31 research outputs found

    Marginal zone lymphoma international prognostic index: a unifying prognostic index for marginal zone lymphomas requiring systemic treatment

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    Background Marginal zone lymphomas (MZL), comprised of three unique but related subtypes, lack a unifying prognostic score applicable to all the patients in need for systemic chemotherapy and/or immunotherapy. Methods Patients from the prospective NF10 study (NCT02904577) with newly diagnosed MZL and receiving frontline systemic therapy at diagnosis or after observation were used to train a prognostic model. The primary endpoint was progression-free survival (PFS) from start of treatment. The model was externally validated in a pooled analysis of two independent cohorts from the University of Iowa and Mayo Clinic Molecular Epidemiology Resource and the University of Miami. Findings We identified 501 eligible patients. After multivariable modeling, lactate dehydrogenase (LDH) above upper normal limit, hemoglobin <12 g/dL, absolute lymphocyte count <1 x 10(9)/L, platelets <100 x 10(9)/L, and MZL subtype (nodal or disseminated) were independently associated with inferior PFS. The proposed MZL International Prognostic index (MZL-IPI) combined these 5 factors, and we defined low (LRG, 0 factors, 27%), intermediate (IRG, 1-2 factors, 57%) and high (HRG, 3+ factors, 16%) risk groups with 5-y PFS of 85%, 66%, and 37%, respectively (c-Harrell = 0.64). Compared to the LRG, the IRG (Hazard Ratio [HR] = 2.30, 95% CI 1.39-3.80) and HRG (HR = 5.41, 95% CI 3.12-9.38) had inferior PFS. Applying the MZL-IPI to the pooled US cohort (N = 353), 94 (27%), 192 (54%), and 67 (19%) patients were classified as LRG, IRG, and HRG, respectively, and the model was validated for PFS (log-rank test p = 0.0018; c-Harrell = 0.578, 95% CI 0.54-0.62). The MZL-IPI was also prognostic for OS in both the training and the external validation sets. Interpretation MZL-IPI is a new prognostic score for use in all patients with MZL considered for systemic treatment. Copyright (c) 2024 The Author(s). Published by Elsevier Ltd. This is an open access article under the CC BY license(http://creativecommons.org/licenses/by/4.0/)

    Lymphoma occurring during pregnancy: antenatal therapy, complications, and maternal survival in a multicenter analysis

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    Co-author Aimee Kroll-Desrosiers is a doctoral student in the Clinical and Population Health Research Program in the Graduate School of Biomedical Sciences (GSBS) at UMass Medical School. Full author list omitted for brevity. For the full list of authors, see article.PURPOSE: Lymphoma is the fourth most frequent cancer in pregnancy; however, current clinical practice is based largely on small series and case reports. PATIENTS AND METHODS: In a multicenter retrospective analysis, we examined treatment, complications, and outcomes for Hodgkin lymphoma (HL) and non-Hodgkin lymphoma (NHL) occurring during pregnancy. RESULTS: Among 90 patients (NHL, n = 50; HL, n = 40), median age was 30 years (range, 18 to 44 years) and median diagnosis occurred at 24 weeks gestation. Of patients with NHL, 52% had advanced-stage versus 25% of patients with HL (P = .01). Pregnancy was terminated in six patients. Among the other 84 patients, 28 (33%) had therapy deferred to postpartum; these patients were diagnosed at a median 30 weeks gestation. This compared with 56 patients (67%) who received antenatal therapy with median lymphoma diagnosis at 21 weeks (P < .001); 89% of these patients received combination chemotherapy. The most common preterm complication was induction of labor (33%). Gestation went to full term in 56% of patients with delivery occurring at a median of 37 weeks. There were no differences in maternal complications, perinatal events, or median infant birth weight based on deferred versus antenatal therapy. At 41 months, 3-year progression-free survival (PFS) and overall survival (OS) for NHL were 53% and 82%, respectively, and 85% and 97%, respectively, for HL. On univariate analysis for NHL, radiotherapy predicted inferior PFS, and increased lactate dehydrogenase and poor Eastern Cooperative Oncology Group performance status (ECOG PS) portended worse OS. For HL patients, nulliparous status and "B" symptoms predicted inferior PFS. CONCLUSION: Standard (non-antimetabolite) combination chemotherapy administered past the first trimester, as early as 13 weeks gestation, was associated with few complications and expected maternal survival with lymphoma occurring during pregnancy

    Imaging findings of familial Mediterranean fever

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    Purpose: The aim of this study was to study the imaging findings of familial Mediterranean fever (FMF). Materials and methods: We performed a retrospective review of the medical records and imaging studies of 38 patients with proven FMF, diagnosed between 1992 and 2002. Results: The most common clinical manifestation was recurrent peritoneal attacks with abdominal pain (76.3percent) and fever (42.1percent). Abdominal imaging findings included ileus (n=12), splenomegaly (n=5), hepatomegaly (n=2), ascitis (n=2), focal peritonitis (n=2), mesenteric streaking (n=1), and enlarged mesenteric lymph node (n=1). One patient developed fatal peritoneal mesothelioma, and 13.1percent of the patients developed amyloidosis with sonographic findings of renal parenchymal disease or cardiomyopathy. Arthritis was second in frequency, occurring in 34.2percent of patients; radiographs were normal (n=4) or showed joint effusion and periarticular soft tissue swelling (n=4) due to synovitis. One patient developed seronegative destructive arthropathy. Skin lesions were noted in 23.6percent of patients. Pleuritis was encountered in 13.1percent and pericarditis in 5.2percent. Polyarteritis nodosa (PAN) was present in two patients, multiple sclerosis in one, and autoimmune hemolytic anemia in one patient. Conclusion: FMF predominantly involves abdominal viscera but can affect other organs. The majority of patients have nonspecific imaging findings, and the radiologic diagnosis is rarely considered. Amyloidosis, mesothelioma, and destructive arthropathy are potential serious complications of FMF. PAN, multiple sclerosis, and autoimmune hemolytic anemia are probably rare associations or rather than coincident with FMF. © 2006 Elsevier Inc. All rights reserved.Aharoni D, 2000, ABDOM IMAGING, V25, P297, DOI 10.1007-s002610000006; Barzilai A, 2000, J AM ACAD DERMATOL, V42, P791, DOI 10.1067-mjd.2000.103048; Belange G, 1998, REV MED INTERNE, V19, P427, DOI 10.1016-S0248-8663(98)80867-9; BELLIN MF, 1989, ANN RADIOL, V32, P302; COOK GC, 1991, ANN SAUDI MED, V11, P576; ESHEL G, 1997, QJM, V90, P643; GOKALP HZ, 1992, CLIN NEUROL NEUROSUR, V94, P261, DOI 10.1016-0303-8467(92)90101-8; Ince E, 2002, RHEUMATOL INT, V21, P213, DOI 10.1007-s00296-001-0168-5; LAURENS A, 1976, NOUV PRESSE MED, V5, P1834; Lidar M, 2002, CLIN CHEST MED, V23, P505, DOI 10.1016-S0272-5231(01)00002-8; Livneh A, 1997, ARTHRITIS RHEUM, V40, P1879, DOI 10.1002-art.1780401023; Livneh A, 1999, Curr Opin Pulm Med, V5, P326, DOI 10.1097-00063198-199909000-00011; LOSSOS A, 1993, J CLIN NEURO-OPHTHAL, V13, P141; Majeed HA, 1998, ANN TROP PAEDIATR, V18, P13; Majeed HA, 2000, PEDIATR SURG INT, V16, P72, DOI 10.1007-s003830050019; Ozen S, 2001, SEMIN ARTHRITIS RHEU, V30, P281, DOI 10.1053-sarh.2001.19958; RIMON D, 1989, POSTGRAD MED J, V65, P776; Schwartz T, 2000, SEMIN ARTHRITIS RHEU, V29, P286, DOI 10.1016-S0049-0172(00)80015-3; Tekin M, 2000, ACTA PAEDIATR, V89, P177; TEKIN M, 1999, NEPHROL DIAL TRANSPL, V14, P469; Topcuoglu MA, 1997, J NEUROL, V244, P510; Uthman I, 2001, RHEUMATOL INT, V20, P145, DOI 10.1007-s002960100103; Wikstrom M, 1998, ABDOM IMAGING, V23, P147, DOI 10.1007-s002619900308; Zissin R, 2003, BRIT J RADIOL, V76, P22, DOI 10.1259-bjr-3205183106

    Parent–infant vocalisations at 12 months predict psychopathology at 7 years

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    This study investigated the utility of adult and infant vocalisation in the prediction of child psychopathology. Families were sampled from the Avon Longitudinal Study of Parents and Children (ALSPAC) birth cohort. Vocalisation patterns were obtained from 180 videos (60 cases and 120 randomly selected sex-matched controls) of parent-infant interactions when infants were one year old. Cases were infants who had been subsequently diagnosed aged seven years, with at least one psychiatric diagnostic categorisation using the Development and Wellbeing Assessment. Psychopathologies included in the case group were disruptive behaviour disorders, oppositional conduct disorders, attention deficit hyperactivity disorder, pervasive development disorder, and emotional disorders. Associations between infant and parent vocalisations and later psychiatric diagnoses were investigated. Low frequencies of maternal vocalisation predicted later development of infant psychopathology. A reduction of five vocalisations per minute predicted a 44% (95%CI: 11% to 94%; p-value=0.006) increase in the odds of an infant being a case. No association was observed between infant vocalisations and overall case status. In sum, altered vocalisation frequency in mother - infant interactions at one year is a potential risk marker for later diagnosis of a range of child psychopathologies
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