Coronavirus Does It Again: Post-COVID-19 Hemophagocytic Lymphohistiocytosis (HLH).

Hemophagocytic lymphohistiocytosis (HLH) is a hematological disorder that results from an uncontrolled activation of the immune system, which can then lead to multisystem organ failure. Given the nonspecific nature of this illness, it can go undetected for too long, thereby causing permanent damage to organ systems. In adults, HLH has been associated with a number of infectious etiologies, particularly viral infections. Coronavirus disease 2019 (COVID-19), caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), has led to a global pandemic and has been associated with acute respiratory distress syndrome (ARDS). Among its other manifestations, COVID-19 has also been linked to HLH. In this report, we describe a case of a male patient who presented with multisystem organ failure and was found to have HLH. Since no clear etiology for his HLH could be elicited, it was determined to be a result of his recent COVID-19 infection.

Burden of bone disease in chronic pancreatitis: A systematic review and meta-analysis.

BACKGROUND: Bone disease is an under-recognized cause of morbidity in chronic pancreatitis (CP). Over the past decade, publications of original studies on bone disease in CP has warranted synthesis of the evidence to ascertain the true burden of the problem. AIM: To quantify the prevalence of osteopenia, osteoporosis, and fragility fractures in CP patients and investigate the associated clinical features and outcomes. METHODS: A systematic search identified studies investigating bone disease in CP patients from Cochrane Library, Embase, Google Scholar, Ovid Medline, PubMed, Scopus, and Web of Science, from inception until October 2022. The outcomes included prevalence of osteopenia, osteoporosis, and fragility fractures, which were meta-analyzed using a random-effects model and underwent metaregression to delineate association with baseline clinical features. RESULTS: Twenty-one studies were included for systematic review and 18 studies were included for meta-analysis. The pooled prevalence of osteopenia and osteoporosis in CP patients was 41.2% (95%CI: 35.2%-47.3%) and 20.9% (95%CI: 14.9%-27.6%), respectively. The pooled prevalence of fragility fractures described among CP was 5.9% (95%CI: 3.9%-8.4%). Meta-regression revealed significant association of pancreatic enzyme replacement therapy (PERT) use with prevalence of osteoporosis [coefficient: 1.7 (95%CI: 0.6-2.8); P < 0.0001]. We observed no associations with mean age, sex distribution, body mass index, alcohol or smoking exposure, diabetes with prevalence of osteopenia, osteoporosis or fragility fractures. Paucity of data on systemic inflammation, CP severity, and bone mineralization parameters precluded a formal meta-analysis. CONCLUSION: This meta-analysis confirms significant bone disease in patients with CP. Other than PERT use, we observed no patient or study-specific factor to be significantly associated with CP-related bone disease. Further studies are needed to identify confounders, at-risk population, and to understand the mechanisms of CP-related bone disease and the implications of treatment response.

A Rare Case of Undifferentiated Rhabdoid Carcinoma of the Colon.

Rhabdoid carcinoma of the colon is a rare type of malignancy that belongs to the family of malignant rhabdoid tumors (MRTs). This is infrequently encountered in clinical settings therefore data regarding treatment is lacking. Herein we discuss an elderly female patient who presented with severe abdominal pain in the setting of a gastrointestinal perforation. Imaging showed a large mass in the transverse colon, which was found to be an undifferentiated carcinoma of the colon with rhabdoid features. We highlight this case to discuss the clinical course of the patient, thereby adding to the limited literature that is available, as well as review the few studies that have, thus far, documented this rare presentation.

Recurrence of Angioimmunoblastic T-cell Lymphoma in a Patient Successfully Treated for Hodgkin's Lymphoma: A Case Report.

Hodgkin's lymphoma (HL) is a common and potentially curable malignancy that has an overall good prognosis when timely treatment with chemoradiation is delivered. Recurrence of malignancy is one complication seen in patients successfully treated for HL. In most cases, the recurring malignancy can be a solid tumor or leukemia. While recurrence of a non-HL (NHL) has been reported, this is relatively uncommon. Angioimmunoblastic T-cell lymphoma (AITL) is a rare nodal appearing, peripheral T-cell lymphoma and represents 2% of all NHLs. Its clinical features include generalized lymphadenopathy, varying constitutional symptoms, and autoimmune-related hematologic findings, such as hemolytic anemia and or thrombocytopenia. Diagnosis is made based on histological and immunohistochemical (IHC) findings, which show evidence of T-cells, follicular T-cell markers, and characteristic genomic features including mutations of T-cell receptor or T-cell receptor signaling genes. It is a characteristically aggressive cancer with a poor prognosis if untreated and therefore requires prompt diagnosis. While sporadic AITL is rare on its own, data on AITL occurrence in patients previously treated for HL is lacking. We present a peculiar case of an 80-year-old patient who was diagnosed and treated for stage IV Hodgkin's disease only to be later diagnosed with AITL.

Hemorrhagic Pericardial Effusion From Apixaban Use: Case Report and Literature Review.

Direct oral anticoagulants (DOACs) have revolutionized therapy for stroke prophylaxis in patients with non-valvular atrial fibrillation. These medications are generally well tolerated and are not associated with the inconvenience of repeat international normalized ratio (INR) checks. While bleeding in general is a common side effect associated with DOACs, especially from a gastrointestinal source, spontaneous hemorrhagic pericardial effusions are not seen frequently. Herein, we present a case of a patient who developed a hemorrhagic pericardial effusion three days after the initiation of apixaban. We also summarize the current data that is available showing this side effect and highlight an important risk factor that may predispose patients to this complication.

Pylephlebitis With Splenic and Mesenteric Vein Thrombosis in a Patient With Diverticulitis.

Diverticulitis is a common gastrointestinal complaint that refers to inflammation of colonic diverticula. Its incidence has increased partly due to the increase in prevalence of diverticulosis, which results from poor dietary habits and chronic constipation. An acute diverticulitis episode can vary in severity, ranging from outpatient management of mild abdominal discomfort to inpatient admission requiring emergent surgery. Some common complications associated with diverticulitis include bowel wall perforation, microperforation, abscess formation, bowel obstruction, and colonic fistulas. A lesser-known complication of diverticulitis is pylephlebitis. Pylephlebitis refers to thrombosis of the portal vein resulting from sepsis secondary to an intra-abdominal or pelvic infection. Initially thought to be most associated with appendicitis, literature has emerged that implicates diverticulitis as the most likely culprit. Less frequently, pylephlebitis can also include thrombosis of the abdominal vasculature that drains into the portal vein such as the mesenteric veins and splenic vein. Despite antibiotic therapy, mortality in patients with pylephlebitis is high as it can lead to bowel ischemia, liver failure, or liver abscesses. While antibiotic therapy is the mainstay of treatment, anticoagulation can also be used in conjunction, especially when thrombosis extends beyond the portal vein. Herein, we present a case of a patient who was diagnosed with pylephlebitis with thrombosis extension into the splenic and mesenteric veins, which resulted from an episode of severe sigmoid diverticulitis. Our patient was treated medically with antibiotics and anticoagulation and underwent a loop transverse colostomy with full recovery. He was discharged with intravenous antibiotics and long-term anticoagulation. We present this case to highlight a rare complication of an otherwise common pathology and describe our management that led to a positive outcome for this patient.

Recurrent Chylous Pleural Effusions in a Patient Treated With Dasatinib.

Dasatinib is a second-generation BCR-ABL tyrosine kinase inhibitor (TKI) that is approved for the treatment of chronic myeloid leukemia (CML), a myeloproliferative disorder seen commonly in adults over the age of 50. Dasatinib is superior in tolerance and efficacy to first-generation TKIs such as imatinib, given its ability to target mutation products that are resistant to first-generation TKIs. One of the common side effects of dasatinib includes pleural effusion which can be seen in up to 25% of patients on treatment. These effusions are predominantly exudative; however, they tend to resolve upon discontinuation of the drug. While infrequent, chylous effusions have been reported with the use of dasatinib; they tend to resolve following discontinuation of the drug. We present a case of a patient who was treated with dasatinib and developed a chylous effusion which was refractory to the discontinuation of the medication. Our patient was switched to imatinib and since his first episode, he has had multiple reaccumulation requiring thoracentesis, all of which have revealed chylous pleural fluid as per fluid analysis. We present this case to highlight a rare adverse effect of dasatinib which, via an unknown mechanism, can potentially lead to irreversible damage to the lymphatic duct resulting in recurrent chylous effusions.

An Atypical Presentation of Twiddler's Syndrome: A Case Report.

The prevalence of implanted pacemaker/defibrillator devices continues to rise. Automatic implantable cardioverter-defibrillator (AICD) lead displacement (Twiddler's syndrome) is an uncommon form of the device malfunction, usually presenting with cardiac symptoms. We present a case of Twiddler's syndrome with an atypical presentation, accompanied by critical alkalosis on arterial blood gas. Considering Twiddler's syndrome as part of the differential diagnosis in patients with implanted devices and utilizing remote ICD interrogation may improve the care of patients presenting with device malfunction.

Infliximab-Induced Non-specific Interstitial Pneumonitis in a Patient With Ulcerative Colitis (UC).

Infliximab belongs to the family of tumor necrosis factor (TNF) alpha inhibitors and since its development, it has revolutionized treatment for both rheumatological diseases and inflammatory bowel disease (IBD). In IBD specifically, it has shown to result in symptomatic, endoscopic, and histological remission which is why it is one of the most widely used treatments for moderate to severe IBD. While common side effects include infections due to immunosuppression, cytopenias and hepatotoxicity, interstitial lung disease (ILD) has been infrequently reported to result from inflixiamb use. We present the case of a patient with ulcerative colitis (UC) who achieved remission with infliximab, however, after about two years of infusions, developed evidence of non-specific interstitial pneumonitis (NSIP). Extensive work up was done to rule out infections, mixed connective tissue disorders, and hypersensitivity pneumonitis. Although lung biopsy remains the gold standard for diagnosing NSIP; clinical, laboratory, and radiographic findings were sufficient in establishing this diagnosis. Initiation of empiric high dose steroids, and cessation of infliximab infusions showed improvement in respiratory status and resolution of lung findings. This case highlights the importance of recognizing adverse effects of infliximab on the pulmonary status of an IBD patient given that infliximab mediated ILD does not adhere to a specific timeline. Considering that respiratory function may be compromised post any number of infusions, it is imperative to acknowledge patients' respiratory complaints and initiate prompt investigation and evaluation for this rare complication.

Transformed Waldenström Macroglobulinemia Responsive to Tafasitamab Plus Lenalidomide: A Case Report.

The histologic transformation (HT) of Waldenström macroglobulinemia (WM) into diffuse large-cell lymphoma is an uncommon but poor-prognostic event for which there is no standard therapy. Knowledge of this entity is mainly derived from largely retrospective studies, which report abysmal average survival rates even with the utilization of first-line chemoimmunotherapy and especially in patients who meet the high-risk criteria based on prognostic indices used for WM. We present the case of a 75-year-old man with high-risk, transformed WM who was ineligible for standard chemoimmunotherapy (due to pancytopenia and multiple comorbidities) and was consequently treated with tafasitabmab, an anti-CD19 monoclonal antibody plus lenalidomide. Tafasitamab plus lenalidomide (TAF/LEN) is a recently approved therapy for relapsed or refractory de novo diffuse large-cell lymphoma (DLCL) but has not been previously studied in transformed low-grade lymphomas or WM. We show that TAF/LEN resulted in a complete and durable response of the DLCL by PET/CT and a complete bone marrow response of lymphoplasmacytoid cells, including the normalization of complex cytogenetic abnormalities. The extraordinary response of our patient to TAF/LEN suggests that this combination may be an effective and tolerable therapy for transformed WM as well as relapsed or refractory non-transformed WM. Clinical trials of TAF/LN for the treatment of Waldenström macroglobulinemia are recommended.

Correction: Transformed Waldenström Macroglobulinemia Responsive to Tafasitamab Plus Lenalidomide: A Case Report.

[This corrects the article DOI: 10.7759/cureus.32403.].