Transfusion management and hemoglobin-based oxygen carrier treatment in a patient with anti-Rh17 antibody.

BACKGROUND: A 54-year-old Hispanic OPos female with known history of anti-Rh17 antibodies was diagnosed with Philadelphia-Chromosome positive (Ph+) acute lymphoblastic leukemia (ALL). Rh17, also known as Hr0, is a high-frequency antigen composed of several epitopes on the RhCE protein. Anti-Rh17 antibodies can be made by individuals with missing or varied C/c, E/e antigens. Anti-Rh17 antibodies are clinically significant given multiple case reports of hemolytic disease of the fetus and newborn (HDFN). Finding compatible units for patients with anti-Rh17 can be particularly difficult given that only 1 in 100,000 people are Rh17 negative. STUDY DESIGN AND METHODS: Search for compatible units was conducted by the American Rare Donor Program (ARDP) with no leads. After chemotherapy induction and despite erythropoiesis stimulating agent administration, the patient's hemoglobin continued to trend down to a nadir of 2.8 g/dL. Here we report transfusion of incompatible pRBC to this patient with critically symptomatic anemia. HBOC-201 (Hemopure) was obtained and administered under an emergency compassionate/expanded access designation from the Food and Drug Administration (FDA) under an emergency Investigational New Drug (IND) application. RESULTS AND DISCUSSION: Overall difficulties in this case included the challenge of finding compatible units, dilemma of transfusing incompatible units in a patient with severe anemia and obtaining alternatives to blood products. This case report demonstrates the successful use of HBOC-21 in treating life-threatening anemia.

Risk factors for massive transfusion in obstetrical hemorrhage and consideration of a whole blood program.

BACKGROUND: Postpartum hemorrhage (PPH) is one of the leading causes of obstetric complications. The goal of this study was to identify risk factors for obstetric (OB) massive transfusion (MT) and determine the feasibility of developing a low-titer group O RhD-positive whole blood (LTO + WB) protocol for OB hemorrhage. STUDY DESIGN AND METHODS: A retrospective study of OB patients who received transfusion within 24 h. MT patients were those who received >3 U of pRBC within 1 h or > 10 U in 24 h. Patient demographics, OB history, comorbidities, blood type, antibody status, and known risk factors for PPH and maternal-fetal outcomes were compared. Logistic regression was used for univariate and multivariate analyses. RESULTS: Of the 610 transfused OB patients, 12.0% (n = 73) required MT. Groups were well matched for body mass index (BMI), maternal comorbidities, and history of spontaneous vaginal deliveries. The incidence of the previous cesarean section was higher in the MT group. Exactly 93.9% of patients were RhD-positive and 3.77% of all patients possessed an antibody on pretransfusion testing. Patients with MT had a longer length of stay (LOS), higher rate of intensive care unit (ICU) admission, fetal death, and hysterectomy. Multivariate analysis found age >35, PPH, placenta percreta, accreta, and increta to be significant (p < .05) risk factors for MT. DISCUSSION: Patients over 35 years and those with abnormal placentation are at increased risk of requiring MT. With a time to delivery of 2 days, potential MT patients can be identified early, and with a 94% rate of RhD-positive+, they are eligible to receive low-titer O whole blood (LTOWB) providing hemostatic resuscitation with reduced donor exposure.

Efficacy and Safety of Whole Blood Transfusion in Non-Trauma Patients.

Whole blood (WB) transfusion for trauma patients with severe hemorrhage has demonstrated early successful outcomes compared to conventional component therapy. The objective of this study was to demonstrate WB transfusion in the non-trauma patient. Consecutive adult patients receiving WB transfusion at a single academic institution were reviewed from February 2018 to January 2020. Outcomes measured were mortality and transfusion-related reactions. A total of 237 patients who received WB were identified with 55 (23.2%) non-trauma patients. Eight patients (14.5%) received pre-hospital WB. The most common etiology of non-traumatic hemorrhage was gastrointestinal bleeding (43.6%, n = 24/55). Approximately half of the non-trauma patients (n = 28/55) received component therapy. Transfusion-related events occurred in 3 patients. This study demonstrated that non-trauma patients could receive WB transfusions safely with infrequent transfusion-related events. Future studies should focus on determining if outcomes are improved in non-trauma patients who receive WB transfusions and defining specific transfusion criteria for this population.

Triple whammy: a rare case of epithelioid hemangioendothelioma with synchronous angiomyomatous hamartoma complicated by Actinomyces meyeri.

Epithelioid hemangioendothelioma is a rare vascular malignancy that originates from vascular endothelial or pre-endothelial cells and is composed of epithelioid or histiocytoid cells. This malignancy has an incidence of approximately one per one million individuals and can occur in various regions of the body including the lungs, liver, bones, and soft tissues. The behavior of this cancer can range from indolent to aggressive and diagnosis and treatment are often delayed due to variable presentations and lack of established treatment guidelines. Here we present the case of a 27-year-old Hispanic male that presented with right groin pain, abdominal pain, and a fifty-pound weight loss over one year. The patient had a complex hospital course during which he was found to have an angiomyomatous hamartoma of his right groin area, postsurgical right inguinal wound infection with Actinomyces meyeri, and epithelioid hemangioendothelioma distal to the right iliac bifurcation. The patient is currently pending further imaging studies to evaluate candidacy for surgical resection and following with oncology for chemotherapeutic options.

A Surprising Cause of Liver Abscesses in a Post-Chemotherapy Patient: Herpes Simplex Virus.

Herpes simplex virus (HSV) hepatitis is a rare complication of HSV infection, and a rare cause of hepatitis. It is often fatal, especially if the diagnosis and treatment are delayed. Herein, we describe the case of a 31-year-old female with a history of receiving cytotoxic cancer chemotherapy five months prior who presented with a one-week history of worsening abdominal pain and fever. She was noted to have an outbreak of genital herpes at the time of presentation. Computed tomography (CT) scan of the abdomen showed innumerable hypodensities compatible with hepatic micro-abscesses. A specimen from a subsequent liver biopsy revealed HSV-type cytopathic changes and nuclear staining with an anti-HSV immunohistochemical stain. She was initially started on high-dose oral valacyclovir for genital herpes and was noted to have rapid clinical improvement prior to the histopathologic diagnosis of HSV hepatitis. She achieved full recovery while on oral valacyclovir. This is the first reported case of HSV hepatitis treated with oral valacyclovir and the third reported case of HSV hepatitis mimicking pyogenic abscesses on abdominal imaging. With the high mortality rate associated with HSV hepatitis, one should consider the diagnosis in all patients with multifocal liver lesions of unknown etiology, especially if genital herpes is present at the time of presentation, or in patients who are immunocompromised.

Pandora's Box: Disseminated Coccidioidomycosis Associated with Self-Medication with an Unregulated Potent Corticosteroid Acquired in Mexico.

Coccidioidomycosis (CM), caused by the dimorphic fungi Coccidioides immitis and C. posadasii, typically presents as acute or chronic pulmonary disease. However, disseminated disease occurs in about 1% of patients. Disseminated CM may affect multiple organ systems, including cutaneous, osteoarticular, and central nervous system sites. Here, we present a case of disseminated CM in a patient from a border city in Texas. The patient had a history of uncontrolled diabetes mellitus and was also taking an over-the-counter medication acquired in Mexico that contained a potent corticosteroid. The patient presented with seizures and was found to have a brain infarct, cavitary lung lesions, synovitis of the knee, multiple skin lesions, and chorioretinitis. The patient had a very high complement fixation titer for Coccidioides; fungal spherules were seen in a skin biopsy specimen, and Coccidioides grew in culture from a sample of synovial fluid and the skin biopsy specimen. This case illustrates the dissemination potential of Coccidioides, the danger of unregulated pharmaceuticals, the importance of thorough history taking, and recognizing risk factors that contribute to disseminated CM.

Accuracy of Intraoperative Frozen Section in Detection of Acute Invasive Fungal Rhinosinusitis.

CONTEXT.­: Acute invasive fungal rhinosinusitis (AIFRS) is an aggressive form of fungal sinusitis, which remains a significant cause of morbidity and mortality. Early diagnosis and intervention are keys to improving patient outcomes. Intraoperative consultation has shown promise in facilitating early surgical intervention, but the accuracy of frozen section has not been clarified in this setting. OBJECTIVES.­: To assess the accuracy of frozen-section diagnosis in patients with clinically suspected AIFRS. DESIGN.­: All cases of clinically suspected AIFRS during a 10-year period (2009-2019) were retrospectively reviewed. The frozen-section results were compared with the final permanent sections as well as the tissue fungal culture results, following which the accuracy of frozen section was determined. RESULTS.­: Forty-eight patients with 133 frozen-section evaluations for AIFRS were included in the study. Thirty of 48 patients and 61 of 133 specimens were positive for AIFRS on final pathology. Of 30 positive patients, 27 (90%) had at least 1 specimen diagnosed as positive during intraoperative consultation; among the 61 positive specimens, 54 (88.5%) were diagnosed as positive during intraoperative consultation. Of 72 negative specimens, all were interpreted as negative on frozen section. Thus, frozen sections had a sensitivity of 88.5% (95% CI, 0.78-0.97), specificity of 100% (95% CI, 0.94-1), positive predictive value of 100% (95% CI, 0.92-1), and negative predictive value of 90.6% (95% CI, 0.82-0.97). CONCLUSIONS.­: This study represents the largest series assessing the diagnostic accuracy of frozen section analysis in AIFRS. These findings are useful in frozen section-informed intraoperative decision making.

A rare case of NXP-2 positive dermatomyositis.

Dermatomyositis is an idiopathic inflammatory myopathy with variable cutaneous manifestations. Several autoantibodies each with distinct clinical phenotypes are associated with the disease. Here we present the case of a 36-year-old Laotian woman with hypothyroidism who presented with severe proximal and distal muscle weakness, dysphagia, diffuse rash, and anasarca that was diagnosed with NXP-2 (nuclear matrix protein 2) antibody positive dermatomyositis. The patient's hospitalization was complicated by disease resistant to conventional therapy.

Case Report: Fulminant Murine Typhus Presenting with Status Epilepticus and Multi-Organ Failure: an Autopsy Case and a Review of the Neurologic Presentations of Murine Typhus.

Murine typhus (MT) is an important cause of febrile illness in endemic areas, and there is an epidemiologic resurgence of this infection currently transpiring in Texas and California. Fatal cases and severe neurological complications are rare. A fatal case of MT in a middle-aged man is reported with a course culminating in multi-organ failure and refractory status epilepticus. An autopsy revealed hemorrhagic pneumonia, acute tubular necrosis, and ischemic necrosis in the liver, adrenals, and brain. We have also reviewed the neurologic complications of MT.