Chronic lymphocytic leukaemia (CLL) is a lymphoproliferative disorder characterised by an accumulation of monoclonal B lymphocytes, with an increased risk of secondary cancers. The coexistence of CLL and chronic myeloid leukaemia (CML) is a rare phenomenon, with three main types being classified: CML preceding CLL, CLL preceding CML and simultaneous occurrence. The coexistence of these chronic leukaemias poses a complex clinical challenge, with the underlying mechanisms of their association remaining enigmatic. Here, we present a report of an elderly male with a long history of CLL, who was subsequently diagnosed with secondary CML. LEARNING POINTS: The development of chronic myeloid leukaemia (CML) subsequent to chronic lymphocytic leukaemia (CLL) is an uncommon occurrence, challenging conventional expectations of disease evolution in chronic leukaemia.Extensive and appropriate testing is necessary to promptly identify secondary CML in CLL patients.Targeted therapy with dasatinib, a tyrosine kinase inhibitor, may demonstrate efficacy in reducing leukocytosis and BCR-ABL1 levels in patients with coexisting CLL and CML.
Statins can commonly cause myopathy. Most of the time, stopping the culprit drug should solve the problem. However, if the drug has been discontinued but muscle weakness continues to worsen, immune-mediated myopathy should be taken into consideration.
Microscopic polyangiitis is a rare autoimmune vasculitis, that could present with renal-pulmonary symptoms, posing diagnostic challenges in patients with preexisting kidney disease. Timely diagnosis is crucial to improve patient outcomes.
Kaposi's sarcoma (KS) is a malignancy that commonly appears as lesions on the skin or mucosal surfaces but can also develop in other organs. This cancer is usually caused by the human herpesvirus 8 (HHV-8), recently known as Kaposi's sarcoma-associated herpesvirus (KSHV). KS is rare in the general population but can develop in kidney transplant recipients with varying incidence due to immunocompromised status from immunosuppression. The main aim of the present systematic review was to identify the prevalence and treatment of KS in kidney transplant patients. PubMed, Cochrane Library, and Google Scholar databases were searched for studies until October 2023. Full-text studies with similar research objectives were included, while non-English articles, reviews, case reports, ongoing clinical trials, and studies evaluating KS in HIV patients or after other solid organ transplants were excluded. All studies were observational; therefore, methodological quality was assessed using the Newcastle-Ottawa Scale. The statistical analyses were performed with the Comprehensive Meta-Analysis (CMA) software (Biostat, Inc. Englewood, NJ). The pooled analysis from the 15 studies included showed that KS develops in 1.5% of kidney transplant recipients and is more prevalent in African (1.7%) and Middle Eastern (1.7%) recipients than in Western recipients (0.07%). KS was also significantly more prevalent among male recipients than female recipients (OR: 2.36; p < 0.0001). Additionally, cyclosporine-based immunosuppression accounts for most KS incidences (79.6%) compared to azathioprine-based immunosuppression (28.2%). Furthermore, reduction or withdrawal of immunosuppression alone resulted in 47.8% KS complete remissions. Post-kidney transplantation KS is more frequent among males and patients of Middle Eastern and African origin. However, the gender difference may be attributed to most patients undergoing kidney transplants being male. Therefore, if gender balance is considered in future studies, then the difference might be insignificant. Based on our results, we can concur that the mainstay treatment for post-transplant KS is reduction or withdrawal of immunosuppression. However, the patients should be closely monitored to avoid KS recurrence and kidney rejection. Furthermore, there is an increased risk for KS with the use of cyclosporine-based immunosuppression. However, this does not mean that the withdrawal of this immunosuppression agent might result in improved KS outcomes because the withdrawal of azathioprine with or without cyclosporine reduction has also led to improved outcomes.
Key Clinical Message: Acquired hemophilia A (AHA) can present as life-threatening bleeding during the postpartum period. Prompt treatment allows patients with AHA to achieve complete remission and have normal subsequent pregnancies. Abstract: Acquired hemophilia A (AHA) is a rare bleeding disorder caused by the production of autoantibodies against factor VIII (FVIII). AHA can present with severe bleeding, especially in postpartum patient. We report a 38-year-old woman who presented in an emergency department with severe postpartum hemorrhage 2 weeks after cesarean section. Her investigation showed an isolated prolongation of partial thromboplastin time (PTT), low factor VIII assay and a factor VIII inhibitor test, resulting in abnormal Bethesda units which consistent with AHA. This case report highlights the importance of early diagnosis and treatment of AHA. With timely and appropriate management, most patients can achieve a good outcome.
Subcutaneous panniculitis-like T-cell lymphoma (SPTL) is a rare subtype of non-Hodgkin lymphoma that manifests as panniculitis-like skin lesions. It frequently co-occurs with hemophagocytic lymphohistocytosis, a life-threatening hyperinflammatory syndrome. The majority of SPTL cases express αß T-cell receptors (SPTL-AB) and have a favorable prognosis with oral immunosuppressive agents. We report a 37-year-old male patient with HIV infection who had a history of low-grade fever for one year, multiple tender subcutaneous nodules on both thighs, and cytopenia. He received several courses of antibiotics without significant improvement. A random skin biopsy showed lobular panniculitis and he was treated with steroids, but his fever recurred after steroid withdrawal. A second skin biopsy confirmed the diagnosis of SPTL. A bone marrow examination revealed hemophagocytic lymphohistiocytosis. He was successfully treated with cyclosporin A and prednisolone and achieved a complete response after one year of drug discontinuation. Panniculitis-like skin lesions have various etiologies and may present as a clinical mimic of lupus erythematosus panniculitis. The selection of an optimal site for skin biopsy is crucial to avoid erroneous diagnoses and adverse outcomes. We report a case of SPTL in an HIV-positive patient, which illustrates this diagnostic challenge.
Acute respiratory distress syndrome (ARDS) is a life-threatening lung injury characterized by rapid onset of widespread inflammation in the lungs. Multiple risk factors, including pneumonia, non-pulmonary sepsis, aspiration of gastric contents or inhalation injury, have been reported, to cause ARDS. We present a case of a healthy young woman in her first trimester with vaping-induced lung injury who presented with spontaneous pneumothorax and acute respiratory distress syndrome with concomitant influenza A and rhinovirus infection followed by methicillin-resistant Staphylococcus aureus pneumonia.
Eosinophilia with pulmonary involvement is characterized by the presence of peripheral blood eosinophilia, typically >500 cells/mm3, nonspecific pulmonary symptoms, and radiographic evidence of pulmonary disease. Clinical, laboratory, and radiologic features can be overlapping in these diseases, thus, it is wise to approach eosinophilia with pulmonary involvement systematically to determine the diagnosis and provide definitive treatment for a better outcome. The authors present a case of idiopathic chronic eosinophilic pneumonia in a patient with a long history of chronic obstructive pulmonary disease (COPD) which was resolved by corticosteroid.
Drug-induced immune haemolytic anaemia (DIIHA) is a rare but serious complication affecting approximately 1 in 1,000,000 patients, but its incidence might be underestimated due to misdiagnosis. Several factors should be considered to ensure an accurate diagnosis, including previous medical history, comorbidities, drug history, the temporal relationship between drug exposure and symptom onset, haemolytic features, and comorbidities in suspected cases. The authors report a case of DIIHA caused by combination chemotherapy with carboplatin and paclitaxel complicated with haeme pigment-induced acute kidney injury. LEARNING POINTS: Drug-induced immune haemolytic anaemia (DIIHA) should be suspected in patients with abrupt immune haemolytic anaemia with a temporal relationship between drug exposure and symptom onset.The main management of DIIHA consists of urgent discontinuation of the suspected drug and supportive treatment with close monitoring, resulting in a favourable outcome in most cases; the role of corticosteroids in DIIHA remains unclear.Haeme pigment-induced acute kidney injury is induced by intravascular haemolysis where urinalysis reveals elevated haemoglobin.
INTRODUCTION: Several studies have shown an inconsistent relationship between postimplantation pocket hematoma and cardiac implantable electronic device (CIED) infection. In this study, we performed a systematic review and meta-analysis to explore the effect of postimplantation hematoma and the risk of CIED infection. METHODS: We searched the databases of MEDLINE and EMBASE from inception to March 2020. Included studies were cohort studies, case-control studies, cross-sectional studies, and randomized controlled trials that reported incidence of postimplantation pocket hematoma and CIED infection during the follow-up period. CIED infection was defined as either a device-related local or systemic infection. Data from each study were combined using the random effects, generic inverse variance method of Der Simonian and Laird to calculate odds ratios (OR) and 95% confidence intervals (CI). RESULTS: Fourteen studies were included in final analysis, involving a total of 28 319 participants. In random-effect model, we found that postimplantation pocket hematoma significantly increases the risk of overall CIED infection (OR = 6.30, 95% CI: 3.87-10.24, I 2 = 49.3%). There was no publication bias observed in the funnel plot as well as no small-study effect observed in Egger's test. CONCLUSIONS: Our meta-analysis demonstrated that postimplantation pocket hematoma significantly increases the risk of CIED infection. Precaution should be taken during device implantation to reduce postimplantation hematoma and subsequent CIED infection.
INTRODUCTION: Heart failure (HF) is one of the world leading causes of admission and readmission. Recent studies have shown that the presence of depression is associated with hospital readmission in patients after an index admission for heart failure (HF). However, there is disagreement between published studies regarding this finding. We performed a systematic review and meta-analysis to evaluate the effect of depression on readmission rates in HF patients. EVIDENCE ACQUISITION: We searched the databases of MEDLINE and EMBASE from inception to March 2020. Included studies were published study evaluating readmission rate of HF patients, with and without depression. Data from each study were combined using a random-effects model, generic inverse variance method of DerSimonian and Laird to calculate risk ratios and 95% confidence intervals. EVIDENCE SYNTHESIS: Ten studies were included in the meta-analysis with a total of 53,165 patients (6194 patients with depression). The presence of depression was associated with an increased risk of readmission in patients with HF (pooled HR=1.54, 95% CI: 1.22-1.94, P<0.001, I2=55.4%). In a subgroup analysis, depression was associated with an increased risk of readmission in patients with HF in both short-term (≤90 days) follow-up (pooled HR=1.75, 95% CI: 1.07-2.85, P=0.025, I2=76.0%) and long-term (>90 days) follow-up (pooled HR=1.58, 95% CI: 1.32-1.90, P<0.001, I2=0.0%). CONCLUSIONS: Our meta-analysis demonstrated that depression is associated with an increased risk of hospital readmission in patients with HF.
Heart Failure , Patient Readmission , Depression/epidemiology , Heart Failure/epidemiology , Hospitalization , Humans
Nuclear scintigraphy is functional imaging and can be combined with anatomical imaging to improve diagnostic yield. Detection of parathyroid lesion by technetium-99m methoxyisobutylisonitrile (Tc-99m MIBI) can facilitate an appropriate operative approach in a patients with primary hyperparathyroidism. Tc-99m MIBI is concentrated in highly cellular or metabolically active tissues, which have abundant mitochondria. False-positive scintigraphic findings could be from head-and-neck carcinomas, thyroid neoplasm, and multinodular goiter. In addition, multiple organs outside of the neck region, such as lung and breast, can take up the Tc-99m MIBI. Herein, we report the occurrence of abnormal focal uptake in the breast region during the preoperative localization of parathyroid adenoma and later discovered breast carcinoma.
Cystadenoma, Papillary/diagnosis , Genital Diseases, Male/diagnosis , Scrotum/pathology , von Hippel-Lindau Disease/diagnosis , Adult , Cystadenoma, Papillary/genetics , Cystadenoma, Papillary/pathology , Cystadenoma, Papillary/surgery , Genital Diseases, Male/genetics , Genital Diseases, Male/pathology , Genital Diseases, Male/surgery , Humans , Male , Scrotum/surgery , Ultrasonography , von Hippel-Lindau Disease/genetics , von Hippel-Lindau Disease/pathology , von Hippel-Lindau Disease/surgery
Pheochromocytomas are rare neuroendocrine tumors arising from chromaffin cells of the adrenal gland. Because of their highly variable clinical spectrum, these tumors often go undiagnosed and result in life-threatening complications. The typical presentations include episodic headache, palpitations and sweating accompanied with sustained or paroxysmal hypertension. However, less than half of pheochromocytoma patients have these classic symptoms. Many patients present with atypical symptoms, which could be overlooked. Our case represents an unusual presentation of pheochromocytoma, which is not well recognized as a possible manifestation. A 60-year-old woman presented with light-intensity-related nausea, which progressed to severe vomiting with hypovolemic shock. An unexpected adrenal mass was found during sonographic evaluation of the volume status. Pheochromocytoma was confirmed by 24-hour urine fractionated metanephrines and a computed tomography (CT) scan. In pheochromocytomas, the elevation of circulating catecholamines activates alpha-adrenergic receptors in the area postrema, which then initiates the emetic cascade. Light-intensity activity-related nausea and vomiting, especially when present with other symptoms of catecholamine excess, could be considered as a clinical presentation of pheochromocytomas.
Hypopituitarism/etiology , Intracranial Aneurysm/complications , Pituitary Gland/diagnostic imaging , Aged , Follicle Stimulating Hormone/blood , Humans , Hypopituitarism/blood , Hypopituitarism/diagnostic imaging , Intracranial Aneurysm/blood , Intracranial Aneurysm/diagnostic imaging , Magnetic Resonance Imaging , Male , Prolactin/blood , Testosterone/blood , Thyroxine/blood
BACKGROUND: Anaplastic lymphoma kinase (ALK) gene rearrangement is detected in 3% to 13% of non-small cell lung carcinoma patients, and these patients benefit from ALK inhibitors. The aim of this study was to determine the prevalence, the clinical and histological characteristics and the treatment outcomes of ALK-rearranged lung adenocarcinoma using immunohistochemistry (IHC) IHC, reverse transcription polymerase chain reaction (RT-PCR) and fluorescence in situ hybridization (FISH) methodologies. METHODS: A total of 268 pulmonary adenocarcinoma patients were screened for ALK expression by ALK IHC, which was confirmed by FISH and/or RT-PCR for ALK gene rearrangement. The treatment outcomes of ALK-rearranged patients were retrospectively reviewed. RESULTS: ALK gene rearrangement was identified in 26 cases (9.7%) with no EGFR co-mutation, and it showed significant associations with younger age, female sex and non-smoker status (p < 0.05). A cribriform growth pattern was identified as the dominant histologic feature, and a solid signet ring cell component was focally present in a minority of the cases. Among 12 ALK-rearranged patients with conventional treatment, seven cases in the early stage of disease were cured and alive, and five patients in the late stage of the disease progressed and died, with a median overall survival (OS) at 14 months. Of the 14 patients receiving crizotinib, all of them had clinical benefit from crizotinib treatment, with one patient having a complete response (CR), 12 patients having a partial response (PR) and one patient having stable disease (SD). On the cutoff date, six of 14 patients were continuing crizotinib treatment with a median time of response of 7.5 (3-13) months, while eight patients had disease progression, and five of them died with a median OS at 8 months. CONCLUSION: ALK gene rearrangement tended to occur in younger, non-smoking, female patients. ALK IHC is a reliable screening method to detect ALK gene rearrangement. Crizotinib therapy provided treatment benefit in ALK-rearranged adenocarcinoma patients especially in advanced stages of the disease.
Adenocarcinoma/genetics , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Biomarkers, Tumor/genetics , Gene Rearrangement , Lung Neoplasms/genetics , Receptor Protein-Tyrosine Kinases/genetics , Adenocarcinoma/drug therapy , Adenocarcinoma/secondary , Adult , Aged , Aged, 80 and over , Anaplastic Lymphoma Kinase , Female , Follow-Up Studies , Humans , Immunoenzyme Techniques , In Situ Hybridization, Fluorescence , Lung Neoplasms/drug therapy , Lung Neoplasms/pathology , Lymphatic Metastasis , Male , Middle Aged , Neoplasm Staging , Prognosis , RNA, Messenger/genetics , Real-Time Polymerase Chain Reaction , Reverse Transcriptase Polymerase Chain Reaction , Young Adult
