Vitamin B12 deficiency can cause extensive hematologic alterations such as pancytopenia, macrocytosis, hypersegmentation of neutrophils, and hypercellular bone marrow with blastic differentiation. These dysplastic changes can sometimes be so profound that they mimic myelodysplastic syndromes or even acute leukemia, leading to extensive workup and aggressive treatment measures. We present a patient who was referred to our tertiary care medical center for treatment of suspected acute myeloid leukemia on the basis of peripheral smear and bone marrow biopsy findings, and induction chemotherapy was considered. However, the patient was found to have vitamin B12 deficiency, with improvement in pancytopenia and blastic changes with parenteral vitamin B12 supplementation. This highlights the importance of recognizing that dysplastic changes in patients with vitamin B12 deficiency could be misleading.
Despite the advances in the treatment and management, esophageal cancers continue to carry a dismal prognosis with an overall 5-year survival rate ranging from 15 to 25%. Delayed onset of symptoms and lack of effective screening methods and guidelines for diagnosis of the early disease contribute to the high mortality rate of esophageal cancers. Detection of esophageal cancer at their early stage is really a challenge for physicians including primary care physicians, gastroenterologists and oncologists. Although imaging, endoscopy and biopsy have been proved to be useful diagnostic tools for esophageal cancers, their diagnostic accuracy is unsatisfactory. In addition, expensive costs, invasiveness and special training operator have limited the clinical application of these tools. Recently, tumor-associated antigens (TAAs) and their antibodies have been reported to be potential markers in esophageal cancer screening, diagnosis, monitoring and prognostication. Because TAAs and their antibodies have the advantages of inexpensive cost, noninvasiveness and easy access, they have attracted much attention as an affordable option for early esophageal cancer diagnosis. In this review, we summarized the advances in TAAs and their antibodies in esophageal cancer screening, diagnosis, monitoring and prognostication.
Acquired inhibitors of coagulation are a group of rare but potentially life-threatening blood disorders characterized by the presence of autoantibodies directed against clotting factor. Autoantibody against factor VIII is the most common form of clotting factor inhibitor, a condition also known as acquired hemophilia A. We present a clinical series of nine patients diagnosed and treated for acquired hemophilia A at our institution. Among these nine patients, there were five men and four women with a median age of 64 years. All patients presented with bleeding diathesis. Factor eight inhibitor bypassing agent and/or recombinant factor VIIa were predominantly used for control of active bleeding. For elimination of autoantibodies, either steroids alone or the combination of steroids with rituximab or oral cyclophosphamide was used. Despite aggressive measures, two of the patients had a poor outcome; seven of the nine patients (77%) had a good clinical outcome. Acquired hemophilia A should be strongly suspected in any patient presenting with bleeding and a prolonged activated partial thromboplastin time. Early initiation of factor bypassing agents such as activated prothrombin complex concentrates or recombinant factor VIIa, along with the use of immunosuppressive agents, can be lifesaving.
Hemophagocytic lymphohistiocytosis (HLH) is an underrecognized disorder due to the variability of its presentation and the fact that in adults, its diagnosis is based on cumbersome, pediatric-based criteria. Data regarding demographics, underlying causes, clinical features, laboratory results, complications, treatments received, and clinical outcomes were collected and analyzed in 41 patients who were diagnosed and treated at University of Arkansas for Medical Sciences between 2007 and 2019. In this group, 51% were male, the median age at diagnosis was 47 years, and 85% (35/41) met the HLH-2004 diagnostic criteria (5/8 variables). When evaluating seven extended variables easily obtained by routine laboratory test, 93% (38/41) of patients met 8 out of 15 criteria. The overall mortality in our patient population was 54% (22/41). The 30-day and 1-year overall survival estimates were 0.73 (95% confidence interval 0.56, 0.84) and 0.46 (95% confidence interval 0.29, 0.62), respectively. Thirty-five patients (85.4%) received HLH-directed therapy, and 19 patients (46.3%) achieved remission. The most common regimen for treating HLH was dexamethasone plus etoposide (53.7%). The patients with malignancy-related HLH had a worse prognosis than those without underlying malignancy, with a 73.33% (11/15) vs 34.62% (9/26) mortality (P ¼ 0.02). In conclusion, despite increasing recognition, HLH remains an enigmatic disorder with increased mortality, even more so with malignancy-associated HLH.
e21645 Background: C-Reactive Protein (CRP) is an acute phase reactant which is elevated in inflammatory conditions and malignancy. Elevated CRP levels are associated with poor prognosis in many cancers and has been reported in patients with melanoma treated with ICI. We examined the association between CRP levels during therapy with ICI in patients with advanced lung cancer and radiological response. Methods: A retrospective study of patients with metastatic lung cancer (stage IV) who were treated with immunotherapy from 2016 to 2019 was conducted. CRP levels were checked at or soon after initiation of therapy and at frequent intervals thereafter. Association between CRP levels and response was determined. Results: 36 patients had initial (at the beginning of therapy, baseline) and subsequent CRP levels recorded during the first course of immunotherapy. Among the 36 patients, 16 (44%) had radiological response or stable disease, and 20 (55%) had radiological progression. The median age was 69 years (range 50 – 89 years); male/female 35/1, Caucasian/African American/other 26/9/1. There was no significant difference in distribution of initial CRP levels between patients with response vs progression as assessed by Kruskal-Wallis test (P-val: 0.20). Logistic regression modeling was employed to examine the association between final CRP level, the last measurement while on ICI therapy (adjusted for baseline CRP level) and odds of observing a response. A unit increase in final CRP level was associated with 4% decrease in odds of observing a response (Odds Ratio, 95% CI: 0.96, 0.92-1.00, P-val: 0.05). Conclusions: Baseline-adjusted final CRP levels were inversely associated with likelihood of observing a response in advanced lung cancer subjects treated with ICI. Further validation in prospective studies is warranted.
Chlorambucil (CHL) was used in combination with prednisolone in the treatment of nine children with frequently relapsing nephrotic syndrome. Serial electroencephalograms were obtained to evaluate CHL central nervous toxicity, before, during and after treatment with this agent. EEG abnormalities were observed in two of the nine children during chlorambucil therapy. EEG changes were diffuse spike and wave complexes and disappeared after discontinuation of therapy. There were no other neurological abnormalities and more particularly, no seizures or myocloni were observed. According to the literature, chlorambucil central nervous toxicity is found almost exclusively in childhood nephrotic syndrome. Strict neurological supervision of patients treated with chlorambucil is recommended.
INTRODUCTION Aggressive NK-cell leukemia ( ANKL) is a systemic lymphoproliferative disorder of natural killer (NK) cells frequently associated with Epstein-Barr virus (EBV) which has been grouped under histiocytic/dendritic neoplasms in the 2008 WHO classification of hematopoietic neoplasms. Due to rarity of diagnosis, the current available literature is limited to case reports and case series. This population-based study using Surveillance Epidemiology and End result program (SEER) is the largest to explore demographic characteristics, survival patterns and long-term outcomes in patients with ANKL in the United States. MATERIALS AND METHODS We utilized SEER 18 November 2020 submission database to select all patients (> 1 years of age) diagnosed from 2000-2018 with ANKL using International Classification of Diseases for Oncology edition 3 (ICD-O-3) code of 9948/3. Patients were divided into various cohorts based on age (<60 years, 60-79 years, >80 years), sex, race (Caucasians, African American, Asian/Pacific islander and American Indians/Alaska natives) and median household income of county of residence (< $ 50,000 vs > $50,000). We used SEER*stat to calculate age adjusted incidence rate using 2000 US standard population. Kaplan Meier curve was utilized to calculate 5-year overall survival. Cox proportional hazard model was used for multivariate analysis of factors associated with survival and p<0.05 was considered significant. RESULTS A total of 140 patients were identified with ANKL. The median age at diagnosis is 58 years. The crude, age-adjusted to 2000 US standard population and age-specific incidence rate of HCD in Unites states is < 1/100, 000 respectively. The incidence in males is 1.9 times that of females- 92 males (65.7%) and 48 females (34.2%). Between 2001-2018, minimum number of cases were diagnosed in 2010 (n=2).Out of the cohort, 107 patients were White (76.4%), 21 patients were Asian or Pacific islander (15 %), 9 patients were African Americans (6.4%) and 3 patients were American Indian/Alaska native (2.1%). The median overall survival was 7 months (95% CI; 0-16). Survival rates at 1year, 2 years and 5 years were found to be 45.3% , 37.1 % and 31.1% respectively [Figure 1]. 5 year overall survival rates are as follows- Whites ( 69.7% ), Blacks ( 63.1%), American Indian (59.9%), Asian or Pacific Islander ( 66.8%). On multivariate analysis, black race was associated with poor outcomes (p 0.008), whereas sex, income and age had no significant effect on cancer outcomes. CONCLUSIONS Our study shows that ANKL is a rare hematological malignancy in general population with a poor median survival of less than one year. Males are twice more likely to be affected than females with poor outcomes in africo-americans. Figure 1 Figure 1. Disclosures No relevant conflicts of interest to declare.
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