A Case of Essential Thrombocythemia and IgA Nephropathy with Literature Review of the Concurrence.

Myeloproliferative neoplasms such as essential thrombocythemia (ET) have been associated with glomerular disease on rare instances. A case of ET associated with immunoglobulin A nephropathy (IgAN) is described in a 57-year-old man with a history of hypertension. Progressively worsening renal function was noted in the patient along with unexplained mild thrombocytosis. Pathological review of renal biopsy identified IgAN concurrently with newly diagnosed JAK2-mutated ET. The patient was started on aspirin therapy and closely monitored for his renal function. A literature review of the association of ET and renal disease revealed nine cases of ET associated with IgAN, focal segmental glomerulosclerosis, and fibrillary glomerulonephritis. Comparison of the pathological features of the renal biopsies within the cases noted mesangial proliferation as a common finding, which has been described to be potentiated by platelet-derived growth factor (PDGF). This commonality may represent a link between ET and glomerular disease which deserves further attention in future cases. Improved management of such cases depends on the recognition of the combined occurrence of ET and glomerular diseases and uncovering the shared pathogenesis between platelets and glomeruli

MABs for TMAs: When Plasma Exchange Is Not Enough

Monoclonal antibody (mAb) therapies are being directed at an increasing selection of disorders, particularly within the realm of hematology. Thrombotic microangiopathies (TMA) such as thrombotic thrombocytopenic purpura (TTP) and complement-mediated atypical hemolytic uremic syndrome (aHUS) are examples of disease processes that are amenable to intervention with such novel therapies. The mAb-based treatments are particularly helpful in treatment of these TMAs when plasma exchange does not yield clinical benefit.This presentation will describe a case of TTP and a case of aHUS that saw successful treatment with mAb-based therapy. The diseases will be compared using clinical presentation, laboratory results, and peripheral smear images to promote their recognition. Furthermore, pathophysiology differences of the diseases will be emphasized to explain the indications of the unique therapies.The presentation will focus on caplacizumab and eculizumab mAb therapies. Caplacizumab has found a place in the treatment of TTP refractory to other therapies, since its FDA approval in 2019 following results from the double-blind, controlled HERCULES trial (Scully et al NEJM 2019). This trial showed that caplacizumab reduced recurrence of TMA events by almost 67% more than placebo. This mAb will be compared to Eculizumab which has been FDA approved for aHUS since 2011 based on prospective studies. Long term data presented by Menne et al BMC 2019 shows only 4% of patients who remained on eculizmab experienced relapsed TMA events

Diagnosing Yersinia Enterocolitis under the Disguise of Pseudoappendicitis

Background: Yersiniosis due to Yersinia enterocolitica causes pseudoappendicitis syndrome presenting with right lower abdominal pain, fever and leukocytosis. Timely recognition of this entity is important as treatment with antibiotics leads to rapid recovery and can save patient from an unwarranted appendectomy. Case discussion: A 35-year-old female presented with three day history of right lower quadrant abdominal pain, nausea and vomiting. No fevers or diarrhea were reported. She had food from vendors prior to these symptoms. She was afebrile and physical exam revealed exquisite tenderness upon palpation of the right side of the abdomen, without rebound or rigidity. Laboratory data identified white blood cell count of 12,100/ul and normal amylase, lipase and liver enzymes. Computerized tomography scan of the abdomen discovered marginal proximal appendiceal wall thickening and inflammation of the ascending colon. The overall clinical picture was consistent with acute yersiniosis mimicking appendicitis and hence patient was started on IV ampicillin-sulbactam, pending stool culture and PCR results. At 24 hours, patient reported significant improvement with normalization of leukocyte count. She was discharged on oral ciprofloxacin and metronidazole for a total duration of 7 days. Her stool culture returned negative while the PCR returned positive for Y. enterocolitica, thus confirming the diagnosis. Conclusion: Current CDC estimates suggest that Yersinia enterocolitica causes almost 640 hospitalizations in the United States every year. Recognizing pseudoappendicitis with history indicative of foodborne illness as a presentation of this uncommon infection is crucial, as treatment with antibiotics leads to prompt recovery

B12 Deficiency: An Overlooked Case of Microangiopathic Hemolytic Anemia

Case Presentation: A 32-year-old female with a history of untreated sarcoidosis and gastric bypass surgery with partially treated B12 deficiency in the past, was admitted for shortness of breath with a primary diagnosis of pancytopenia. Patient had not taken B12 injections for close to 2 years. Her labs were consistent with severe pancytopenia (Hemoglobin 5.4, White blood cell count 1.8, Platelets 78000) with lab evidence of massive hemolysis on peripheral smear (schistocytes) and chemistries (severely increased Lactate dehydrogenase 2377, reticulocytes 2.5% and decreased haptoglobin \u3c30). B12 levels (\u3c50) and folate levels (5.4) were severely depressed. Coombs test was negative on two separate occasions. Patient responded to packed red blood cell transfusion, however she continued to show signs of ongoing hemolysis. Hematology was consulted who started patient for possible immune mediated hemolytic anemia with intravenous immunoglobulin and prednisone. We determined that microangiopathic hemolytic anemia can be explained by B12 deficiency alone, given patient’s pancytopenia prior response to B12 injections. Treatment was simplified with discontinuation of intravenous immunoglobulins and initiations of supplementation with B12 injections and oral folate. Patient complete blood count after a month of treatment with B12 injections showed immense improvement in hemoglobin to 12.5 and WBC to 4.1. Discussion: Massive hemolytic anemia is a rare and potentially lethal complication of B12 deficiency. B12 is usually attributed to megaloblastic anemic with severe forms of deficiency causing pancytopenia’s. However, severe B12 deficiency is an under-documented etiology for treatable microangiopathic hemolytic anemia. Severe hemolysis with low reticulocyte counts, schistocytes on peripheral smear and very high Lactate dehydrogenase should raise the suspicion of severe B12 deficiency. Conclusions: B12 supplements in patients with severely depressed B12 levels with hemolysis is a simple fix for an apparently life-threatening condition and avoids the need for unnecessary interventions

Safety of Antria Cell Preparation Process to Enhance Facial Fat Grafting with Adipose Derived Stem Cells

The use of adipose tissue transfer in plastic and reconstructive surgery is not new and has been studied for more than a century but problems such as unpredictability in results and a low rate of graft survival due to partial necrosis were always among major concerns [1]. However, emerging information regarding the potential of adipose derived stem cells, new methods of cell extraction, graft preparation and injection techniques have increased the popularity of fat transfer and the efforts toward development of cell based therapies for various diseases from Adipose Derived Stem Cells (ADSC’s) and Stromal Vascular Fraction (SVF) of the adipose tissue. Although the mechanism of action of those stem cells is not fully known, their paracrine activities and transformation to various cell types can be responsible for reported clinical outcomes [1,2]. Many clinicians and researchers report better outcomes in fat grafting upon addition of SVF cells [1,2]. This study aims to investigate the long-term (3 years) safety of Antria’s cell preparation process utilizing a digestive enzyme in SVF assisted fat grafting. The outcomes of this study was utilized to conduct further safety and efficacy studies to obtain regulatory and marketing approval for a novel SVF extraction method in the U

The utility of S100B level in detecting mild traumatic brain injury in intoxicated patients.

BACKGROUND: S100B is a serum protein known to elevate in patients with brain injury, but it is unknown whether it can predict intracranial pathology in intoxicated patients following mild traumatic brain injury (MTBI). We performed a systematic review and meta-analysis of the English language literature to address this question. MAIN OUTCOMES AND RESULTS: Four prospective cohort trials of serum S100B levels on acutely intoxicated patients with MTBI were included in this meta-analysis. Prevalence of intracranial pathology in the pooled cohort of the intoxicated MTBI patients was 10%, lower than the 15-30% reported in the literature for the general MTBI population. Standard mean difference of serum S100B levels between patients with and without intracranial pathology on CT was 0.73 μg/L (Z = 18.33, P \u3c 0.001). Following sensitivity analysis and hierarchical summary receiver-operating characteristic models, three remaining articles were used for pooled estimates that found that S100B had a sensitivity of 0.96 (95% CI: 0.84-1.00, I2 = 0%) and specificity of 0.63 (95% CI: 0.58-0.68, I2 = 86.8%) with a high negative predictive value (100%, 95% CI: 95.14-100, I2 = 0%) and a negative LR of 0.06 (95% CI: 0.01-0.31). CONCLUSIONS: Serum S100B levels may have utility in ruling out intracranial pathology in intoxicated patients, however more study and comparison with other serum biomarkers of brain injury are necessary before this becomes the accepted standard of care