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Biomedical subjects

M Narbaitz

Publications and source records attributed to M Narbaitz.

7 recordsLinked to original sources

Catastrophic antiphospholipid syndrome and Kikuchi-Fujimoto disease: the first case reported.

The case of a man with diagnosis of Kikuchi-Fujimoto disease (KFD) and catastrophic antiphospholipid syndrome (CAPS) is reported. He presented prolonged fever, lymphadenopathies, arthralgia, weight loss, hepatosplenomegaly and positive IgM for cytomegalovirus. While he was empirically treated with tuberculostatic drugs, he suddenly developed systemic inflammatory response syndrome, multiple organ failure and distal necrosis. On suspicion of severe sepsis, antibiotics, corticoids and recombinant human activated protein C (XIGRIS) were administrated. Exhaustive laboratory searching was negative. Histopathologic examinations of lymph node first disclosed malignant lymphoma but later KFD was confirmed. One month later, laboratory tests showed the presence of antiphospholipid antibodies (aPL). The patient was discharged after two months of hospitalization. This case exhibits a KFD complicated by definite CAPS. Cytomegalovirus could be involved in the development of both CAPS and KFD. Because of the severity of the case, we believe that XIGRIS noticeable improved the altered coagulation profile associated with CAPS.

Adult↗

[Primary esophageal lymphoma in a patient with AIDS].

Primary esophageal lymphomas are extremely rare. We report a primary esophageal T cell lymphoma of a diffuse large cell phenotype B in a patient with AIDS. Also we reviewed other published cases. The diagnosis of this complication should be considered in HIV seropositive patients with progressive displagia and endoscopic findings of masses, polyps or ulcerations and, specially in those unresponsive to antifungal or antiviral therapy. Biopsy and histopathologic studies are needed to confirm the diagnosis.

Adult↗

Bcl-2 molecular analysis in paraffin-embedded biopsies from diffuse large B-cell lymphomas.

Translocation t(14; 18) has been observed in 50-85% of follicular and in 30% of diffuse non-Hodgkin lymphomas. About half of follicle center lymphoma (FCL) undergo histological conversion at relapse to more aggressive diffuse large B-cell lymphoma (DLBCL). This report correlates the molecular bcl-2/IgH rearrangement by PCR and Bcl-2 immunohistochemical (IHC) expression in a series of high grade DLBCLs with and without FCL remnant. Twenty-three paraffin-embedded lymph nodes from DLBCL patients were analyzed. Eleven patients showed FCL remnant (Group A) and 12, did not (Group B). Single PCR from paraffin extracted DNA followed by Southern transfer of products, hybridisation with internal oligoprobes for the MBR/JH and MCR/JH bcl-2 rearrangements and IHC analysis of Bcl-2 expression, were performed. PCR analysis was positive in 34.8% of patients. Bcl-2/IgH gene rearrangements were observed in 8 (34%) cases and 7 (30%) showed Bcl-2 expression on large noncleaved B-cells (centroblasts). All patients from Group A showed IHC positive reaction on FCL remnant (small cleaved cells) but only 2 (18%) were positive in DLBCL areas, suggesting either the loss of the bcl-2 expression on the transformed lymphoma, or, alternatively, the development of a second disease when the first lymphoma transforms. Group B patients showed a clear correlation between PCR and IHC studies. Our results suggest a similar frequency of t(14; 18) in DLBCLs to that reported in Europe and USA series. The discordance observed between PCR and IHC, particularly in Group A, points out the necessity to perform both studies in order to detect bcl-2 gene involvement in DLBCLs.

Adult↗

[Hairy cell leukemia. An alternative method for the detection of minimal residual disease using flow cytometry].

In patients with hairy cell leukemia (HCL) who received chemotherapeutic treatment and achieved complete remission (CR), minimal residual disease (MRD) can be detected in bone marrow biopsies using immunohistochemical (IHC) techniques. In this study, we investigated the value of flow cytometry (FCM) and IHC to detect MRD and to establish whether MRD+ could predict relapse. A total of 15 HCL patients in CR were studied. Samples of bone marrow (BM) and peripheral blood (PB) were processed by FCM with triple staining of the following monoclonal antibodies (mAbs): CD20, CD22, CD11c, CD103, CD25, anti-Kappa and anti-Lambda light chains. Reference values were obtained from normal samples of peripheral blood and bone marrow. FCM detected MRD in 64% of the patients. BM samples were more demonstrative than peripheral blood for MRD detection in HCL. IHC was performed in paraffin-embedded BM biopsies using CD20 and DBA44 mAbs. MRD+ was detected in 46% of patients. Although not statistically significant, FCM appeared more sensitive compared with IHC. Detection of MRD by either of these methods in our series did not predict hematological relapse. The results show that FCM is a useful alternative method to detect MRD in HCL and that a longer, follow-up is required to establish the predictive outcome of MRD+ patients.

Antibodies, Monoclonal↗

[Gastric solitary plasmacytoma associated with +Helicobacter pylori infection].

Solitary gastric plasmacitomas are infrequent tumors. They account for 5% of the extramedullary plasmacitomas. We report an unusual case in a 14 years old boy. The patient has had gastric symptoms for 2 years prior to an endoscopic examination. A fungating, ulcerated lesion was observed in the antrum. The biopsies showed a monoclonal, Lambda positive, diffuse, plasmocitic proliferation infiltrating the mucosa. Also a moderate number of Helicobacter pylori were identified in the gastric pits and numerous lymphoid follicules were observed in the deep portion of the mucosa. In view of the presence of HP infection the patient was treated with Orneprazole and Clarithromycin. Endoscopic examination and biopsies performed 3 and 5 months later showed a complete remission of the gastric lesion. At the time of this report the patient is in good physical condition, has recovered his weight and has grown 5 cm. Differential diagnosis with plasmo limpho in chronic gastritis and with lympheicitic lymphoma with plasmocitoid features had to be done. The macroscopic appearance of the gastric lesion, the absence of other inflammatory cells and monoclonality of the plasmocitic infiltration ruled out chronic gastritis. The negative staining for CD 20 as well as the abscence of lymphoid cells in the mucosal infiltrate give support: to the diagnosis of plasmocitoma. The close association between gastric MALT lymphoma and HP infection has been reported as well as its regression after antibiotic treatment for its erradication. In our review of the literature we failed to find any references to the association of HP with gastric plasmocitoma nor to its regression after antibiotic therapy.

Adolescent↗

IgA from HIV+ haemophilic patients triggers intracellular signals coupled to the cholinergic system of the intestine.

IgA was obtained from HIV-infected haemophilic patients and the intracellular signals triggered by its reaction with isolated rat intestinal strips were studied. HIV+ IgA stained intestinal microvilli with a granular immunofluorescence pattern and bound to the muscarinic acetylcholine receptor (mAChR), displacing the specific muscarinic cholinergic antagonist QNB in a non-competitive manner. It triggered the signals that are the consequence of mAChR stimulation in the intestine. Thus, it decreased cAMP synthesis and increased guanosine 3':5'-cyclic monophosphate (cGMP) formation and phosphoinositide (PI) turnover of the intestine. In addition, it stimulated prostaglandin E2(PGE2) synthesis by intestinal strips. Through its effect on PGE2 synthesis, HIV+ IgA could have a dual action. On the one hand, it could enhance immunosuppression at a local level, favouring pathogen growth and subsequent intestinal dysfunction. On the other hand, PGE2 could directly increase intestinal motility and electrolyte/fluid loss. Both effects could be involved in intestinal damage in AIDS.

Animals↗

Variant (8;22) translocation in lymphoblastic lymphoma.

Occasional cases with the chromosomal translocations that juxtapose the c-myc oncogene to Ig genes have been reported in large cell, small lymphocytic, immunoblastic, and follicular lymphomas. In this report, we present a 20 year-old female with lymphoblastic lymphoma of B-cell type and a translocation t(8;22), in which cytogenetic, immunologic, and molecular studies were performed. To the best of our knowledge this is the first report of this association.

Adult↗