Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0021051 (immunodeficiency)
 71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Plasmablastic microlymphoma (PML) is defined as the accumulation of monotypic but polyclonal plasmablasts in lymphoid tissues involved in human herpes virus 8 (HHV-8)-positive multicentric Castleman's disease (MCD). So far, the nature of this very rare condition remains poorly determined. In this study, we describe a human immunodeficiency virus (HIV)-seropositive patient who developed a PML in the setting of HHV-8-positive MCD. In contrast to the cases previously reported, most of the plasmablasts in our patient were localized within the germinal center (GC) of lymphoid follicles. These plasmablasts expressed the multiple myeloma-1/interferon regulatory factor-4 (MUM1/IRF4) protein as well as IgMlambda in a monotypic fashion. They did not show any immunoreactivity with antibodies directed against Pax-5, CD20, CD79a, CD10, CD30, CD23, CD138, epithelial membrane antigen (EMA) or BCL-6. These cells exhibited a high proliferation rate, expressed the HHV-8 latent nuclear antigen-1, and secreted the HHV-8 viral homologue of human interleukin-6. Polymerase chain reaction analysis did not demonstrate any clonal rearrangement of the genes coding for the heavy chain of the immunoglobulin. Moreover, no Epstein-Barr virus (EBV) RNA transcript could be found, using in situ hybridization. The present case illustrates that PML may arise within the GC of lymphoid follicles in the absence of EBV coinfection. In our opinion, PML occurring in MCD likely represents a variant of HHV-8-positive MCD in which lytic HHV-8 replication is particularly prominent, due to a local or systemic immune imbalance.
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PMID:Plasmablastic microlymphoma occurring in human herpesvirus 8 (HHV-8)-positive multicentric Castleman's disease and featuring a follicular growth pattern. 1761 57

HLA-DR expression on monocytes as a marker for the functioning of the immune system is known to be severely depressed in immunodeficiency. Up to now, other markers for the function of the immune system are scarce. In the peripheral blood of patients with open heart surgery the expression of the membrane peptidases neprilysin/CD10 and aminopeptidase N/CD13, was determined on granulocytes in comparison to the monocytic HLA-DR expression. We used the QuantiBRITE flow cytometry system, which yields an absolute antigen expression value (antibodies bound per cell) and may be useful in standardizing surface antigen expression analysis. This system makes use of a highly purified phycoerythrin-labeled antibody with a 1:1 fluorochrome-to-protein ratio, and multilevel calibrated beads with known absolute phycoerythrin fluorescence. Our results show that both membrane peptidases on granulocytes show a similar time-course of expression after heart surgery as do HLA-DR molecules on monocytes, with a decrease from days one to three and a subsequent recovery to normal values. In future analyses a possible relationship between the immunodeficiency of patients and a diminished expression of both membrane peptidases on granulocytes has to be investigated.
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PMID:CD13 and CD10 expression of granulocytes as markers for the functioning of the immune system: quantification of the expression of membrane molecules using 1:1 labeled monoclonal antibodies and flow cytometry. 1860 79

In this case report we present a 44-year-old woman with a 2-weekhistory of vaginal bleeding. Gynaecological examination revealed the presence of a polypoid neoformation in the endometrial cavity with a maximum diameter of 4 cm. Histological analysis showed a classic leiomyoma infiltrated by a second monomorphic, highly undifferentiated neoplasia. Immunohistochemical analysis revealed a negative reaction for cytokeratin, CD10, inhibin, CD99, CD20, CD3, TdT and CD34, and positivity for CD45, MPO, CD68 and CD117. A diagnosis of myeloid sarcoma in myometrial leiomyoma was made. The following days the patient showed the onset of an acute myeloid leukaemia M5a. Forty days after diagnosis the patient died for complications related to immunodeficiency caused by therapy. Especially when a common antibody panel reveals negativity for epithelial, mesenchymal and lymphoid markers, this case underlines the importance of considering myeloid sarcoma in differential diagnosis of undifferentiated tumours arising in an extramedullary site in order to avoid errors and permit optimal therapeutic management.
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PMID:[Myeloid sarcoma in uterine leiomyoma]. 1868 22

Primary effusion lymphoma (PEL) is very rare type of non-Hodgkin's lymphoma (NHL) usually confined to the body cavities such as the pleural space, pericardium, and peritoneum. PEL is a human herpes virus-8 (HHV-8)-associated lymphoma and commonly observed in human immunodeficiency virus (HIV)-infected patients. However, HIV-infected patients are extremely fewer in Japan in comparison with those in Western countries; PEL is usually not associated with HIV infection in Japan. This report presents seven Japanese cases of PEL. In situ hybridization revealed that the PEL cells were negative for EBV in all cases. An immunocytological analysis showed that only one case was positive for HHV-8, and PEL cells were positive for CD20 in all cases. MUM1 was positive, but CD10 and CD138 were negative in six cases. One case each was positive for CD30 and BCL-6. The phenotypic patterns of HIV-related is BCL6-/MUM1+/CD138+, thus, the phenotypic findings observed by immunocytochemistry in this study were somehow different from those reported in Western countries. However, the cytomorphological features of PEL cells showed large cell size, abundant basophilic cytoplasm, coarse chromatin, and occasional binucleated or multinucleated cells, similar to a large cell immunoblastic and anaplastic large cell lymphoma, indicating that the cytomorphological characteristics of PE cells in Giemsa and Papanicolaou stain were consistent with those reported abroad. The prognosis for PEL in these cases was poor, but the survival time was variable ranging from 1 month to 54 months, and was different from that of Western cases. No p16/CDKN2A expression was observed, and one case showed PEL cells with a BLIMP1 mutation.
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PMID:Cytologic and immunocytochemical features of EBV negative primary effusion lymphoma: report on seven Japanese cases. 1921 41

Epstein-Barr virus positive diffuse large B-cell lymphoma (EBV+ DLBCL) of the elderly is a rare B-cell lymphoproliferative disorder (B-LPD) that occurs in patients > 50 years with no known history of immunodeficiency or lymphoma. Patients present with moderate to severe clinical B-symptoms. These lesions show complete effacement of normal tissue/nodal architecture by large atypical lymphoid cells/immunoblasts and Hodgkin/Reed-Sternberg-like giant cells with variable amounts of inflammatory cells in the background. The ratio of neoplastic to inflammatory cells, degree of mitoses and necrosis can be quite variable; hence EBV+ DLBCL of the elderly was historically divided into low grade polymorphic and high grade monomorphic types. Further studies have shown both types to be different points in the spectrum of disease, and are all high grade lymphomas. The neoplastic large lymphoid cells show expression of CD20/CD79a and PAX-5, with variable expression of CD30, LMP-1 and EBNA-2, but CD15, CD10 and BCL6 are generally negative. Neoplastic cells show EBER positivity and high Ki-67 expression. Differential diagnoses include EBV+ B-LPD, classical Hodgkin lymphoma and EBV-DLBCL. EBV+ DLBCL of the elderly is highly aggressive with a median survival of 2 years. These patients are less responsive to standard chemotherapy compared with other B-LPD.
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PMID:Epstein-Barr virus positive diffuse large B-cell lymphoma of the elderly. 1925 22

Plasmablastic lymphoma (PBL) is very rare, and predominantly occurs in Human immunodeficiency virus (HIV)-positive individuals. It shows a strong affinity for the oral cavity and for the Epstein-Barr virus (EBV) positive. We investigated the clinicopathologic characteristics of six cases of PBL in Koreans. All patients were HIV-negative and without underlying immunodeficiency. The age distribution was bimodal, and four patients were older than 60 years. Male predominance was observed with male to female ratio of 5:1. The organs primarily involved were the terminal ileum, stomach, oral cavity, tonsil, nasal cavity and meninges. The tumors were histologically typical of PBL. Three of them were composed of monomorphic large immunoblastic or plasmablastic cells, and classified as PBL of the oral mucosa type. Another three cases were classified as PBL with plasmacytic differentiation. Five cases revealed loss of B-cell antigens with CD138 or MUM1 substitution. CD10 was positive in two cases (PBLs of the oral mucosa type), and one of them unexpectedly expressed cytokeratin. EBV was detected in one case (PBL with plasmacytic differentiation). Four patients succumbed to PBL in a relatively short period of time. We suggest that PBL is not strongly associated HIV or EBV in Koreans, and that it shows a variable organ distribution without an oro-nasal predilection.
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PMID:Human immunodeficiency virus-negative plasmablastic lymphoma in Korea. 1937 56

We describe a case of human immunodeficiency virus-associated T-lymphoblastic leukemia/lymphoblastic lymphoma in a 43-year-old Italian man with a history of human immunodeficiency virus infection lasting 9 years. Immunoperoxidase stains showed that neoplastic cells were positive for CD3, TdT, CD45, CD10, CD1a, CD2, CD7, CD5, and CD43 (focal). The proliferation rate was approximately 70%, assessed by Ki-67/MIB-1 staining. Flow cytometry of the marrow aspirate revealed an intermediate/cortical T-lymphoblastic phenotype: negative for surface CD3 and positive for cytoplasmic CD3, CD1a, TdT, CD2, CD7, CD5, and CD8, with partial coexpression of dimCD4. Analysis of T-cell receptor gamma polymerase chain reaction products showed clonality. T-lymphoblastic leukemia/lymphoblastic lymphoma is a very rare occurrence in the clinical setting of human immunodeficiency virus infection. It is not listed in the World Health Organization classification of lymphomas associated with human immunodeficiency virus infection. Only 4 cases of human immunodeficiency virus-associated T-lymphoblastic leukemia/lymphoblastic lymphoma are reported in the current medical literature.
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PMID:Human immunodeficiency virus-associated precursor T-lymphoblastic leukemia/lymphoblastic lymphoma: report of a case and review of the literature. 1942 18

A 40-year-old female, HIV positive, stage C, since 4 years, complained of a right cervical lymph node swelling. Two years before, the patient had been diagnosed with follicular B-cell non-Hodgkin lymphoma (FL); she had been treated with four cycles of multiagent chemotherapy plus rituximab, the last cycle being administered 10 months before coming to our attention. An ultrasound (US) guided fine-needle cytology (FNC) showed an atypical lymphoid cell proliferation. The phenotype evidenced by flow cytometry (FC) analysis was D5: 10%, CD19: 49%, CD23: 10%, FMC7: 0%, CD10: 40%, CD10/19: 40%, lambda light chain 40%, kappa light chain 0%. FDG-positron emission tomography (PET/CT) scan showed positivity in the corresponding cervical area. Since low LDH values and a reduced lymph node size were observed, the lymph node was therefore excised; the histology revealed a reactive hyperplastic lymph node with florid follicular pattern. A subsequent PCR analysis, performed on DNA extracted from a whole histological section, did not evidence IgH rearrangement. The patient is currently undergoing strict clinical and instrumental follow-up, including PET every 3 months; after 13 months, she is alive without recurrence of lymphoma. Clonal B-cell populations in non-lymphomatous processes have been described in mucosa-associated lymphoid cell populations and reactive lymph nodes, and are considered non-malignant, antigen driven, proliferations of B-lymphocytes determined by an abnormal response to bacterial or viral antigen stimulation. The present case occurred in an HIV patient and was clinically complex because of the patient's history of FL. This experience suggests much attention in the evaluation of radiological, cytological, and FC data and in clinical correlation in patients suffering from autoimmune or immunodeficiency syndromes.
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PMID:Clonal B-cell population in a reactive lymph node in acquired immunodeficiency syndrome. 1958 4

We report a rare case of human immunodeficiency virus (HIV)- and human herpes virus-8 (HHV-8)-negative primary effusion lymphoma (PEL)-like lymphoma presenting with lymphomatous effusions complicated by cardiac tamponade. A 68-year-old woman was hospitalized with generalized weakness in June 2006. Echocardiogram revealed the presence of pericardial effusion and she had the signs of cardiac tamponade. Urgent pericardial drainage relieved her symptoms. Chest computed tomography showed bilateral pleural effusions along with pericardial effusion. Cytologic findings of both effusions were suggestive of malignancies, including malignant lymphoma. Immunocytochemical studies with a panel of antibodies, including CD20 and CD79a, could not provide a definite diagnosis. Flow cytometric analysis of pleural effusion revealed that tumor cells were positive for CD10 and CD19, but negative for CD20, CD23, surface immunoglobulin, and T-cell associated antigens. Clonal rearrangement of the immunoglobulin heavy chain gene was detected by Southern blot analysis. Polymerase chain reaction proved to be negative for HHV-8. The serology test for HIV was negative. After a diagnosis of HHV-8-negative PEL-like lymphoma, she was treated with CHOP chemotherapy (cyclophosphamide, doxorubicin, vincristine and prednisolone). However, she died of progressive lymphoma 7 months after the diagnosis. PEL-like lymphomas are of B-cell origin. In some cases of PEL-like lymphoma, tumor cells may be negative for representative markers of B-cell phenotype such as CD20 and CD79a.
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PMID:[HIV- and HHV-8-negative primary effusion lymphoma-like lymphoma presenting with lymphomatous effusions complicated by cardiac tamponade-a case report]. 1962 Aug 17

Diffuse large B-cell lymphoma that develops in the setting of long-standing chronic inflammation is typically associated with Epstein-Barr virus, and usually presents as tumor mass involving body cavities, as in pyothorax-associated lymphoma. It is listed as a distinct entity in the latest World Health Organization lymphoma classification. We report four cases that were incidentally discovered on histologic examination, one each in a splenic false cyst, a long-standing hydrocele, an atrial myxoma, and metallic-implant wear debris. Microscopic foci of atypical (neoplastic) large lymphoid cells were found within the contents of the cysts or curettage material, or within the stroma of the atrial myxoma. Despite the diverse clinical scenarios, all cases showed a homogeneous phenotype: positivity for B-lineage markers (CD20+, CD79a+, PAX5+), non-germinal center immunophenotype (CD10-, BCL6-/+, MUM-1+), and positivity for Epstein-Barr virus with type III latency (LMP1+, EBNA2+). The last feature supports the hypothesis that the lymphoma has arisen in a setting of 'local immunodeficiency' as a result of long-standing chronic inflammation in an enclosed space, a characteristic pathogenetic mechanism of diffuse large B-cell lymphoma associated with chronic inflammation. These cases therefore expand the spectrum of this entity to include new clinical scenarios for the development of this lymphoma type.
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PMID:Diffuse large B-cell lymphoma associated with chronic inflammation as an incidental finding and new clinical scenarios. 2006 8


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