Synchronous Primary Central Nervous System and Pulmonary Lymphoma in a 7-Year-Old Female with Unspecified T-Cell Immunodeficiency.

Primary central nervous system lymphoma (PCNSL) is rare in children with immunocompromise as an important risk factor. A 7-year-old girl with unspecified T-cell immunodeficiency presented with left-sided weakness and was found to have a right-sided frontal lobe mass on imaging. The mass was resected; histopathology and molecular studies evidenced diffuse large B-cell lymphoma. Prior chest imaging had revealed left upper lobe mass, and repeat chest imaging revealed multiple pulmonary nodules, initially concerning for metastasis. Video-assisted thoracoscopic surgical wedge resection of the lung mass was performed; the molecular profile was distinct from the PCNSL, suggesting synchronous de novo lymphomagenesis of brain and pulmonary primaries.

Trends in Bone Marrow Sampling and Core Biopsy Specimen Adequacy in the United States and Canada: A Multicenter Study.

OBJECTIVES: To assess bone marrow (BM) sampling in academic medical centers. METHODS: Data from 6,374 BM samples obtained in 32 centers in 2001 and 2011, including core length (CL), were analyzed. RESULTS: BM included a biopsy (BMB; 93%) specimen, aspirate (BMA; 92%) specimen, or both (83%). The median (SD) CL was 12 (8.5) mm, and evaluable marrow was 9 (7.6) mm. Tissue contraction due to processing was 15%. BMB specimens were longer in adults younger than 60 years, men, and bilateral, staging, and baseline samples. Only 4% of BMB and 2% of BMB/BMA samples were deemed inadequate for diagnosis. BM for plasma cell dyscrasias, nonphysician operators, and ancillary studies usage increased, while bilateral sampling decreased over the decade. BM-related quality assurance programs are infrequent. CONCLUSIONS: CL is shorter than recommended and varies with patient age and sex, clinical circumstances, and center experience. While pathologists render diagnoses on most cases irrespective of CL, BMB yield improvement is desirable.

Reproducibility of TEG Parameters in Stable Cirrhotics.

BACKGROUND: Thromboelastography (TEG) has become the standard of care in liver-transplant surgery to identify real-time abnormalities in the coagulation cascade. To our knowledge, no studies have been performed to measure the intrasubject reproducibility of TEG parameters in cirrhotic patients. OBJECTIVE: To perform a validation study to determine the reproducibility of TEG in cirrhosis. METHODS: We recruited 30 patients with stable cirrhosis and tested 25 of them. Two blood specimens were drawn 1 hour apart; we measured the TEG parameters R time, K time, angle, maximum amplitude (MA), and functional fibrinogen (FF), along with conventional coagulation parameters. Reproducibility was assessed using the intraclass coefficient test. The TEG parameters were then compared with conventional coagulation test results. RESULTS: The K time, angle, MA, and FF results showed excellent reproducibility (r > 0.7; P <.001). Platelets and fibrinogen correlated with MA and K time; prothrombin time (PT) and activated partial thromboplastin time (aPTT) were inversely correlated with MA. CONCLUSION: All parameters were reproducible when measured 1 hour apart. TEG may be suitable to investigate coagulation characteristics in patients with clinically stable cirrhosis; however, further studies are needed in patients with more advanced cirrhosis, in whomblood product use may be more prevalent.

Equal 3-Year Outcomes for Kidney Transplantation Alone in HCV-Positive Patients With Cirrhosis.

Kidney transplantation alone in clinically compensated patients with cirrhosis is not well documented. Current guidelines list cirrhosis as a contraindication for kidney transplantation alone. This is an Institutional Review Board-approved retrospective study. We report our experience with a retrospective comparison between transplants in hepatitis C virus-positive (HCV(+)) patients without cirrhosis and HCV(+) patients with cirrhosis. All of the patients were followed for at least a full 3-year period. All of the deaths and graft losses were recorded and analyzed using Kaplan-Meier methodology. One- and three-year cumulative patient survival rates for noncirrhotic patients were 91% and 82%, respectively. For cirrhotic patients, one- and three-year cumulative patient survival rates were 100% and 83%, respectively (P = NS). One- and three-year cumulative graft survival rates censored for death were 94% and 81%, and 95% and 82% for the noncirrhosis and cirrhosis groups, respectively (P = NS). Comparable patient and allograft survival rates were observed when standard kidney allograft recipients were analyzed separately. This study is the longest follow-up document in the literature showing that HCV(+) clinically ompensated patients with cirrhosis may undergo kidney transplantation alone as a safe and viable practice.

Liver transplant for neuroendocrine tumor metastatic to the liver: literature review and report of extirpation at 16-year recurrence.

New information has demonstrated that there are few long-term disease-free survivors after a liver transplant for neuroendocrine tumors. All studies have limited follow-up to 10 years after a transplant. We present the case of a recurrent metastatic carcinoid in a patient 16 years after an orthotopic liver transplant. The subject initially presented with worsening chronic diarrhea, hypoglycemia, and confusion with massive hepatomegaly. The postoperative pathology report showed 80% to 90% of the liver tissue replaced by biopsy-proven synaptophysin-positive intrahepatic tumor with neuroendocrine differentiation. At the time of his liver transplant, he also underwent a distal pancreatectomy and splenectomy. Nuclear medicine tumor location studies, ultrasound, and computed tomography studies were performed at regular yearly intervals for 8 years on follow-up. Sixteen years after his orthotopic liver transplant, a retroperitoneal mass was detected showing neuroendocrine differentiation. Older studies focusing on an orthotopic liver transplant for highlighted clinical features would positively predict long-term survival. Older studies found the following features to be predictive of long-term survival in liver transplant for neuroendocrine tumors: age < 55 years, < 50% replacement of liver with metastatic neoplastic tissue and carcinoid type. These features were identified on multiple studies as positive predictors of disease-free survival. These studies were limited to, at most, 10-year follow-up. Newer studies have examined molecular features such as expression of E-cadherin and Ki-67 as positive predictors of long-term survival. However, no study has determined the full natural history of these tumors and for how long these patients should be followed. This anecdotal report highlights that late recurrence can occur.

Primary antiphospholipid syndrome and necrotizing pancreatitis: a diagnostic challenge.

The objective of this study was to report an unusual case of primary antiphospholipid syndrome (APS)-associated severe necrotizing pancreatitis. Since the APS was first recognized in the 1980s, a number of manifestations of the disorder have been described. We report primary APS presenting as severe necrotizing pancreatitis. This is the first such case to date that fulfills the revised Sapporo classification criteria. A 38-year-old previously healthy woman presented with new-onset hypertensive emergency and acute kidney injury. She subsequently developed severe epigastric pain attributable to necrotizing pancreatitis and extensive splenic infarcts. Biopsies of both the pancreas and kidney revealed thrombotic microangiopathy. Her lupus anticoagulant was positive on both weeks 1 and 12 of her disease course. A diagnosis of primary APS was made. Despite 6 months of aggressive care, she died of sepsis.

Risk factor assessment of young patients with acute myocardial infarction.

UNLABELLED: The Middle East represents an attractive area for young individuals to seek employment, where they are exposed to numerous environmental conditions. The pursuit of a better standard of living has driven hundreds to the Middle East over the recent decades. This influx has also resulted in a predisposition to premature coronary artery disease (CAD). The aim of this study was to provide an overview of the risk factors in patients younger than 45 years, presenting with acute myocardial infarction (AMI). Out of the 148 patients analyzed, 137 were males and 11 females. 119 were from South Asia and 29 were Arabs. Their mean age was 36 ± 4.2 years. Smoking was the most prevalent risk factor in both groups at 67.6%. This was followed by hypertension, family history of CAD, hyperlipidemia and Diabetes mellitus. There was no significant difference in the clinical risk factor profile between these two groups. ST elevation myocardial infarction (STEMI) was noted in 67.6%, while 32.4% patients suffered a Non ST elevation myocardial infarction (NSTEMI). 84.5% received coronary stents, 8.8% had lone thrombus aspiration or balloon angioplasty only, while the rest were treated by conservative medical management or referred for coronary artery bypass surgery. CONCLUSION: There is no significant difference in the CAD risk profile between young South Asian and Arab patients. Preventive strategies focused on risk factor reduction, especially smoking cessation, should be implemented to protect young adults in the most productive years of their life.

Approach to intraoperative consultation for donor liver biopsies.

CONTEXT: As demand for organs to treat end-stage liver disease increases, donor livers once deemed only marginally suitable for donation are being considered for transplantation. Pathologists are increasingly being asked to evaluate these livers for acceptability. This article provides guidelines for frozen section evaluation of livers for transplantation. OBJECTIVE: This article concentrates on the histopathologic features of transplant suitability with appropriate clinicopathologic correlation for the practicing pathologist. Recommendations for proper handling and sampling of tissue are discussed. Relative and absolute contraindications as well as artifacts and benign conditions are emphasized. DATA SOURCES: Sources include a compilation of the authors' experiences in academic and community liver transplantation centers. In addition, relevant medical literature was reviewed, as well as Web sites specializing in organ transplantation, such as Transplant Pathology Internet Services and the Organ Procurement and Transplantation Network. CONCLUSIONS: Malignancy and extensive necrosis in the liver are absolute contraindications to transplantation. Evaluation of macrosteatosis, fibrosis, hepatitis, and necrosis depends on the severity of disease and correlation with the clinical situation. Donor age of greater than 60 years does not preclude transplantation. Artifacts and benign conditions need to be understood to prevent wastage of precious organs and to ensure that an appropriate organ is provided for the recipient.

Porcine intact and wounded skin responses to atmospheric nonthermal plasma.

Thermal plasma is a valued tool in surgery for its coagulative and ablative properties. We suggested through in vitro studies that nonthermal plasma can sterilize tissues, inactive pathogens, promote coagulation, and potentiate wound healing. The present research was undertaken to study acute toxicity in porcine skin tissues. We demonstrate that floating electrode-discharge barrier discharge (FE-DBD) nonthermal plasma is electrically safe to apply to living organisms for short periods. We investigated the effects of FE-DBD plasma on Yorkshire pigs on intact and wounded skin immediately after treatment or 24h posttreatment. Macroscopic or microscopic histological changes were identified using histological and immunohistochemical techniques. The changes were classified into four groups for intact skin: normal features, minimal changes or congestive changes, epidermal layer damage, and full burn and into three groups for wounded skin: normal, clot or scab, and full burn-like features. Immunohistochemical staining for laminin layer integrity showed compromise over time. A marker for double-stranded DNA breaks, γ-H2AX, increased over plasma-exposure time. These findings identified a threshold for plasma exposure of up to 900s at low power and <120s at high power. Nonthermal FE-DBD plasma can be considered safe for future studies of external use under these threshold conditions for evaluation of sterilization, coagulation, and wound healing.

Severe hepatitis C virus recurrence is nearly universal after donation after cardiac death liver transplant.

OBJECTIVES: The rate of hepatitis C virus recurrence after donation after cardiac death liver transplant is not clearly defined. MATERIALS AND METHODS: This is a retrospective review of 39 donations after cardiac death-liver transplant recipients. Biopsies were performed at 6, 12, 24, and 36 months for all hepatitis C virus positive donation after cardiac death recipients. RESULTS: The 6-, 12-, 24-, and 36-month severe hepatitis C virus recurrence rates were 60%, 73%, 87%, and 94%. A histologic comparison group of 26 long-surviving hepatitis C virus positive donation after neurologic death recipients had severe hepatitis C virus recurrence 27%, 31%, 42%, and 52% of the time. Six of the 19 hepatitis C virus donation after cardiac death patients developed cirrhosis at a median of 56 months (range, 14-119 months). There was no significant 3-year allograft and patient survival difference between hepatitis C virus and nonhepatitis C virus donation after cardiac death recipients. The factors most associated with decreased survival in the entire cohort included biliary and vascular complications. Organs procured by our institution's attending surgeons were associated with a better 3-year allograft survival. CONCLUSIONS: Severe hepatitis C virus recurrence was nearly universal but did not lead to increased graft loss when compared with nonhepatitis C virus donation after cardiac death at 3 years. These data may justify early interferon treatment in these at-risk patients.

Falsely elevated tacrolimus levels caused by immunoassay interference secondary to beta-galactosidase antibodies in an infected liver transplant recipient.

Careful interpretation of tacrolimus levels is essential to ensure optimal immunosuppressive therapy while avoiding toxicity. Interference with tacrolimus assays may be an underreported event that has the potential to result in negative patient outcomes through unnecessary modifications of therapy. We describe a 55-year-old liver transplant recipient who had falsely elevated tacrolimus levels that led to the eventual disruption of his immunosuppressive therapy and subsequent rejection of his allograft.Although his increased tacrolimus levels did not correlate with clinical signs and symptoms of tacrolimus toxicity, interruption of therapy in this patient was supported by an acute infection and a slight elevation in serum creatinine concentration. Tacrolimus levels were analyzed by using an antibody conjugated magnetic immunoassay method, and levels as high as 79.7 ng/ml were observed, despite discontinuation of tacrolimus. We conducted an evaluation for assay interference by using an alternative assay method(microparticle enzyme immunoassay), by testing plasma samples that were not hemolyzed, and by analyzing levels of an unrelated drug that uses the same technology as the initial tacrolimus assay. beta-galactosidase antibodies were ultimately confirmed as the cause of the immunoassay interference. Inpatients receiving tacrolimus, spuriously high tacrolimus levels should be carefully evaluated, and drastic adjustments to therapy should be made only within the context of clinical toxicity.

Fatal fat embolism in isolated vertebral compression fracture.

Fat embolism after long bone and pelvic fractures as well as orthopedic interventions is a well-documented phenomenon, but it is highly unusual after isolated vertebral fractures. We report a case of fatal fat embolism in a 78-year-old man after an isolated vertebral compression fracture with no related orthopedic intervention. A high index of suspicion is necessary for early diagnosis and successfully treating this unusual complication.

Classical Hodgkin lymphoma concurrently evolving in a patient with marginal zone B-cell lymphoma of the spleen.

Combination of the splenic marginal zone B-cell lymphoma (SMZL) and classical Hodgkin lymphoma (cHL) is extremely rare. We report a unique case with concurrent SMZL and cHL. The patient was a 63-year-old man who presented with fatigue and anemia, showing a splenomegaly and retroperitoneal lymphadenopathy. A splenectomy revealed monotonous marginal zone lymphocytic infiltrates and numerous large Reed-Sternberg-like cells. Flow cytometry revealed a kappa light-chain-restricted CD5 (-), CD23 (-) B-cell population. DNA polymerase chain reaction analysis confirmed the presence of clonal rearrangement of the immunoglobulin heavy-chain gene. Immunohistochemical studies revealed that the large atypical cells were CD30 (+), CD15 (weakly +), CD20 (-), CD45 (-), Pax5 (weakly +), BOB.1 (-), and Oct2 (-), indicating the coexistence of SMZL with cHL. After the chemotherapy, the patient achieved a clinical/radiologic remission, whereas cHL was detected in liver and bone marrow subsequently. The case indicates that both components of lymphoma can present concurrently as a composite form of lymphoma and both need to be treated adequately.

Diagnosis of T-cell lymphoma in body fluids: cytologic features and unusual flow cytometric findings.

OBJECTIVE: To demonstrate the unique morphologic and phenotypic features observed in cases of T-cell lymphomas presenting as effusions. STUDY DESIGN: Cytologic slides and flow cytometric histograms of 8 cases of body fluids with T-cell lymphoma were retrospectively reviewed. Morphologic features, flow cytometric histograms and immunophenotypes of the cells were evaluated. RESULTS: Three of the 8 cases showed 1 or more of the following: intermediate-to-large cells with an increased nuclear-cytoplasmic ratio, finely granular or vacuolated cytoplasm and round or convoluted vesicular nuclei with a prominent single or multiple nucleoli. Flow cytometric studies of these 3 cases showed an abnormal scatter pattern in the myelomonocytic region of the histograms. Phenotypic analysis revealed variable expression of a T-cell phenotype. The remaining cases showed the conventional morphologic and flow cytometric features of a T-cell lymphoma. CONCLUSION: Morphologic alterations of neoplastic T-cells in body fluids can result in a variety of potentially incorrect diagnoses. The unusual flow cytometric histogram can serve as a useful clue for the diagnosis of T-cell lymphoma in body fluids but could be a potential pitfall for a false negative. Detailed cytologic evaluation combined with flow cytometric study can improve diagnostic accuracy.