Chronic Atrophic Gastritis Presenting as Hemolytic Anemia due to Severe Vitamin B12 Deficiency.

Vitamin B12 is an essential nutrient which plays an important role in neurological function, hematopoiesis, and DNA synthesis. Low levels usually stem from either poor intake or a malabsorptive process. Presently, the most common cause of vitamin B12 deficiency is food-bound cobalamin malabsorption, which occurs when there is impaired release of vitamin B12 from ingested food due to an outstanding factor preventing the release of the nutrient from its transport protein. Such causes include achlorhydria, gastritis, gastrectomy, or the use of PPIs or antacids. A rarer cause is autoimmune chronic atrophic gastritis, resulting in pernicious anemia. In this disease process, there is destruction of parietal cells and thus a reduction in intrinsic factor, which is essential to the absorption of vitamin B12. Deficiency will result in a variety of abnormalities including but not limited to pancytopenia, paresthesias, and neuropsychiatric symptoms. A rare manifestation of vitamin B12 deficiency is hemolytic anemia, which occurs due to intramedullary and extramedullary dysfunction. This case describes a 46-year-old male with no past medical history who presented with chest pain, fatigue, and progressive weakness, found to have hemolytic anemia, ultimately attributed to vitamin B12 deficiency. Antiparietal cell antibodies and intrinsic factor antibodies (IFA) were both negative. Still, the patient underwent an endoscopy with biopsies of the stomach; pathology was consistent with chronic metaplastic atrophic gastritis. The patient improved with intramuscular vitamin B12 supplementation. This case highlights both a rare cause and presentation of vitamin B12 deficiency. Patients with autoimmune chronic atrophic gastritis should have antiparietal cell or intrinsic factor antibodies. Still, seronegative patients have been reported, like this patient. Additionally, hemolytic anemia secondary to vitamin B12 deficiency is uncommon. The presentation will usually mirror that of a thrombotic microangiopathy (TMA), including hemolytic anemia with schistocytes on peripheral blood smear and thrombocytopenia, as it did in this patient. This clinical entity is described as pseudothrombotic microangiopathy and is crucial to identify in order to prevent the initiation of invasive treatment strategies such as plasmapheresis.

Trends of Ordering Hypercoagulability Work-Up at an Academic Medical Center.

BACKGROUND: Venous thromboembolism is a significant clinical event, with an annual incidence of 1-2 per 1000 population. Risk factors include recent surgery, prolonged immobility, oral contraceptive use, and active cancer. Inherited risks include protein C and S deficiencies, antithrombin deficiency, factor V Leiden mutation and prothrombin. These factors can be tested to guide therapy, but current evidence suggests that testing for inherited thrombophilia is not recommended in most inpatient settings. In the era of high value care, hypercoagulable testing for VTE creates a financial burden for the hospital and patients. We performed a retrospective chart review of hypercoagulable orders on VTE patients at our institution. METHODS: Institutional Review Board approval was obtained. A total of 287 adult patients admitted over a 3-month period with the diagnosis of VTE were included. Patients were identified via ICD-10 codes and data were collected from electronic medical records. Patient characteristics, provoked versus unprovoked VTE, and relative contraindications for hypercoagulability work-up were analyzed. Our primary outcome was to assess the appropriateness of thrombophilia testing in VTE patients based on screening guidelines. Our secondary outcome was to analyze the cost burden of ordering these tests. RESULTS: A total of 287 patients were included in our data analysis. Patient risk factors for VTE were malignancy, previous DVT, immobilization, surgery 3 months prior, and central line placement. Fifty-seven of 287 patients had at least one hypercoagulable test ordered during hospitalization which did not adhere to guidelines. Misuse of testing occurred during active thrombosis, active anticoagulation, presence of risk factors, first episode of VTE, and malignancy. The cost of ordering these 5 thrombophilia tests totaled over $40,000. CONCLUSION: In our study, numerous patients were tested without compliance to standard recommendations, which created financial and value-based burdens on our health care system. Increased awareness among clinicians is thus warranted to ensure high value care.

Diagnostic Enigma: Spindle Cell Sarcoma of the Aorta Presenting as Pulmonary Embolism and Chronic Anaemia.

Primary aortic sarcoma is a rare and aggressive malignancy with only approximately 190 cases reported in the literature. While angiosarcoma and intimal sarcomas represent an estimated 67.7% of malignant aortic tumours, spindle cell sarcomas are even more exclusive, consisting of only 0.9% of malignant aortic tumours. Differentiated from other malignant aortic tumours, spindle cell sarcomas are of mesenchymal origin and usually express vimentin and osteopontin. Clinical presentations are variable and nonspecific, ranging from back pain, abdominal pain or elevated blood pressure, misleading to differentials like pulmonary emboli or aortic aneurysms such as in our case here. In this article, we discuss the finding of an extremely rare aortic sarcoma masquerading as a pulmonary embolism. The patient underwent surgical resection; however, the course was complicated by the development of brain metastases and intracranial haemorrhage. The literature is expanding regarding the evolution of adjuvant chemotherapy and radiation therapy in the treatment of these patients. The exact pathogenesis of spindle cell sarcomas is unknown but thought to be related to the MDM2-p53 pathway. The development of spindle cell sarcomas may be related to Li-Fraumeni syndrome, which should be on the differential for these patients. This case highlights the importance of identifying aortic sarcomas in patients who present with signs and symptoms of peripheral embolization as the diagnosis can be easily misconstrued for thrombus or aortic aneurysm, leading to a delay in proper and timely management. We herein emphasize that aortic sarcomas should be included in the clinician's working differential due to the poor prognosis and outcomes that these aggressive tumours carry. LEARNING POINTS: Malignant aortic tumours are rare and can present with a multitude of symptoms ranging from constitutional symptoms to abdominal discomfort to unexplained hypertension. Spindle cell sarcomas represent 1 of the least common malignant aortic tumours reported in the literature.Malignant aortic tumours have a poor prognosis, and of the various types of malignant aortic tumours, aortic sarcomas have a particularly poor prognosis with a 5-year survival rate of 8%.The exact pathophysiology of these malignancies is unknown but is thought to be related to the MDM2-p53 pathway and may be related to Li-Fraumeni syndrome.

Iatrogenic Cushing's Syndrome Following Intra-Articular Triamcinolone Injection in an HIV-Infected Patient on Cobicistat Presenting as a Pulmonary Embolism: Case Report and Literature Review.

BACKGROUND: Iatrogenic Cushing's syndrome (ICS) typically develops after long-term exposure to corticosteroids, but it can occur after a single dose in patients treated with cobicistat or ritonavir for HIV. We present a patient who developed ICS due to the interaction between cobicistat and triamcinolone, a review of the literature, and what to our knowledge is the first case of ICS presenting as a pulmonary embolism. CASE PRESENTATION: A 55-year old male with a past medical history of human immunodeficiency virus, undetectable for 15 years and currently on elvitegravir/cobicistat/emtricitabine/tenofovir alafenamide, received 2 intra-articular injections of triamcinolone one month apart for a Baker's cyst in his right knee. He used nasal fluticasone for 9 days in-between the injections. After his second knee injection, he developed easy bruising and friable skin. Over the coming months, he experienced weight gain and Cushingoid facies. Four months after the knee injections he developed a pulmonary embolism and deep vein thrombosis treated with warfarin. The Cushingoid facies prompted an evaluation and diagnosis of ICS along with hypothalamic pituitary adrenal axis suppression. CONCLUSION: This case demonstrates the need to monitor patients on pharmacological boosters with any exposure to corticosteroids, whether it be injected, inhaled, topical, oral or intravenous, as it can lead to profound adrenal suppression and ICS.

Small Bowel Gastrointestinal Stromal Tumor as a Gateway for Streptococcus anginosus Causing Multiple Liver Abscesses.

Gastrointestinal stromal tumors (GISTs) are the most common type of mesenchymal neoplasm of the gastrointestinal tract but consist of only 1% of all primary gastrointestinal neoplasms. Differentiated from other spindle cell tumors, GISTs are uniquely positive for CD117 expression which allows for molecular targeting therapy with imatinib mesylate (Gleevec). Clinical presentations are variable, ranging from asymptomatic to vague symptoms of abdominal pain, early satiety, abdominal distention or gastrointestinal bleeding. Very rarely, patients can present with tumor-bowel fistula and intra-abdominal abscesses. In this article, we discuss a rare presentation of a middle-aged male with multiple liver abscesses found to have a primary small bowel GIST. This patient received prompt intravenous antibiotics; however, hepatic abscesses can be easily misinterpreted as cystic hepatic metastases which can delay appropriate therapy. Streptococcus anginosus was found to be responsible for the formation of the liver abscesses visualized on computed tomography (CT) scan. Similar to Streptococcus bovis, knowledge in the literature is arising about the association between S. anginosus and gastrointestinal malignancies. This case highlights the importance of identifying concomitant primary GISTs with intra-hepatic abscesses, as these lesions can be easily misconstrued as liver metastases and consequently mismanaged. We herein emphasize that hepatic abscesses are a potential sequela of GISTs and should thus prompt further investigation for potential malignancies, if warranted, so that there is no delay in treatment of these gastrointestinal tumors.

A Unique Presentation of Spontaneous Compartment Syndrome due to Acquired Hemophilia A and Associated Malignancy: Case Report and Literature Review.

Hemophilia is a bleeding diathesis that is most commonly congenital and causes a tendency for significant bleeding during procedures and often manifests as hemarthrosis. However, more rarely, hemophilia can be acquired. Our paper focuses on acquired hemophilia A (AHA), which is caused by the development of an autoantibody (an inhibitor) to factor VIII. A 61-year-old man with a past medical history of type II diabetes mellitus, hypertension, hyperlipidemia, hypothyroidism, and obstructive sleep apnea presented to the emergency department with severe right lower extremity pain and swelling of 2-day duration. He was found to have compartment syndrome and underwent emergent fasciotomy of his right leg. After surgery he still had significant bleeding, despite transfusions and administration of fresh frozen plasma (FFP) by the surgical team. He was later diagnosed with AHA, but was not adequately responsive to factor VII, factor VIII, steroids nor rituxan and unfortunately had his right lower extremity amputated. He had a prolonged hospital course, which included Streptococcus bovis bacteremia and a code stroke for which head computed tomography (CT) showed probable metastasis. It was acknowledged he had probable metastatic colon cancer, which was not confirmed as the patient transitioned to hospice care. Rather than hemarthrosis, patients with AHA tend to have bleeding in soft tissue or the gastrointestinal tract. AHA can have underlying causes, such as malignancy. AHA associated with malignancy is associated with poorer outcomes and tends to improve with treatment of the underlying malignancy. Therefore, it is important to quickly identify these patients and screen them for underlying etiologies.

S100 Staining Adds to the Prognostic Significance of the Combination of Perineural Invasion and Lymphovascular Invasion in Colorectal Cancer.

Studies have suggested that perineural invasion (PNI) and lymphovascular invasion (LVI) serve as independent prognostic factors in colorectal cancer (CRC). Currently, little is known regarding the combination of PNI and LVI as prognostic factors, independent of stage. We hypothesized that this combination was a better prognostic marker than either PNI or LVI alone, and that S100 staining would detect PNI not seen with hematoxylin and eosin (H&E). Surgical pathology slides were retrospectively reviewed for 151 stages I to IV CRC patients who had surgery between January 1, 2008 and December 8, 2008 at 3 Hackensack Meridian Health hospitals in New Jersey. PNI and LVI were detected by H&E staining and a subset of 127 patient samples were additionally examined for PNI by S100 staining. Correlation between staining characteristics and patient outcomes was assessed using the Pearson χ tests and the Fisher exact tests. Survival was analyzed using Kaplan-Meier methods. Of the 151 cases reviewed, 30.5% were positive for PNI and 35.1% were positive for LVI by H&E. The use of S100 staining for PNI enabled its detection in 27 additional cases. Median time from patient diagnosis to death was significantly shorter for patients who were positive for both PNI and LVI (P<0.001). PNI and LVI were individual markers for poor survival in CRC patients and their combined presence had an even worse outcome. Failure to detect PNI on H&E can be overcome by S100 staining.

Hypercalcemia in T-Cell/Histiocyte-Rich Large B-Cell Lymphoma: An Unusual Presentation of a Rare Disease and Literature Review.

T-cell/histiocyte-rich large B-cell lymphoma (THRLBCL) is an extremely rare morphologic subtype of diffuse large B-cell lymphoma (DLBCL), accounting for only 1-3% of total cases. It is considered an aggressive lymphoma with a poor prognosis. Hypercalcemia has been described as an uncommon presenting symptom of patients with DLBCL in several case reports. Here, we report an unusual case of severe hypercalcemia in a patient who was ultimately diagnosed with T-cell/histiocyte-rich B-cell lymphoma. A 69-year-old male patient presented to our hospital with nausea, vomiting, weakness and unintentional weight loss. His initial blood tests showed a serum calcium level of 16.1 mg/dL and serum creatinine level of 3.25 mg/dL. He had high intact parathyroid hormone (PTH, 6.8 pg/mL), mildly elevated 25-hydroxyvitamin D and serum PTH-related peptide (PTHrP). To exclude malignancy, computed tomography (CT) scans of the chest, abdomen and pelvis were performed which were unremarkable. A bone marrow biopsy was performed to detect any hidden hematologic malignancy which showed large mononuclear cells with prominent nucleoli and occasional Reed-Sternberg cells, consistent with the diagnosis of THRLBCL. Subsequent positron emission tomography demonstrated diffuse fluorodeoxyglucose (FDG) uptake. This case reports a unique presentation of a rare subtype of non-Hodgkin's lymphoma. We highlight the importance of pursuing a thorough workup for causes of hypercalcemia as well as understanding the underlying mechanisms of severe hypercalcemia in malignancy.