Splenic Involvement Research Articles

A Review on Colonic Ischemia due to Vasoconstrictors

Intestinal ischemia results due to reduced blood flow to the intestine. Hypoperfusion of mesenteric vasculature can be due to occlusive or nonocclusive etiology. Nonocclusive mesenteric ischemia (NOMI) is due to arterial spasm from vasoconstrictors. Colon ischemia has a reported mortality rate ranging from 6 to 25%, depending upon the causal agent and comorbidities. This review's scope was to examine the body of published literature regarding outcomes of iatrogenic NOMI and compare cocaine-related NOMI with other causes of iatrogenic large bowel ischemia. A literature search was conducted on Pubmed and Google scholar, using "Mesenteric Ischemia" and "Vasoconstrictor" as the Mesh terms. Twenty-two articles (19 case reports, 3 case series) were finally included in our review. Among study subjects, Abdominal pain was the presenting complaint in 88.88% of patients, and bloody bowel movements were reported in 81.48% of patients. Diagnostic modalities used included colonoscopy (59.26%), sigmoidoscopy (23.07%), computed tomography (37.04%), plain abdominal films (11.54%), and laparotomy (19.23%). Combining findings from all the diagnostic modalities revealed pan-colonic involvement in 11.54% of patients, proximal colon in 23.08% of patients, 7.68% of patients had involvement of transverse colon and descending/ sigmoid colon were involved in 55.56%. Splenic flexure region involvement was noticed in 30.77% of cases. Most of the patients had more than one region of bowel involved. Findings of severe colon ischemia, including ulcers, hemorrhages, and gangrene, were found in 70.37% of patients on colonoscopy or autopsy. Nineteen patients (70.37%) were managed conservatively with broad-spectrum antibiotics, intravenous fluids, and bowel rest. Two of them died due to septic shock, while the remaining 17 recovered without any further complications. Eight patients (29.63%) required surgical management, and two of them had septic shock, causing death. In this series, nonoperative management had a success rate of 89%, while surgical management had a success rate of 75%. Based on the available reported dataset, mean hospitalization days for patients managed non-operatively were 4.31 (Range 2-10). For patients requiring surgery, it was 21 (range 4-60) due to sepsis and multiorgan failure, complicating the colon ischemia and prolonging the stay. Significant differences were found between cocaine and non-cocaine vasoconstrictor-induced large bowel NOMI regarding surgery and length of hospital stay (7 days vs 4 days), but the difference in mortality and hospital score did not reach statistical significance. Our article's message is that in patients with acute abdominal pain where a diagnosis of colon ischemia is being entertained, care should be taken not to miss out on the potential role of vasoconstrictors, including cocaine.

Identification of hub genes associated with the pathogenesis of diffuse large B-cell lymphoma subtype one characterized by host response via integrated bioinformatic analyses.

BackgroundHost response diffuse large B-cell lymphoma (HR DLBCL) shares features of histologically defined T-cell/histiocyte-rich B-cell lymphoma, including fewer genetic abnormalities, frequent splenic and bone marrow involvement, and younger age at presentation. HR DLBCL is inherently less responsive to the standard treatment for DLBCL. Moreover, the mechanism of infiltration of HR DLBCL with preexisting abundant T-cells and dendritic cells is unknown, and their associated underlying immune responses incompletely defined. Here, hub genes and pathogenesis associated with HR DLBCL were explored to reveal molecular mechanisms and treatment targets.MethodsDifferentially expressed genes were identified in three datasets (GSE25638, GSE44337, GSE56315). The expression profile of the genes in the GSE53786 dataset was used to constructed a co-expression network. Protein-protein interactions analysis in the modules of interest identified candidate hub genes. Then screening of real hub genes was carried out by survival analysis within the GSE53786 and GSE10846 datasets. Expression of hub genes was validated in the Gene expression profiling interactive analysis, Oncomine databases and human tissue specimens. Functional enrichment analysis and Gene set enrichment analysis were utilized to investigate the potential mechanisms. Tumor Immune Estimation Resource and The Cancer Genome Atlas were used to mine the association of the hub gene with tumor immunity, potential upstream regulators were predicted using bioinformatics tools.ResultsA total of 274 common differentially expressed genes were identified. Within the key module, we identified CXCL10 as a real hub gene. The validation of upregulated expression level of CXCL10 was consistent with our study. CXCL10 might have a regulatory effect on tumor immunity. The predicted miRNA (hsa-mir-6849-3p) and transcription factor (IRF9) might regulate gene expression in the hub module.

Circulating melanin-containing cells and neutrophils with phagocytized melanin granules in a horse with disseminated melanoma.

An 18-year-old, grey, Thoroughbred Cross gelding was referred to the Cummings School of Veterinary Medicine at Tufts University following a 3-week history of low-grade fever of unknown origin, distal limb swelling, and weight loss. Clinical examination identified a few black, round, smooth nodules along the ventral aspect of the proximal tail. Transabdominal ultrasound showed a markedly enlarged heterogenous spleen, hyperechoic liver nodules, and evidence of peritonitis with fibrin deposition. A mature neutrophilia was noted on complete blood count with variable numbers of phagocytized granules within neutrophils. The granules did not stain with Perl's Prussian blue, and were intensely positive when stained with Fontana-Mason, consistent with melanin. On necropsy, the spleen occupied approximately one-third of the abdominal cavity and was diffusely firm with abundant black pigment on cut section. The medullary space of the 18th thoracic vertebra was also diffusely blackened. The splenic, mediastinal, and tracheobronchial lymph nodes were five times the normal size and diffusely pigmented. The final anatomic diagnosis was disseminated malignant melanoma with extensive splenic involvement and hemolymphatic and vascular neoplastic dissemination. To the authors' knowledge, this is the first full report to identify circulating neutrophils containing phagocytized melanin granules, which confirmed an antemortem diagnosis of disseminated melanoma.

Hirschsprung disease and other gastrointestinal motility disorders in patients with CCHS.

Congenital central hypoventilation syndrome (CCHS) is an autonomic nervous system dysfunction due to PHOX2B gene mutation. Little is known about gastrointestinal motility disorders in CCHS patients. This study aims to describe the spectrum of gastrointestinal motility disorders in CCHS and provide PHOX2B genotype-phenotype correlation with Hirschsprung Disease (HD). We reviewed the records of 72 CCHS patients seen at Children's Hospital Los Angeles from 1999 to 2019. Data collected included demographics, PHOX2B genotype, ventilator dependence, medical and surgical history, and gastrointestinal motility studies. Of the 72 patients, 31% had HD, 50% females, and 60% had 20/27 PARM. Rectosigmoid HD formed 73% of the cases whereas long segment (up to splenic flexure involvement) forms represented 23%. Four patients had total colonic aganglionosis, including one patient with 20/25 PARM genotype. One HD patient was identified with colonic myopathy in the residual segment. One patient was found to have achalasia type 1.Conclusion: Nearly one third of our CCHS patients had HD. Although most had 20/27 PARM, 2 patients had 20/25 PARM. Thus, CCHS patients with constipation are at risk for HD regardless of genotype. Colonic myopathy may coexist in treated HD with refractory constipation. Achalasia may occur in patients with CCHS. What is Known: • Patients with CCHS have motility disorders and present with esophageal dysmotility and constipation as a manifestation of their autonomic nervous system dysfunction. • About 20% of patients with CCHS have Hirschsprung disease and previously described to be associated with NPARM and 20/27 PARM genotype. What is New: • Thirty-one percent of CCHS patients in our series have Hirschsprung disease (HD). •HD, including the more severe total colonic aganglionosis was found in a patient with 20/25 PARM genotype suggesting that CCHS patients withconstipation should be screened for HD regardless of genotype.

T-cell neoplasms in the spleen

Hematopoietic neoplasms involving the spleen are uncommon, but T cell neoplasms involving the spleen are extremely rare. The rarity of splenic involvement by T cell neoplasms has resulted in a limited body of literature describing their splenic characteristics. As a result, our purpose in this review article is to provide and summarize some of the characteristics seen by different T cell neoplasms that may involve the spleen.

Postmortem Findings in Italian Patients With COVID-19: A Descriptive Full Autopsy Study of Cases With and Without Comorbidities.

BackgroundDescriptions of the pathological features of coronavirus disease-2019 (COVID-19) caused by the novel zoonotic pathogen severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) emanate from tissue biopsies, case reports, and small postmortem studies restricted to the lung and specific organs. Whole-body autopsy studies of COVID-19 patients have been sparse.MethodsTo further define the pathology caused by SARS-CoV-2 across all body organs, we performed autopsies on 22 patients with COVID-19 (18 with comorbidities and 4 without comorbidities) who died at the National Institute for Infectious Diseases Lazzaro Spallanzani-IRCCS Hospital, Rome, Italy. Tissues from the lung, heart, liver, kidney, spleen, and bone marrow (but not the brain) were examined. Only lung tissues were subject to transmission electron microscopy.ResultsCOVID-19 caused multisystem pathology. Pulmonary and cardiovascular involvement were dominant pathological features. Extrapulmonary manifestations included hepatic, kidney, splenic, and bone marrow involvement, and microvascular injury and thrombosis were also detected. These findings were similar in patients with or without preexisting medical comorbidities.ConclusionsSARS-CoV-2 infection causes multisystem disease and significant pathology in most organs in patients with and without comorbidities.

HL-290: Prognostic Impact of CD20 Positivity on Outcomes in Pediatric Hodgkin Lymphoma

Context In adults, CD20-positive Hodgkin lymphoma (HL) has shown a trend toward better overall survival, though it has limited applications in the current treatment protocols. Studies on the impact of CD20 positivity on outcomes of pediatric HL is limited. Objective The primary objective was to compare the overall survival (OS) and event-free survival (EFS) of CD20-positive and -negative patients in pediatric HL. The secondary objective was to compare clinical presentation and stage between the two groups. Design We retrospectively reviewed the records of patients less than or equal to 18 years of age diagnosed with HL during the period of August 2003 to December 2018. Patients who were lost to follow-up were excluded. CD20 positivity was determined based on cutoff of 10% positivity. EFS and OS were evaluated by Kaplan-Meier methods. Clinical presentation and stage were compared between the two groups using the Chi-squared test. Setting The study was done at a tertiary care centre. Results The total number of patients were 299 with a median age of 10 years with predominantly males (84%). Eighty-five (28.43%) patients were CD20-negative. There was no significant difference in age, sex, B symptoms, bone marrow involvement, extra-nodal involvement, infra-diaphragmatic disease, liver involvement and stage between CD20-positive and CD20-negative patients. Splenic involvement was higher in CD20-negative patients compared to CD20-positive patients (82.7% vs 17.3%, p =0.001). Twenty-six patients progressed, nine of whom died. The total number of deaths were 12, nine of whom died due to disease and three of whom died due to bleomycin toxicity. Median follow up duration was 5.9 years. Median EFS and OS was not reached. There was no significant difference between EFS (HR: 0.96, CI: 0.43–2.18, p= 0.93) and OS (HR: 0.52, CI: 0.11–2.37, p=0.36) between CD20-positive and -negative patients. Conclusions We report the largest data on impact of CD20 in pediatric HL in this study. Splenic involvement was higher in CD20-negative patients. There was no significant difference in EFS and OS of CD20-positive patients in pediatric HL, the number of events being a limitation. Our study suggests that CD20 does not influence clinical outcome.

A case of spleen hydatid cyst

The hydatid cyst of the spleen results from the development of the hydatid worm Echinococcus granulosus, in the splenic parenchyma. Humans are the accidental intermediate host in the development cycle of hydatid disease. Splenic involvement is the third most common location after the liver and lung. Echography and sometimes tomography are needed to confirm the diagnosis. In the absence of truly effective medical treatment, splenic hydatidosis often leads to surgery. Resection of the protruding dome is the treatment of choice due to low morbidity and absence of mortality. We report the case of 27 years old, with no particular medico-surgical history, referred to our hospital for the treatment of a splenic hydatid cyst discovered fortuitously during a radiological assessment. The patient was operated by laparoscopy. The treatment consisted of sterilization of the contents of the cyst with hydrogen peroxide, removal of the contents and resection of the salient dome, the results were unremarkable.

Myeloid, mast cell, histiocytic and dendritic cell neoplasms and proliferations involving the spleen.

Splenic involvement and consequent splenomegaly are usually seen as part of systemic involvement by myeloid neoplasms as well as mast cell and histiocytic neoplasms. Primary splenic involvement by these neoplasms is rare. Splenectomy is usually not performed for establishing a diagnosis of these entities. However, in rare instances, the pathologist may need to evaluate the spleen secondary to splenic rupture or palliative splenectomy to alleviate symptoms related to splenomegaly. This review article describes the clinicopathologic features of a broad group of myeloid, mastocytic, and histiocytic proliferative and neoplastic disorders.

Radiographic Splenic Artery Involvement Is a Poor Prognostic Factor in Upfront Surgery for Patients with Resectable Pancreatic Body and Tail Cancer.

The prognostic impact of radiographic splenic vessel involvement in pancreatic cancer remains unclear. We evaluate its oncological significance in resectable pancreatic body/tail cancer. We retrospectively review 102 cases of resectable pancreatic cancer and 51 of borderline resectable pancreatic cancer (BRPC) who underwent pancreatectomy for pancreatic body/tail cancer. Resectable pancreatic body/tail cancer was classified into one of three categories based on radiographic splenic vessel involvement. Among 102 cases of resectable pancreatic cancer, 37 (36.3%), 35 (34.3%), and 30 cases (29.4%) were classified as no splenic vessel involvement (Rnone), splenic vein involvement (RV), and splenic artery involvement (RA), respectively. Disease-free survival (DFS) among patients with Rnone, RV, RA, and BRPC was 58.5, 18.4, 10.8, and 9.2months, respectively. Patients with RV and RA had significantly poorer DFS than patients with Rnone (P = 0.010, P < 0.001, respectively). Median survival among Rnone, RV, RA, and BRPC was 80.6, 23.4, 15.1, and 21.3months, respectively. Patients with RV and RA had significantly poorer survival than patients with Rnone (P = 0.001, P < 0.001, respectively) and had short survival similar to that of those with BRPC. Multivariate Cox proportional hazard analysis detected preoperative CA19-9 ≥ 37IU/L, radiologic splenic vein involvement, radiologic splenic artery involvement, intraoperative bleeding ≥ 500ml, transfusion, positive washing cytology, and noncompletion of adjuvant therapy as independent prognostic factors. Radiographic splenic artery involvement is a poor prognostic factor in resectable pancreatic body/tail cancer and may have a role in stratification of treatment strategy.

Sarcoidosis related to checkpoint and BRAF/MEK inhibitors in melanoma.

Therapy for advanced melanoma has deeply changed in the last decade with the introduction of checkpoint and BRAF/MEK inhibitors. Granulomatous reactions have been reported related to these drugs. We performed a systematic review of all the cases described in the medical literature by the search (("Melanoma"[Mesh]) AND ("Sarcoidosis"[Mesh] OR "Granuloma"[Mesh])). Ninety-one patients under immunotherapy were included in the analyses. The time from the initiation of the immunotherapy until the onset of sarcoidosis or sarcoid-like reaction (SLR) was 7.1months (SD 9). Peripheral lymph nodes as the mode of onset were seen more frequently in patients under CTLA-4 inhibitors (p=.016) whereas in patients under BRAF/MEK inhibitors used to be in the form of specific skin lesions (p=.006). Chest X-ray stage I-II was the rule in the CTLA-4 and PD-1 groups. On the contrary, stage 0 accounted for 80% of the patients in the BRAF/MEK group examined for pulmonary involvement. Specific skin involvement was the most common manifestation apart from pulmonary involvement. It was more frequent in patients under BRAF/MEK inhibitors and especially in the form of papules. Splenic involvement was found also more frequently in patients under CTLA-4 inhibitors. Specific treatment for sarcoidosis/SLR was prescribed in 50 patients (58.8%), without differences among groups. Almost all patients presented a good prognosis independently of the decision made regarding their previous immunotherapy. CONCLUSION: Physicians should bear in mind the possibility of sarcoidosis/SLR after the initiation of checkpoint or BRAF/MEK inhibitors in patients diagnosed with advanced melanoma, especially in the form of skin involvement and mediastinal and peripheral lymph nodes. It is important to achieve an accurate diagnosis to rule out the possibility of cancer involvement. What to do with these drugs is yet to be clarified. It seems reasonable to prioritize cancer treatment so it is not mandatory to stop these drugs.

Anaesthetic complications and management of a great dane presenting with acute respiratory distress

Anaesthesia can be challenging in animals with acute respiratory distress. This report details the management of a seven-year-old female great dane who initially presented with acute onset tachypnoea and retching....

Fluorodeoxyglucose-Positron Emission Tomography Predicts Bone Marrow Involvement in the Staging of Follicular Lymphoma.

Standard bone marrow biopsy (BMB) and bone involvement with follicular lymphoma (FL) on positron emission tomography (PET)/computed tomography (CT) both predict early clinical failure in FL. The key clinical question is whether PET/CT findings can obviate the need for BMB. The goal of this study was to determine the value of PET/CT in determining bone involvement in FL, using posterior iliac crest BMB as the gold standard. A total of 548 patients with newly diagnosed grade 1-3A FL were included. The presence, pattern, and location of bone involvement, spleen involvement, and standardized uptake values (SUVs) in the L3 vertebral body were recorded for all patients and compared with the BMB report. Excluding patients with focal bone lesions on PET/CT, the sensitivity and specificity of PET/CT in detecting bone or marrow involvement, compared with BMB, were 53% and 88%, respectively. The sensitivity and specificity of spleen involvement on PET/CT in predicting a positive BMB were 55% and 86%, respectively. An L3 SUVmax of less than 2.0 resulted in a negative predictive value (NPV) of 96% for marrow involvement on BMB; an L3 SUVmean below 1.4 resulted in an NPV of 100%. In newly diagnosed FL, PET/CT-detected bone and splenic involvement is highly specific for a positive BMB, and very low SUV values (<2.0 SUVmax and < 1.4 SUVmean ) in the lumbar spine have a high NPV for a negative BMB. Routine BMB may be obviated in these patients. BMB remains necessary to definitively exclude bone marrow involvement in a large majority of patients with a negative PET. Predicting early clinical failure in follicular lymphoma (FL) is important but difficult. Bone marrow involvement by FL is associated with early clinical failure, and determining this involvement is a key component of the initial staging. This study highlights that in certain patients, positron emission tomography/computed tomography is sufficient in determining bone or marrow involvement, without the need for a confirmatory bone marrow biopsy (BMB). An algorithm is provided based on these findings to help clinicians determine which patients would benefit from BMB and when it can be avoided.

Clinicopathologic characteristics of follicular lymphoma in hepatitis C virus-infected patients.

Follicular lymphoma (FL) is the most common subtype of indolent non-Hodgkin lymphoma. It has been hypothesized that chronic hepatitis C virus (HCV) infection stimulates IGH-BCL2 clone proliferation, leading to development of FL. Furthermore, regression of FL after antiviral treatment without chemotherapy has been reported in HCV-infected patients. To clarify the relationship between HCV and FL, we compared the prevalence of IGH-BCL2 translocation and other clinicopathologic characteristics between HCV-infected and HCV-uninfected FL patients and determined the impact of HCV eradication on the oncologic outcomes of HCV-infected FL patients. The study included HCV-infected patients (cases) with FL seen at our institution during 2004-2018. Cases were matched with HCV-uninfected FL patients (controls) according to year of lymphoma diagnosis, sex, and hepatitis B serology. We studied 19 cases and 57 controls. More cases than controls had splenic involvement of FL (26% vs 5%, P = 0.02), higher histologic grade (grade 3 in 56% vs 24%, P = 0.01), absent or weak CD10 expression (42% vs 11%, P = 0.005), and absent BCL2 expression (33% vs 4%, P = 0.004). Compared to controls, cases had a lower rate of detection of IGH-BCL2 translocation (31% vs 68%, P = 0.02). Finally, cases with a sustained virologic response (virologic cure of HCV) had a better 10-year overall survival rate than did cases not treated with antivirals or controls (P = 0.001). In conclusion, HCV-infected patients with FL have unique clinicopathologic characteristics including improved overall survival with HCV eradication. The pathogenesis of FL in HCV-infected patients seems unrelated to antiapoptotic effect of IGH-BCL2 rearrangement.

The Incidence of Venous Thromboembolism and Impact on Survival in Hodgkin Lymphoma

BackgroundThrombosis increase the acute and long-term morbidity and mortality in malignancy patients. We analyzed venous thromboembolism (VTE) in patients with Hodgkin lymphoma, the impact of VTE on survival, predisposing factors for VTE, and predicting value of Khorana and ThroLy score models. Patients and MethodsWe included 150 adult patients with Hodgkin lymphoma between January 2010 and 2018 at our university hospital. ResultsVTE was observed in 31 patients (20.7%). The types of VTE were 18 upper and 3 lower extremity deep vein thrombosis and 10 pulmonary embolism (1 with lower extremity deep vein thrombosis). Twenty-nine patients developed VTE during the treatment with a median time of episode as 5 months. In logistic regression analysis, a body mass index of >32 kg/m2, high fibrinogen levels, initial thrombocytosis and leukocytosis, splenic and extranodal involvement, presence of a central venous line, advanced stage, line of treatment status of thromboprophylaxis, VTE timing, and better Eastern Cooperative Oncology Group performance scores were observed to be related with VTE. Kaplan Meier survival analysis showed a negative impact of VTE on survival. Khorana and ThroLy risk assessment models were found predictive for VTE (P = .000 and P = .003, respectively), although only ThroLy score was associated with the survival. ConclusionThromboprophylaxis and precautions for VTE in patients with Hodgkin lymphoma according to validated risk assessment models can improve prognosis and quality of life owing to the impact of VTE on survival in the study.

Splenic infarction is not rare in granulomatosis with polyangiitis.

Splenic involvement is rarely reported in patients with ANCA-associated vasculitides (AAVs), particularly in those with granulomatosis with polyangiitis (GPA) and is in fact considered to be underestimated. We aimed to investigate the frequency of splenic lesions-mainly infarction-and related factors in patients with AAVs. Patients with AAV whose abdominal or thoracic computed tomographies (CTs) were performed at or after diagnosis were included in the study. CT images were examined for splenic lesions. Overall, 69 patients (median age at diagnosis 54 [IQR 24] years; 55% with GPA, 29% with microscopic polyangiitis, and 16% with renal-limited disease) were included in the analysis. Splenic pathologies were detected in 19 (28%) patients; 12/19 (63%) splenomegaly and 7/19 (37%) splenic infarction. All patients with splenic infarction exhibited GPA with PR3-ANCA-positive serology. Three of these seven patients had autosplenectomy. Patients with splenic infarction were younger at diagnosis (p = 0.018) with also significantly higher ear-nose-throat (ENT) (%100 vs 37; p = 0.002) and eye involvement (%50 vs %12; p = 0.044) than patients without splenic infarction. Splenic pathologies are not rare in AAVs. Furthermore, infarction can help separate GPA from MPA. In young patients with GPA, particularly those with ENT and eye involvements, physicians should consider splenic infarction.Key Points• Splenic infarction is more common than previously thought in ANCA-associated vasculitides, particularly in granulomatosis with polyangiitis.• Detecting splenic infarction can help differentiate granulomatosis with polyangiitis from other subgroups.

Distal pancreatectomy for pancreatic neoplasia: is splenectomy really necessary? A bicentric retrospective analysis of surgical specimens

BackgroundWhile distal pancreatectomy with splenectomy (DPS) is the reference treatment for pancreatic body and tail neoplasia, oncological benefits of splenectomy have never been demonstrated. Involvement of spleen, splenic hilum and lymph nodes (LN) was therefore assessed on DPS specimens. MethodsAll DPS pancreatic neoplasia specimens obtained in 2 Brussels University Hospitals over 15 years (2004–2018) were reviewed retrospectively, using both preoperative radiological imaging and postoperative pathological analyses of splenic parenchyma, hilar tissue and LN. ResultsThe total of 130 DPS specimens included 85 adenocarcinomas, 37 neuroendocrine neoplasms and 8 other carcinomas. Tumours involved the pancreatic body without tail invasion for 59 specimens (B, Body group), and the pancreatic tail with/without body for 71 (T, Tail group). At pathology, direct splenic and/or hilar involvement was observed in 13 T specimens (13/71, 18.3%), but in none belonging to the Body group. The observed numbers of splenic hilar LN (only reported in 49/130 patients) were low, only one T adenocarcinoma had positive splenic LN in addition to direct splenic involvement. ConclusionSplenectomy remains justified during pancreatectomy for neoplasia involving the pancreatic tail, but in case of pancreatic body tumours, its benefits should be questioned in the light of absent splenic LN/parenchymal involvement.

Higher prevalence of splenic artery aneurysms in hereditary hemorrhagic telangiectasia: Vascular implications and risk factors.

BackgroundSplenic artery aneurysm (SAA) is a rare but potentially fatal condition. Rupture results in 25% mortality up to 75% in pregnant women with 95% fetal mortality. Brief reports suggest an increased risk of developing SAA in patients with HHT.MethodsWe analyzed enhanced multidetector CT data in 186 HHT patients matched (gender and ± 5 year old) with 186 controls. We screened for SAA and recorded diameter of splenic and hepatic arteries and hepatic, pancreatic and splenic parenchymal involvements. We determined by univariate and multivariate analysis, the relationship with age, sex, genetic status, cardiovascular risk factors (CVRF) and visceral involvement.ResultsSAA concerned 24.7% of HHT patients and 5.4% of controls, p<0.001. Factors associated with increased risk of SAA in HHT were female gender (p = 0.04, OR = 2.12, IC 95% = 1.03–4.50), age (p = 0.0003, OR = 1.04, 95% CI = 1.02–1.06) and pancreatic parenchymal involvement (p = 0.04, OR = 2.13, 95% CI = 1.01–4.49), but not type of mutation, hepatic or splenic parenchymal involvements, splenic size or splenic artery diameter or CVRF.ConclusionsWe found a 4.57 higher rate of SAA in HHT patients without evidence of splenic high output related disease or increased CVRF. These results suggest the presence of a vascular intrinsic involvement. It should lead to screening all HHT patients for SAA. The vasculopathy hypothesis could require a change in management as screening of all systemic arteries and even the aorta and to further research in the field.

Characteristics of extranodal classic Hodgkin lymphoma: a retrospective analysis of 175 patients

Aim To find out the characteristics of extranodal classical Hodgkin lymphoma (cHL). Patients and methods A retrospective analysis was done on 175 patients with cHL who attended the Oncology Center of Mansoura University from January 1, 2008 to May 31, 2016, with a median follow-up of 2.93 years (range, 0.05–10.2 years). Results One (0.6%) patient was diagnosed with primary extranodal cHL and 48 (27.4%) patients had secondary extranodal cHL. Liver (25.3%), lung (24.1%), and the bone (20.3%) were the most frequent extranodal sites. A total of 27 (55.1%) patients had one extranodal site, 16 (32.7%) patients had two extranodal sites, and six (12.2%) patients had more than or equal to three sites. Overall, 42 (85.71%) patients with extranodal sites were less than 45 years old (P=0.019), 15 (30.6%) had splenic involvement (P=0.003), 39 (79.6%) patients had absolute monocyte count /absolute lymphocyte count (monocyte-lymphocytes ratio) more than or equal to 0.305 (P Conclusion Most of the extranodal cHL cases were secondary (97.96%) and significantly associated with unfavorable clinical features and lower response rate to standard treatment. To our knowledge, this is the first study to demonstrate that extranodal cHL is statistically associated with elevated monocyte-lymphocytes ratio.

Clinical Characteristics and Treatment Outcomes in Children, Adolescents, and Young-adults with Hodgkin's Lymphoma: a KPHOG Lymphoma Working-party, Multicenter, Retrospective Study

BackgroundHodgkin's lymphoma (HL) constitutes 10%–20% of all malignant lymphomas and has a high cure rate (5-year survival, around 90%). Recently, interest has increased concerning preventing secondary complications (secondary cancer, endocrine disorders) in long-term survivors. We aimed to study the epidemiologic features and therapeutic outcomes of HL in children, adolescents, and young adults in Korea.MethodsWe performed a multicenter, retrospective study of 224 patients aged < 25 years diagnosed with HL at 22 participating institutes in Korea from January 2007 to August 2016.ResultsA higher percentage of males was diagnosed at a younger age. Nodular sclerosis histopathological HL subtype was most common, followed by mixed cellularity subtype. Eighty-one (36.2%), 101 (45.1%), and 42 (18.8%) patients were classified into low, intermediate, and high-risk groups, respectively. Doxorubicin, bleomycin, vinblastine, dacarbazine was the most common protocol (n = 102, 45.5%). Event-free survival rate was 86.0% ± 2.4%, while five-year overall survival (OS) rate was 96.1% ± 1.4%: 98.7% ± 1.3%, 97.7% ± 1.6%, and 86.5% ± 5.6% in the low, intermediate, and high-risk groups, respectively (P = 0.021). Five-year OS was worse in patients with B-symptoms, stage IV disease, high-risk, splenic involvement, extra-nodal lymphoma, and elevated lactate dehydrogenase level. In multivariate analysis, B-symptoms and extra-nodal involvement were prognostic factors for poor OS. Late complications of endocrine disorders and secondary malignancy were observed in 17 and 6 patients, respectively.ConclusionThis is the first study on the epidemiology and treatment outcomes of HL in children, adolescents, and young adults in Korea. Future prospective studies are indicated to develop therapies that minimize treatment toxicity while maximizing cure rates in children, adolescents, and young adults with HL.