Nicholas J. Olson

Blood component transfusion and wastage rates in the setting of massive transfusion in three regional trauma centers

The purpose of massive transfusion protocols (MTPs) is to provide large quantities of blood products rapidly to exsanguinating patients. The expected rates of blood product transfusion and wastage in this setting have not been defined. This study was undertaken to assess the transfusion and wastage rates for bleeding patients requiring emergency issue of blood components at three American Level I trauma centers.

Factor V Inhibitors: A Diagnostic and Therapeutic Challenge

Historically, inhibitors to coagulation factor V (FV) most often have developed in patients treated with bovine thrombin, a topical hemostatic agent used during surgical procedures. With the advent of newer hemostatic agents, and the concurrent diminished use of bovine thrombin, the incidence of FV inhibitors has fallen. Nevertheless, FV inhibitors are occasionally seen on an idiopathic basis as well as in association with medications, malignancies, autoimmune disorders, pregnancy, and infections. Factor V inhibitors may present with life-threatening bleeding or thrombosis, or they may be discovered incidentally as a coagulation screening test abnormality. Management of patients with FV inhibitors is challenging and consists of control of bleeding and eradication of the inhibitor. In this short overview we review the role of platelet and plasma FV in hemostasis and discuss the unique characteristics, clinical features, diagnosis, treatment, and prognosis associated with FV inhibitors.

Successful use of cryocrit for monitoring response to therapeutic plasma exchange in type 1 cryoglobulinemia

Waldenstrom macroglobulinemia (WM) is a clinical syndrome that is defined as lymphoplasmacytic lymphoma with bone marrow involvement and IgM monoclonal gammopathy of any level. In some instances WM can result in a type I cryoglobulinemia with very high cryocrits, which is unusual in type II and III cryoglobulinemia. We describe a case of an 80 year old male with WM, severe type I cryoglobulinemia, and an extremely elevated cryocrit (69%). Over the course of five weeks we performed nine therapeutic plasma exchanges (TPE), and after seven treatments his cryocrit had decreased to 6% with improvement in his symptoms. By monitoring his cryocrit throughout his TPE sessions, we were able to assess his response to treatment, determine the ideal length of treatment in addition to his symptomatic improvement. J. Clin. Apheresis 31:403–404, 2016.

A novel case of an aggressive superficial spindle cell sarcoma in an adult resembling fibrosarcomatous dermatofibrosarcoma protuberans and harboring an EML4-NTRK3 fusion

A subset of soft tissue sarcomas often harbors recurrent fusions involving protein kinases. While some of these fusion events have shown utility in arriving at a precise diagnosis, novel fusions in otherwise difficult to classify sarcomas continue to be identified. We present a case of a 40‐year‐old female who noted a lower back nodule in 2010 that was initially labeled as a dermatofibrosarcoma protuberans with fibrosarcomatous transformation. The lesion recurred the following year and metastasized to the groin 6 years later. Because of some morphologic peculiarities, molecular characterization was pursued in the metastatic focus, which revealed the neoplasm was negative for the COL1A1‐PDGFB fusion. However, anchored multiplex polymerase chain reaction for targeted next‐generation sequencing (Archer Dx) detected an EML4‐NTRK3 fusion, which was confirmed by reverse transcription‐PCR, Sanger sequencing and RNA sequencing analysis of the recurrent and metastatic specimens. Although various soft tissue neoplasms involving fusions with NTRK genes are well‐reported, the current case could not be easily classified in any of the established entities. Nevertheless, it raises interesting questions regarding the importance of classification, prognosis, and treatment for some of these tyrosine kinase fusion‐driven sarcomas.

Synchronous Pulmonary Adenofibroma and Solitary Fibrous Tumor: Case Report and Review of the Literature

Pulmonary adenofibroma (PAF) is a rare neoplasm that may be related to solitary fibrous tumor (SFT). A subset of PAFs harbor the NAB2-STAT6 fusion that is typical of SFT, but a significant proportion do not. Their distinction is clinically important as SFTs can potentially have an aggressive clinical course, while there has been no report of a PAF behaving in a malignant fashion. We report a case of a 60-year-old male who developed a SFT and PAF in the same lung. The SFT harbored a NAB2-STAT6 fusion, while the PAF did not have any identifiable fusion. This case represents the first instance of a single patient with both of these tumors occurring simultaneously in the same lung.

Central xanthoma of the jaw in association with Noonan syndrome

Xanthomas are histiocytic lesions of the skin, soft tissue, and bone and are generally considered to be reactive in nature. When they arise in the bones of the jaw, they are referred to as central xanthomas. New evidence supports the hypothesis that central xanthomas are a separate and distinct entity from their extragnathic counterparts. Noonan syndrome (NS) is an autosomal dominant disorder that has been associated with giant cell lesions, which also commonly occur in the jaw. We present a case of a 15-year-old boy with NS who presented with a radiolucent lesion of the mandible that on excision was found to be a central xanthoma. Although giant cell lesions have been well described in NS, xanthomas of the jaw have not been reported. We will also discuss the entities that must be excluded before making a diagnosis of central xanthoma, as this can affect both treatment and follow-up.

Dedifferentiated Solitary Fibrous Tumor: A Concise Review

Solitary fibrous tumor (SFT) is a unique mesenchymal neoplasm that was originally believed to be of submesothelial origin. Eventually, SFT expanded to include what was previously called hemangiopericytoma in other regions of the body that had similar immunohistochemical and morphologic features. Although most are benign, many studies have tried to identify histologic features that predict which tumors will behave in an aggressive manner. Recently, dedifferentiation has been described in rare cases of SFT and does appear to correlate with a more aggressive clinical course. Dedifferentiated SFT occurs in a similar age range and location as conventional SFT and can resemble multiple different malignant entities. Utilization of ancillary studies and thorough tissue sampling is important to reach the correct diagnosis. The morphologic features, immunohistochemistry, molecular alterations, and prognosis will be discussed.

Hematuria in a Former Smoker

A 75-year-old woman presented with painless, massive hematuria. She was a former smoker but had no history of a bleeding disorder. Laboratory studies revealed a hemoglobin level of 6.7 g/dL [reference interval (RI) 11.2–15.7], prothrombin time (PT)2 of 47.9 s (RI 12.5–15.5), activated partial thromboplastin time (aPTT) of >160 s (RI 25–35), and a thrombin time of …

Fatal hemorrhage due to a spontaneous factor V inhibitor with lupus anticoagulant properties.

: Factor V inhibitors are rare and have varied clinical presentations. We report on a 76-year-old female admitted to the hospital for pneumonia and treated with multiple antibiotics. Her baseline prothrombin time was 15.6 s and the activated partial thromboplastin time was 35 s. On admission day 10, she developed arm weakness and brain imaging showed a subdural hematoma. The prothrombin time was now 59.1 s with an activated partial thromboplastin time of more than 160 s and a normal thrombin time. A mixing study did not correct the clotting times and coagulation factor assays showed a nonspecific inhibition pattern. Only factor V activity remained low with serial dilutions, however, and a 70 Bethesda Unit inhibitor was identified. Aggressive supportive care was initiated but the patient succumbed to the effects of the intracranial hemorrhage. Factor V inhibitors may display lupus anticoagulant properties and may cause catastrophic bleeding. Our case illustrates that these inhibitors can arise quickly and supports an association with antibiotics.

Scleromatous Changes in an Abdominal Wall Graft: Graft-Versus-Graft Disease, or Chronic Graft Rejection?

Abstract: Abdominal wall transplants are relatively new procedures that are frequently performed in conjunction with multivisceral transplants. The skin of the abdominal wall transplant is often the first site for graft rejection to manifest itself. Prompt recognition can lead to appropriate treatment before the involvement of the underlying viscera. However, the signs of graft rejection are nonspecific and can overlap with other entities. We present a case of a patient who received a multivisceral and abdominal wall transplant from 2 different donors, who presented with acute and eventually chronic graft rejection of the abdominal wall graft. Serial biopsies performed during the course of her treatment demonstrated progressive sclerotic changes in the dermis. Because these changes were confined to the abdominal wall graft, they could represent either chronic graft rejection or graft-versus-graft disease. To date, graft-versus-graft disease has not been documented in these patients. This case illustrates the possibility that patients with multidonor transplants may be at an increased risk for graft failure secondary to multiple potential etiologies.