without significant pruritic urticaria and has reported a vast improvement in quality of life.
with sebaceous glands and transdermal migration of water-soluble antigens leading to mast cell release of histamine. IgG antibodies can inhibit IgE- mediated mast cell activation through direct allergen neutralization or through the inhibitory receptor FcγRIIb. This case raises questions of a potential mechanistic interaction, especially given the robust response to omalizumab and IVIG treatment.
Discussion: An association between IgG3 subclass deficiency and aquagenic urticaria has never been documented. The pathophysiology of aquagenic urticaria remains elusive, however proposed mechanisms include water antigens interacting
MYSTERY CASE PRESENTATIONS – RESIDENTS
PSEUDO-THROMBOTIC MICROANGIOPATHY SECONDARY TO B12 DEFICIENCY Karam Rahat MD, Ricardo Vallejo-Calzada MD, Graham Unis, MD, Drishti Chawla MD; Department of Medicine, Ochsner Medical Center, New Orleans, LA.
Introduction: Pseudo-thrombotic microangiopathy (TMA), a rare presentation of vitamin B12 deficiency, shares many features with thrombotic thrombocytopenic purpura (TTP). TTP management
higher level of care due to concern for TTP given her altered mental status and concurrent renal impairment. Further evaluation revealed a vitamin B12 and folate deficiency and a PLASMIC score of 2. ADAMTS13 activity was normal. Intravenous vitamin B12 and folate repletion were initiated with improvements seen in her mental status and blood counts. An intrinsic blocking factor returned positive. The patient was discharged with monthly vitamin B12 injections and hematology follow-up. Discussion: Vitamin B12 deficiency has a variable presentation with a myriad of potential hematologic and neurologic sequelae. Pseudo-TMA represents a rare presentation of B12 deficiency often confused with TTP. Management of TTP involves plasma exchange and often requires specialized care. Therefore, recognition of factors that may distinguish Pseudo-TMA from TTP is critical.
often includes plasma exchange and other therapies that are neither therapeutic nor benign in regard to vitamin B12 deficiency.
Case: A 58-year-old female with no known history was brought by her family after being found on the bathroom floor with somnolence and confusion. Her initial workup was notable for a Hgb of 2.6 g/ dL, a MCV of 95fL, and platelets of 71k/µL. LDH 3500 Unit/L, Haptoglobin 19 mg/dL, negative direct Coomb’s test, elevated bilirubin with depressed reticulocyte index, reticulocyte 0.7%, reticulocyte index 0.06, elevated PT/aPTT and fibrinogen of 315 were also reported. Peripheral smear demonstrated schistocytes, teardrop cells, and hypersegmented neutrophils. The patient was transferred to a
HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS Anastasia Graffagnini, MD, Andriane Melanthiou, MD, Jasleen Hora, MD, Jennings Field, MD, Shyam Mani, MD, Ibrahim El Abbassi, MD; Department of Medicine, Leonard J Chabert Medical Center, Houma, LA.
Introduction: Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome resulting from defective macrophages and cytotoxic T cells. HLH can be triggered by infections, malignancies, or autoimmune disorders. Patients can present with recurrent fevers, cytopenias, liver dysfunction, and a sepsis-like syndrome. Case: A 55-year-old male with atrial fibrillation, lung mass, hypertension, hyperlipidemia, diabetes, heart failure with a preserved ejection fraction, obstructive
sleep apnea, prior COVID-19 infection, and morbid obesity presented with four days of altered mental status, shortness of breath and a productive cough with clear sputum. In addition, the patient endorsed dysuria with malodorous urine and bilateral extremity swelling. Prior outpatient evaluation for her lung mass revealed atypical pneumocyte hyperplasia with background macrophages including vacuolated forms. Repeat biopsy performed to further evaluate the suspicious lung mass and adenopathy revealed predominantly lymphoplasmacytic inflammatory 13
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