BCL-6 Overexpression Predicts Poor Response to Reduced Immune Suppression Therapy in Children with Post-Transplantation Lymphoproliferative Disease (PTLD).

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 4342-4342
Author(s):  
Christopher C. Porter ◽  
Xiayuan Liang ◽  
Edythe A. Albano
Keyword(s):  
Immune Suppression ◽  
Poor Response ◽  
Lymphoproliferative Disease ◽  
Response To Treatment ◽  
Liver Transplants ◽  
Post Transplantation ◽  
Heart Transplants ◽  
Hematopoietic Stem ◽  
Follicular Hyperplasia ◽  
Archived Specimens

Abstract Background: PTLD is a well known complication of organ transplantation. While the understanding of this disease is increasing, the diagnosis and treatment remain challenging. The clinical, morphological, and molecular findings in PTLD vary widely. Reduction of immune suppression is the treatment of choice, but is effective in only 20–50% of patients. Mutations in the BCL6 gene have been demonstrated in some lymphomas and PTLD and may have prognostic implications. A single study, in which most subjects were adults, has suggested that BCL6 mutation in PTLD predicts a lack of response to reduced immunosuppression (IS). Objectives: To determine if BCL-6 overexpression predicts poor response to reduced IS or decreased survival in children with PTLD. Methods: Clinical data of patients identified with PTLD at The Children’s Hospital (TCH) from 1992–2002 were obtained, including age, type of organ transplanted, subtype of PTLD, treatment received, response to treatment, relapse free survival (RFS), and overall survival (OS). Archived specimens of lesions obtained at the time of diagnosis of PTLD were evaluated for the presence of BCL-6 expression by immunohistochemistry. Results: Between 1992 and 2002, 486 organ transplants (211 heart, 107 hematopoietic stem cell (HSC), 86 liver, 82 kidney) were performed at TCH. During that same period, 20 children were diagnosed with 21 cases of PTLD. Eleven patients had liver transplants (12.7% of transplants), 5 had heart transplants (2.4%), 3 had HSC transplants (2.8%), and 1 had a kidney transplant (1.2%). Histologic classifications of PTLD included 1 follicular hyperplasia, 9 polymorphic PTLD, and 11 lymphomas. Only 5 of 20 patients responded to reduced IS. Overall mortality was 45% (9/20). BCL-6 was overexpressed in 7 of 18 evaluable specimens from 20 patients. None of the patients with BCL-6 overexpression responded to reduced IS (p=0.05). BCL-6 overexpression was associated with monomorphic histology (p=0.02), but did not predict RFS or OS. Conclusions: BCL-6 expression is associated with monomorphic histology and non-response to reduced IS in children with PTLD. Larger, prospective studies of BCL-6 mutation and expression are needed to verify the clinical significance of these findings.

Successful allografting for relapsing pediatric ALL: The results of two decades.

2021 ◽  
Vol 39 (15_suppl) ◽  
pp. e22000-e22000
Author(s):  
O. Bulent Zulfikar ◽  
Basak Koc
Keyword(s):  
Bone Marrow ◽  
High Risk ◽  
Residual Disease ◽  
Lymphoblastic Leukemia ◽  
Treatment Options ◽  
Poor Response ◽  
Response To Treatment ◽  
Laboratory Findings ◽  
Hematopoietic Stem ◽  
Allogeneic Hsct

e22000 Background: The prognosis of children and adolescents with acute lymphoblastic leukemia (ALL) has dramatically improved. This success is associated with both by multiagent chemotherapy regimens and by definition of clinical, biological and treatment response that allow the administration of risk-adapted therapy, including allogeneic hematopoietic stem cell transplantation (HSCT). In treatment of relapsed and resistance ALL, allogeneic HSCT continues to play a major curative role. In the present study, we desciribed the patients who underwent HSCT in our clinic in the last 21 years. Methods: From 1999 to 2020, 147 patients who diagnosed with ALL and treated with the COG protocols at the Istanbul University Oncology Institute were retrospectively reviewed and 17 of them relapsed. HSCT was applied 7 of the relapsed cases and also 3 resistant cases who had suitable matched donors. The demographic features, laboratory findings and treatment responses of 10 patients were recorded from the patients’ medical records. Results: All 10 patients were B-ALL with median diagnosis age of 79.5 months (range: 32-195) and 5 were male. Characteristics of patients given in Table 1. HSCT was performed due to late relaps in 7 patients. Three of the 7 relaps were only bone marrow and other 4 had combined. Patient #2 had both breast, conjunctiva and bone marrow for the 1st relaps and only conjunctiva for the 2nd one and this patient had also t(9;22) in the 1st relaps. Patient #3 had bone marrow+central nervous system relaps and patient #7 had bone marrow for the 1st relaps and testis and bone marrow for the 2nd one. Other 3 had poor response to treatment and Minimal Residual Disease (MRD) was high in End of Introduction (EoI) and End of Consolidation (EoC)). All patients had allogeneic HSCT and 8 are alive. Conclusions: HSCT remains the standard-of-care treatment for ALL patients who carry high-risk features predicting leukemia recurrence and for those experiencing high-risk first relapse or multiple relapses. Additionally, defining the indications of HSCT are dynamic and it could change according to treatment options as well as new molecular and biological findings. It is important to identify the patients who have high relapse risk and HSCT should have priority in patients whom MRD is high in EoI and EoC.[Table: see text]

Erdheim-Chester Disease Presenting with Histiocytic Colitis and Cytokine Storm

2017 ◽  
Vol 26 (2) ◽  
pp. 183-187
Author(s):  
George P. Christophi ◽  
Yeshika Sharma ◽  
Quader Farhan ◽  
Umang Jain ◽  
Ted Walker ◽  
...  
Keyword(s):  
Periodic Acid ◽  
Poor Response ◽  
Rare Type ◽  
Periodic Acid Schiff ◽  
Hematopoietic Stem ◽  
Hemodynamic Compromise ◽  
Erdheim Chester Disease ◽  
Erdheim Chester ◽  
Chester Disease ◽  
Segmental Colitis

Background: Non-Langerhans histiocytosis is a group of inflammatory lymphoproliferative disorders originating from non-clonal expansion of hematopoietic stem cells into cytokine-secreting dendritic cells or macrophages. Erdheim-Chester Disease (ECD) is a rare type of non-Langerhans cell histiocytosis characterized by tissue inflammation and injury caused by macrophage infiltration and histologic findings of foamy histiocytes. Often ECD involves the skeleton, retroperitoneum and the orbits. This is the first report documenting ECD manifesting as segmental colitis and causing cytokine-release syndrome.Case presentation: A 68-year old woman presented with persistent fever without infectious etiology and hematochezia. Endoscopy showed segmental colitis and pathology revealed infiltration of large foamy histiocytes CD3-/CD20-/CD68+/CD163+/S100- consistent with ECD. The patient was empirically treated with steroids but continued to have fever and developed progressive distributive shock.Conclusion: This case report describes the differential diagnosis of infectious and immune-mediated inflammatory and rheumatologic segmental colitis. Non-Langerhans histiocytosis and ECD are rare causes of gastrointestinal inflammation. Prompt diagnosis is imperative for the appropriate treatment to prevent hemodynamic compromise due to distributive shock or gastrointestinal bleeding. Importantly, gastrointestinal ECD might exhibit poor response to steroid treatment and other potential treatments including chemotherapy, and biologic treatments targeting IL-1 and TNF-alpha signaling should be considered.Abbreviations: AFB: acid-fast bacilli; ECD: Erdheim-Chester Disease; IBD: inflammatory bowel disease; PASD: periodic acid-Schiff with diastase; TB: tuberculosis

Criteria positive and criteria negative anaphylaxis, with a focus on undifferentiated somatoform idiopathic anaphylaxis: A review and case series

2020 ◽  
Vol 41 (6) ◽  
pp. 436-441 ◽  
Author(s):  
Daniel A. Rosloff ◽  
Kunal Patel ◽  
Paul J. Feustel ◽  
Jocelyn Celestin
Keyword(s):  
Diagnostic Criteria ◽  
Statistical Significance ◽  
Case Series ◽  
Poor Response ◽  
Response To Treatment ◽  
Fisher Exact Test ◽  
Subjective Symptoms ◽  
Significant Difference ◽  
Physical Findings ◽  
Idiopathic Anaphylaxis

Background: Undifferentiated somatoform (US) idiopathic anaphylaxis (IA) is considered a psychogenic disorder characterized by a lack of observable physical findings and poor response to treatment. Although failure to diagnose true anaphylaxis can have disastrous consequences, identification of US-IA is crucial to limit unnecessary expenses and use of health care resources. Objective: To better define the presentation and understand the potential relationship between US-IA and underlying psychiatric comorbidities. Methods: We retrospectively reviewed 110 visits by 107 patients to our institution for evaluation and management of anaphylaxis over a 1-year period. The patients were classified as having either criteria positive (CP) or criteria negative (CN) anaphylaxis based on whether they met Second National Institute of Allergy and Infectious Disease/Food Allergy and Anaphylaxis Network Symposium criteria for the clinical diagnosis of anaphylaxis. Patient characteristics, including objective and subjective signs and symptoms, and the presence of psychiatric diagnoses were collected and analyzed. Statistical significance was assessed by using the Fisher exact test. A literature review of US-IA and other psychogenic forms of anaphylaxis was performed. Results: Patients with CP anaphylaxis were more likely to present with hypotension, wheezing, urticaria, and vomiting than were patients with CN anaphylaxis. The patients with CN anaphylaxis were more likely to present with subjective symptoms of sensory throat tightness or swelling compared with patients with CP anaphylaxis. No significant difference was detected in the prevalence of psychiatric conditions between the two groups. Conclusion: Patients who met previously established diagnostic criteria for anaphylaxis were more likely to present with objective physical findings than those who did not meet criteria for true anaphylaxis. CN patients who presented for treatment of anaphylaxis were more likely to present with subjective symptoms. Formal diagnostic criteria should be used by clinicians when evaluating patients with suspected anaphylaxis.

POSSIBILITIES OF USING RUXOLITINIB TO IMPROVE THE RESULTS OF allo-HSCT (LITERATURE REVIEW)

2019 ◽  
Vol 65 (3) ◽  
pp. 330-336
Author(s):  
Irina Gribkova ◽  
I. Ishmatova ◽  
Mariya Davydovskaya ◽  
K. Kokushkin
Keyword(s):  
Stem Cell ◽  
Hematopoietic Stem Cell Transplantation ◽  
Literature Review ◽  
Stem Cell Transplantation ◽  
Clinical Improvement ◽  
Cell Transplantation ◽  
Graft Versus Host Disease ◽  
Post Transplantation ◽  
Hematopoietic Stem ◽  
Graft Versus Host

The aim of the study was to systematize and summarize the current available data on ruxolitinib use in patients with myelofibrosis (MF) prior to allogeneic hematopoietic stem cell transplantation (allo-HSCT) to improve its results. The review includes data from foreign and domestic articles found in the PubMed and elibrary.ru databases describing the results of the use of ruxolitinib in patients with MF prior to allo-HSCT, including clinical cases, original scientific studies and reviews. It is reported that ruxolitinib therapy is safe, reduces mortality in the early post-transplantation period, reduces the incidence of acute and chronic graft-versus-host disease, and decreases the frequency of relapses. Clinical improvement with ruxolitinib therapy prior to allo-HSCT can be considered a prognostically favorable factor.

scholarly journals Oral mucositis in patients with acute myeloid leukemia treated with allogeneic hematopoietic stem cell transplantation in relation to the conditioning used prior to transplantation

2021 ◽  
Author(s):  
Aleksandra Wysocka-Słowik ◽  
Lidia Gil ◽  
Zuzanna Ślebioda ◽  
Agnieszka Kręgielczak ◽  
Barbara Dorocka-Bobkowska
Keyword(s):  
Acute Myeloid Leukemia ◽  
Cell Transplantation ◽  
Oral Mucositis ◽  
Myeloid Leukemia ◽  
Hematopoietic Cell Transplantation ◽  
Conditioning Regimen ◽  
World Health ◽  
Post Transplantation ◽  
Hematopoietic Stem ◽  
Acute Myeloid

AbstractThis study was designed to investigate the frequency and severity of oral mucositis in patients with acute myeloid leukemia after allogeneic hematopoietic cell transplantation, in relation to the type of conditioning used. Eighty patients diagnosed with acute myeloid leukemia were assigned to two groups based on the conditioning regimen used before transplantation. The intensity of oral inflammatory lesions induced by chemotherapy (oral mucositis) was evaluated according to a 5-point scale recommended by World Health Organization. Oral mucosa was investigated in all patients before the transplantation and during two subsequent stages of the post-transplantation procedure in relation to the conditioning regimen used. Mucositis in the oral cavity was observed in the majority of patients (66%) in the first week after transplantation, whereas the largest percentage of patients suffering oral lesions (74%) occurred in the second week after transplantation. A significantly higher percentage of patients with mucositis was observed in the group which underwent myeloablation therapy (74% of MAC and 50% of RIC patients in the first week; 83% of MAC and 53% of RIC patients in the second examination).The severity of mucositis after transplantation was higher in the MAC patients compared to the RIC patients. The highest mean value of the mucositis index was recorded in the second week in the MAC group (1.59). In AML sufferers receiving allo-HSCT, oral mucositis is a significant complication of the transplantation. This condition is more frequent and more severe in patients after treatment with myeloablation therapy.

scholarly journals Predictive Factors of the Responses to Treatment of Mycoplasma pneumoniae Pneumonia

2021 ◽  
Vol 10 (6) ◽  
pp. 1154
Author(s):  
Eun Lee ◽  
Yun Young Lee
Keyword(s):  
Pleural Effusion ◽  
Mycoplasma Pneumoniae ◽  
Predictive Factors ◽  
Respiratory Virus ◽  
Clinical Laboratory ◽  
Poor Response ◽  
Response To Treatment ◽  
Slow Response ◽  
Response Group ◽  
Time Of Admission

The prevalence of refractory Mycoplasma pneumoniae (MP) pneumonia is increasing. The present study aimed to identify the predictive factors of responses to treatment of MP pneumonia in children. A total of 149 children were diagnosed with MP pneumonia, of whom 56 were included in the good response group, 75 children in the slow response group, and 18 children in no response or progression group. Data on the clinical, laboratory, and radiologic features were retrospectively obtained through medical chart reviews. The severity of pneumonia, based on the extent of pneumonic lesions on chest x-ray (adjusted odds ratio (aOR), 10.573; 95% confidence intervals (CIs), 2.303−48.543), and lactate dehydrogenase (LDH) levels (aOR, 1.002; 95% CIs, 1.000–1.004) at the time of admission were associated with slow response to treatment of MP pneumonia. Pleural effusion (aOR, 5.127; 95% CIs, 1.404–18.727), respiratory virus co-infection (aOR, 4.354; 95% CIs, 1.374–13.800), and higher LDH levels (aOR, 1.005; 95% CIs, 1.002–1.007) as well as MP-specific IgM titer (aOR, 1.309; 95% CIs, 1.095–1.564) were associated with no response or progression of MP pneumonia. The area under the curve for the prediction of no or poor response in MP pneumonia using pleural effusion, respiratory virus co-infection, LDH levels, and MP-specific IgM titer at the time of admission was 0.8547. This study identified the predictive factors of responses to treatment of MP pneumonia in children, which would be helpful in establishing a therapeutic plan and predicting the clinical course of MP pneumonia in children.

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