Adult-onset Still's disease with concurrent thrombotic microangiopathy: Observations from pooled analysis for an uncommon finding.

BACKGROUND: Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disorder that is characterized by quotidian fevers, arthritis, and an evanescent rash. Occurrence of concurrent thrombotic microangiopathy (TMA) in AOSD is rare. The treatment aspects of TMA in AOSD are actively being debated. METHODS: Medline search using MeSH terms and snowballing yielded a total of 29 articles with co-occurrence of AOSD and thrombotic thrombocytopenic purpura (TTP) including our own. Pooled data were synthesized for descriptive analysis. RESULTS: Median age was 35 years with a majority of females (68.96%). A majority of these studies/patients were either Asian (34.48%) or Caucasian (31.03%). Concurrent TMA at the time of AOSD diagnosis was seen in 65.51% patients. Only 3/29 patients had ADAMTS13 level less than 10%, consistent with TTP and 3/29 were diagnosed with hemolytic uremic syndrome (HUS). The remainder were diagnosed clinically. Complication rate was high, and 15/29 (51.72%) patients died or had permanent neurological/renal/vision/gangrenous complications. Median and mean ferritin peak was observed to be higher (7458 and 12 349, respectively) in patients who either died/had partial remission, compared to those who had complete response (3257 and 10 899, respectively), p = .829. CONCLUSIONS: A majority of patients with AOSD-associated TMA either died or had permanent complications. TMA was diagnosed alongside AOSD in 65% patients, while the rest developed TMA during the course of their disease. Blurred vision may precede TMA and could help risk-stratify high-risk AOSD patients clinically. Glycosylated ferritin remains low several weeks to months after disease remission and may be used to monitor severity of disease process. Further studies are necessary to confirm the existing vascular endothelial growth factor hypothesis in AOSD-associated TMA.

Thrombotic Thrombocytopenic Purpura Without Evidence of Microangiopathic Hemolytic Anemia: A Case Report.

Diagnosis of thrombotic thrombocytopenic purpura (TTP) is challenging due to varied clinical presentations and is primarily based on ADAMTS13 activity assay, however clinical suspicion to include TTP as a potential diagnosis relies on multiple scoring systems all involving hemolysis as a prime feature. Here, we report a case of TTP without any evidence of microangiopathic hemolytic anemia (MAHA). A 65-year-old male admitted with a Glasglow come scale of 3 was intubated and sedated on admission. Complete blood count was concerning for a hemoglobin (Hb) of 5.8 g/dL, and a platelet count of 76 k/µL. The patient had a bleeding episode while placing a central line; the repeat platelet count was found to be 35 k/µL, further dropping to 14 k/µL the next day. Coagulation studies now reflected PT of 19.8 sec, aPTT of 38.7 sec, and fibrinogen of 212 mg/dL. The peripheral smear showed no evidence of hemolysis. TTP was kept low on the differential and haematological anomalies were attributed to possible disseminated intravascular coagulation (DIC) from sepsis and liver disease. ADAMSTS13 was incidentally checked upon admission, later resulting in <5% activity with a Bethesda titer inhibitor of 3.2. The patient was immediately initiated on plasmapheresis along with prednisone. Additionally, rituximab and caplacizumab were added. Plasmapheresis was continued for ten sessions until the platelet count reached 167 k/µL. At the time of discharge, laboratory values revealed platelets of 251 k/µL and hemoglobin of 8.8 g/dL. We recognize that the diagnosis of TTP is challenging because of its diverse clinical manifestations and constrained availability of ADAMTS13 testing. Clinical prediction scores have been developed to estimate the pretest probability of severe ADAMTS13 deficiency, however, they all include the presence of MAHA. Atypical presentation of TTP has been previously acknowledged however continues to remain under-recognized.

Cultural competency education in the medical curriculum to overcome health care disparities.

Background: Our increasingly diverse population demands the adoption of transcultural approaches to health care delivery. Training courses in medical education have been developed across the country for cultural competency, but have not been standardized or incorporated consistently. This study sought to formulate an educational intervention in medical training using the concepts of cultural competency and humility to improve understanding of cultural disparities in health care. Methods: This study used three domains of Tools for Assessing Cultural Competence Training (TACCT) by the Association of American Medical Colleges. Participants included 106 fourth-year medical students and 19 internal medicine residents at Louisiana State University in Shreveport in 2022. The training session included a lecture introducing cultural and structural competency for 30 minutes followed by three workshops based on the TACCT domains of key aspects of cultural competence, understanding the impact of stereotyping on medical decision-making, and cross-cultural clinical skills. The participants were given a pre- and postsession questionnaire. Results: After the session, 68% of students rated their understanding of cultural competency as excellent. For methods of teaching-lecture versus workshop versus both-66% rated the combination as excellent. Conclusion: The rudimentary understanding of cultural competency and cultural humility improved after the session.

Sickle Cell Trait: Is It Always Benign?

Sickle cell disease is a well-known homozygous inherited hemoglobinopathy that causes vaso-occlusive phenomena and chronic hemolysis. Vaso-occlusion results in sickle cell crisis and can eventually lead to complications involving multiple organ systems. However, the heterozygous counterpart, sickle cell trait (SCT) has less clinical significance as these patients are generally asymptomatic. This case series examines three unrelated patients with SCT ranging from the age of 27 to 61 years, who presented with pain in multiple long bones. Hemoglobin electrophoresis confirmed a diagnosis of SCT. Radiographic images of the affected sites showed osteonecrosis (ON). Interventions included pain management and bilateral hip replacement in two of the patients. Historically, vaso-occlusive disease in patients with SCT with no evidence of hemolysis or other hallmark findings of sickle cell disease is rare. There are limited reported cases of ON in SCT patients. Clinicians should explore other hemoglobinopathies not tested on routine hemoglobin electrophoresis and alternative risk factors for ON in these patients.

Duodenal Metastasis From Primary Lung Adenocarcinoma: A Diagnostic and Therapeutic Challenge.

Distant metastasis from primary lung cancer is mostly seen in the liver, brain, adrenal glands and bones. Small bowel, specifically duodenum is a relatively unusual site for distant metastasis from lung carcinoma. This case reports a rare scenario of upper gastrointestinal bleeding caused by duodenal metastasis by a primary lung adenocarcinoma. A 43-year-old woman presented to the emergency department with complaints of progressive hemoptysis for the past three weeks. Esophagogastroduodenoscopy (EGD) revealed a 2.5 cm x 2.5 cm fungating villous mass-like structure in the first portion of the duodenum, with a normal-appearing esophagus and stomach. Biopsies were performed, which were histologically consistent with poorly differentiated malignant. The immunohistochemical (IHC) staining was consistent with metastatic disease from primary lung adenocarcinoma. Due to its rarity, there are no solidified guidelines for the management of duodenal metastasis from lung carcinoma. Our case was challenging due to the extensive metastasis and low functional status of the patient and was ultimately managed with home hospice.

Co­existence of triple­negative essential thrombocythemia and double transcript chronic myeloid leukemia: A case report.

Chronic myeloproliferative neoplasms (MPN) include polycythemia vera (PV), primary myelofibrosis, essential thrombocythemia (ET) and chronic myeloid leukemia (CML). Overlapping MPNs are rare; however, they can occur in the same individual. The present case report describes a patient with both triple-negative ET and CML. A 64-year-old woman was followed-up at our hematology clinic at Feist Weiller Cancer Center, Louisiana State University Health Shreveport (Shreveport, LA, USA) since 2000 after she was diagnosed with JAK2V617F-negative ET. The patient remained stable on hydroxyurea until 2012, when they underwent a bone marrow biopsy for progressively increasing white blood cell counts, and the pathology was consistent with CML; PCR for BCR-ABL was positive for both P210 and P190 transcripts. The patient was then initiated on dasatinib. After dasatinib, they were given a trial of imatinib, and were later transitioned to nilotinib and finally to bosutinib (2019) due to unchanged thrombocytosis. Next-generation sequencing from a bone marrow biopsy in 2019 demonstrated an EZH2 mutation that may be associated with triple-negative ET. CML was in major molecular response at that time. The patient was continued on bosutinib with hydroxyurea, after which hydroxyurea was changed to anagrelide due to worsening anemia and persistent thrombocytosis. However, bosutinib and anagrelide were discontinued due to worsening pulmonary hypertension. The patient was noted to have peripheral blasts of 14% by flow cytometry, after which they underwent a repeat bone marrow biopsy in 2022, which showed extensive myelofibrosis. BCR-ABL transcripts were undetectable. Given their accelerated myelofibrosis, the patient was started on a hypomethylating agent, decitabine/cedazuridine, along with darbepoetin for anemia in June 2022. Given their persistent thrombocytosis, the patient was also started on peginterferon α. Most studies reporting two clonal processes in the same patient have been for PV and CML. To the best of our knowledge, this is the first reported case of triple-negative ET with double transcript CML in the same individual.

Rectum to Medulla Oblongata: Colorectal Cancer Metastasizing to the Brainstem.

Metastasis with colorectal cancer (CRC) is commonly seen in the liver, lungs and peritoneal cavity. Brainstem involvement with CRC is not studied with no prior reported cases. We report a case of CRC, admitted for apneic spells and dry cough and later found to have metastasis to the left anterolateral medulla oblongata. A 28-year-old male, with a past medical history of asthma, and colorectal adenocarcinoma metastatic to the brain, presented to the emergency department with complaints of a dry cough, altered mental status and shortness of breath. He was seen at urgent care before and was given a week of oral levofloxacin for presumptive pneumonia without any relief. Physical examination was concerning for stridor with clear lung fields. MRI brain showed previously noted post-operative right frontoparietal craniotomy changes and a new 9 x 8 x 8 mm ring-enhancing intra-axial lesion centered at the left anterolateral medulla oblongata indicative of brainstem metastatic disease. The patient was intubated for airway protection and underwent a suboccipital craniotomy for resection of the left pontomedullary mass, and histopathology was positive for metastatic adenocarcinoma, colorectal primary with hemorrhagic necrosis. He had a tracheostomy placed post multiple failed extubation trials and a gastrostomy tube for oral feeds. Goals of care were addressed with the patient and family, and a decision was made for home hospice.

Not Another Pleural Effusion: A Unique Case of Metastatic Endometrial Stromal Sarcoma.

The etiology of complicated pleural effusion can be vast. We present a unique case of an unsuspected metastatic endometrial stromal sarcoma (ESS) in an asymptomatic patient with an incidentally found complicated pleural effusion. A 69-year-old female with no pertinent past medical history was referred to pulmonology for an effusion noted on a routine chest X-ray. Her surgical history was significant for a hysterectomy. At the time of evaluation in the pulmonology clinic, the patient was asymptomatic with stable vital signs. Computed tomography of her chest showed a complex pleural effusion which was drained by cardiothoracic surgery. Fluid analysis results were positive for estrogen and progesterone receptor-positive mesenchymal tumor. Follow-up imaging was negative for any other metastasis. Appropriate management and drainage of this asymptomatic pleural effusion resulted in the diagnosis of a rare malignancy. Given the good clinical prognosis of mesenchymal tumors, the patient was appropriately treated and doing well. We present the case of a patient who was found to have a rare malignancy rather than a benign chronic pleural effusion, as previously suspected. This neoplasm represented a metastatic ESS, especially in this patient's setting of a hysterectomy.

Therapeutic Plasma Exchange in Severe Rhabdomyolysis: A Case-Control Study.

INTRODUCTION:  Rhabdomyolysis is a serious condition that can cause acute kidney injury (AKI), compartment syndrome, severe metabolic and electrolyte derangement leading to arrhythmias, and even death. Total plasma exchange (TPE) has been used as a treatment modality to clear myoglobin, but the evidence is limited. In this study, we aim to investigate the use of TPE in critically ill rhabdomyolysis patients. METHODS:  We retrospectively chart reviewed adult patients admitted to the intensive care unit (ICU) with a diagnosis of rhabdomyolysis between 2012 and 2021. We dichotomized patients into two groups based on whether TPE was used or not in addition to standard care. PRISMA machines with TPE2000 filters and either 5% albumin or fresh frozen plasma were used in the TPE group. RESULTS:  The patients' age ranged from 23 years to 87 years (mean 49.4, SD 18.1), and 51% were male. Initial creatinine ranged from 0.6 to 16mg/dL (mean 3.4, SD 2.7), creatinine phosphokinase (CPK) from 403-93,232 U/L, and myoglobin from 934 to >20,000. The Sequential Organ Failure Assessment (SOFA)scores on admission ranged from 6 to 17 (mean 7.23, SD 3.40). Overall, 28.78% (N=19) of the patients received therapeutic plasma exchange. The overall mortality in our study was 31.9%, with the length of ICU stay ranging from 1-25 days (mean 7.10, SD 5.91) among survivors. Older age and the presence of shock were predictive of mortality in univariate and multivariate analyses. There was no statistically significant association in mortality between the TPE and non-TPE groups (36.84% in TPE vs. 36.17% in the non-TPE group, OR 0.7209, p=0.959). Only two patients in the non-TPE group developed CKD/ESRD on long-term follow-up. CONCLUSION: Our study showed that TPE administration in critically ill patients with rhabdomyolysis did not improve mortality or length of ICU stay. Further studies are required to elucidate its indication and effect on long-term renal outcomes.