ABSTRACT
Cold agglutinin hemolytic anemia (cAHA) is a rare autoimmune disorder characterized by the production of cold agglutinins. We present a case of secondary cAHA in a 23-year-old female with severe anemia and unexplained hemolysis. The patient exhibited findings indicative of hemolysis and a positive direct antiglobulin test (DAT) with complement alone. Additional investigations revealed incidental lung infiltrates, negative serology for infections and autoimmune diseases, and a low cold agglutinin titer. The patient showed a favorable response to doxycycline and supportive therapy, including multiple packed red blood cell transfusions. At the two-week follow-up, the patient had a stable hemoglobin level with no evidence of ongoing hemolysis. This case highlights the importance of considering secondary cAHA in patients with cold symptoms or unexplained hemolysis. Primary cAHA patients may require more aggressive treatment, including rituximab and sutilumab.
ABSTRACT
The expression of hormone receptors (estrogen and progesterone) and human epidermal growth factor receptor-2 (HER2) has been used for both therapeutic and prognostic purposes in the management of breast cancer. The presence of a discordant receptor status complicates the approach to treatment in patients with synchronous bilateral breast cancer. We describe the case of a 45-year-old female with synchronous bilateral breast cancer with a triple-negative tumor and a contralateral HER2-positive tumor and discussed the impact of this on the approach to therapeutic management.
ABSTRACT
PURPOSE: Patients with relapse and/or refractory multiple myeloma (RRMM) have a high disease burden with poor health-related quality of life (HRQoL) which worsens with each additional relapse. We aimed to review the impact of triplet, doublet, monotherapies, and salvage autologous stem cell transplantation on the HRQoL of RRMM patients. METHODS: We performed a comprehensive literature search of Medline/PubMed, Wiley Cochrane Library, EMBASE, Scopus, CINAHL, and Clinicaltrials.gov to identify clinical studies in RRMM patients with HRQoL as an outcome measure. The ISOQoL and CONSORT-PRO extension guidelines were used to assess the quality of HRQoL reporting. We synthesized the result using a qualitative analysis. RESULTS: A total of 10,245 RRMM patients enrolled in 28 eligible studies received either a triplet, doublet regimen, monotherapy, or salvage autologous stem cell transplantation. The EORTC QLQ-C30 was the most used questionnaire, and compliance with HRQoL reporting standards is generally poor among studies without an additional HRQoL publication. Most of the current therapeutic options are at best able to maintain HRQoL at baseline but not improve it. The methodological and reporting heterogeneity among the studies complicates generalizations. CONCLUSIONS: Many of the current treatment regimens for RRMM have demonstrated clinical effectiveness in trials. Unlike newly diagnosed MM, these regimens are less likely to result in significant improvement in HRQoL in RRMM. This should be communicated to patients before initiating therapies. IMPLICATIONS FOR CANCER SURVIVORS: Individualized therapeutic approach for RRMM should be chosen based on a shared decision-making process that aligns clinical efficacy with patients' treatment priorities and HRQoL.
ABSTRACT
Acute mesenteric vein thrombosis (MVT) is an uncommon cause of mesenteric ischemia and portal hypertension. The common risk factors for the development of MVT include surgery, acute-intraabdominal inflammatory disorders, malignancies, and other prothrombotic states. However, MVT can occur in the absence of these risk factors. We describe the case of a 40-year-old man with a new diagnosis of hemoglobin E thalassemia and MVT and discuss the relationship between the hemoglobinopathy and thrombosis based on evidence from existing literature. The case emphasizes the importance of a high index of suspicion in the diagnosis of MVT in other to reduce complications and improve overall outcomes.
ABSTRACT
BACKGROUND: Smoldering multiple myeloma (SMM) is an intermediate pre-malignant condition with individuals having a distinct risk of progression to overt myeloma. The optimal management option has remained controversial due to the heterogeneous nature of the condition in which progression to overt diseases is variable. The question of who, when, and what to use for the treatment of SMM remains equivocal. We performed a systematic review of randomized controlled trials and summarized the current evidence supporting the best approach to the management of SMM. METHODS: A comprehensive literature search of Medline/PubMed, PubMed Central, Embase, Scopus, Web of Science, Wiley Cochrane Library, CINAHL, clinicaltrial.gov, and conference proceedings of ASCO, ASH, EHA, and ESMO was performed on October 25, 2020. Synthesis of the result was done using narrative analysis. RESULT: Of the total 1560 identified records, 10 eligible studies involving 1157 patients made up of 580 in the intervention group and 577 in the control group were included in this review. Three early trials of melphalan and prednisone fail to demonstrate any significant impact on disease progression with major toxicities reported. Three trials on bisphosphonate monotherapy show reduced skeletal-related events without any clinical effect on disease progression. Lenalidomide monotherapy or as part of a combination therapy demonstrates superiority in delaying disease progression over observation. Only Lenalidomide and dexamethasone combination demonstrated superior overall survival over observation across the trials. CONCLUSION: Trials of lenalidomide in a less intensive approach has shown promise in delaying disease progression and should be investigated further in clinical trials.
