scholarly journals Langerhans Cell Histiocytosis: A Single Institution Retrospective Analysis of Eleven Patients

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 5458-5458
Author(s):  
Vittorio Stefoni ◽  
Alessandro Broccoli ◽  
Beatrice Casadei ◽  
Enrico Derenzini ◽  
Letizia Gandolfi ◽  
...  
Keyword(s):  
Radiation Therapy ◽  
Retrospective Analysis ◽  
Steroid Therapy ◽  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
Unknown Etiology ◽  
First Line ◽  
First Line Therapy ◽  
Line Therapy

Abstract Langerhans cell histiocytosis (LCH), is a rare disorder which has a substantially unknown etiology, pathophysiology, and may manifest through a variety of clinical presentations ranging from solitary eosinophilic granuloma to severe multisystem disease. LCH is more common in children, although it can affect any age; the most common sites of involvement are bone, skin, and lung. From a histological point of view LCH derives from accumulation of proliferating cells with surface markers and ultrastructural features similar to cutaneous Langerhans cells, intermixed with inflammatory cells, particularly eosinophils. Below, a retrospective analysis of LCH patients treated at our institution. Between 1997 and 2013 we have treated 11 LCH patients, including 6 females and 5 males with a median age at time of diagnosis of 42.9 years (range 22.2-62.3). All diagnoses were reviewed by our pathologist. With regard to the site at onset, 9 patients had bone involvment, among these, four patients had only bone involvment, the other five patients also lung, oral cavity and lymph nodes. At time of onset 4 patients showed no symptoms, while the remaining 7 showed a variety of symptoms ranging from B symptoms to tinnitus, dizziness, and other neurological symptoms such as diplopia. Among the study group 6 patients had multisystemic involvement. All patients except one had CT scan performed before, during, and at follow-up, the remaining patient was studied and followed through follow-up with PET scan. As first-line therapy 8 patients underwent chemotherapy, 2 patients radiation therapy, 1 patient required only steroid therapy. The most frequently used chemotherapy regimen for these 8 patients was MACOP-B, a third generation, CHOP-like regimen. Responses to first-line therapy were as follows: 7 complete remissions (CR), resulting with chemotherapy (5), radiation therapy and steroid therapy, two partial remissions (both obtained with chemotherapy) and two stable diseases (1 with chemotherapy and 1 with radiation therapy). Two patients relapsed, of whom one has ran several lines of chemotherapy, including autologous stem cell transplantation. Both are alive at the time of the last follow-up. To date all patients are alive but one, who died of pulmonary embolism while he was in stable disease. Six patients are in CR (60%), two in SD (20%) and two in PD (20%). In conclusion, our monocentric experience of 11 LCH patients confirms what reported in the literature in terms of heterogeneity of presentation, age, sites of involvement, symptomatology and treatment demanded. Coming to the the results our retrospective analisys shows that ten of the eleven study population patients (90.9%) are to date still alive after a significant median time of follow-up; six out of these ten patients (60%) are in CR. Disclosures No relevant conflicts of interest to declare.

Late Relapse in Hodgkin Lymphoma (HL): A Retrospective Analysis of Patients Enrolled on Clinical Trials at the Istituto Nazionale Tumori of Milan (INT-MI)

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 2697-2697 ◽  
Author(s):  
Simonetta Viviani ◽  
Alberto Mussetti ◽  
Oreste Di Bartolo ◽  
Antonello Cabras ◽  
Pinuccia Valagussa ◽  
...  
Keyword(s):  
Clinical Trials ◽  
Retrospective Analysis ◽  
Salvage Therapy ◽  
Prognostic Score ◽  
Late Relapse ◽  
First Line ◽  
First Line Therapy ◽  
Conventional Dose ◽  
Line Therapy

Abstract Background The majority of relapses in HL occur within the first three years from end of first-line treatment. Late relapses, occurring after more than five years, are rare events and there is no consensus on the optimal treatment. Aims of study The aim of our retrospective analysis was to assess the proportion of relapses occuring > 5 years in patients enrolled on clinical trials at INT-MI between 1974 and 2007, to evaluate prognostic factors, treatment outcome and survival. Patients and Methods From 1974 to 2007, 1089 consecutive HL patients, previously untreated with chemotherapy (CT), were enrolled on clinical trials at the INT-MI; 959 patients in complete remission (CR) after first-line CT or combined modality treatment were included in this study. In all patients who relapsed >5 years from end of frontline treatment a second biopsy was performed to prove HL histology at relapse. Failure-free survival (FFS) was calculated from the date of late relapse until documented relapse from CR after salvage therapy, disease progression during or within 3 months from end of salvage therapy, or death, whichever came first. Overall survival (OS) was calculated from the date of late relapse to last follow-up visit or death. Results A total of 31 complete responders after first-line therapy relapsed after > 5 years. Median time from end of first-line therapy to relapse was 115 months (range, 63-299). Main patient characteristics at late relapse were as follows: males: 71%; median age: 48 years (range, 20-67); classical histology: 81%; stage III/IV: 55%; B Symptoms: 48%; extranodal disease: 26%; > 3 involved sites: 52%; bulky disease: 13%; GHSG prognostic score ³ 1: 52%; MSKCC prognostic score ³ 1: 61%. Conventional dose salvage chemotherapy (CT) was delivered to 64.5% of patients: 9 patients were retreated with the same regimen employed as front-line therapy (MOPP alternated with ABVD), 5 were retreated with ABVD alone, 6 received non cross-resistant regimens. High-dose chemotherapy (HDCT) with hematopoietic stem cell reinfusion (ASCT) was delivered to 35.5% of patients. With a median follow-up of 9 years, 10-year FFS was 57% (95% Confidence Interval CI: 33-75) and OS was 64% (95% CI: 41-80). In univariate analysis only age > 48 years was associated with an inferior FFS [44 % (95% CI:18-68) vs 92% (95% CI:54-99), p=<0.001] and OS [40% (95%CI:16-65) vs 100% , p=0.03]. There was no significant difference in the outcome of patients treated with HDCT+ASCT compared to those treated with conventional dose CT [FFS 75% (95%CI: 30-93) vs 63% (95%CI: 36-82), p=0.78; OS 71% (95%CI: 26-92) vs 64% (95%CI: 34-83),p=0.21]. Conclusions: This retrospective cohort of patients with late relapse HL is to date one of the largest case series and with the longest follow up. Besides the rarity of these cases, late relapse of HL appears to have a good prognosis. Classical prognostic scores for relapsed HL have no role in this setting. In the subgroup analyses, being older than 48 years conveys a worse prognosis. HDCT and ASCT does not appear to confer an important survival advantage over conventional dose CT. Figure 1. Figure 1. Disclosures Viviani: Takeda Italia SpA: Consultancy; Teva Italia SpA: Consultancy; Italfarmaco SpA: Consultancy; Takeda International: Consultancy. Corradini:Celgene: Consultancy; Novartis: Consultancy.

scholarly journals Vemurafenib as first line therapy in BRAF-mutated Langerhans cell histiocytosis

2015 ◽  
Vol 73 (1) ◽  
pp. e29-e30 ◽  
Author(s):  
Julien Haroche ◽  
Fleur Cohen-Aubart ◽  
Jean-François Emile ◽  
Jean Donadieu ◽  
Zahir Amoura
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
First Line ◽  
First Line Therapy ◽  
Line Therapy

Evaluation in the Context of Daily Practice of Follicular Lymphoma patients' Outcome Before and After Approval of Rituximab in First Line Therapy in This Indication: Results of a Retrospective Analysis of 247 Unselected Patients with a 5-Year Median of Follow-up,

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 3698-3698
Author(s):  
Emmanuelle Nicolas Virelizier ◽  
Celine Segura-Ferlay ◽  
Izabela-Irina Domnisoru ◽  
Philippe Rey ◽  
Thérèse Gargi ◽  
...  
Keyword(s):  
Clinical Trials ◽  
Follicular Lymphoma ◽  
Retrospective Analysis ◽  
Response Rate ◽  
Daily Practice ◽  
First Line ◽  
First Line Therapy ◽  
Line Therapy ◽  
Before And After

Abstract Abstract 3698 Background: Randomized trials had demonstrated that rituximab improved outcome in newly diagnosed follicular lymphoma (FL) and this anti-CD20 monoclonal antibody was approved in France since 2004 in combination of chemotherapy (CT) for LF in first line therapy. As these results were obtained in selected patients with strict inclusion and exclusion criteria used in clinical trials, whether the improvement of patient's outcome is valuable in unselected population of FL patients with various medical history, different CT regimens is questionable. We performed a retrospective analysis to evaluate if the management and the outcome of FL have significantly changed in our institution before and after rituximab approval in 2004. Patients and Methods: all adult patients referred for first line LF were included and separated into cohort 1 diagnosis between 1996 and 2003 (103 patients) and cohort 2 between 2004 and 2010 (144 patients). Baseline clinical and biological datas, Follicular Lymphoma International prognostic Index (FLIPI), type of therapy, treatment response, progression free survival (PFS) and overall survival (OS) were compared. Results: There were no statistical differences in patients' characteristics between the 2 cohorts, including FLIPI score. In cohort 2, 71% received in first line therapy a rituximab based regimen (19% in monotherapy and 52% with CT). In cohort 1, 58% of patients were treated with CT and 10% with rituximab, 2% in monotherapy and 8% in combination with CT. Response rate were significantly improved in cohort 2 compared to cohort 1: complete response rate was 74.3% versus 57.8%, P <.001. With a median follow-up of 115.5 and 46.5 months in cohort 1 and 2, the 2-year PFS was 67% for cohort 1 and 82% for cohort 2 (P =.0039), respectively. The 2-year OS was also improved between the two periods (98% versus 87%, P =.0007). We could confirm the prognostic value on OS of the FLIPI score in whole series, in cohort 1 before the rituximab era but not in cohort 2. In fact, the 2-year OS was respectively 100% in low risk, 98% in intermediate risk and 95% in high risk (P =.14)/ Conclusions: These datas confirms the improvement of FL patients' outcome observed in clinical trials after the approval of rituximab in first line therapy prescript in a context of daily practice whatever age, medical history, and type of CT. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Clinical Features and Outcomes of Unifocal Adult Langerhans Cell Histiocytosis

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 1667-1667
Author(s):  
Marie Hu ◽  
Gaurav Goyal ◽  
Jason Young ◽  
Karen Rech ◽  
N. Nora Bennani ◽  
...  
Keyword(s):  
Local Recurrence ◽  
Complete Remission ◽  
Research Funding ◽  
Langerhans Cell Histiocytosis ◽  
Advisory Board ◽  
First Line ◽  
Multisystem Disease ◽  
First Line Therapy ◽  
Line Therapy

Purpose: Langerhans cell histiocytosis (LCH) is a rare histiocytic disorder that presents with a wide spectrum of clinical diseases, ranging from single-organ lesions to systemic disease. Although previously thought of as an immune disorder, LCH was reclassified as an inflammatory myeloid neoplasm in the 2016 Histocyte Society classification after discovery of BRAF V600E or MAP2K1 gain-of-function mutations and evidence of clonality in most LCH patients. In this revised classification, LCH was divided into single-system LCH, pulmonary LCH, or multisystem LCH. However, there is a lack of data on clinical features and outcomes in the subgroup of "unifocal" non-pulmonary LCH in adults. In this study, we sought to address the gaps in knowledge for unifocal adult LCH utilizing our institution's experience over 20 years. Methods: We retrospectively reviewed the medical records of 189 adult patients (defined as >18 years old at diagnosis) with histopathologically confirmed LCH who were seen at our tertiary referral center between 1997 and 2018. Of these, 44 met criteria for unifocal LCH at diagnosis after careful exclusion of other sites of disease involvement. Results: We included 44 adult patients with unifocal LCH at diagnosis, with median age 42 years (range 19 to 88) and 55% males. 84% were Caucasians and 50% were smokers. Most commonly involved disease sites included bone (43%), skin (25%), pituitary (14%), and gastrointestinal (11%), with common presenting symptoms of head pain/swelling (25%), skin rash (20%), abdominal pain/diarrhea (11%), and diabetes insipidus (9%). Resection/excision was the most common first line therapy in 24 patients (63%); none had local recurrence and 3 patients developed recurrence at a new site. Radiation was the second most common therapy in 6 patients, with an overall response rate of 83%; none had local recurrence and 1 patient had recurrence at a new site. Other less common first-line treatments included resection followed by radiation (2), topical immunosuppression (2), dexamethasone (1), cladribine (1), smoking cessation (1), and observation (1) (Table 1). Cladribine used as first-line therapy for pituitary LCH resulted in progressive disease, but cladribine used as second-line therapy in 2 cases (including one who had progressed to multisystem disease) resulted in partial remission with no further recurrence in both cases. Patients were followed for a median of 3.8 years (range 0.1 to 18.8), with 5 patients lost to follow-up. By time of last follow-up, 11 (28%) had developed recurrence: 1 had local recurrence, 5 developed disease at a new site within the same system (skin or bone), and 5 developed multisystem disease (Figure 1). Median time to recurrence was 2 years (range 0.2 to 6.6). 2 out of 5 patients tested for BRAF had a V600E mutation, both of whom had isolated unifocal bone disease and remained in complete remission following resection at time of last follow-up. Median overall survival (OS) from time of diagnosis was not reached and overall 5-year OS was 94%. 3 patients died, only 1 of progressive LCH. Conclusion: In our study, most patients with unifocal adult LCH achieved a complete remission with surgical resection or local radiation. None of the patients treated with resection or radiation developed local recurrence, but around 1 in 5 developed distant recurrence within 5 years. However, the overall prognosis was very good, and none of the patients in the cohort progressed to "high-risk" organ (liver, spleen, or bone marrow) involvement or pulmonary involvement. Further studies are warranted to assess the role of MAPK-ERK mutations in the prognosis of unifocal LCH. Disclosures Bennani: Seattle Genetics: Other: Advisory board; Adicet Bio: Other: Advisory board; Adicet Bio: Other: Advisory board; Adicet Bio: Other: Advisory board; Kite Pharma: Other: Advisory board; Kite Pharma: Other: Advisory board; Kite Pharma: Other: Advisory board; Purdue Pharma: Other: Advisory board; Seattle Genetics: Other: Advisory board; Purdue Pharma: Other: Advisory board; Seattle Genetics: Other: Advisory board; Purdue Pharma: Other: Advisory board; Bristol-Myers Squibb: Research Funding; Bristol-Myers Squibb: Research Funding; Bristol-Myers Squibb: Research Funding. Vassallo:Sun Pharmaceuticals: Research Funding; Sun Pharmaceuticals: Research Funding; Pfizer: Research Funding; Bristol Myers Squibb: Research Funding; Bristol Myers Squibb: Research Funding.

scholarly journals GCT-28. RECURRENCE PATTERN AND SURVIVAL FOR RELAPSED INTRACRANIAL NON-GERMINOMATOUS GERM CELL TUMORS: A SINGLE-INSTITUTION EXPERIENCE

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii333-iii333
Author(s):  
Lei Wen ◽  
Zhaoming Zhou ◽  
Qingjun Hu ◽  
Juan Li ◽  
Mingyao Lai ◽  
...  
Keyword(s):  
Germ Cell ◽  
Germ Cell Tumors ◽  
Late Recurrence ◽  
Salvage Chemotherapy ◽  
Recurrence Time ◽  
First Line ◽  
Primary Tumor Site ◽  
First Line Therapy ◽  
Line Therapy

Abstract PURPOSE Intracranial non-germinomatous germ cell tumors (NGGCTs) have lower overall survival than germinoma because relatively higher recurrence usually occurs after first line therapy. METHODS Between January 2003 and December 2018, 111 consecutive patients diagnosed with NGGCTs reviewed. Those who progressed after first line therapy were included in this study. Data of first line treatment, salvage treatment, clinicopathological features and survival were collected and analyzed. RESULTS Totally, thirty patients (30/111, 27.0%) relapsed in our cohort, including 19 patients with accurate relapse information detail, and 11 patients who died of disease progression during follow up but without exact time and site of relapse. The median OS from diagnosis of the disease was 49.2 months (95% CI: 14.1 to 84.3 months) and 3-year OS was 54.3%. Patients who received both CSI and chemotherapy relapsed less than those who received reduced volume of radiotherapy or only CSI or only chemotherapy (22.5% vs. 45.5%, p=0.034). Of 19 patients who had detail information of recurrence time and site, the median time from diagnosis of disease to relapse was 9.5 months (2.2 to 72.1 months). Regarding to recurrence site, most patients relapsed in primary site (10/19, 52.6%) or distant intracranial (6/19, 31.6%). The recurrence site of other 3 patients were spinal (n=1), ventricular (n=1) and peritoneal (n=1). CONCLUSION Protracted follow-up is recommended because late recurrence is not uncommon. Primary tumor site and distant intracranial are the most prevalent relapsed location. Patients who relapsed could benefited from both CSI and salvage chemotherapy.

scholarly journals A Female with Evans Syndrome Presented with Pancytopenia

2021 ◽  
Vol 10 (2) ◽  
pp. 114-117
Author(s):  
Md Rezaul Karim Chowdhury ◽  
Md Haroon Ur Rashid ◽  
Md Mahbub Hossain ◽  
Shafayet Hossain Riyan
Keyword(s):  
Oral Prednisolone ◽  
Low Grade ◽  
Evans Syndrome ◽  
First Line ◽  
Indirect Bilirubin ◽  
First Line Therapy ◽  
Line Therapy ◽  
Immune Neutropenia ◽  
Chronic Course

Evans syndrome is an uncommon haematological disorder characterised by autoimmune haemolytic anaemia (AIHA), immune thrombocytopenia (ITP) and/or immune neutropenia. It may occur in all ethnic groups, all ages and has no sex predilection. The direct antiglobulin test (DAT) is almost invariably positive. This condition generally runs a chronic course and is characterised by frequent exacerbations and remissions. Corticosteroids and/or intravenous immunoglobulin (IVIG) are the most commonly used first line therapy. Here we report a case of a female who presented with severe shortness of breath, palpitation and low grade fever and on examination she was found severely pale and mildly icteric. Her CBC and PBF showed pancytopenia. Indirect bilirubin and LDH were raised and direct Coomb’s test was positive. She was labeled as a case of Evans syndrome and responded to oral prednisolone. On subsequent follow-up her haematological profiles remained normal. J Enam Med Col 2020; 10(2): 114-117

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