6 research outputs found
Acute Myeloid Leukemia Presenting as Granulocytic Sarcoma (Chloroma) of the Tongue: A Case Report
Chloroma or Granulocytic Sarcoma or Myeloid Sarcoma or Myeloblastoma is a tumor composed of immature myeloid precursor cells or blasts. It is an extra medullary manifestation of AML, which is extremely rare but well documented. It can herald, follow or occur with a diagnosis of primary AML. It can also be seen in relapse [1]. The usual sites of involvement are skin, soft tissue and lymph nodes. Intra oral myeloid sarcoma is infrequent and particularly chloroma in the tongue is further uncommon. A thorough review of literature yielded only three reported cases of chloroma of the tongue [2-4]. In this case report, we describe a case of a 36-year-old female who presented with two weeks of dysphagia due to a large tongue lesion accompanied by pancytopenia. The tongue lesion was strongly suspicious of a chloroma. Bone marrow biopsy confirmed a diagnosis of AML and a resolution of the chloroma was observed with induction chemotherapy for AML
Patients with MEN1 are at an increased risk for venous thromboembolism VTE risk in MEN1
Background: Multiple endocrine neoplasia type 1 (MEN1) is a rare inherited disorder predisposing to the development of multiple functional and non-functional neuroendocrine tumors (NETs). Only uncommon MEN1-associated functional NETs such as glucagonomas (<1%) and ACTH-producing tumors (<5%) are known to be associated with hypercoagulability. It is unknown if patients with MEN1 generally have increased risk of VTE.
Methods: We queried a prospective natural history study of germline mutation positive MEN1 patients (n=286) between 1991-2019 for all lifetime events of VTE. Search terms were: DVT, thromb, embol, PE, pulmonary embolism, clot, hematology consult, anticoagulant, coumadin, lovenox, xarelto, warfarin, aspirin, rivaroxaban and apixaban. Incidence rates were calculated accounting for age and sex. Comparison was made to published incidence rates in healthy populations, different types of cancer, and Cushing's syndrome.
Results: Thirty-six subjects (median age 45 years, range 16-75) experienced a VTE event, yielding a prevalence rate of 12.9%. The age-sex adjusted incidence rate of VTE is 9.11 per 1,000 patient-years, with a sex-adjusted lifetime incidence rate of 2.81 per 1,000 patient-years. MEN1-associated lifetime incidence rates are ~two-fold higher than the estimated annual incidence rate in the general population and are comparable to known risk in the setting of various types of cancer. Approximately 80% were diagnosed with pancreatic NETs, of which 24% were insulinomas. Fourteen patients (42%) experienced peri-operative VTE events.
Conclusions: MEN1 patients have an increased risk of VTE. Further mechanistic investigation and validation from other MEN1 cohorts are needed to confirm the increased prevalence of VTE in MEN1
Nanomedicine in coronary artery disease
Nanomedicine is one of the most promising therapeutic modalities researchers are working on. It involves development of drugs and devices that work at the nanoscale (10–9 m). Coronary artery disease (CAD) is responsible for more than a third of all deaths in age group >35 years. With such a huge burden of mortality, CAD is one of the diseases where nanomedicine is being employed for preventive and therapeutic interventions. Nanomedicine can effectively deliver focused drug payload at sites of local plaque formation. Non-invasive strategies include thwarting angiogenesis, intra-arterial thrombosis and local inflammation. Invasive strategies following percutaneous coronary intervention (PCI) include anti-restenosis and healing enhancement. However, before practical application becomes widespread, many challenges need to be dealt with. These include manufacturing at the nanoscale, direct nanomaterial cellular toxicity and visualization
Are Venous Thromboembolic Events Increased in MEN1 Patients?
Background: Multiple endocrine neoplasia type 1 (MEN1) is a rare inherited disorder in which patients develop multiple simultaneous hormone-secreting tumors. Most common tumors include: anterior pituitary adenomas (50%), multi-gland parathyroid adenomas (95%), and gastroenteropancreatic neuroendocrine tumors (40-80%). Only rare MEN1 associated glucagonomas (<1%), and ACTH-producing neuroendocrine tumors (<5%) are known to increase risk of hypercoagulability. It is unknown if patients with MEN1 syndrome have increased risk of venous thrombolytic events (VTE), defined as a deep-vein thrombosis and/or pulmonary embolism.
Methods: We queried a prospective natural history study of MEN1 patients who tested positive for germline MEN1 mutations (n=287) between 1991-2019 (54 patients on our current protocol were followed before 1991; the earliest was 1971). All lifetime events of VTE were included. Search terms included: DVT, thromb, embol, PE, pulmonary embolism, clot, hematology consult, anticoagulant, coumadin, lovenox, xarelto, warfarin, aspirin, rivaroxaban and apixaban. After initial screening, 10 patients were removed due to insufficient clinical data. Kaplan-Meier analysis was performed to compare age of death between the two cohorts. Results were expressed as mean ± standard deviation.
Results: Thirty-four subjects (mean 57.5 years-old, 17 women) were identified with any VTE, yielding a prevalence rate of 13.4%. The incidence of VTE corresponded to 264 events per 100,000 patient-years, which was ~2-fold higher than the estimated annual incidence rate in the general population (104-183/100,000 patient years).1 Kaplan-Meier analysis revealed no significant difference in survival between the two groups (non-VTE cohort mean 81.1 years ± 2.23; VTE cohort mean 77.4 years ± 3.45; p = 0.96). Thirty-two events occurred during the surveillance period at our institution; 9 individuals had more than one VTE. At the time of VTE, 80% had hyperparathyroidism (mean PTH ± SD; 97.56 pg/mL ± 90.76), 21% had hyperprolactinemia (prolactin 25.7μg/L ± 43.41), 62.5% had hypergastrinemia (mean gastrin 1100.9 pg/mL ± 3127.8), and 84.6% had non-functional pancreatic neuroendocrine tumors. One patient was identified to have a Factor V Leiden mutation, 3 patients had lupus anti-coagulant. Eleven patients experienced events within a post-surgical period of 3 months.
Conclusions: Hypercoagulability in MEN1 has been previously unidentified. Our cohort data suggests a two-fold increase in the incidence of VTE as compared to the general population, with a high risk occurring within the perioperative period. Further mechanistic investigation and validation from other cohorts are needed to confirm the increased prevalence of VTE in this population.
1Heit, John A., et al. Epidemiology of venous thromboembolism. Nat Rev Cardiol 2015 Aug;12(8): 464-474