative for p27(kip1) or showed a faint positive staining in a minority of the neoplastic cells. Nine of 19 cases (47%) of MCL showed a bcl1 rearrangement involving the major translocation cluster region. These findings demonstrate that p27(kip1) immunostaining is a valuable additional marker for the differential diagnosis of small B-cell NHL infiltrates in BM biopsies. The reduction or lack of p27(kip1) protein expression in MCL, as well as in hairy cell leukemia, might be an important event in the pathogenesis of these disorders.
Biopsy↗
Primary intestinal non-Hodgkin's lymphoma and Epstein-Barr virus: high frequency of EBV-infection in T-cell lymphomas of Mexican origin.
Epstein-Barr virus is universally associated with endemic Burkitt's lymphoma (BL) and undifferentiated nasopharyngeal carcinoma and can be detected in a significant proportion of cases of Hodgkin's disease (HD) and peripheral T-cell lymphoma, but only rarely in sporadic B-NHL. The frequency of EBV-positivity in certain neoplasms shows important geographic variations. Both HD and sporadic BL from Latin America have shown higher rates of EBV-association than cases from Western countries. In T-NHL, the frequency of EBV-positivity is influenced by the site of the primary tumor and the phenotype of the neoplastic cells. Nasal and nasal-type T-NHL, which show a T/NK-cell phenotype with expression of CD56 are virtually always EBV-associated, whereas only a proportion of nodal, gastrointestinal and pulmonary T-NHL are EBV-infected. A recent investigation of primary intestinal lymphomas of Mexican origin demonstrated EBV-positivity in all examined cases of T-NHL and BL and a proportion of other B-NHLs. The presence of EBV was independent of the presence or absence of enteropathy. Two of 6 cases studied showed CD56 expression. The high rate of EBV-positivity independent of histologic subtype is in contrast to the low to intermediate rates of EBV-positivity found in cases of intestinal T-NHL from Western countries and indicates that geographic differences in the frequency of EBV-association of lymphoid neoplasms might also extend to a fraction of peripheral T-cell lymphomas.
Burkitt Lymphoma↗
Post-transplantation lymphoproliferative disorders in Mexico: an aggressive clonal disease associated with Epstein-Barr virus type A.
Post-transplantation lymphoproliferative disorders (PT-LPDs) are a complication of immunosuppression with variable clinical behavior and frequent Epstein-Barr virus (EBV) association. There is geographic variation in the association of EBV with certain tumors and a lack of studies of PT-LPDs from developing countries, so we decided to study in detail a series of PT-LPDs from Mexico to identify similarities and differences between populations in Mexico and those in Europe and the United States. We used paraffin-embedded tissue from eight PT-LPDs (six from men, two from women) that arose after renal transplantation. Clinical data, morphologic features, and clonality on the basis of immunoglobulin (Ig) light chain restriction, as well as polymerase chain reaction (PCR) for Ig heavy chain genes, were studied. The presence of EBV was investigated with PCR, immunohistochemical analysis for latent membrane protein (LMP)-1, and in situ hybridization for EBV early RNA transcripts. In addition, the subtype of EBV based on the EBNA 2A and 2B genes and the presence of a 30-bp deletion in the LMP-1 gene were investigated by PCR. Seven (87.5%) of eight cases presented with gastrointestinal involvement; five patients died. Three cases were polymorphic PT-LPDs, four were monomorphic large cell lymphomas (one diffuse large cell, three immunoblastic), and one was unclassifiable. All showed a B-cell phenotype, with a clonal population demonstrated in seven of the eight cases. Tumor cells expressed EBERs in all of the cases and LMP-1 in six of seven evaluable cases. Seven of seven cases showed EBV subtype A. Two (25%) of eight cases had the 30-bp LMP-1 deletion. This study shows that PT-LPDs in Mexico are clonal disorders associated with EBV subtype A. In contrast to series from Europe and the United States, our cases showed a significantly higher incidence of gastrointestinal tract involvement (P < .001), and a lower incidence of the 30-bp LMP-1 deletion, although this was not statistically significant (P < .28).
Adult↗
Primary non-Hodgkin's lymphoma of the intestine: high prevalence of Epstein-Barr virus in Mexican lymphomas as compared with European cases.
Recent studies in Western European populations have shown that peripheral T-cell non-Hodgkin's lymphomas (T-NHLs) are associated with Epstein-Barr virus (EBV) in a higher percentage than sporadic B-cell NHL (B-NHLs), and that the frequency of EBV-positivity might be influenced by the primary site of the tumor. Because of the geographic differences in EBV expression in Burkitt's lymphoma (BL) and Hodgkin's disease (HD), and the lack of studies of sporadic NHL from developing countries, we decided to survey the presence of EBV in a series of primary intestinal lymphomas from patients in Mexico and in Western Europe, and to analyze whether EBV status is influenced by tumor phenotype, and geographic or ethnic determinants. Paraffin-embedded tissue from 43 primary intestinal NHLs (19 cases from Mexico and 24 from Western Europe) were examined, including 17 high grade B-NHLs, 9 low grade B-NHLs, and 17 T-NHLs; 6 of which were enteropathy associated T-cell lymphomas. The distribution of histologic subtypes was similar in both groups. The presence of EBV was investigated with a combined approach using a nested polymerase chain reaction technique as well as immunohistochemistry for latent membrane protein-1 and in situ hybridization for EBV early RNA transcripts (EBER 1/2) RNAs. The median age of the Mexican patients was significantly lower than the median age of the European patients (32 v 62 years). This difference was most pronounced in patients with T-cell lymphoma (24 v 63 years). EBER-positive tumor cells were detected in 13 of the 43 (30%) cases of primary intestinal lymphoma, including 5 of 26 sporadic B-NHL (3 high grade and 2 low grade), and 8 of 17 T-NHL, all of which were classified as pleomorphic, medium and large cell. The rates of EBV-positivity were markedly different for European and Mexican cases. Whereas 7 of 7 (100%) T-NHL and 5 of 12 (42%) sporadic B-NHL of Mexican origin were EBER-positive, only 1 of 10 T-NHL and 0 of 14 sporadic B-NHL from Europe showed EBER expression in tumor cells. Latent membrane protein was positive in only 2 of 43 cases, 1 of which was an EBER-negative high grade B-NHL from Mexico that showed intact total mRNA in control hybridization. CD30 expression was found in 4 of 8 EBV-positive T-NHL and in none of the EBV-positive B-NHL. In contrast to European cases, intestinal NHLs from Mexico show a very high frequency of EBV-positivity, which is not limited to T-NHL, but includes a significant proportion of B-NHL. This study strongly suggests that similar to HD and probably BL, there are important epidemiologic differences in EBV association in intestinal T-cell NHL between European and Mexican populations. These differences might be the result of environmental factors, for example, earlier contact with childhood viruses on intestinal lymphomagenesis.
Adolescent↗
Clinical and pathological characterization of posttransplantation lymphoproliferative disorders in kidney transplant recipients: single institution experience.
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Adolescent↗
Extramedullary tumors of lymphoid or myeloid blasts. The role of immunohistology in diagnosis and classification.
The diagnosis of primitive hematologic malignancies in extramedullary sites (lymphoblastic lymphoma of T- or B-cell type and myeloid sarcoma) on paraffin-embedded tissue sections is difficult and often impossible because of the primitive morphology of the neoplastic cells. The authors studied 21 extramedullary tumors of lymphoid or myeloid blasts. They used a panel of 22 antibodies on frozen sections and 9 antibodies on paraffin sections to determine the spectrum of immunophenotypes and to develop a practical panel for diagnosis. All but two of the cases could be classified as lymphoid or myeloid using immunohistologic analysis. Thirteen cases were classified as lymphoblastic lymphoma/acute lymphoblastic leukemia (LBL/ALL); 10 were classified as precursor T (CD7+, CD3+/-, CD45+) and 3 as precursor B-cell (CD19+/-CD10+CD45-) type. Five cases were classified as myeloid sarcoma (CD13+ myeloperoxidase+, lysozyme+). Two LBL/ALL coexpressed either CD33 (1 case) or CD15 (1 case), and one myeloid sarcoma coexpressed TdT and CD7. One case appeared to be truly mixed lineage, coexpressing CD3 with myeloperoxidase and lysozyme, and two cases expressed no lineage-specific antigens. There were clinical differences between the three major tumor types, and within the category of T-precursor LBL/ALL, classification according to stage of thymocyte differentiation was associated with distinctive clinical features. In conclusion, the spectrum of immunophenotypes detected on frozen section was similar to that reported by flow cytometry on peripheral blood and bone marrow specimens. The most useful antigens on frozen sections were CD7 and CD3 (T cell), CD10 and CD19 (B cell), and CD13 (myeloid). TdT was coexpressed by one myeloid sarcoma and was undetectable in 40% of LBL/ALL. On paraffin sections, myeloperoxidase and lysozyme were reliable markers of myeloid lineage, but none of the markers used on paraffin sections distinguished between LBL/ALL of T- and B-precursor types. Both B-LBL/ALL and myeloid sarcomas were often CD45- on paraffin sections, which may be a obstacle in determining the diagnosis. These distinctions appear to have clinical relevance.
Adolescent↗
Peripheral neuroectodermal tumor of the chest wall in a patient treated for Hodgkin's disease.
The occurrence of second malignancies after treatment for Hodgkin's disease is a well recognized phenomenon. In this report we describe a case of a 22-year-old male who developed a peripheral neuroectodermal tumor of the chest wall four years after completing successful treatment with combined chemotherapy and radiotherapy for Hodgkin's disease.
Adult↗
Association of Epstein-Barr virus latent membrane protein and Hodgkin's disease in Mexico.
Epstein-Barr virus (EBV) has been demonstrated in association with Hodgkin's disease (HD) in approximately 40 to 50% of cases in series from North America, Europe, and Japan. However, few data are available concerning this association in developing countries. Recent studies, including mostly a pediatric population from Peru and a pediatric population from Honduras, showed a higher percentage of EBV positivity compared with those in developed countries. To determine the prevalence of EBV in Hodgkin's disease in a Mexican adult population, we analyzed 50 formalin-fixed, paraffin-embedded cases of HD by a three-step technique using a monoclonal antibody to the latent membrane protein of EBV. All the cases were confirmed immunophenotypically as HD (CD15+ and/or CD30+, CD45-). Reed-Sternberg cells and variants were positive for LMP in 35 cases (70%). The staining was found both on the cell surface and/or within the cytoplasm with enhancement of the Golgi area. EBV latent membrane protein was found in 1/1 case (100%) of diffuse lymphocyte-predominant HD, 10/20 cases (50%) of nodular-sclerosis, 18/22 cases of mixed-cellularity (81%), and 6/7 (86%) cases of lymphocyte-depleted HD. Our results show a high prevalence of EBV in HD in this Mexican adult population. All histologic subtypes of HD in the population analyzed appear to be strongly associated with EBV, in contrast to the strong association of mixed cellularity HD in developed countries. Patient age and gender were not predictive of the presence of EBV.
Adolescent↗
Primary carcinoid of the common bile duct. Immunohistochemical characterization of a case and review of the literature.
A 39-year-old woman with a primary carcinoid of the common bile duct is presented. The tumor showed argyrophilia; and, by immunohistochemical studies, chromogranin, serotonin, and somatostatin were demonstrated. The patient has no symptoms 42 months after surgical treatment. To the authors' knowledge, this is the first report of a carcinoid of the common bile duct as studied by immunohistochemistry. When six choledochal carcinoids were reviewed, the following characteristics were observed: in none of the cases were endocrine symptoms present; women were affected more frequently than men; the carcinoids occurred in younger patients than did adenocarcinomas; metastases were present in half of the patients; and, as a group, carcinoids seemed to have a better prognosis than adenocarcinomas in this location.
Adult↗
[Xanthogranulomatous cholecystitis. Clinical entity or pathologic curiosity].
Xanthogranulomatous cholecystitis is an uncommon variety of gallbladder inflammation. Its histologic appearance is quite specific but its clinical characteristics, prognostic and physiopathologic implications are unknown. The present study has the aim of analyzing a group of 40 patients with xanthogranulomatous cholecystitis and another of 80 patients with non-xanthogranulomatous inflammation. The clinical characteristics, risk factors, morphologic expression, complications and mortality of both groups were compared. Diffuse xanthogranulomatous cholecystitis comprised 1.8% of all cholecystectomies done between January 1976 and July 1987. This variety was more frequent in males (p less than .05) without any specific risk factors. There was a higher frequency of acute pyogenic inflammation sometimes associated to extrinsic compression of the biliary duct and fistulization, but without any difference in the overall complication rate or mortality. We conclude that xanthogranulomatous cholecystitis is a specific entity which occurs with a higher frequency in males and that has a different clinical behavior than that of other types of gallbladder inflammation.
Adult↗
[Hepatitis C virus infection and membranoproliferative glomerulonephritis].
BACKGROUND AND METHODS: A possible association between hepatitis C virus infection (HCV) and membranoproliferative glomerulonephritis (MPGN) or membranous glomerulonephritis has recently been reported. The pathogenesis of this entity appears to be immunologically mediated. The purpose of this report is to describe the clinical, laboratory, and histopathological features of three patients with chronic HCV infection, without hepatitis B virus disease or autoimmune diseases, but with glomerular disease. RESULTS: All three patients had chronic hepatopathy stigmata, ascitis, peripheral edema, and normal blood pressure values. Laboratory results showed mild liver function abnormalities and normal levels of blood nitrogenous waste products. Microscopic hematuria, hypoalbuminemia, and variable proteinuria without hypercholesterolemia were found in all cases. All three had positive rheumatoid factor. Only one patient had positive antinuclear antibodies and antimitochondrial antibodies at low levels, and another displayed low C3 and C4 serum levels. Renal histology in the three cases showed type I membranoproliferative glomerulonephritis and hepatic cirrhosis in the liver biopsy. CONCLUSIONS: This report supports the association between chronic HCV infection and membranoproliferative glomerulonephritis. However, further studies are needed to establish more firmly the association as well as the mechanisms of pathogenesis and causality between them.
Adult↗
[Lymph node biopsy using fine-needle aspiration. Clinical and cytohistological correlation in 179 cases].
We reviewed the institutional experience with fine needle aspiration biopsy (FNAB) of peripheral lymph nodes in the Instituto Nacional de la Nutrición Salvador Zubiran from January 1988 to December 1992. A total of 298 FNAB had been performed in 288 patients. Aspirates with just blood, few stromal cells or with desiccation changes were excluded (63 cases), as well as those in whom it was not possible to establish a clinical or morphologic correlation (56 cases). In the 179 cases included, the FNAB diagnosis was histologically confirmed in 137 and in 42 cases the correlation was established by clinical follow-up and response to treatment. According to the diagnosis, aspirates were divided in benign and malignant in the analysis. We observed 70 aspirates (39%) with benign lesions and 109 (61%) with primary or metastatic neoplastic cells. The most frequent benign diagnosis was hyperplasia followed by epithelial metastatic neoplasms. In 25 aspirates there was a discrepant cyto-histologic diagnosis but only six were false negatives. No false positive or complications were identified. The FNAB in our cases showed: sensitivity 95%, specificity 100%, positive predictive value 100% and negative predictive value 91.4%. Our data support the value of FNAB as a reliable, safe, inexpensive and easy to perform method in the diagnosis of lymph node enlargements. Thus, we believe this method should be recommended as the initial approach in the study of lymphadenopathies.
Biopsy, Needle↗
[High prevalence of the Epstein-Barr virus in a Mexican population with Hodgkin's disease].
The Epstein-Barr virus (EBV) has been demonstrated in association with cases of Hodgkin's disease (HD) indicating that, in at least in some cases, EBV may play a pathogenic role in the development of HD. To determine the prevalence of EBV in HD in a Mexican adult population, we studied 39 formalin-fixed and/or B-5, paraffin embedded samples of patients with HD, by immunoperoxidase; in situ hybridization was done in 32 of the 39 cases. We analyzed the presence of the latent membrane protein (LMP) and EBV-specific DNA sequences. Reed-Sternberg cells and mononuclear variants were positive for LMP in 28 cases (72%). LMP staining was found both on the cell surface and/or within the cytoplasm with enhancement in the Golgi area. The LMP was found in the single case of diffuse lymphocyte predominant HD, in 6/12 cases of nodular sclerosis (50%), in 10/15 cases of mixed cellularity (67%) and in all eleven cases of lymphocyte depleted HD (100%). No EBV-specific DNA sequences were found by in situ hybridization. Our results show: 1) a higher association of EBV in our population compared to the approximately 48% reported in developed countries; 2) all histologic subtypes of HD in Mexico appear to be strongly associated with EBV in contrast to the strong association with only mixed cellularity seen in Western populations; 3) the high prevalence of EBV in HD in Mexico may be a function of histology, ethnic groups, socioeconomic factors and/or geography.
Adolescent↗
[Pancreatoduodenectomy in the treatment of carcinoma of Vater's ampulla].
OBJECTIVE: To analyze clinical characteristics, diagnostic methods, intraoperative findings, morbidity & mortality and outcome of a series of patients with ampullary adenocarcinoma. DESIGN: Retrospective analysis. SETTING: Tertiary-care medical center. PATIENTS: Thirty four consecutive patients who underwent radical resection between 1960 and 1992. RESULTS: The most frequent findings were jaundice (91%), weight loss (44%) and palpable gallbladder (42%). Thirty-three patients (97%) underwent Whipple resection and one total pancreatectomy (3%). The postoperative mortality was 15%. Overall survival at 1, 5, 10 and 14 years was 67, 36, 25 and 17% respectively. Fourteen patients died of tumor recurrence developed more than five years after the resection. CONCLUSION: Radical resection for ampullary carcinoma remains the procedure of first choice in these patients.
Adenocarcinoma↗
[Molecular biology in medicine. IX. In situ hybridization].
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Animals↗