Myelodysplastic Neoplasms (MDS): The Current and Future Treatment Landscape.

Myelodysplastic neoplasms (MDS) are a heterogenous clonal disorder of hemopoietic stem cells characterized by cytomorphologic dysplasia, ineffective hematopoiesis, peripheral cytopenias and risk of progression to acute myeloid leukemia (AML). Our understanding of this disease has continued to evolve over the last century. More recently, prognostication and treatment have been determined by cytogenetic and molecular data. Specific genetic abnormalities, such as deletion of the long arm of chromosome 5 (del(5q)), TP53 inactivation and SF3B1 mutation, are increasingly associated with disease phenotype and outcome, as reflected in the recently updated fifth edition of the World Health Organization Classification of Hematolymphoid Tumors (WHO5) and the International Consensus Classification 2022 (ICC 2022) classification systems. Treatment of lower-risk MDS is primarily symptom directed to ameliorate cytopenias. Higher-risk disease warrants disease-directed therapy at diagnosis; however, the only possible cure is an allogenic bone marrow transplant. Novel treatments aimed at rational molecular and cellular pathway targets have yielded a number of candidate drugs over recent years; however few new approvals have been granted. With ongoing research, we hope to increasingly offer our MDS patients tailored therapeutic approaches, ultimately decreasing morbidity and mortality.

IgA plasma cell myeloma presenting as cold agglutinin-induced haemolytic transfusion reaction.

Cold agglutinins produced in the setting of B cell neoplasms, such as lymphoplasmacytic lymphoma and plasma cell myeloma, can mediate autoimmune haemolytic anemia. Transfusion of these patients can exacerbate cold agglutinin-mediated haemolysis. Moreover, the workup for these reactions represents a diagnostic challenge due in part to false negative direct antiglobulin tests (DATs). Here, we report an anaemic patient who after a red blood cell (RBC) transfusion performed without blood warming, experienced a DAT-negative haemolytic transfusion reaction, and was later diagnosed with IgA-multiple myeloma, which showed an uncommon granular pattern by CD138 immunohistochemistry. Extensive workup excluded other diagnostic possibilities, including the presence of Donath-Landsteiner antibodies and cryoglobulins. Successful treatment with CyBorD (cyclophosphamide, bortezomib and dexamethasone) achieved complete remission, and additional RBC transfusions using warmers were completed uneventfully.

Central Centrifugal Cicatricial Alopecia Associated With PDL1 Loss and Increased Expression of Caspase 3: A Case Series.

ABSTRACT: Central centrifugal cicatricial alopecia (CCCA) is a scarring alopecia that disproportionately affects patients with skin of color. Genetic studies have revealed that approximately 30% of CCCAs are associated with peptidyl arginine deiminase 3 misfolding mutations. Patients with CCCA usually have a poor prognosis with progressive and permanent hair loss. To further characterize CCCA, we evaluated the inflammatory milieu, PDL1, and caspase 3 expression. The data support the idea of CCCA being a CD4-predominant T-cell process. The loss of PDL1 and increase in caspase 3 expression raises the possibility of involvement of the PD1/PDL1 pathway in CCCA.

Dendriform pulmonary ossification in a patient with sickle cell disease.

Dendriform pulmonary ossification (DPO) is a rare condition characterised by the formation of bones in lung tissues. This case report describes the first reported case of DPO in a patient with sickle cell disease (SCD), identifying DPO as a potential pulmonary consequence of SCD.

Two useful tools: to improve patient engagement and transition from the hospital.

We use an Internet-based health assessment and feedback system to examine the range of needs and diverse experiences of 520 hospitalized adults in transition and the factors most strongly associated with their self-reported health confidence. Our results strongly suggest that patient engagement prior to admission and the quality of care coordination and communication during hospitalization can greatly enhance successful transition from the hospital back to the community. Hospitals are complex institutions. This report illustrates how the Internet or a straightforward graphic can make the complexity less overwhelming to patients and efficiently increase their health confidence for transitions.

Who's behind an HCAHPS score?

BACKGROUND: The Hospital Consumer Assessment of Health Care Providers and Systems (HCAHPS) survey asks patients how frequently their physicians treated them with courtesy and respect, listened carefully, and explained things in a way they could understand. Such summary reports may obscure differences among the types of physicians involved. A study was conducted to examine the association between ratings for different physician types and the overall HCAHPS rating of physicians. METHODS: A mixed-methods study included closed-ended surveys and in-depth interviews of patients on a hospitalist teaching service. The three HCAHPS physician communication items were used to interview patients about their communication experiences with emergency medicine (EM) physicians, hospitalists, and specialists. The association between the overall score and the scores of each physician type was examined using Spearman correlation coefficients and linear regression. Qualitative data from additional in-depth interviews were analyzed using the constant comparative method to identify recurrent themes. RESULTS: Ninety-six patients were recruited for the survey, and additional in-depth interviews were conducted with the first 30 patients. Hospitalist and specialist scores were significantly associated (p values < .05) with overall scores. Recurrent themes regarding determinants of patients' ratings were categorized in three broad domains: individual physician behavior, team communication, and system issues. The influence of each domain differed across physician types. DISCUSSION: Physician communication scores may be most strongly influenced by patient experiences with hospitalists and specialists rather than with EM physicians. Several team communication and system issues represent opportunities for improving physician communication.