scholarly journals Characteristics and Follow-Up of Organizing Pneumonia Associated with Haematological Malignancies

2022 ◽  
Vol Volume 15 ◽  
pp. 301-310
Author(s):  
Huihui Zeng ◽  
Yiming Ma ◽  
Xue He ◽  
Shan Cai ◽  
Ping Chen ◽  
...  
2020 ◽  
Vol 35 (10) ◽  
pp. 949-960
Author(s):  
Else Toft Würtz ◽  
Johnni Hansen ◽  
Oluf Dimitri Røe ◽  
Øyvind Omland

Abstract Environmental asbestos exposure and occupational asbestos exposure increase the risk of several types of cancer, but the role of such exposures for haematological malignancies remains controversial. We aimed to examine the risk of haematological malignancies: first, in subjects exposed early in life, independently of any occupational exposure occurring later; second, in subjects exposed occupationally. We established an environmentally exposed cohort from four schools located near the only former asbestos cement production plant in Denmark. We identified nearly all pupils in the seventh grade and created an age and sex-matched 1:9 reference cohort from the Danish Central Population Register. Participants were born 1940–1970 and followed up in national registers until the end of 2015. Occupational asbestos exposure was assessed for all participants using two different job exposure matrices. The school cohort included 12,111 participants (49.7% girls) and the reference cohort 108,987 participants. Eight subgroups of haematological malignancy were identified in the Danish Cancer Registry. These cases were analysed for combined overall haematological malignancy, a combined subgroup of lymphomas and a combined subgroup of leukaemias. The data were analysed using Cox regression (hazard ratios (HR)) including other cancers and death as competing risks. Haematological malignancy was identified in 1125 participants. The median follow-up was 49.3 years (0.1–63.4). Early environmental asbestos exposure was not associated with an increased risk of haematological malignancy. Long-term occupational asbestos exposure was associated with overall haematological malignancy (HR 1.69, 95% CI 1.04–2.73); in particular for the leukaemia subgroup (HR 2.14, 95% CI 1.19–3.84). This large follow-up study suggests that long-term occupational asbestos exposure is associated with increased leukaemia risk. However, further studies are needed to confirm these observations.


Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 4001-4001
Author(s):  
David Sutton ◽  
Jeffrey Faint ◽  
Anandram Seetharam ◽  
Alan Macwhannell ◽  
Stephen Harding ◽  
...  

Abstract Abstract 4001 Introduction Serum free light chain (FLC) measurements are used routinely to identify monoclonal immunoglobulin (M-Ig) production in patients. In addition, summated kappa + lambda concentrations have recently been shown to have prognostic value in chronic lymphocytic leukaemia (CLL), Hodgkins lymphoma and HIV patients; presumably reflecting immune stimulation. A recently developed companion assay to FLC testing measures intact immunoglobulin heavy chain/light chain (HLC) pairs in serum calculating Ig'κ/Ig'λ ratios as a reflection of clonality. Here we report on a computational approach combining FLC and HLC measurements to identify clonal disease, hyper- and hypo-gammaglobulinemia, and as markers of immune dysfunction. Furthermore, we comment on the potential of such an algorithm to risk stratify haematological and non-haematological malignancies. Patients, Materials and Methods 1468 patients referred to the Royal Wolverhampton Hospital for haematological evaluation were enrolled on to the study. Median patient age was 64 years, with a slight female preponderance (60%). Patient sera were analysed at presentation using routine electrophoresis tests (serum protein electrophoresis, SPEP and immunofixation, IFE). FLC and HLC (IgG, IgA and IgM) were measured by nephelometric immunoassay. Each immunoassay result was assessed with respect to individual normal ranges (FLCκ = 3.3–19.4mg/L, FLCλ = 5.71–26.30mg/L, IgGκ = 4.03–9.78g/L, IgGλ = 1.97–5.71g/L, IgAκ = 0.48–2.82g/L, IgAλ = 0.36–1.98, IgMκ = 0.29–1.82g/L, IgMλ = 0.17–0.94g/L) and ratios (FLCκ/FLCλ = 0.26–1.65, Gκ/Gλ = 0.98–2.75, Aκ/Aλ = 0.8–2.04, Mκ/Mλ = 0.96–2.3). The results were used to create an algorithm which assessed the degree of abnormality individually and with respect to the other results generated. Diagnosis was recorded ∼3 months after the analysis and patients were followed for up to 3 years. Results 293/1468 (19%) samples had an abnormal SPEP of which 95/293 were confirmed by IFE including: 10 intact immunoglobulin Multiple Myeloma (MM, 6 IgGκ, 1 IgGλ, 2 IgAκ, 1 IgAλ), 6 light chain MM (LCMM, 4 FLCκ, 2 FLCλ), 2 Waldenstrom macroglobulinemia (2 IgMκ), 1 IgMκ cryoglobulinemia, 3 CLL, 1 mantle cell lymphoma, 3 other lymphoma, 1 IgGκ plasmacytoma, and 68 MGUS patients, 26/68 were confirmed MGUS (1 FLCκ, 13 IgGκ, 4 IgGλ, 4 IgAκ, 2 IgMκ, 2 IgMλ), 42 were laboratory findings requiring follow up (1FLCκ, 3 FLCλ, 19 IgGκ, 5 IgGλ, 4 IgAκ, 1 IgAλ, 8 IgMκ, 1 IgMλ). FLC/HLC algorithm identified 85/95 IFE positive patients; of the 10 patients reported not identified by the algorithm, 3 had oligoclonal banding indicative of infection and 7 had monoclonal bands secondary to other diagnoses (including 2 colon cancer patients and 2 rheumatoid arthritis patients). In addition the algorithm identified 15 IFE negative patients with M-Ig production including: 2 AL amyloid patients, 1 asymptomatic LCMM, 1 patient with ∼1g/L FLCκ (lost to follow up), 1 follicular lymphoma patient and 1 CLL; diagnosis was not available for 9/15 patients. Furthermore, the algorithm identified 205/1468 patients as having elevated polyclonal FLC (cFLC) >50mg/L without evidence of clonal production. In a subset analysis comparing matched numbers of patients with elevated or normal cFLC concentrations, cFLC >50mg/L predicted all cause mortality (logistic regression odds ratio 9.96, 95% CI 4.72–21.0, p<0.001), and was associated with poorer overall survival (Kaplan Meier p<0.001, Cox regression hazard ratio 8.73, 95% CI 4.47–17.02). Discussion MM presents with disparate and vague symptoms pervasive in an elderly population. Current guidelines recommend SPEP and FLC analysis reflexing to IFE for confirmation in the event of abnormality. However, SPEP and IFE require interpretation making the assessment variable. Standardised immunoassays (FLC+HLC) may obviate the need for interpretation and in this study identified additional haematological malignancies. Furthermore, standard assessments can be used to identify M-Ig, but in the absence of this finding may offer little information pertaining to the symptoms that prompted referral. By contrast this algorithm identified patients with elevated cFLC which in agreement with other reports was associated with increased risk of mortality. Further work is required to assess the algorithm, and determine if cFLC measurements should be used as a prompt for additional diagnostic investigations. Disclosures: Faint: The Binding Site Group, Ltd.: Employment. Harding:The Binding Site Group Ltd.: Membership on an entity's Board of Directors or advisory committees. Mirbahai:The Binding Site Group, Ltd: Employment.


Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 5480-5480
Author(s):  
A. Paisiou ◽  
C. M. Vadikolia ◽  
K. Stefanaki ◽  
E. Goussetis ◽  
I. Peristeri ◽  
...  

Abstract Background: Post transplant lymphoproliferative disorder (PTLD) is a heterogeneous complication of HSCT. It comprises a spectrum of pathogenetic mechanisms and clinical manifestations. It is mostly associated with EBV infection, either as a consequence of reactivation in the post transplant period or less frequently from primary infection. WHO classification defines four major histopathologic subtypes: 1. early plasmacytic hyperplasia and infectious mononucleosis (IM)-like 2. polymorphic lesions which may be polyclonal or focally monoclonal (P-PTLD) 3. monomorphic lesions that fulfill criteria for aggressive B- or T/NL-cell neoplasm (M-PTLD) 4. classic Hodgkin-type Lymphomas (CHL-PTLD). Indistinguishable categories, such as Hodgkin-like P-or M-PTLD might represent laborious diagnostic dilemmas. In literature, PTLD occurs in less than 1% of non T-cell-depleted grafts from matched siblings compared with as high as 25% in unrelated donor (VUD) recipients. There are several risk factors such as the type of conditioning and the use of ATG, the degree of immunosuppression and complications such as concurrent infections and GvHD. Objectives: To present our experience regarding the frequency, presentation and outcome of EBV related PTLD in a paediatric population of allo-HSCT recipients with haematological malignancies, to assess the risk factors and inquire into the heterogeneity in clinical presentation and outcome. Patients-Methods: From January 2004 until December 2014, 177 allo-HSCTs for myeloid and lymphoid malignancies were performed in patients aged 3 months to 19 years. PTLD was recorded in 15 (8 male) with median age of 13.24 yrs (range 5.0-17.9yrs). 11 patients had VUD, 4 patients had an haploidentical related donor and the grafts depleted of T-cells. 12 patients had peripheral blood and 3 had bone marrow as the source of HSCs. 4 were given a fully matched graft, 6 had 1 HLA antigen or allele mismatched donors, 1 patient had a 2 HLA antigen mismatched donor and 4 had 5/10 HLA matched haploidentical donors. All except for 2 patients were EBV IgG positive and the two seronegative patients had an EBV IgG positive donor. The conditioning regimens used were Busulfan or Treosulfan based in 11 patients with the addition of a combination of Cyclophosphamide, Fludarabine, Melphalan, VP16 and ATG. The latter was administered to all patients. GvHD prophylaxis consisted of Cyclosporin A and Methotrexate. Five patients (1 male) are alive and well with a median follow up of 43.1 months (range 8.3-69.2). Results: Neutrophil and platelet engraftment occurred at a median of 20 (range 14-24) and 21 days (range 14-48) respectively. Acute GvHD (stage II-IV) was recorded in 9 patients. Serial quantitative EBV-DNA/ml was employed routinely and the median day of confirmation of a rising level above 1.0x103/ml was day +58 post transplant. Median EBV copies at diagnosis were 2.3x104/ml(range 1.3x103/ml-2.15x105/ml). EBV positivity was immediately recorded post DLI in 3 patients and reactivation occurred post subsequent DLI. Immune reconstitution had not been achieved in any patient at the time of diagnosis. Median WBC count was 4.1x109/lt (1.04X109/lt - 8.78x109/lt) and all patients had absolute lymphopenia. 9 patients had documented other viral and/or bacterial infections. 2 patients developed M-PTLD, consistent unequivocally with DLBCL, 7 patients had P-PTLD and 6 patients had IM-like PTLD with plasmacytic hyperplasia only. All had anti-CD20, two patients had additional chemotherapy. Although responsive to EBV treatment, 3 died of primary disease recurrence while PTLD failed to regress in 7 patients who died of a multitude of complications. With a median follow up of 43,1 months (range 8.3-69.2), 5 patients (1 male) are alive and well. Conclusions: Nearly all HSCT recipients are EBV infected or will be infected eventually, yet only a fraction develop EBV driven PTLD. In our population the incidence was found to be 8.47%. PTLD exhibits a spectrum of features ranging from non specific and reactive to life threatening, indistinguishable from lymphoma. Administration of ATG, the type and extend of HLA mismatch, multiple sites of disease, immune suppression and concurrent infections heighten the risk of systemic manifestation. Rising EBV loads are strongly associated with impending PTLD, which can occur even with an overall modest viral load, thus requiring prompt recognition and early intervention. Disclosures No relevant conflicts of interest to declare.


2011 ◽  
Vol 17 (4) ◽  
pp. 493-498 ◽  
Author(s):  
Attiya Haroon ◽  
Futoshi Higa ◽  
Kenji Hibiya ◽  
Shusaku Haranaga ◽  
Satomi Yara ◽  
...  
Keyword(s):  

Author(s):  
Shunsuke Mori ◽  
Yukinori Koga ◽  
Mineharu Sugimoto

We treated 21 patients with organizing pneumonia (OP) associated with rheumatoid arthritis (RA) or related to biological disease-modifying antirheumatic drugs (DMARDs) at our institution between 2006 and 2014. Among these cases, 3 (14.3%) preceded articular symptoms of RA, 4 (19.0%) developed simultaneously with RA onset, and 14 (66.7%) occurred during follow-up periods for RA. In the case of OP preceding RA, increased levels of anti-cyclic citrullinated peptide antibodies and rheumatoid factor were observed at the OP onset. RA disease activity was related to the development of OP in the simultaneous cases. In the cases of OP developing after RA diagnosis, 10 of 14 patients had maintained low disease activity with biological DMARD therapy at the OP onset, and among them, 6 patients developed OP within the first year of this therapy. In the remaining four patients, RA activity was not controlled at the OP onset. All patients responded well to systemic steroid therapy, but two patients suffered from relapses of articular and pulmonary symptoms upon steroid tapering. In most of the RA patients, DMARD therapy was introduced or restarted during the steroid tapering. We successfully restarted a biological DMARD that had not been previously used for patients whose RA would otherwise have been difficult to control. In this study, we also perform a review of the literature on RA-associated or biological DMARD-related OP and discuss the pathogenesis and management of OP occurring in RA patients.


2018 ◽  
Vol 10 (1) ◽  
pp. 25-35 ◽  
Author(s):  
Beatrice J Edwards ◽  
Xiaotao Zhang ◽  
Ming Sun ◽  
Juhee Song ◽  
Peter Khalil ◽  
...  

ObjectivesA growing number of patients with cancer are older adults. We sought to identify the predictors for overall survival (OS) in older adults with solid tumour and haematological malignancies between January 2013 and December 2016.MethodsRetrospective cohort study. A comprehensive geriatric assessment was performed, with a median follow-up of 12.8 months. Analysis: univariate and multivariate Cox proportional hazards regression analysis.ResultsIn this study, among the 455 patients with last follow-up date or date of death, 152 (33.4%) died during the follow-up. The median follow-up is 12.8 months (range 0.2–51.1 months) and the median OS is 20.5 months (range 0.3–44.5 months). Among all older patients with cancer, predictors of OS included male gender, cancer stage, malnutrition, history of smoking, heavy alcohol use, frailty, weight loss, major depression, low body weight and nursing home residence. Traditional performance scores (Eastern Cooperative Oncology Group (ECOG) and Karnofsky Performance Scale (KPS)) were predictors of OS. Independent predictors included age >85 years and haematological malignancies. Among solid tumours (n=311) in addition to the above predictors, comorbidity, gait speed and vitamin D deficiency were associated with OS.ConclusionsWe identified specific geriatric factors associated with OS in older patients with cancer, and comparable in predictive ability to traditional performance scores such as KPS and ECOG. Prospective studies will be necessary to confirm our findings.


2019 ◽  
Vol 16 ◽  
pp. 147997311985382 ◽  
Author(s):  
Ying Zhou ◽  
Lei Wang ◽  
Mei Huang ◽  
Jingjing Ding ◽  
Hanyi Jiang ◽  
...  

Cryptogenic organizing pneumonia (COP) is characterized by good response to corticosteroids, but frequent relapses after reduction or cessation of treatment are noted. The incidence, risk factors of relapse, and long-term outcomes of patients with COP remain undetermined. Patients with COP from September 2010 to December 2017 were enrolled. Hospital and office records were used as data sources. Clinical information, lab examinations, chest radiographs, treatment courses, and follow-up data were collected. Relapse group was defined as worsening of clinical manifestations in combination with progression of radiographic abnormalities in the absence of identified causes. Eighty-seven patients with COP were enrolled. Of them, 73 patients were treated with corticosteroids with relapse rate yielding 31.5% (23 of 73). Eleven patients were treated with macrolides and none of them relapsed. Fever was more common (65.2% vs. 32.0%, p = 0.004), C-reactive protein (CRP) was higher (31.5 ± 39.4 mg/L vs. 17.5 ± 32.2 mg/L, p = 0.038), and diffusion capacity for carbon monoxide (DLCO) % predicted was lower (45.9 ± 14.2% vs. 57.6 ± 18.5%, p = 0.050) in relapse group compared to nonrelapse group. Four patients who presented with organizing pneumonia (OP) as the first manifestation were ultimately diagnosed with OP secondary to autoimmune disease in follow-up. We showed relapse was common in COP patients treated with corticosteroids, but the prognosis was favorable. Fever, elevated CRP, and a reduced DLCO were related to relapse. As OP may not always be cryptogenic, a careful follow-up should be programmed to diagnose the underlying systemic disease.


Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 4881-4881
Author(s):  
Marcio Andrade-Campos ◽  
Abelardo Barez ◽  
Soledad Noya ◽  
M Angeles Fernández-Galán ◽  
Jose Balanzat ◽  
...  

Abstract Introduction: Patients with type1 Gaucher's disease (GD1) have an increased risk of gammopathy (RR,33 Taddei TH 2009), multiple myeloma (RR,25.), other haematological malignancies (RR,3.45) and overall cancer risk (RR, 1.80). The Spanish Registry of Gaucher Disease (SpRGD) was established in 1993 in response to the need to group individual experiences in the diagnosis and management of this disease, increasing knowledge related to general characteristics and to know the real incidence and prevalence in the Spanish population. Registration is open to all physicians involved in the management of patients with GD and offers free enzymatic analysis, biomarkers and molecular analysis for the diagnosis and monitoring of patients (www.feeteg.org). Aim: to analyses the incidence of malignancies in adults GD patients. Patients and methods: A review of the SpRGD to obtain data form patients over 20 years of age at May, 2016 was performed. Physicians on charge fulfilled a survey in which they inform about the incidence of malignancies and follow-up information. Ethical approval was obtained from the institutional board and all patients has signed an inform consent before to be included into the SpRGD. Results: Of the 281 adult patients (³20 years) included, 279 were GD1 and 2 GD3. The average age of the entire cohort was 52.3 (23-90), of which 140 men, 141 women. Of these, 27 (9.6%) patients with GD1, 5 homozygous for N370S and 22 heterozygous for N370S had the presence of a malignancy and / or monoclonal gammopathy (MGUS), two of them had more than one neoplasia. Male / female: 11/16, mean age 60.2 (25-90), median follow-up of 16.5 years (4-23). Six have died by the tumor complications. All MGUS (N=12) were identified at GD diagnosis, they were 6 males and 6 females mean age 55.5 y (10-82) of them 50% under 60 years of age. Sixteen patients developed seventeen different neoplasms, with a female predominant (11, 68.7%). Only eight patients were under therapy at the time of neoplasia diagnosis (table1). Mean time on therapy 7.4 years (1.2-13-6). Neoplasms were registered (M/ F): B cell malignancies: Hodgkin lymphoma 1 (M), chronic lymphocytic leukemia 1 (M), multiple myeloma 1 (M), myeloid neoplasms: chronic myeloid leukemia: 1 (F), myelodysplastic syndrome: 1 (F), solid tumors: melanoma: 1 (F), meningioma: 2 (F), uterine cancer: 3 (F), gastric carcinoma 1 (F), cancer colon 2 (F), breast cancer 1 (F), prostate adenocarcinoma: 1(M), lung cancer 1 (M), liver carcinoma 1 (M), thyroid cancer 1 (F). Conclusions: It has been widely reported the highest incidence of haematological malignancies among patients with GD. Nevertheless in this cohort of Spanish patients, the incidence of solid tumors is similar to haematological neoplasms in general and higher than B cell lymphoid. Probably the incidence of malignancy in this population and during this monitoring period is similar to the expected in Spanish general population found in 0.21% / year, however females showed two times risk increase for malignancies and this aspect warranty further studies. This work has been carried out with aid for research FIS PS15/00616 and FEETEG Disclosures No relevant conflicts of interest to declare.


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