scholarly journals A case of primary intestinal lymphangiectasia with non-Hodgkin lymphoma

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Doudou Hu ◽  
Xianghua Cui ◽  
Wanlei Ren ◽  
Jian Zhang ◽  
Xin Guan ◽  
...  

Abstract Background Primary intestinal lymphangiectasia (PIL) is a rare protein-losing enteropathy characterized by the loss of proteins, lymphocytes, and immunoglobulins into the intestinal lumen. Increasing evidence has demonstrated an association between PIL and lymphoma. Case presentation A 54-year-old man with a 20-year history of abdominal distension and bilateral lower limb edema was admitted. Laboratory investigations revealed lymphopenia, hypoalbuminemia, decreased triglyceride and cholesterol level. Colonoscopy showed multiple smooth pseudo polyps in the ileocecal valve and terminal ileum and histological examination showed conspicuous dilation of the lymphatic channels in the mucosa and submucosa. A diagnosis of PIL was made. Three years later colonoscopy of the patient showed an intraluminal proliferative mass in the ascending colon and biopsy examination confirmed a malignant non-Hodgkin lymphoma. Then the patient was been underwent chemotherapy, and his clinical condition is satisfactory. Conclusion Our report supports the hypothesis that PIL is associated with lymphoma development.

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Christina Bergqvist ◽  
François Hemery ◽  
Arnaud Jannic ◽  
Salah Ferkal ◽  
Pierre Wolkenstein

AbstractNeurofibromatosis 1 (NF1) is an inherited, autosomal-dominant, tumor predisposition syndrome with a birth incidence as high as 1:2000. A patient with NF1 is four to five times more likely to develop a malignancy as compared to the general population. The number of epidemiologic studies on lymphoproliferative malignancies in patients with NF1 is limited. The aim of this study was to determine the incidence rate of lymphoproliferative malignancies (lymphoma and leukemia) in NF1 patients followed in our referral center for neurofibromatoses. We used the Informatics for Integrated Biology and the Bedside (i2b2) platform to extract information from the hospital’s electronic health records. We performed a keyword search on clinical notes generated between Jan/01/2014 and May/11/2020 for patients aged 18 years or older. A total of 1507 patients with confirmed NF1 patients aged 18 years and above were identified (mean age 39.2 years; 57% women). The total number of person-years in follow-up was 57,736 (men, 24,327 years; women, 33,409 years). Mean length of follow-up was 38.3 years (median, 36 years). A total of 13 patients had a medical history of either lymphoma or leukemia, yielding an overall incidence rate of 22.5 per 100,000 (0.000225, 95% confidence interval (CI) 0.000223–0.000227). This incidence is similar to that of the general population in France (standardized incidence ratio 1.07, 95% CI 0.60–1.79). Four patients had a medical history leukemia and 9 patients had a medical history of lymphoma of which 7 had non-Hodgkin lymphoma, and 2 had Hodgkin lymphoma. Our results show that adults with NF1 do not have an increased tendency to develop lymphoproliferative malignancies, in contrast to the general increased risk of malignancy. While our results are consistent with the recent population-based study in Finland, they are in contrast with the larger population-based study in England whereby NF1 individuals were found to be 3 times more likely to develop both non-Hodgkin lymphoma and lymphocytic leukemia. Large-scale epidemiological studies based on nationwide data sets are thus needed to confirm our findings.


2004 ◽  
Vol 78 (7) ◽  
pp. 972-977 ◽  
Author(s):  
Jennifer Trofe ◽  
Joseph F. Buell ◽  
E Steve Woodle ◽  
Thomas M. Beebe ◽  
Michael J. Hanaway ◽  
...  

2007 ◽  
Vol 18 (4) ◽  
pp. 351-359 ◽  
Author(s):  
Yawei Zhang ◽  
Rong Wang ◽  
Theodore R. Holford ◽  
Brian Leaderer ◽  
Shelia Hoar Zahm ◽  
...  

2004 ◽  
Vol 128 (6) ◽  
pp. 689-692
Author(s):  
Erica Jacobson ◽  
Gregory Sharp ◽  
Jeffrey Rimmer ◽  
Bruce MacPherson

Abstract Immunotactoid glomerulopathy is one of several renal disorders characterized by the extracellular deposition of nonamyloid fibrillary deposits. There is considerable debate as to whether immunotactoid glomerulopathy should be distinguished from fibrillary glomerulonephritis, a closely related entity. Currently, the distinction is based on fibril size and arrangement. We report the case of a 59-year-old woman in whom a diagnosis of immunotactoid glomerulopathy was made after a 2-year history of proteinuria. Electron microscopy of her renal biopsy showed randomly arranged microtubular subepithelial and mesangial deposits, which measured 34 nm in average diameter. She was later discovered to have circulating immunoglobulin G heavy chains without associated light chains (γ-heavy-chain disease) and, subsequently, non-Hodgkin lymphoma, follicular lymphoma, grade I (World Health Organization classification). Approximately 100 cases of γ-heavy-chain disease have been reported in the literature since it was originally described by Franklin in 1964. However, while there are 10 reports in the literature of heavy-chain disease with fibrillary deposits in the kidney, none fit the criteria for immunotactoid glomerulopathy.


2020 ◽  
Vol 2020 ◽  
pp. 1-5
Author(s):  
Fernando Naranjo-Saltos ◽  
Alejandro Hallo ◽  
Carlos Hallo ◽  
Andres Mayancela ◽  
Alejandra Rojas

Intestinal lymphangiectasia is a pathological dilation of enteric lymphatic vessels resulting in lymph leakage to the intestinal lumen. This chronic lymph leakage leads to a state of immunosuppression secondary to the loss of humoral and cellular components of the immune system and represents a potential risk factor for opportunistic infections. We report a case of protein-losing enteropathy in a seemingly immunocompetent patient. An intestinal histopathological study revealed the unusual association of lymphangiectasia and intestinal cryptococcosis. Although cryptococcal infection is common in immunocompromised patients, intestinal involvement is rarely reported. We found no reports on the association of intestinal cryptococcosis in patients with lymphangiectasia. This case report is the first to describe intestinal cryptococcosis associated with intestinal lymphangiectasia.


Blood ◽  
2005 ◽  
Vol 106 (2) ◽  
pp. 668-672 ◽  
Author(s):  
Andrea Altieri ◽  
Justo Lorenzo Bermejo ◽  
Kari Hemminki

Abstract Non-Hodgkin lymphoma (NHL) consists of a heterogeneous group of tumors. Population-based data on the familial risk for specific histopathologic subtypes have not been established. Such data are useful for clinical counseling and for searching tumor subtypes sharing common genetic pathways. We used the Swedish Family-Cancer Database to calculate standardized incidence ratios (SIRs) for histopathology-specific subtypes of NHL in 4455 offspring with NHL whose parents or siblings were affected with different types of lymphoproliferative malignancies. A familial history of NHL significantly increased the risk for NHL (SIRparent = 1.8; SIRsibling = 1.9) and for diffuse large B-cell lymphoma (SIRparent = 2.3), follicular lymphoma (SIRsibling = 2.3), and B-cell lymphoma not otherwise specified (NOS) (SIRsibling = 3.4). For a parental history of histopathology-specific concordant cancer, the risks were significantly increased for diffuse large B-cell lymphoma (SIR = 11.8), follicular NHL (SIR = 6.1), plasma cell myeloma (SIR = 2.5), and chronic lymphocytic leukemia (SIR = 5.9). Familial clusters for NHL seemed stronger in females and in siblings. Our study provides the first quantification of the familial risks for NHL by histopathology. The present findings give evidence for a strong familial association of NHL, with little differences in the magnitude of risks for various histopathologic subtypes. The patterns of risks in parents and siblings support the hypothesis of an autosomal-dominant component for diffuse large B-cell NHL and a recessive one for follicular NHL. (Blood. 2005;106:668-672)


Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 4648-4648
Author(s):  
Wendy Cozen ◽  
Engels A. Eric ◽  
James R. Cerhan ◽  
Martha Linet ◽  
Leslie Bernstein ◽  
...  

Abstract Subtle differences in immune response may play a role in non-Hodgkin lymphoma (NHL) etiology. Because adult immune response may be influenced by early childhood exposures, we examined the role of childhood crowding, history of atopic disease, and other childhood immune-related exposures on the risk of non-Hodgkin lymphoma in a multi-center case-control study. Interviews were completed with 1,321 cases ascertained from population-based cancer registries in Seattle, Detroit, Los Angeles and Iowa, and with 1,057 frequency-matched controls, selected by random-digit dialing and from the Health Care Financing Administration (HCFA) database. The association between NHL risk in relation to atopy and other exposures was assessed using multivariable logistic regression methods. Most types of allergy were associated with protection from NHL, with hay fever especially protective against all NHL combined (Odds Ratio [OR] = 0.71, 95% confidence interval [CI]= 0.54–0.94), diffuse large B-cell lymphoma [DLBCL] (OR=0.61, 95% CI=0.41–0.91), and follicular lymphoma (OR=0.70, 95% CI=0.45–1.09). A history of eczema increased risk of follicular lymphoma (OR=1.92, 95% CI= 1.08–3.41) but not DLBCL (OR=1.06, 95% CI= 0.55.2.04). Asthma in childhood was not associated with risk of NHL. Risk of DLBCL (OR =1.72, 95% CI=1.17–2.52), but not follicular lymphoma (OR=1.15, 95% CI=0.75–1.76) was elevated for the youngest compared to the oldest of siblings. Neither number of siblings nor years between births of siblings were significantly associated with risk. These results suggest that some immune-related exposures may affect NHL risk.


2020 ◽  
Author(s):  
Cong Li ◽  
Fangxiao Lu ◽  
Lei Tao ◽  
Haifeng Yu ◽  
Xi Chen ◽  
...  

Abstract Background Several studies have reported on the incidence of interstitial pneumonia (IP) among patients with non-Hodgkin lymphoma (NHL) that have been treated with chemotherapy plus rituximab, however, the best means of prophylactically preventing IP remains unclear. This retrospective study was designed to explore the prophylactic effect of trimethoprim-sulfamethoxazole (TMP-SMX) on IP and to identify IP-associated risk factors in NHL patients. Methods Between March 2013 and April 2018, 498 patients (264 male, 53%) with B cell NHL undergoing first-line rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone (RCHOP)-like chemotherapy treatment were enrolled in this study. Results These patients had a median age of 56 years, and 311 of these patients (62.4%) were administered prophylactic TMP-SMX. IP occurred in 65 patients (13.1%), with once daily prophylactic TMP-SMX treatment leading to a significant reduced IP rate (21.4% vs. 8.0%; p<0.001). Among patients treated with TMP-SMX, 2 (1.2%) exhibited rashes, 38 (12.2%) suffered from nausea and vomiting, 52 (16.7%) showed signs of neutropenia, and 18 (5.8%) suffered kidney dysfunction. Being male, having a history of diabetes, and not having undergone prophylactic TMP-SMX treatment were all found to be significantly associated with IP risk in both univariate and multivariate analysis. Disease progression was observed in 55/311 (17.7%) patients that underwent prophylactic TMP-SMX treatment and in 63/187 (33.7%) patients that did not (p<0.001). Conclusions Overall, these results reveal that IP is common in B cell NHL patients undergoing chemotherapy plus rituximab treatment, with the prophylactic administration of once daily oral TMP-SMX significantly reduces the IP incidence.


2020 ◽  
Vol 17 (3) ◽  
Author(s):  
Mark P. ◽  
Najihah Hanim A. ◽  
Eshamsol Kamar O. ◽  
Suhaila A. ◽  
Irfan M.

Lymphoma is generally a nodal disease and arises from lymphoid tissues or organs. Extranodal lymphoma accounts for almost a third of malignant lymphomas. Squamous cell carcinoma accounts for 90 % of laryngeal carcinoma, while extranodal Non Hodgkin Lymphoma (NHL) attributes only less than 1% of laryngeal neoplasms. Less than 100 of such cases been reported in literature since 1952. As to our best knowledge, no such case was ever reported in our country. We report a case of a 58-year-old gentleman who presented the typical history of laryngeal malignancy however the pathology turned out to be as NHLof Diffuse Large B-cell subtype.


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