A Case of Rosai-Dorfman Disease Presenting as an Isolated Pleural Plaque.

Rosai-Dorfman disease (RDD) is a rare non-Langerhans histiocytic disorder primarily involving lymph nodes. Extranodal RDD has a heterogenous presentation, and isolated pulmonary involvement is rare. We report the only case of RDD presenting as an isolated pleural mass. Our patient was a 55-year-old female with multiple comorbidities who presented with chest pain. Imaging revealed an enlarging pleural-based lesion. She underwent resection of the pleural mass, showing an atypical histiocytic infiltrate in a prominent background of collagenous fibrosis. Immunohistochemistry shows CD1a-negative and S100-positive atypical histiocytic cells demonstrating emperipolesis, confirming the diagnosis of RDD. She is currently on six-month CT surveillance with no recurrence of the disease. This case highlights the unique pulmonary presentation of RDD. It also underscores that observations may be appropriate in isolated asymptomatic pleural involvement cases.

Impact of Treatment Parameters on Racial Survival Differences in Oropharyngeal Cancer: National Cancer Database Study.

OBJECTIVE: To investigate how differences in treatment parameters account for survival differences between races of patients with oropharyngeal squamous cell carcinoma (OPSCC). STUDY DESIGN: Retrospective cohort study. SETTING: National Cancer Database. METHODS: Data of patients with OPSCC undergoing radiation therapy (RT) or concurrent chemoradiation therapy as primary treatment were obtained from the National Cancer Database from 2004 to 2016. We analyzed 4 treatment-related time intervals to determine their impact on survival between races when controlling for human papilloma virus (HPV) status. Cox proportional hazards models, stepwise logistic regressions, covariate adjustments, and propensity score matching were performed. RESULTS: A total of 3152 patients were identified (2877 White, 275 Black). In HPV- cases, Black patients with prolonged radiation duration had a significantly worse overall survival as compared with White patients (hazard ratio, 1.77; 95% CI, 1.03-3.05; P = .039). In a logistic regression model, the only covariate that was significantly associated with prolonged RT was facility type. When further adjusted for facility type, the survival difference between Black and White patients with HPV- status and prolonged RT times was no longer significant (hazard ratio, 1.55; 95% CI, 0.90-2.69; P = .116). CONCLUSIONS: There is a significant disparity in overall survival between Black and White patients with HPV- OPSCC when RT duration is prolonged. Clinicians should be aware of the negative impact of prolonged RT, especially in Black patients, so that they can attempt to decrease treatment-related time intervals. Facility type was also found to affect the outcomes of patients with OPSCC, and efforts should be made to improve patient access to well-equipped, high-volume facilities.

Severe Platelet Transfusion Refractoriness in Association with Antibodies Against CD36.

Platelet-transfusion refractoriness (PTR) is common in patients with hematological malignancies. The etiology of immune PTR is typically human leukocyte antigen (HLA) antibodies (Abs) from pregnancy or previous transfusion. Herein, we report PTR in the setting of induction chemotherapy for acute myelogenous leukemia (AML) from Abs against CD36/glycoprotein (GP)IV. A 66-year-old African American woman presented with anemia and thrombocytopenia. She was found to have transfusion-dependent AML, and a 7 + 3 regimen (7 days of standard-dose cytarabine and 3 days of an anthracycline antibiotic or an anthracenedione, most often daunorubicin) was initiated. The patient developed profound thrombocytopenia, with platelet nadir of 0 by day 13. The results of HLA antibody screening were negative. However, the results of a screening test for platelet-specific antibodies screen showed Abs against cluster of differentiation (CD)36. The platelets of the patient lacked expression of CD36, and DNA analysis showed mutations in the CD36 gene. HLA Ab-mediated PTR is common in patients with hematological malignancies. However, once HLA Abs are excluded, other less-frequent Abs should be considered, particularly in patient populations of Asian, African, or Middle Eastern descent.

A 30-Year-Old Carrier of Gaucher Disease with Multiple Myeloma.

We are reporting a case of a 30-year-old male with no past medical history who presented with new onset of renal failure, anemia, and splenomegaly and was diagnosed with multiple myeloma. Given the splenomegaly and the patient's Jewish heritage, blood tests were done and the patient was found to be a Gaucher disease carrier. The association of Gaucher disease and multiple myeloma has previously been reported; however, we want to describe the case of a young Gaucher disease carrier who developed multiple myeloma and provide a review of the literature.

Corrigendum to "High on Cannabis and Calcineurin Inhibitors: A Word of Warning in an Era of Legalized Marijuana".

[This corrects the article DOI: 10.1155/2016/4028492.].

ABO identical and washed blood transfusions as candidate strategies to reduce early mortality in acute promyelocytic leukemia.

BACKGROUND: Despite dramatically improved long term outcomes seen with all-trans retinoic acid therapy, and now arsenic trioxide, in acute promyelocytic leukemia (APL), early mortality remains a substantial challenge. Recent data from a single center study and the Surveillance, Epidemiology and End Results (SEER) registry report 30day mortality rates of 26% (n=18 of 70) and 17% (n=238 of 1400), respectively. Early deaths are predominately due to hemorrhage. Patients with APL invariably have abnormal laboratory hemostasis tests. The standard of practice is to prophylactically transfuse platelets, plasma and cryoprecipitate to mitigate abnormal platelet counts, PT/PTT and fibrinogen levels. Standard blood bank practice is to transfuse platelets, plasma and cryoprecipitate largely without regard to ABO blood group (platelets, cryoprecipitate), and, in some centers, transfusing ABO non-identical universal donor group AB plasma. Evidence from observational studies suggests that use of ABO non-identical blood components may be associated with increased bleeding. We hypothesized that use of ABO identical blood components and saline washed transfusions (red cells and platelets) would be associated with reduced early mortality in APL by avoidance of transfusion induced hemostatic dysfunction. METHODS: This is a single center cohort study of APL patients treated in an 800 bed university community and referral hospital. Novel approaches to transfusion support, based upon randomized trials, include implementation of ABO identical platelet transfusions for all patients with acute leukemia in 1990, use of only ABO identical cryoprecipitate in 2005, and washed transfusions of red cells and platelets for all patients with acute leukemia <50years of age beginning in 2006. Plasma transfusion has always been ABO identical. Two comparison populations were recent literature reports and the New York State Cancer Registry. We characterized 30 day mortality in APL patients seen in our institution since 2000 as a convenience sample comparable to literature reports, beginning approxcimately when ATRA therapy became uniform for induction therapy. Only patients receiving their induction therapy in our hospital were included. RESULTS: Of 41 patients there were 2 early (30 day) deaths (5%; a 71-81% reduction from expected). Early mortality at 100 days was 7% (n=3). The 30 day mortality in the younger cohort <50years of age (n=16) receiving washed transfusions was 0%. Restricting the analysis to patients treated since 2006 (ABO identical transfusions, mostly washed) (n=27; mean age 43 years; median 41 years; range 12-79), the early mortality rate at 30days was 3.7%. Long-term survival (5 years) of our APL patients was similar to New York State Cancer Registry and literature reports (80-83%). DISCUSSION: APL patients supported with transfusion regimens including ABO identical blood components, with or without washing, experienced early mortality at 30 days that was strikingly improved (71% to 86% lower) compared with that reported in the recent literature (3.7% to 5% vs. 17% to 26%). If these observed low rates of early mortality are related to transfusion practices, avoidance of ABO immune complex formation, and subsequent interference with hemostasis, is a plausible contributing mechanism. These favorable results provide a rationale for randomized trials of relatively simple and inexpensive approaches to reducing early hemorrhagic mortality in APL: use of ABO identical transfusions and washing to remove supernatant plasma.

Bilateral Myelomatous Pleural Effusion in a Patient with IgA Kappa Multiple Myeloma.

Multiple myelomas is a neoplastic plasma cell disorder that accounts for one percent of all cancers and 13% of hematologic malignancies. Although primarily known to be a bone marrow disorder, it can metastasize to extramedullary sites or it can present as a solitary extramedullary plasmacytoma. Primary pleural effusion from myeloma is rare, occurring in less than one percent of the patients. The following case report highlights a case of bilateral pleural effusion, directly attributable to multiple myeloma after other causes were ruled out. The diagnosis was made using cytology and immunohistochemical (IHC) staining of the pleural fluid. Myelomatous pleural effusion (MPE) is a poor prognostic feature heralding an aggressive underlying disease state, as represented in this case.

Rapid response to intravenous vitamin K may obviate the need to transfuse prothrombin complex concentrates.

BACKGROUND: Patients on warfarin who present with bleeding or who require an urgent procedure are commonly treated with intravenous (IV) vitamin K, which is supplemented with repletion of the vitamin K factors using either plasma or a prothrombin complex concentrate (PCC). In some such cases, use of vitamin K alone could be adequate to achieve acceptable hemostasis. STUDY DESIGN AND METHODS: An algorithm emphasizing the use of vitamin K alone in patients presenting with non-life-threatening bleeding was encouraged, with repeat testing of the international normalized ratio (INR) within 5 hours. Depending on the INR result, patients received no factor repletion or plasma or PCC, as judged by the physician. Leftover samples from a separate cohort of patients with supratherapeutic INRs (INR > 4.0) were studied for clotting factor evaluation. RESULTS: A total of 46 pre- and postinfusion INRs were evaluable from 41 patients. Median INR decreased from 5.8 to 2.5, with a median dose of 5 mg after a median time of 4.0 hours postinfusion. A total of 27 of 46 (59%) postinfusion samples showed an INR of 2.5 or less. Samples from patients with the highest INR showed the greatest decline in INR. Samples from supratherapeutic INR patients showed very high Factor VIII:C (200%) and a normal activated partial thromboplastin time in 23 of 50 (46%). CONCLUSION: Use of IV vitamin K as sole therapy for urgent partial reversal of warfarin for non-life-threatening bleeding may provide adequate hemostasis within 5 hours, avoiding the need for clotting factor repletion.

High on Cannabis and Calcineurin Inhibitors: A Word of Warning in an Era of Legalized Marijuana.

Tacrolimus, a potent immunosuppressant medication, acts by inhibiting calcineurin, which eventually leads to inhibition of T-cell activation. The drug is commonly used to prevent graft rejection in solid organ transplant and graft-versus-host disease in hematopoietic stem cell transplant patients. Tacrolimus has a narrow therapeutic index with variable oral bioavailability and metabolism via cytochrome P-450 3A enzyme. Toxicity can occur from overdosing or from drug-drug interactions with the simultaneous administration of cytochrome P-450 3A inhibitors and possibly P-glycoprotein inhibitors. Tacrolimus toxicity can be severe and may include multiorgan damage. We present a case of suspected tacrolimus toxicity in a postallogeneic hematopoietic stem cell transplant patient who was concurrently using oral marijuana. This case represents an important and growing clinical scenario with the increasing legalization and use of marijuana throughout the United States.