Impact of Patient Blood Management on Red Blood Cell Utilization in an Urban Community Teaching Hospital: A Seven-Year Retrospective Study.

OBJECTIVES: This study aimed to showcase how implementing a patient blood management (PBM) program effectively cuts unnecessary red blood cell (RBC) transfusions in a New York City urban community teaching hospital. METHODS: Analyzing seven years from 2013 to 2019, a retrospective review of RBC transfusions was conducted. RESULTS: Following the introduction of PBM, considerable improvements were observed annually. These included a drop in mean pretransfusion hemoglobin levels from 7.26 g/dL (2013) to 6.58 g/dL (2019), a 34% reduction in yearly RBC unit transfusions, and fewer units given to patients with pre-Hgb levels ≥ 7 g/dL (from 1210 units in 2013 to 310 units in 2019). Furthermore, this study noted a decline in two-unit RBC orders when Hgb levels were ≥ 7 g/dL from 65 orders in 2013 to merely 3 in 2019. The estimated total cost savings attributed to the six-year PBM program duration after full implementation in 2014 amounted to USD 2.1 million. CONCLUSIONS: Overall, PBM implementation significantly decreased RBC transfusions and enhanced transfusion practices. The findings emphasize that successful PBM strategies do not always necessitate extensive resources or increased budgets but instead rely on the application of intuitive methods, as evidenced by this study.

Enlarging perineal endometrioma developing after colpoperineorrhaphy.

Although endometriosis is not infrequent, the occurrence of perineal endometriomata is relatively rare and is generally attributed to seeding of endometrial cells to the perineal body during obstetrical trauma. A 45-year-old female with a history of a colpoperineorrhaphy during menses and a remote history of obstetric perineal trauma presented with an enlarging perineal mass. Excision of the mass led to a clinical and histopathologic diagnosis of perineal endometriosis. Elective surgery disrupting the vaginoperineal epithelium performed during menses may facilitate the seeding of endometrial cells to the perineal body and formation of perineal endometriomata.

Atypical Sjögren's Syndrome Initially Presenting as Lymphocytic Interstitial Pneumonitis followed by Immune Thrombocytopenia.

BACKGROUND: Sjögren's syndrome is an autoimmune disease characterized primarily by decreased exocrine gland function leading to eye and mouth dryness. Extraglandular manifestations occur less frequently. Case Report. A 74-year-old man with hypertension was admitted with productive cough and fever. On physical examination, he had bilateral lower lung decreased breath sounds. A chest radiograph showed bibasilar patchy infiltrate. Laboratory studies revealed hemoglobin of 11.9 g/dL, white blood cell count of 16,000/uL, and platelet count of 250,000/uL. Empiric antibiotic therapy was begun for suspected community acquired pneumonia, and then he was discharged home. However, his cough recurred. Chest computed tomography demonstrated adenopathy throughout the mediastinum and multiple ill-defined patchy groundglass opacities with a lower lobe prominence. He underwent a transbronchial biopsy to rule out malignancy; however, it showed lymphocytic interstitial pneumonitis. Antinuclear antibody was 1 : 80 homogeneous, and anti-SSA antibody was 6.3 AI (normal <1.0 AI). The patient was treated with prednisone 20 mg/day with marked improvement in his symptoms. Repeat chest computed tomography showed decreased groundglass opacities and decreased mediastinal lymph nodes. After more than a year, he was readmitted due to petechiae on his buccal mucosa and a platelet count of 2000/µL. The patient was started on prednisone 80 mg/d and intravenous immunoglobulin 80 g/d for 2 consecutive days. The platelet count eventually increased to 244,000/µL. CONCLUSION: We report a rare presentation of Sjogren's syndrome manifesting as acute lymphocytic interstitial pneumonitis and followed by immune thrombocytopenia. Both extraglandular manifestations responded well to corticosteroid therapy.

Acute Hepatitis in a Yemeni Immigrant Associated with Khat: A "Biological Amphetamine" Carried in Cultures.

Viral infections, alcohol, hepatic steatosis, autoimmunity medications and herbal supplements are common etiologies of hepatitis. Khat (Catha Edulis) is a commonly used recreational substance in East African and Middle Eastern countries. Khat has been reported in the literature to be associated with hepatotoxicity, which can present in several forms, including chronic liver disease. The possible pathogenesis of liver injury could be secondary to biochemical components of Khat itself or additives such as pesticides or preservatives. An autoimmune mechanism of liver injury has also been postulated, supported by sparse evidence. We present a case of a Yemeni immigrant with acute hepatitis whose fear about social norms and breaching confidentiality made it challenging to identify Khat as being the underlying cause. A 34-year-old man from Yemen presented with right upper quadrant pain of one day duration. He had predominantly elevated transaminases with mild elevation in bilirubin. His investigations were negative for the viral, metabolic or biliary etiology. A persistent focus on clinical history and the well-established physician-patient relationship revealed a history of Khat use. The liver biopsy finding of lobular hepatitis was compatible with drug-induced liver injury and established the finding of Khat hepatotoxicity. Subsequently, the patient improved with conservative management.

Colonic Ganglioneuroma: A Rare Lesion With Extremely Different Presentations and Outcomes in Two Patients.

Ganglioneuroma (GN) of the gastrointestinal tract is an extremely rare neuroectodermal tumor. Patients with ganglioneuromas have different presentations depending on the location, extent and size of the lesion. We present two cases of ganglioneuromas that had different clinical presentations and outcomes.

A Rare Case of Cerebellar Glioblastoma Mimicking Acute Stroke in an Elderly Patient.

BACKGROUND Glioblastoma (GB) is a common brain tumor that usually presents in the cerebral hemisphere. Very rarely, these tumors can present in the cerebellum. The tumor tends to have a diffuse infiltrative growth that follows the white-matter pathway. Cerebellar GB is often difficult to diagnose on imaging and a biopsy is often needed for diagnosis. Here, we present the case of an elderly woman who presented with symptoms suggestive of acute stroke. CASE REPORT An 82-year-old woman presented for intermittent dizziness that started 2 weeks prior to the presentation and had been progressively worsening. She had a prior history of stroke and was noted to have decreased motor strength and sensation to touch on the left side. A cranial nerve examination was normal, as was finger-nose testing. Magnetic resonance imaging (MRI) of the brain with and without contrast showed an enhancing lesion in the left posterior cerebellum producing a mass effect in the left lateral ventricle. The differential diagnosis included cerebellitis with abscess, neoplastic process with necrosis, and, less likely, a sub-acute infarction A suboccipital craniotomy with cerebellar biopsy-diagnosed cerebellar GB. CONCLUSIONS We report the unique presentation of cerebellar GB in an elderly woman who presented with left-sided weakness, elevated blood pressure, dizziness, vasogenic edema in the left cerebellum, and a mass effect on the fourth ventricle, mimicking acute stroke.

Lung mass with bilateral mediastinal hypermetabolic lymphadenopathy in a chronic smoker indicative of lung cancer?

We present the case of an asymptomatic woman, a chronic heavy smoker, who presented with an incidental lung mass and mediastinal lymphadenopathy. Bronchoscopy with transbronchial biopsy and endobronchial ultrasound-guided transbronchial needle aspiration did not show malignancy. A positron emission tomography/computed tomography scan showed increased uptake with a standardized uptake value of 26.4 in the mediastinal lymph node and an additional hypermetabolic right supraclavicular lymph node. Surgical biopsy of the supraclavicular node revealed non-necrotizing granuloma. Discussion of the clinical dilemma is provided.

Malignant Epithelioid Neoplasm of the Brain.

Malignant epithelioid tumors have been described in various organ systems, but are rarely seen in the brain. They are aggressive tumors and have high mortality. In certain cases, the immunohistochemistry (IHC) findings may not be sufficient to clarify the diagnosis. In these cases, next-generation genetic sequencing may play a role in clarifying the diagnosis. In addition to lab testing, a thorough history and physical exam are necessary to rule out other sources of the tumor such as melanoma. Patients presenting with neurological symptoms are cared for by a wide variety of physicians, hence it is important to raise awareness of rare tumors in order to provide timely and appropriate management and referral for these patients. We present the case of a middle-aged woman who was diagnosed with a 'malignant epithelioid neoplasm' of the brain, a rare variety of tumors. We also give the clinical course of this illness.

A Rare Case of Splenic Pneumocystis jirovecii in a HIV-Positive Patient.

INTRODUCTION: Human immunodeficiency virus (HIV) positive individuals with the CD4 count less than 200 cells/mm3 are at risk for opportunistic infections. Pneumocystis jirovecii, a fungal pathogen, is a common cause of opportunistic infections with predominantly pulmonary involvement. Disseminated P. jiroveciii infection presenting with hepatosplenic lesion is extremely rare. Case Summary. A 31-year-old male with HIV with and acquired immunodeficiency syndrome (AIDS) presented with diarrhea for 3 weeks. He had splenomegaly and inguinal lymphadenopathy on physical examination. Laboratory parameters revealed anemia and hypoalbuminemia, while stool studies for infectious etiology and fecal leucocyte were negative. Computed tomography (CT) of the chest and abdomen depicted consolidation of the lungs and a large splenic mass. He underwent fiberoptic bronchoscopy with transbronchial biopsy which was consistent with P. jirovecii pneumonia. He also had a ultrasound-guided core biopsy of the splenic mass which revealed necrotizing granulomas with Pneumocystis jirovecii infection on Grocott-Gomori's methenamine silver (GMS) stain and was initiated on treatment for P. jirovecii with sulfamethoxazole with trimethoprim. CONCLUSION: Malignancy and atypical infection are key differentials in patients presenting with hepatosplenic lesions. HIV positive patients are at increased risk of AIDS-related lymphoma. Tissue diagnosis is often required for further evaluation. Disseminated P. jirovecii presenting with splenic mass and liver lesion is extremely rare.

A Rare Case of Disseminated Mycobacterium avium-intracellulare Presenting as Proctitis.

Mycobacterium avium intracellulare (MAI) infections are common in Human Immuno-deficiency Virus (HIV) positive patients. MAI infection can have localized or disseminated presentation, patients with low CD4 count presenting with disseminated infection. Fever, abdominal pain, diarrhea, and weight loss are generally the presenting symptoms of disseminated MAI. We present a rare case of a patient with HIV and low CD4 count presenting with proctitis as manifestation of disseminated MAI infection. A 25 year-old-man with HIV came to the emergency room (ER) with complaints of intermittent rectal bleeding for two months. His CD4 count was less than 20 cells/µL. He was MSM (men having sex with men) and has receptive anal intercourse with men. His stool work-up was unrevealing for infectious etiology. Swabs for gonorrhea and chlamydia were negative. Colonoscopy revealed erythematous, congested, friable rectal mucosa with two superficial ulcers. Biopsies of the ulcer were positive for acid fast staining bacteria and the culture grew MAI. His blood culture was negative for growth of acid-fast bacteria (AFB). However, liver biopsy performed for elevated alkaline phosphatase of 958 units/L revealed noncaseating granuloma. Gastro-duodenoscopy with duodenal biopsy did not reveal any mucosal abnormality. He was managed as with disseminated MAI infection using clarithromycin, ethambutol, and rifabutin in addition to HAART therapy. Interval Colonoscopy in 20 months showed resolution of rectal ulcer. The gut is often involved in patients with disseminated MAI infection, with the duodenum being the most common site. MAI infection should be suspected as possible etiology for proctitis in HIV positive patient with low CD4 count, as proctitis, though infrequent can be the sole presentation for disseminated MAI infection in patients with HIV and low CD4 count.

Cytophagic Histiocytic Panniculitis (CHP) in a Patient with SLE Found after Autopsy: When a Rash Is "Complicated!"

INTRODUCTION: Cytophagic histolytic panniculitis (CHP) is a clinical disorder characterized by nodular panniculitis of the subcutaneous adipose tissue. It was first described in 1980 by Winkelmann. Histologically it is described as an infiltration of the adipose tissue by T- lymphocytes and phagocytic macrophages (also known as "bean bag cells"). Most of the cases are reported under the age of 50 and is a rare cause of panniculitis. We report a case of CHP in a young patient who presented to our emergency room (ER). CASE SUMMARY: A 39-year-old African American woman who presented to our hospital with lethargy, progressive confusion, and generalized rash involving both lower extremities of 1 week duration. She had a history of pancytopenia and focal proliferative and membranous lupus nephritis classes 3 and 5. Her physical examination was remarkable for bilateral lower extremity pitting edema and a desquamating rash on both of her legs. The Nicolsky sign was positive. She was noted to be hypotensive and was started on intravenous fluids and broad spectrum antibiotics. Routine laboratory tests revealed severe pancytopenia, with a hemoglobin of 3.9 g/dl, white blood cell count 600/ul, and platelet count of 11000/ul. Within an hour of arrival to the ER she developed acute respiratory failure. She was intubated and placed on mechanical ventilation. She developed shock requiring vasopressors. No imaging could be done due to her unstable condition. Four hours after her initial presentation she developed asystole and expired. Postmortem histopathology of the adipose tissue revealed CHP. CONCLUSION: CHP can be rapidly fatal. The treatment involves high dose of intravenous steroids and immunosuppressants such as cyclosporine.

A Rare Case of DRESS (Drug Reaction with Eosinophilia and Systemic Symptoms) Syndrome with Cholecystitis in a Patient on Levetiracetam.

Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a rare idiosyncratic drug reaction with a mortality of up to 10%. As the name suggests, it is characterized by skin rash, eosinophilia, and systemic symptoms resulting from the involvement of visceral organs. We present a case of DRESS in a patient who was on both lamotrigine and levetiracetam, where levetiracetam turned out to be the inciting agent. The interesting features of the case include the onset of symptoms about 70 days after the initiation of levetiracetam, the lack of prominent eosinophilia and the involvement of the gall bladder, which was previously unknown with Levetiracetam. It also reinforces the importance of using the RegiSCAR score in the diagnosis of DRESS. The symptoms resolved over the next few months after drug withdrawal.

Atypical Radiographic Presentation of Cryptococcus Pneumonia in a Newly Diagnosed HIV Patient.

BACKGROUND: Cryptococcus infection is an opportunistic infection that occurs primarily among immunocompromised patients, and the morbidity and mortality of this infection is high if left unrecognized and untreated. There are no clinical or radiographic characteristics typical of cryptococcal pneumonia, and its clinical and radiological presentations often overlap with other diagnoses. CASE PRESENTATION: We present a case of a 25-year-old man from Ghana admitted for an altered mental state, weight loss, neck pain, fever, and photophobia. He was diagnosed with Cryptococcus neoformans meningitis by cerebrospinal fluid culture and with disseminated cryptococcal infection by a positive Cryptococcus blood test. Diffuse micronodular opacities were found in a miliary pattern in the upper portions of both lungs upon imaging, which suggested miliary tuberculosis; thus, the patient was started on antituberculosis therapy. The patient underwent flexible fiber optic bronchoscopy, and transbronchial biopsy of the right lung showed bronchopneumonia with fungal spores consistent with filamentous Cryptococcus neoformans, which grew in tissue culture of the right lung. Interferon-gamma release assay, Mycobacterium tuberculosis PCR, and acid-fast bacilli staining of the bronchoalveolar lavage were negative for the M. tuberculosis complex. CONCLUSION: The similarities in clinical and imaging findings among patients with acute immunodeficiency syndrome with coinfections make diagnoses difficult; thus image-guided biopsies are essential to confirm diagnoses.

Malignant Peritoneal Mesothelioma Without Asbestos Exposure.

Malignant mesothelioma is a rare neoplasm of the serosal linings. Mesothelioma has been linked to asbestos exposure, with prior asbestos exposure linked to 33-50% of malignant peritoneal mesotheliomas. We describe a case of malignant peritoneal mesothelioma (MPM) without any prior exposure to asbestos in a 40-year-old Hispanic female who presented to the emergency department with worsening abdominal pain and distension. She had a history of beta thalassemia trait and iron deficiency anemia. Examination revealed a distended abdomen with protruding umbilicus and positive shifting dullness. Laboratory tests showed anemia. Computed tomography (CT) of the abdomen revealed massive complex ascites suspicious of a malignant process. Ascitic fluid analysis showed serum ascites albumin gradient (SAAG) of 1.1 g/dL with a total protein of 5.2 g/dL. She underwent laparoscopic peritoneal biopsy which yielded epithelioid type malignant mesothelioma. She was started on chemotherapy with cisplatin and pemetrexed. The last follow-up was 27 months after the diagnosis. MPM is a rare and life-threatening malignancy. Frequently, the symptoms are non-specific. This poses a diagnostic challenge for physicians and probably the reason why the diagnosis is often delayed, especially in the absence of risk factors.

Small-Cell Type, Poorly Differentiated Neuroendocrine Carcinoma of the Gallbladder: A Case Report and Review of the Literature.

The poorly differentiated small-cell type, neuroendocrine carcinoma (NEC) of the gallbladder is a very uncommon subtype of a neuroendocrine tumor of the gastrointestinal tract. Nonsecretory NEC by virtue of its nonspecific and subtle clinical presentation of the tumor is usually diagnosed at an advance stage with presenting symptoms related to either locally advance disease or from metastatic disease. Though the radiologic imaging does identify the gall bladder cancer, the tumor lacks a specific diagnostic test; therefore, the diagnosis is almost always confirmed on histopathologic and immunohistochemical staining. We present a case of a poorly differentiated, small-cell neuroendocrine tumor of the gallbladder. The patient died within 3 months after the definitive diagnosis was made. Survival from this deadly malignancy can be improved with aggressive surgical treatment followed by chemotherapy and radiotherapy on a case-by-case scenario. The systemic chemotherapy remained the treatment of choice for an unresectable tumor (Chen et al., 2014).

Case of Gastric Diffuse Large B-Cell Lymphoma.

The gastrointestinal (GI) tract is the predominant site of extra nodal lymphoma involvement. In the United States (US), gastric lymphoma is the most common extra nodal site of lymphoma. Most of these lesions are either extra nodal marginal zone B cell lymphoma of mucosa associated lymphoid tissue (MALT) type or diffuse large B cell lymphoma (DLBCL). We report a case of diffuse large B-Cell Gastric Lymphoma who initially presented with sore throat, dysphagia and hiccups for a few months. Esophagogastroduodenoscopy showed lower esophageal stenosis and a large, infiltrative, ulcerated, circumferential mass at the gastro esophageal junction and cardia. Histopathology showed diffuse large B cell lymphoma. Positron emission tomography scan showed advanced disease with presence of lymph nodes on both sides of the diaphragm. The patient was considered to have Stage IV gastric lymphoma. Subsequently, he was treated with R-CHOP regimen (rituximab, cyclophosphamide, hydroxydaunorubicin, oncovin (vincristine), and prednisone).

Co-Occurrence of Rarest Type of Dysphagia Lusoria (Type N-1) and Eosinophilic Esophagitis in a Cognitively Disabled Individual.

Dysphagia is an expressive symptom, described by an individual as "difficulty in swallowing." Dysphagia due to esophageal compression from an aberrant right subclavian artery is rare, and it is termed as "dysphagia lusoria." We present a rare case of co-occurrence of dysphagia lusoria with esophageal eosinophilia in a patient with cognitive disability which portends a case with diagnostic challenge and treatment dilemma. A 31-year-old man with intellectual disability, cerebral palsy, previous history of feeding difficulty, and esophageal food impaction presented with esophageal foreign body impaction. He has no known history of atopy and food allergies. There was no laboratory evidence of peripheral eosinophilia. The IgE-mediated allergic test was unremarkable. His prior presentation revealed a diagnosis of eosinophilic esophagitis. The imaging studies showed proximal esophageal dilatation with extrinsic compression at the level of the upper esophagus. The foreign bodies were removed successfully through the help of upper endoscopy. Subsequent evaluation revealed a rare type of dysphagia lusoria (type N-1) due to an aberrant left subclavian artery arising from the right-sided aortic arch. The patient's family refused further management of artery lusoria. Prolonged stasis of secretions and food in the esophagus can also lead to increased esophageal eosinophils. In our case, it remains undetermined whether increased number of esophageal eosinophils resulted from primary eosinophilic esophagitis or due to prolonged food stasis from esophageal compression caused by an aberrant subclavian artery. However, food impaction right above the compression site makes dysphagia lusoria the likely etiology.

A Rare Etiology of Abnormally Large Gastric Folds: Menetrier's Disease.

Menetrier's disease (MD) is described as hypertrophied giant gastric folds causing hypoproteinemia due to protein losing gastropathy and is associated with higher risk of gastric adenocarcinoma. We present a case of a 58-year-old male who presented to our clinic with Melena and endoscopic work up showed enlarged gastric folds and erythematous mucosa in the antrum and three nonbleeding angioectasias in the duodenum. Mucosa biopsies were negative for H. pylori infection. He underwent polypectomy which showed fundic gland polyps. After 1½ years, EGD was repeated for abnormal computerized tomography of abdomen which showed enlarged gastric folds and biopsy revealed gastric fundic mucosa with foveolar hyperplasia, dilated fundic glands, and chronic gastritis. Stomach biopsy results were consistent with MD. Our patient had progressive disease in one and half years. It is important to follow patient with large gastric folds regular as they can develop MD over time which has increased risk of gastric adenocarcinoma.